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Hidradenoma of the Chin
Abstract
Clear cell hidradenoma is a rare skin tumor in the head and neck. Clear cell hidradenoma of skin is an uncommon soft tissue tumor originating from the sweat glands. The authors report a case of clear cell hidradenoma developing in the chin in a 61-year-old woman, which occurred during the course of 8 months. The clinical and histologic findings of the tumor are documented. The lesion was totally removed by excision and revealed no evidence of recurrence.
... There is no cellular atypia or aggressive behavior, and the overall architecture is rather homogeneous. 26 Hidradenomas do not have a specific immunophenotype and frequently express p40, p63, CK5/6, and CKAE1/AE3. 27 In contrast, hidradenocarcinoma typically presents as a solitary, slow-growing nodule, but histologically it shows aggressive features such as marked cellular atypia, pleomorphism, infiltrative growth, and areas of necrosis. ...
Gene rearrangements or fusions have emerged as critical oncogenic drivers in various cutaneous adnexal neoplasms. This review offers a comprehensive overview of both established and recently identified molecular alterations, with a specific focus on gene fusions. Key alterations discussed include YAP1 rearrangements , CRTC1::MAML2 fusions, BRD3 rearrangements, MYB::NFIB fusions, ETV6::NTRK3 fusions, and PLAG1 rearrangements , alongside rarer fusion transcripts, such as MEF2C::SS18, FOXK1::GRHL1/2, GPS2::GRHL, and RARA::NPEPPS. The article highlights the significance of these genetic changes in tumor biology and their potential therapeutic implications for locally advanced and metastatic skin adnexal tumors. It also addresses diagnostic challenges and molecular distinctions, providing updated insights into adnexal tumors driven by these gene fusions.
A 62-year-old woman, with neuropathic pain and paresthesia in her right forefoot, showed a circumscribed soft tissue swelling on the sole between the second and third metatarsal. Ultrasound (US) imaging showed a well-defined lesion in the second intermetatarsal space, without vascularization sign at Power Doppler (PD). In the first hypothesis, these findings led to Morton’s neuroma. Magnetic Resonance Imaging (MRI), demonstrated a dumbbell-shaped lesion between the II and the III metatarsal heads; it extended cranially to the subcutaneous fat of the dorsal slope. The MRI findings weren’t compatible with a classic Morton’s neuroma and were radiologically undetectable. The patient had a sub-total excisional biopsy. The anatomopathological features were specific to an apocrine hydroadenoma from an ectopic sweat gland. This rare pathology has not been previously described in the literature and it must be considered as a differential diagnosis due to the clinical presentation and the US appearance mimicking Morton’s neuroma.
Lymphoepithelial carcinoma (LEC) of salivary gland origin is a rare malignant tumor with morphological characteristics identical to those of undifferentiated nasopharyngeal carcinoma. It has a marked racial predilection for Inuit and Southeast Asian populations. An association between LEC and Epstein-Barr virus (EBV) infection has previously been reported. LEC most frequently affects the parotid gland, followed by the submandibular glands. To the best of our knowledge, only three LECs arising from the minor salivary gland have been previously described in the English language literature. The current study reports a case of EBV-associated LEC of the minor salivary gland in the hard palate of a 38-year-old Chinese female, and reviews the clinicopathological characteristics of this uncommon tumor.
Clear cell hidradenoma or nodulocystic hidradenoma or acrospiroma are histologically distinct relatively rare tumors of sweat gland duct origin, found mainly in adults with a female preponderance. We report a case of eccrine hidradenoma in a 31-year-old man who presented with an asymptomatic, solitary nodule on occipital region. A few reports are available in literature regarding presence of this tumor on occipital region of young man and present case is being reported because of its rarity in this region of scalp and in this sex.
Clear cell hidradenoma is a rare skin appendage tumor. A 41-year-old female presented with right gluteal mass. Excisional biopsy of the mass was performed. Under the epidermis, an eosinophilic-cytoplasm, uniform-appearance, oval-round-nucleus, benign tumor with cystic and solid components was detected. These results were consistent with clear cell hidradenoma. The patient had not been given postoperative adjuvant treatment and has been under follow up free from disease for 2 years.
A case of lymphoepithelial carcinoma occurring in the left submandibular salivary gland is presented. Histologic features of this rare malignancy are described in detail. This unusual carcinoma has been described frequently in Eskimos and is usually located in the parotid gland. The raised serum IgG to Epstein-Barr virus capsid antigen suggests a causal relationship between Epstein-Barr virus and this type of salivary gland carcinoma.
Background:
Lymphoepithelioma-like carcinoma (LELC) in the salivary glands is a rare but unique malignancy.
Methods:
Sixty-nine patients with salivary gland LELC with long-term follow-up were reviewed for this study.
Results:
There were 52 cases in the parotid gland and 17 cases in the submandibular gland. All patients underwent complete tumor excision, 41 underwent neck dissection, and 39 received postoperative radiotherapy. The 5-year, 10-year, and 15-year overall survival (OS) rates were 90%, 75%, and 54%, respectively. Patients with higher neutrophil/lymphocyte ratio (NLR ≥ 4.0) and advanced stage (stage III and IV) had significantly poorer OS. Patients who received postoperative radiotherapy had significantly better relapse-free survival (RFS). In multivariate analysis, stage, NLR, and neck dissection were associated independently with OS, whereas stage and postoperative radiotherapy were associated independently with RFS.
Conclusion:
Salivary gland LELC is a rare malignancy with a better prognosis that partially attributes to surgery with neck dissection and postoperative radiotherapy. Preoperative NLR is an independent prognostic factor.
A very rare case of nodular hidradenoma with an atypical clinical presentation of the face of a 70-year-old woman is presented. Although this rare benign tumor has the potential of local recurrence and malignant transformation, it is commonly reported in the dermatology, pathology, and oncology literature, with limited clinical information. The purpose of this report was to emphasize the clinical features of this lesion for plastic surgeons.
Undifferentiated carcinoma of the salivary glands is a rare disease, the incidence of which is highest among the Inuit of Greenland and North America. It was demonstrated to be closely related to Epstein- Barr virus (EBV) infection. However, the relation of EBV to this tumor has not been studied to any great extent here in Taiwan because of the small number of cases.
Twelve cases of undifferentiated carcinoma of the salivary glands from the period 1977-1996 were retrieved from the cancer registry at National Taiwan University Hospital. The clinical data were analyzed retrospectively based on the medical records. Eleven formalin fixed, paraffin embedded tissue sections were used for in situ hybridization with an antisense probe complementary to EBV-encoded RNA 1 (EBER1).
Ten of 12 tumors originated from the parotid gