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Chondroid SyringomaMixed Tumor of Skin, Salivary Gland Type
Abstract
The pluripotentiality of the cutaneous organ is well illustrated by the many epithelial structures it can form. This is seen not only in normal skin but in cutaneous neoplasms as well.Neoplasms with microscopic features that indicate both epithelial and mesenchymal origin have been referred to as mixed tumors.1 The term has been applied not only to those of the skin but also to many tumors unrelated to skin or to one another, i.e., basosquamous carcinoma, mixed tumors of salivary gland, mixed tumors of kidney, and other tumors with a mixture of cell types.In this paper we are reporting a study of a cutaneous tumor that has been referred to as mixed tumor of skin, salivary gland type. Because the tumor is relatively uncommon, it has not been investigated in all of its aspects. On the basis of a large collection we have been able to evaluate the
... Chondroid syringoma refers to pleomorphic adenoma developing from the cutaneous appendices [6][7][8]. It is a mixed cutaneous tumor diagnosed on pathology, characterized by subepithelial proliferation of glandular structures with networks of myo-epithelial components in a chondromyxoid stroma [8,9]. ...
... Pleomorphic adenoma of the external auditory canal is rare, but can develop from an ectopic salivary gland or extension of a parotid pleomorphic adenoma via the foramen of Huschke (foramen tympanicum) [15,16]. When the origin is in cutaneous annexes such as the ceruminous glands, it is referred to as chondroid syringoma or ceruminous pleomorphic adenoma [6,[17][18][19]. Chondroid syringoma is mainly located in the head and neck region. ...
... Diagnosis is pathologic, often associating immunohistochemistry [9,20,36,37]. In the present case, the absence of parotid involvement and the pathology results indicated a diagnosis of mixed tumor of cutaneous rather than salivary origin, confirming the hypothesis of primary external auditory canal chondroid syringoma developing from the cutaneous sebaceous glands [6,17,18]. Osteolysis has not often been reported to be associated with chondroid syringoma, and delayed treatment may be implicated in the present case. That no bone lysis was reported in the literature may be due to chondroid syringoma being frequently facial and thus visible, with slow growth. ...
Introduction. – Chondroid syringoma or ceruminous pleomorphic adenoma is a benign mixed cutaneous
tumor. It is the second most frequent glandular lesion in the external auditory canal, but no cases
associated with bone erosion were found in a PubMed search.
Case report. – The present CARE case report details a chondroid syringoma in the external auditory canal
with associated bone lysis, and its management.
Discussion. – Chondroid syringoma is a differential diagnosis for other external auditory canal tumors.
Forms causing bone erosion are rare, but should not challenge the diagnosis. Current treatment consists
in complete and broad surgical resection and prolonged follow-up due to the risk of recurrence and of
malignant transformation
... 1,2 The term 'chondroid syringoma' was introduced by Hirsch and Helwig in 1961 based on their review of 188 cases. 3 The designation 'chondroid' reflects the observed cartilaginous characteristics within tumour stroma, particularly in scalp lesions. 3 In other locations, the stroma may appear myxoid. ...
... 3 The designation 'chondroid' reflects the observed cartilaginous characteristics within tumour stroma, particularly in scalp lesions. 3 In other locations, the stroma may appear myxoid. 4 Headington identified two morphologically distinct types of mixed tumours: apocrine-type and eccrine-type. ...
... Surgical excision remains the mainstay of treatment, with careful consideration to prevent recurrence and malignant transformation. [3][4][5] In conclusion, CS, a rare and mostly benign skin lesion, often poses a diagnostic challenge due to its diverse morphological features and varied clinical presentations. Further research is necessary to elucidate its molecular characteristics and improve diagnostic accuracy and management strategies. ...
Mixed tumours of the skin, also called chondroid syringomas (CS), represent an uncommon subset of cutaneous adnexal neoplasms. These tumours exhibit morphologic differentiation towards primary skin appendages, including hair follicles, sebaceous glands and sweat glands (both apocrine and eccrine). We report a case of CS in a 55-year-old male.
... The term CS was first suggested by Hirsch and Helwig in 1961 for mixed tumours of the skin following a case series study of tumours with benign epithelial, myoepithelial and mesenchymal elements in a chondromyxoid matrix, considered to be derived from sweat glands of the skin [7]. CMT is the recommended terminology for these tumours used by the fifth edition of the WHO Classification of Skin Tumours (2023). ...
... Typically these tumours arise in the head and neck region [2][3][4]. Rare cases have been reported presenting in the skin of abdomen, axilla, penis, vulva, scrotum, hands and feet [5,7,9]. It is more common in men than women [3]. ...
... Hence, there is limited awareness and experience of the presentation of these lesions in this location. The tumour is generally asymptomatic and grows gradually, hence the patient tends to present to clinicians relatively late usually due to cosmetic reasons or trauma [7,9,10]. In a study by Okuda et al., the average time described in the literature from initial presentation to elective excision is 7.9 years with average size of tumour at time of excision of 42mm [10]. ...
Cutaneous mixed tumour (CMT), also known as chondroid syringoma (CS), is a rare benign tumour composed of epithelial, myoepithelial, and mesenchymal components with an incidence of less than 0.01% of primary skin tumours. It is more common in males and typically presents as a painless slow-growing firm mass in the subcutis of the head and neck region. Genital regions are very rarely involved. We present the case of a 50-year-old male with a 10-year history of an asymptomatic gradually enlarging mass in the upper scrotum. A surgical excision was performed. Microscopic examination showed features of CMT. This case highlights the diagnostic challenges associated with scrotal CMT and surgical management of these lesions. Additionally, we endorse the recommended terminology of CMT used by the fifth edition of WHO Classification of Skin Tumours (2023).
... Mixed tumor of the skin, arguably referred to as chondroid syringoma, is a rare and mostly benign skin lesion, comprising epithelial, myoepithelial, and mesenchymal stromal-derived elements [7]. The most commonly reported location is in the head and neck ...
... Mixed tumor of the skin, arguably referred to as chondroid syringoma, is a rare and mostly benign skin lesion, comprising epithelial, myoepithelial, and mesenchymal stromal-derived elements [7]. The most commonly reported location is in the head and neck region with a frequency of less than 0.1% [7,8]. ...
... Mixed tumor of the skin, arguably referred to as chondroid syringoma, is a rare and mostly benign skin lesion, comprising epithelial, myoepithelial, and mesenchymal stromal-derived elements [7]. The most commonly reported location is in the head and neck region with a frequency of less than 0.1% [7,8]. Due to the wide variety of differentiation and metaplastic changes in these lesions, it may present a diagnostic pitfall as it could mimic various skin and adnexal neoplasms. ...
Background/Introduction: Cutaneous mixed tumor is a rare benign neoplasm that exhibits a wide range of metaplastic changes and differentiation in the epithelial, myoepithelial, and stromal components, which is often confused with various other skin lesions. Case report: We present an unusual case of a 58-year-old woman with a mixed tumor of the upper lip, previously misdiagnosed as adnexal carcinoma on a preoperative biopsy. The excision biopsy shows a well-circumscribed lesion composed of various cells and structures featuring folliculo–sebaceous differentiation embedded in a prominent chondromyxoid stroma. The immunohistochemical study proves the various lineages of differentiation and classifies the neoplasm as the less common eccrine subtype of cutaneous mixed tumor. Discussion: The common embryologic origin of the folliculo–sebaceous apocrine complex leads to a great histological variety of cellular components of mixed tumors and the formation of structures that resemble established types of adnexal neoplasms, which could be a diagnostic pitfall, especially on a small incision biopsy.
... In 1859, Billroth [9] delineated it for the very first time as a category of salivary gland tumors having variable amounts of mucoid and cartilaginous material. In 1961, Hirsch and Helwig [10] established the terminology of "chondroid syringoma" elucidating it as a tumor histopathologically distinguished by the identification of elements of sweat glands lying within a cartilagelike stroma. They also elaborated its five specific histopathologic criteria which included presence of cuboidal or polygonal cell nests; tubuloalveolar structures encircled by two or more than two layers of cuboidal cells; ductal structures comprising of one or two rows of cuboidal cells; occasional keratinous or epidermoid cysts; and a diversified stroma or matrix on hematoxylin and eosin (H&E) stain [10]. ...
... In 1961, Hirsch and Helwig [10] established the terminology of "chondroid syringoma" elucidating it as a tumor histopathologically distinguished by the identification of elements of sweat glands lying within a cartilagelike stroma. They also elaborated its five specific histopathologic criteria which included presence of cuboidal or polygonal cell nests; tubuloalveolar structures encircled by two or more than two layers of cuboidal cells; ductal structures comprising of one or two rows of cuboidal cells; occasional keratinous or epidermoid cysts; and a diversified stroma or matrix on hematoxylin and eosin (H&E) stain [10]. Thereafter, Headington [11] explained that CS originates from either eccrine or apocrine glands. ...
... CS is an uncommon lesion having male predominance usually in middle or old age [15,16]. Male to female ratio was 5:1 in the pioneer study by Hirsch and Helwig [10]. It was reported to be 2:1 by Stout and Gorman [17] in their case series of 134 patients of CS. ...
... 1,2 The disease was first described by Billroth as a "cutaneous mixed tumor' and later revised by Hirsch et al., who coined the term ' CS ' in 1961. 3 It arises from the sweat glands and is similar to the pleomorphic adenoma of the salivary glands. The tumor is more common in men and has a biphasic age distribution. ...
... The tumor is more common in men and has a biphasic age distribution. 1,3 CS manifests as slow-growing, subcutaneous, non-tender, non-ulcerating dermal nodules, most often in the head and neck. 1,3,4 The tumor has epithelial and mesenchymal components and sweat-gland parts in a cartilaginous stroma. ...
... 1,3 CS manifests as slow-growing, subcutaneous, non-tender, non-ulcerating dermal nodules, most often in the head and neck. 1,3,4 The tumor has epithelial and mesenchymal components and sweat-gland parts in a cartilaginous stroma. CS is often benign, although rare cases of malignant CS have been described, with local recurrence, metastasis, and death. ...
Chondroid syringoma (CS) is a sporadic skin appendage tumor that manifests as slow-growing, subcutaneous, nontender, non-ulcerating dermal nodules, most often in the head and neck. The tumor is more common in men, has a
biphasic age distribution, and usually attains a size of 0.5 to 3 cm. Larger lesions up to 9 cm in size have been
recorded in the literature, with the majority of them containing malignancies. Rare cases of malignant CS have been
described, with local recurrence and metastasis. We report a case of an 85-year-old man presenting with complaints of
a significantly large swelling in the inner aspect of his right thigh for the past 25 years. Clinical and radiological
differential diagnoses of a large uncomplicated dermoid cyst, sebaceous cyst, epidermal inclusion cysts, neurogenic
tumours, or myxoid tumours with cystic degeneration were suggested. Under general anaesthesia, the lesion was
completely excised with a 1 cm margin of healthy surrounding tissue. The histopathological examination of the
complete surgical specimen was consistent with CS. Patient has been on regular follow-up and has shown excellent
response to treatment, with no evidence of recurrence, metastasis, or disfigurement. With this paper, we report a case
of benign CS in an unusual location, attaining a huge size (>10 cm) and, even then, not turning malignant. We hope to
add to the current knowledge on CS, allowing for early and accurate diagnosis and successful disease management.
... In 1859, Billroth [9] delineated it for the very first time as a category of salivary gland tumors having variable amounts of mucoid and cartilaginous material. In 1961, Hirsch and Helwig [10] established the terminology of "chondroid syringoma" elucidating it as a tumor histopathologically distinguished by the identification of elements of sweat glands lying within a cartilagelike stroma. They also elaborated its five specific histopathologic criteria which included presence of cuboidal or polygonal cell nests; tubuloalveolar structures encircled by two or more than two layers of cuboidal cells; ductal structures comprising of one or two rows of cuboidal cells; occasional keratinous or epidermoid cysts; and a diversified stroma or matrix on hematoxylin and eosin (H&E) stain [10]. ...
... In 1961, Hirsch and Helwig [10] established the terminology of "chondroid syringoma" elucidating it as a tumor histopathologically distinguished by the identification of elements of sweat glands lying within a cartilagelike stroma. They also elaborated its five specific histopathologic criteria which included presence of cuboidal or polygonal cell nests; tubuloalveolar structures encircled by two or more than two layers of cuboidal cells; ductal structures comprising of one or two rows of cuboidal cells; occasional keratinous or epidermoid cysts; and a diversified stroma or matrix on hematoxylin and eosin (H&E) stain [10]. Thereafter, Headington [11] explained that CS originates from either eccrine or apocrine glands. ...
... CS is an uncommon lesion having male predominance usually in middle or old age [15,16]. Male to female ratio was 5:1 in the pioneer study by Hirsch and Helwig [10]. It was reported to be 2:1 by Stout and Gorman [17] in their case series of 134 patients of CS. ...
Chondroid syringoma is a cutaneous adnexal tumor originating from sweat glands origin. It is rare in occurrence and usually benign, having an incidence of 0.01 to 0.098%. As these tumors are uncommon, their diagnosis is missed many times and are misdiagnosed. Hence in any case of facial skin swelling increasing slowly in size, this entity should be kept in mind as one of the possibilities and differential diagnosis. Histopathological examination of the excision biopsy gives the definitive confirmatory diagnosis. Surgically excising the swelling locally along with a surrounding normal tissue cuff is the standard treatment given which prevents recurrence. Hereby we present a 35-year-old case of facial chondroid syringoma having a focal component of eccrine hidrocystoma, keratinous cyst as well as syringocystadenoma papilliferum on the chin that was clinically suspected to be an epidermoid cyst or mucocele.
... However, the first case of mixed tumor of the skin was reported by Nasse in 1892 (2,3). Several decades later in 1961, upon a review of 188 cases of mixed tumour of the skin, the name 'Chondroid syringoma' was coined (4). Although contested, the Hirsch and Helwig (4) chondroid terminology resulted from observation that the biggest number of mostly scalp lesion cases had cartilaginous properties in the tumor stroma (4,5). ...
... Several decades later in 1961, upon a review of 188 cases of mixed tumour of the skin, the name 'Chondroid syringoma' was coined (4). Although contested, the Hirsch and Helwig (4) chondroid terminology resulted from observation that the biggest number of mostly scalp lesion cases had cartilaginous properties in the tumor stroma (4,5). Other tumour locations, reportedly demonstrate a myxoid stroma (5). ...
... The incidence of CS is reportedly <0.36% of all primary skin tumours (2). The tumour affects mostly middle-aged men, and commonly found in various sites of the head and neck region including the scalp, nose, cheek and lips (2)(3)(4). However, from professional literature more cases in the upper lips have been described compared to the lower lips . ...
Mixed tumour of the skin or chondroid syringoma (CS) is a rare and mostly benign neoplasm of the sweat glands. Although CS is frequently located on varied parts of the head and neck region, the lower lip is a rarely reported site. The present report describes a case of CS of the lower lip in a 58‑year‑old male as an expository case to further emphasise the need for proper diagnosis, appropriate treatment and prognostic evaluation. The patient presented with a round, non‑tender, slightly hard and mobile mass beneath the mucocutaneous junction of his left lateral side of the lower lip. Radiology revealed a mass measuring 11x11x7 mm3 in size at a depth of ~2 mm. Furthermore, magnetic resonance T1‑ and T2‑weighted images showed slightly low and high signal intensities, respectively. A provisional diagnosis of benign tumour of the lower lip was made, and surgical excision biopsy taken under local anaesthesia, while considering the patient's cosmetic appearance. Histopathology demonstrated features akin to apocrine gland, chondroid and myxoid stroma consistent with the diagnosis of benign CS. No evidence of recurrence or satellites were recorded after a follow‑up of nearly 2 years. Although rare, a high index of suspicion for CS among other cutaneous adnexal tumours of the lower lip is necessary. In addition, interprofessional collaboration in the management of such oral tumours could enhance patient satisfaction amid prevailing intraoral and aesthetic concerns.
... 1-6 It was first described by Billroth, 7 however the term chondroid syringoma was coined by Hirsch and Helwig since these lesions have sweat gland elements in the background of cartilaginous stroma. 8 Five histopathological criteria have been proposed for its diagnosis including nests of cuboidal/polygonal cells, intercommunicating tubuloalveolar structures, ductal structures, occasional keratinous cysts and a matrix of varying composition. 8 Chondroid syringoma may manifest only some or all five characteristics. ...
... 8 Five histopathological criteria have been proposed for its diagnosis including nests of cuboidal/polygonal cells, intercommunicating tubuloalveolar structures, ductal structures, occasional keratinous cysts and a matrix of varying composition. 8 Chondroid syringoma may manifest only some or all five characteristics. 8 In both our cases, four of the above criteria were present. ...
... 8 Chondroid syringoma may manifest only some or all five characteristics. 8 In both our cases, four of the above criteria were present. on January 7, 2022 by guest. ...
Chondroid syringoma is a benign mixed tumour arising from the sweat glands which can be apocrine (found throughout the surface of the eyelid) or eccrine with a mean age of presentation being 50 years. Here we report two cases of chondroid syringoma of the upper eyelid, one in a young male patient and the other in an elderly woman. Both cases had well-defined lesions involving upper eyelid in the sub-brow region, underwent excisional biopsy, and histopathological findings were consistent with an apocrine type of chondroid syringoma in one case and eccrine type of chondroid syringoma in the second case. It is important to be aware of this entity as a rare cause of eyelid lesion due to the fact that although it is a benign neoplasm, incomplete excision may be associated with recurrence or malignant transformation.
... [1] Mostly found in the head and neck region as reported by Hirsh and Helwig on a 188 case study with the following descending order of frequency : nose, cheek, upper lip, scalp, forehead, and chin. [2] From a clinical standpoint, these lesions are frequently misinterpreted as dermoid or sebaceous cysts, neurofibromas, dermatofibromas, or pleomorphic adenomas of the salivary glands. As a result, making a pre-excision diagnosis of these lesions is often challenging, with definitive identification typically achieved only through excision or biopsy. ...
... Recurrence is infrequent and is often linked to incomplete excision, a consequence of the tumor's lobulated structure and the absence of a comprehensive capsule. [2,8] ...
... Based on its histological origin from sweat glands and cartilage elements, Hirsch and Helwig categorized it as a chondroid syringoma in 1961. 5 The most documented presentation is a painless, gradually growing cutaneous lesion of the head and neck region with a size ranging from 0.5 to 3 cm in middle-aged men in their fifties. ...
... While the four main varieties of stromal elements, including adipose, fibrous, cartilaginous, and myxoid, are well recognized, osteoid substances may also be found less commonly in stromal components. 5 Features suggestive of malignancy include many atypical or mitotic cells, cell necrosis due to rapid proliferation, pericapsular infiltration, and poor differentiation of the cartilaginous elements. ...
The expression “pleomorphic adenoma” has been used synonymously with mixed cutaneous tumors and chondroid syringomas. It originates from eccrine or apocrine skin, salivary glands, and lacrimal glands. Histologically, it comprises an epithelial-lined glandular component embedded in the cartilaginous, myxomatous, or fibrous stroma. These lesions are usually misdiagnosed because they are extremely rare. It commonly affects middle-aged men and has a slow-growing nature. The usual manifestation is a firm nodular lesion in the periorbital region, particularly at eyelid margins. A unique presentation of this tumor was described in a young woman. The tumor presented as a small, static, nontender lump located at the junction of the superior margin of the left eyebrow and forehead. After a thorough clinical assessment, she underwent a complete surgical excision of the lesion. The most probable preoperative clinical impression at that time was that of a sebaceous cyst. However, histopathological examination revealed it to be a pleomorphic adenoma, which, to our knowledge, has never been reported in the literature at this specific anatomical site. Two years after the procedure, the patient’s follow-up was uneventful, and revealed no recurrence of the lesion. Although its incidence is exceptionally low, it should always be considered in the differential diagnosis of cutaneous lesions in the head, neck, and trunk. Complete surgical excision for histopathological assessment is recommended to rule out malignancy and avoid the frequent issue of local recurrence in cases of benign tumors.
... Chondroid synringomas (CSs) are mixed tumors of the skin, having both epithelial and mesenchymal constituents [1]. Hirsch and Helwig gave them the appellation chondroid syringoma, because of the presence of sweat gland elements which are set in a cartilaginous stroma. ...
... Mills suggested that these mixed tumors are monoclonal neoplasms with replicating cells can differentiate into epithelial or mesenchymal entities, thereby accounting for the histological variability of these tumors [7]. Hirsch and Helwig described five histological criteria that make up this diagnosis: collections of cuboidal or polygonal cells, tubuloalveolar structures lined with cuboidal cells, ductal structures with rows of cuboidal cells, some keratinous cysts and a matrix of varying components [1]. The treatment modality of choice for benign CSs is surgical excision. ...
... among all primary skin tumors, and demonstrates a predilection for males (2,3). MTS commonly involves in the head and neck areas; however, a very small number of cases of MTS of the lip have been reported (4)(5)(6)(7). It presents as asymptomatic slow growing, firm subcutaneous or intradermal nodule (3,6). ...
... Among cases of MTS, the frequency of MTS of the lip reported by Stout and Gorman (6), Hirsch and Helwig (5), and Kazakov et al (4) was only 13.4% (18/134 cases), 12.8% (24/188 cases), and 16.0% (39/244 cases), respectively. The frequency among MTSs in the head and neck region is similar to that in the nose and cheek skin regions (4)(5)(6). The frequency at which MTS occurs in the lip is considered to be very low in comparison to the frequency of all primary skin tumors (8). ...
Mixed tumor of the skin (MTS) is a rare neoplasm derived from the sweat glands with a reported frequency of 0.01-0.098% among all primary skin tumors. MTS often occurs in the head and neck region and is characterized by a mixture of epithelial, myoepithelial and stromal components. MTS also shows various morphological patterns, thus the presence of variants with rare components and its rarity make the clinical diagnosis even more difficult. A 47-year-old man was referred due to a painless, slowly growing, exophytic swelling intracutaneous mass of the upper lip. Magnetic resonance imaging revealed that the mass was a solid tumor with a fatty component in the proximal portion, while the distal portion was cystic and possibly contained highly viscous fluid. The mass was located between the skin and the orbicularis oris muscle in the upper lip. Excisional biopsy was performed and the lesion showed two intriguing features: A tumor with extensive lipomatous stroma and some large cysts. It was histopathologically diagnosed as lipomatous MTS with cystic formation in the upper lip. No evident signs of recurrence were observed during follow-up. The present report describes this case and includes a brief literature review of reported cases in the lip, since MTS can be confused with various skin lesions in clinical settings due to this rarity. Recognition by clinicians of different variants of MTSs, including the present case, is important for preventing erroneous diagnosis and treatment.
... The term "Chondroid Syringoma" was first used by Hisch and Helwig in 1961 to describe these relatively rare tumours. 1 They often arise as asymptomatic, firm subcutaneous nodules and are usually present for several years with little to no change in size. Histopathologic criteria include: (1) nests of cuboidal or polygonal cells, (2) tubuloalveolar structures, (3) ductal structures, and (4) a matrix of varying appearances (chondroid or hyaline material). ...
... There can be keratinous cysts lined with squamoid cells present. 1 Immunohistochemistry can be helpful, usually showing expression of a combination of different epithelial and mesenchymal markers: cytokeratins, epithelial membrane antigen (EMA), S-100, vimentin, carcinoembryonic antigen (CEA), and blood group antigen H. 2 Malignant chondroid syringomas (MCS) are much rarer than their benign counterpart, with only 51 reported cases in the literature. The histologic criteria for malignancy have been somewhat arbitrary, as histologically benign appearing tumours have recurred or metastasized. ...
Malignant Chondroid Syringomas (MCS) are very rare malignant tumours arising from cutaneous sweat glands, with only 51 reported cases in the literature. These tumours can metastasize and cause death if not treated adequately. While there are histological criteria to diagnose MCS tumours, there are no established criterion to determine which tumours are more or less likely to metastasize. A systematic review was performed to establish if any features of the primary MCS tumour are associated with risk of metastasis or patient mortality, as well as the efficacy of common treatment options. The literature search was performed using the Ovid Medline and Web of Science databases from inception through March 2020. This yielded 47 case reports corresponding to 51 unique patients. Statistical analysis of the collected data revealed none of the commonly accepted malignant histopathologic findings (including nuclear atypia and/or pleomorphism, mitotic figures, an infiltrative growth pattern, presence of satellite nodules, necrosis, and vascular and/or perineural invasion) of the primary tumour to be significantly more associated with metastatic risk or death. However, gross characteristics of the tumour, including size (greater than 5 cm) and truncal location of the primary lesion, were found to be associated with a higher risk of metastasis. The most effective treatment modality was wide local excision. Overall, primary MCS tumours, especially those greater than 5 cm or located on the trunk, should be treated with a wide local excision and followed closely to confirm no lesion recurrence or distant metastasis.
... Based on their work, they advocated that this entity should be named 'chondroid syringoma', as it was made up of cartilaginous-like material and is thought to be derived from sweat glands. 2 However, this term is now thought to be ill-fitting, as cartilage is not a consistent histopathological feature and the tumours do not look like syringomas. Instead, the term 'cutaneous mixed tumour' is supported by the WHO classification. ...
... However, not all features are required to make a diagnosis. 2,6 Malignant transformation can arise, although this is exceedingly rare. Histopathological risk factors that may indicate malignancy include infiltrative margins, tumour necrosis, cytological atypia, satellite lesions and deep involvement. ...
A 23‐year‐old man presenting with a 1‐year history of a lesion of the right cheek. We highlight this case for awareness as this tumour may mimic other benign lesions, such as pilomatrixoma or benign cysts, as it does not have any uniquely identifying clinical or dermoscopic features. Additionally, it is of concern as malignant transformation can occur and therefore surgery should be considered as both for diagnostic and therapeutic benefit.
... Chondroid syringoma is a rare mixed tumor occuring in the head and neck region that accounts for only 0.01% of all primary skin tumors. 1 Review of literatures shows only a minority of cases arising in periorbital tissues. 2 They are usually slow-growing, asymptomatic, solitary and nonulcerating masses ranging between 0.5 to 3.0 cm in size. 3 A size of 3 cm greater carries a higher risk of malignant potential. ...
... Sometimes tumours may display both features of eccrine and apocrine differentiation 9 The mesenchymal stroma may be chondroid as in the present case, or myxoid, adipocytic or fibrous .Rarely osteoid stroma and mature adipocytes may be observed. 1 Chondroid Syringoma may at times present with certain atypical features, i.e., extensive ossification, extensive cystic changes, predominantly chondromyxoid stroma with scant epithelial component, giant size, and atypical location, that can pose diagnostic challenge in diagnosis. ...
Chondroid syringoma is a rare mixed tumour of sweat gland origin that is characterised by sweat gland elements in a cartiliginous stroma. Its an uncommon cutaneous tumour of head and neck region with a reported incidence rate of 0.01% to 0.1%. Its occurence in periorbital tissues is rare and usually are small in size. Here we report a case of giant chondroid syringoma (>3 cms) arising in the lower eyelid.
... Chondroid syringoma is a rare mixed tumor occuring in the head and neck region that accounts for only 0.01% of all primary skin tumors. 1 Review of literatures shows only a minority of cases arising in periorbital tissues. 2 They are usually slow-growing, asymptomatic, solitary and nonulcerating masses ranging between 0.5 to 3.0 cm in size. 3 A size of 3 cm greater carries a higher risk of malignant potential. ...
... Sometimes tumours may display both features of eccrine and apocrine differentiation 9 The mesenchymal stroma may be chondroid as in the present case, or myxoid, adipocytic or fibrous .Rarely osteoid stroma and mature adipocytes may be observed. 1 Chondroid Syringoma may at times present with certain atypical features, i.e., extensive ossification, extensive cystic changes, predominantly chondromyxoid stroma with scant epithelial component, giant size, and atypical location, that can pose diagnostic challenge in diagnosis. ...
Synovial chondromatosis is a rare, benign disorder which commonly involves the large joints and is characterised by the proliferation of cartilaginous nodules beneath the synovial membrane. We report a case of an elderly male who presented with pain and swelling of the right foot since five years. Proper clinico- radiological and histological evaluation of this case led to the diagnosis of synovial chondromatosis which was managed surgically and patient’s symptoms were relieved. The rare occurrence of this entity in the small joints of hands and feet and the presence of several close differentials, warrants a thorough clinico-radiological and histological work-up to prevent unnecessary surgical exploration.
... En 1961, Hirsch y Helwig denominaron a los tumores mixtos cutáneos como Siringoma Condroide, después de analizar la histopatología y el comportamiento clínico de 188 casos, encontrando que el 80% de ellos se localizaban en la cabeza y el cuello, mientras que el 20% restante se ubicaba en otras partes del cuerpo. 3 Desde entonces, se han reportado varios casos de Siringomas Condroides con localizaciones atípicas. (Tabla 1) ...
Resumen El siringoma condroide (SC) es un tumor benigno infrecuente de las glándulas sudoríparas, que se compone de células epiteliales y estromales mezcladas. Por lo general, se presenta en adultos como nódulos firmes, de crecimiento lento, asintomáticos y localizados predominantemente en la cabeza y el cuello. En este trabajo, se describe un caso de SC con un diagnóstico preoperatorio difícil debido a la ubicación atípica en el brazo, lo que podría confundirse con otros tumores y, en algunos casos, incluso con neoplasias malignas. AbstRAct Chondroid Syringoma (CS), also known as a Mixed Tumor of the skin, is an uncommon benign tumor of the sweat glands that is composed of a mixture of epithelial and stromal cells. It typically presents in adults as slow-growing firm nodules that are asymptomatic and frequently located on the head and neck. In this report, we present a case of CS with a difficult preoperative diagnosis due to its atypical location in the arm, which could be mistaken for other tumors and even some malignant neoplasms. El siringoma condroide (SC), también conocido como tumor mixto cutáneo, es un tumor be-nigno poco común que puede tener un origen apocrino o ecrino. Se caracteriza por presentar com-ponentes epiteliales y estromales mezclados en su estructura. Por lo general, se presenta en adultos como nódulos firmes de crecimiento lento, asintomáticos y localizados con frecuencia en la cabeza y el cuello. Se estima que la incidencia del SC está entre el 0,01% y el 0,098%. 1 Aunque es poco frecuente, se han reportado casos de siringoma condroide maligno (SCM), lo que resalta la importancia de un diagnóstico y trata-miento oportuno. 2 El SC puede presentarse de forma atípica en áreas extrafaciales, lo que dificulta su diag-nóstico, ya que puede confundirse con otros tumores, como veremos en el caso a presentar. cAso clínico Se presenta el caso de un paciente masculino de 36 años de edad, de fototipo V y sin antecedentes mórbidos relevantes, quien acudió a consulta por presentar una tumoración en el brazo derecho de 7 años de evolución, de crecimiento lento, consistencia dura, no dolorosa y sin ulceración. Además, durante los úl-timos tres meses ha experimentado impotencia funcional al esfuerzo. Al examen físico se evidenció una masa redondeada de consistencia firme en la zona distal anterior del brazo derecho, subcutánea, sin cambios epidérmicos, de 3 cm de diámetro, móvil, polilobulada, bien definida, no adherida a planos profundos y con sensibilidad local conservada. (Figura 1) No presentaba adenopatías axilares ni cervicales. A la dermatoscopia no se observaron hallazgos distintivos. 42
... Billroth, in 1859, described a chondroid syringoma for the first time as a type of salivary gland tumor composed of both cartilaginous and muco-myxoid areas [5]. In 1961, Hirsch and Helwig were the first to introduce the term 'chondroid syringoma', while describing a mixed tumor of the skin originating from the eccrine glands [6]. Histologically, the tumor has a distinct histomorphology with both epithelial and stromal components. ...
A chondroid syringoma is a rare primary benign skin adnexal tumor with distinct histomorphology. However, extensive metaplastic bone formation within the tumor can raise diagnostic concerns. Further, the eyelid is quite a rare site of involvement of this tumor. Herein, we report a case of a chondroid syringoma with metaplastic bone formation involving the right upper eyelid in a 60-year-old female patient. The Ki67 labeling index was low. On follow-up for 36 months, no recurrence was noted. Acquaintance with the diverse histomorphological and clinical presentation of this tumor can prevent major diagnostic pitfalls.
... Cutaneous mixed tumors (MTS), also known as chondroid syringoma (CS), are rare benign neoplasms of sweat glands. They are characterized by a composite histological composition of both epithelial and mesenchymal elements [1,2]. Despite being first reported by Nasse in 1892, the etiology of MTS remains unknown, with reported incidence rates varying between 0.01 and 0.098% in previous studies [3]. ...
In this study we aimed to understand and summarize the clinical and pathological characteristics of cutaneous mixed tumors. A retrospective analysis was conducted on the clinical and pathological data of 20 patients diagnosed with cutaneous mixed tumors in our outpatient department between January 2014 and April 2024. The study comprised 20 patients with a male-to-female ratio of 3:2. The mean age of onset was 52.10 ± 15.46 years. All patients presented with solitary cutaneous lesions ranging from 2 to 30 mm in diameter. Notably, none of the patients received a preoperative diagnosis of cutaneous mixed tumor. The clinical manifestations of cutaneous mixed tumors are nonspecific, necessitating histopathological examination for a definitive diagnosis.
... Most notably, reviews of case reports in the 1950s of so named mixed tumors of skin of salivary gland type led to the recognition of salivary-gland-independent cutaneous tumor histogenesis and their reclassification as chondroid syringomas [17,18]. Eventually, further case reports and reviews presented pleomorphic adenomas and myoepitheliomas-a related myoepithelial tumor without ductal differentiation-arising in other organs including, but not limited to esophagus, breast, lacrimal glands, and kidneys [19][20][21]. ...
... [5] Mixed tumor of the skin was first described by Billroth in 1859 according to its histopathological dual composition of epithelial and myoepithelial cells; epithelial elements were in the form of myxoid, cartilaginous, or fibrous components. [6] Then Hirsh and Helwig [7] reviewed a large series of mixed tumors of skin and found chondroid proliferation in the tumor stroma, especially lesions arising from scalp and named it as chondroid syringoma. While lesions from other sites like periocular regions contain myxoid stroma, it was later found that these histological features are similar to PA of salivary glands. ...
A 26-year-old male presented with a cystic mass in the right upper eyelid of 3 years duration. The lesion was 1.0 cm × 1.0 cm in size with a smooth surface, nontender, and freely mobile under the skin. Complete surgical excision of the lesion was done under local anesthesia. The histopathological findings were suggestive of a pleomorphic adenoma (PA) of the right upper eyelid. PA of the eyelid is a rare tumor and needs to be considered in the differential diagnosis of eyelid mass lesions. Complete excision can help prevent future recurrences.
... Chondroid syringoma is exceptionally rare in the periorbital region, particularly in the upper eyelid.This tumor accounts for only 0.01% of all primary skin tumors and occur only rarely in eyelids.Till now 27 cases of chondroid syringoma of ocular 3,5 adnexa have been reported so far Chondroid syringoma is a benign , non-ulcerated and nodular tumor that occur largely on the face, head and neck 5 but also on the extremities and trunk . Histologically, histochemically and ultrastructuraly, their appearance can be very well comparable to that of mixed tumors of salivary [7][8][9] gland origin . This includes the presence of cells with an 10,11. ...
Chondroid syringoma is a benign adnexal mixed tumor of skin characterized by sweat gland elements in a cartilaginous stroma1. It is a tumor found to be arising from the sweat glands which can be apocrine (found throughout the surface of lid) or eccrine (glands of moll) with a mean age of 50 years2. Chondroid syringoma is a rare benign tumour that accounts for 0.01% of all primary skin tumours3 and occurs rarely in the upper eyelid region. Head and neck region involvement is common (nose is the most common site).It is morphologically similar to pleomorphic adenoma or benign mixed tumour of the salivary gland4. A 18-year-old male patient presented to us with a slow growing, painless mass on right upper eyelid since 6 months. Complete surgical excision of the mass was done under local anaesthesia. This report aims to discuss about this rare benign tumor of the eyelid and to study the histopathological aspects of it.
... Hirsch and Helwig coined the term "chondroid syringoma" in 1961 due to the presence of sweat gland features in a cartilage-like stroma. Headington classified chondroid syringomas into apocrine and eccrine types based on histologic appearance [25,26]. Most cases have an unremarkable presentation as a benign mass, and thus, most diagnoses are made on microscopic examination post-excision. ...
A variety of cutaneous disorders can present to the general surgeon either directly or by referral for surgical intervention. Some conditions are commonly seen and operated on by general surgeons which include lipoma, epidermoid cyst, etc. On the other hand, some are uncommon conditions like dermatofibrosarcoma protuberans and chondroid syringoma which require a high index of suspicion to diagnose. Most general surgeons are not familiar with the latest guidelines to treat such uncommon conditions. In this article, we provide a review of uncommon cutaneous disorders requiring surgical intervention that were encountered at our high-volume tertiary care center and a discussion about their etiology, presentation, diagnosis, management and follow-up with one case report of each condition. The objective of this article is to familiarize the general surgeon with these cutaneous disorders which though uncommon, will present in their practice at some point.
... The term "CS" in lieu of mixed tumor of the skin was introduced by Hirsch and Helwig in 1961. [5] CSs most commonly occur in the head and neck with a size ranging from 2 mm to ≥1 cm and usually present with solitary, solid, painless, nonulcerative, subcutaneous, or intracutaneous nodule. They often affect middle-age to elderly patients with a male-to-female ratio of 2:1. ...
Chondroid syringomas (CSs) are benign adnexal skin tumors usually affecting adolescent females in the periorbital region, and may have calcifications. Malignant transformation is very rarely encountered. There is a tendency to either medical treatment or intralesional injection of these lesions when confirmed benign especially if multiple. We herein describe a female with a periorbital benign CS. The presurgery cytology failed to predict the diagnosis, but surgical excision was attempted with good result. Both pathologists and surgeons are encouraged to add this differential in their diagnostic package of facial subcutaneous swellings.
... The term 'chondroid syringoma' was first introduced by Hirsch and Helwig in 1961 to describe this neoplasm because of the presence of sweat gland elements set in a cartilaginous stroma [8] . The term, mixed tumor, was coined by Virchow and Minssen for tumors with microscopic features that indicate both epithelial and mesenchymal origin. ...
... First described in 1859 by Billroth, it was initially called "mixed tumor or myxoid of the skin", by analogy with mixed tumors of the salivary glands [1,2]. In 1961, Hirsch and Helwig introduced the name "chondroidsyringoma" in order to differentiate common mixed tumors of the salivary or lacrimal glands from those, much rarer, of the skin, originating in the sweat glands [2,3]. In the same year, Headington differentiated two types: apocrine and eccrine, characterized respectively by a double cuboid cell base, and small tubular lumens with a single cell base [4]. ...
Myoepithelial tumours are rare and distinct entities with uncertain histogenesis. They occur primarily in major salivary glands and soft tissue around the head and neck. Bony involvement predominantly occurs in facial bones. Their involvement in long bones is extremely rare. There is no sex predisposition with a wide age range, though they are less common in the elderly. Histologically, they show a wide morphological spectrum and cytological heterogeneity with myxoid stroma and chondroid differentiation, with cells ranging from spindled, epithelioid and plasmacytoid forms, causing significant diagnostic dilemmas. The coexpression of at least one epithelial marker and S-100 or GFAP protein is required for the diagnosis. Here we report a rare case of an early adolescent male with a myoepithelial carcinoma of the left proximal tibia, which is owing to an unusual location. This article has shared our experience, managing this rare medical identity with excellent outcomes.
Chondroid syringoma, a mixed tumor of the skin, is an acquired hamartoma that differentiates into hair follicles, sebaceous glands, and apocrine sweat glands. Chondroid syringoma in the ear region is exceptionally rare. However, we present 2 cases of ear chondroid syringoma: 1 involving multiple lesions in the external auditory canal and the other including a single lesion behind the auricle. We reviewed relevant literature to offer insights into the diagnosis and treatment of ear tumors in the future.
Chondroid syringoma (CS) is a rare benign skin adnexal neoplasm. Its incidence is very low among all primary skin tumors. Diagnosing it clinically can be difficult as it closely resembles other benign salivary or adnexal neoplasms. Until now, only a few cases have been reported from the periorbital region. Clinically, it can be mistaken for epidermal cysts, but ectopic salivary glands are a close differential diagnosis. To diagnose it, a complete removal is usually required, followed by a histopathological examination. We present a unique case of a 45-year-old female patient with a CS in her right supraorbital ridge and review the pertinent literature.
Chondroid syringoma (CS) is a benign, slow-growing mixed tumour that arises from the sweat glands and usually presents in the head and neck area. Histopathological examination is important for proper diagnosis, as CS is often confused with epidermal cysts due to its rare presentation. This article presents a man in his 40s with a right upper lip mass that emerged 6 months prior to presentation. An intraoral surgical excision was performed and the histopathological analysis revealed solid epithelial cells that formed multiple, non-branching ducts lined by cuboidal epithelium. Cystic spaces were filled by heterogeneous eosinophilic material embedded in chondromyxoid stroma. Histopathology identified the lesion as an eccrine-variant CS. The patient recovered well.
The clinical presentation of skin appendage tumours for the most part is not as specific and diverse as the histological features of this extremely heterogeneous tumour group. Differential diagnosis of certain appendageal tumours can be extremely subtle, yet greatly influence final treatment outcome. Occasional association of certain entities with inherited tumour syndrome s can be extremely important in terms of hereditary cancer screening. This chapter aims to provide the most important clinical, histological and genetic features for selected, most important cutaneous appendage tumours.
Background
Cutaneous malignant mixed tumor (MMT) is a rare sweat gland–derived tumor characterized by admixed malignant epithelial cells and chondromyxoid stroma. Approximately 50 cases have been described in the literature. Metastasis, which may occur in more than one-third of cases, is most common in the lung.
Methods
We summarized the clinicopathologic features of a patient with cutaneous MMT metastatic to the lungs. A literature review of similar cases was completed using Web of Science, Scopus, and PubMed databases.
Results
A woman in her 70s presented with an enlarging mass on her left eyebrow; histopathologic examination showed large islands of atypical cells with increased mitotic activity, admixed with necrosis on a background of fibrotic and chondromyxoid stroma. Multiple lung nodules were identified during follow-up. Examination of a pulmonary core needle biopsy specimen was consistent with metastatic cutaneous MMT. Literature review identified 10 cases published between 1980 and 2017. Most primary tumors were large (≥4 cm). Local recurrence was uncommon, and the lung was the only metastatic site in 5 cases. Histopathologically, metastatic tumors were described as more cellular, with diminished stromal tissue compared with the primary lesion.
Conclusion
This is 1 of the 11 reports of cutaneous MMT with metastasis to the lungs found in the English-language literature published after 1980. Of note, most reports were published before 1990, making this case study one of the few contemporary descriptions of cutaneous MMT with pulmonary metastases. We think that the present case report will increase the awareness of this rare tumor.
Mixed tumor of the skin (MTS) is a tumor characterized by folliculosebaceous–apocrine differentiation. Because of the wide range of histological variations, understanding the unique features of MTS can help improve diagnosis. This study describes the histopathological characteristics of MTS, mainly apocrine-type MTS (AMT), using 166 cases of AMT. We found that nodular aggregates of myoepithelial cells, mucinous changes in the stroma, and follicular differentiation were standard characteristic features of MTS. Among the cases studied, 67% showed prominent follicular germinative cells and 40% showed prominent lipomatous metaplasia in the stroma. These cases often pose difficulties for the diagnosis of AMT because of insufficient evidence of sweat glands or myoepithelial cell differentiation. This is the first study to examine how the histological features of AMT change as the tumor extends deeper into the dermis. We found that the proportion of AMT with folliculosebaceous differentiation and large lumina increased as it got deeper into the dermis. Histopathological diagnosis of MTS is vital because the clinical symptoms lack specificity. This study enhances our understanding of the histopathological characteristics of MTS.
Chondroid syringoma is a rare adnexal tumor of skin, with few cases diagnosed on fine needle aspiration cytology (FNAC). Hyaline cell-rich chondroid syringoma (HCRCS) is a very uncommon variant of chondroid syringoma described in histopathologic studies. This variant differs from the usual chondroid syringoma in its site of presentation, cytohistologic features, and morphologically mimics malignant neoplasms. To the best of our knowledge, cytologic features of this entity have never been described. This case report highlights some unusual features of this tumor and discusses the cytologic features of HCRCS along with neoplastic mimics.
Fully updated throughout, the second edition of this bestselling book provides a comprehensive guide to the pathology of soft tissue tumors and tumor-like lesions. Reflecting the latest WHO classification throughout, this new edition incorporates advances in clinicopathologic, biologic and genetic studies. As such, detailed coverage of immunohistochemistry and molecular diagnostics is included for each entity. Soft tissue tumors are broadly defined to include metastatic melanomas, carcinomas, and lymphoid proliferations in soft tissue, enabling the reader to distinguish between easily confused entities. Chapters are richly illustrated throughout with high-quality colour images, depicting both typical histology and variants of each entity. Each printed copy of this new edition is also packaged with a password, providing online access to the book's text and images. Written and edited by renowned international leaders in the field, this is an essential guide to all diagnostic modalities in soft tissue pathology.
Although chondroid syringoma rarely occurs outside the head and neck, the majority of malignant chondroid syringomas are identified in the extremities. Here, we present a case of atypical chondroid syringoma in the fifth toe. Diagnosis of chondroid syringoma with atypical cells was made following initial excisional biopsy and histology, necessitating repeated surgery for positive margins. In this case report, we examine the radiopathologic correlation of this diagnosis, detail the imaging findings of benign and malignant chondroid syringomas, and highlight how magnetic resonance imaging can be used to guide surgical planning and treatment course of this potentially malignant tumor.
Chondroid syringomas are rare, benign tumors originating from sweat glands occurring as singular lesions of the head and neck. This case report presents a patient in whom multiple chondroid syringoma lesions were found. Thus illuminating the possibility for such occurrences in the future of which physicians should be aware.
Chondroid Syringoma is a rare benign adnexal skin tumor arising from sweat glands(apocrine or eccrine) situated in the dermis. They are often mistaken for other benign adnexal tumors of the skin and are most commonly seen in the head and neck region and, in individuals above the age of 40 years and predominantly in males. Rarely they can turn malignant and diagnosis is only by histopathology. We had an occasion to treat a 50 years old female patient with this tumor occupying the entire right alanasi. We could treat her well by complete excision with out any deformity of the alanasi. We are reporting this case, since this is the rst case reported arising from the alanasi in the world literature and also review the available literature.
Chondroid Syringoma is a rare benign mixed tumor characterized by gland elements in a cartilaginous stroma. These tumors most commonly occur in the skin of the head and neck district, affecting only rarely the periorbital area.
Below we present two cases of Chondroid Syringoma, the first on the upper lip, and the second in the orbital cavity. Both are characterized by an epithelial component in a cartilaginous stroma.
We present the case report of a 58-year-old man with recurrent chondroid syringoma, which was histopathologically confirmed, who underwent exenteration surgery of the right eye. Furthermore, the patient was receiving postoperative radiation therapy, and presently there is no local and/or distant evidence of disease in the patient.
This chapter on cutaneous appendageal tumours covers the spectrum of lesions displaying eccrine, apocrine, sebaceous and follicular differentiation. It includes cysts, hamartomas and benign and malignant tumours. Traditionally, appendageal tumours have been classified according to rigid criteria separating lesions with eccrine, apocrine, sebaceous and follicular differentiation. This separation is clearly artificial and it is now acknowledged that lesions regarded as strictly eccrine may well have apocrine differentiation and lesions classified as purely follicular may exhibit apocrine differentiation reflecting the close embryological relationship between the pilosebaceous unit and the apocrine gland.
The chapter summarizes important clinical and histological findings of cutaneous skin adnexal tumors. Thus, a well-trained clinician is able to diagnose some cutaneous adnexal tumors on the basis of clinical findings or at least is able to give a spectrum of relevant differential diagnosis. Depending on the morphological differentiation, a distinction is made between follicular tumors containing structures of the hair follicle, sweat gland tumors with eccrine or apocrine differentiation, and sebaceous skin tumors. In addition to benign tumors, the morphological criteria for malignant skin adnexal tumors are described. With regard to the different growth patterns and the biological behavior, we give important recommendations for biopsy technique and subsequent surgical treatment.
Whilst benign cutaneous mixed tumour is common, malignant cutaneous mixed tumour is rare. There are only eleven accepted cases of the malignant counterpart in the literature. In none was there residual benign tumour tissue present to suggest that they arose from malignant transformation of the benign tumour. We report a very rare case of a malignant transformation of a benign cutaneous mixed tumour in an eighty-four year old female. Other unusual features in this case included considerable involvement of bone in the primary lesion and the histological picture of extreme pleomorphism and active mitoses, not seen in other reported cases of the malignant tumour.
Two cases of eccrine tumour of sweat gland origin found in the hand are presented. They were on-tender rounded masses of eight months to two years duration, beneath and adherent to the pidermis but movable on underlying tissue. The pre-operative diagnosis in each case was that of sebaceous cyst. Histology revealed one to be an eccrine spiradenoma and the other a chondroid syringoma. The lesions were considered unusual in that such tumours usually occur on the trunk, head and back and are rarely found in the hand.
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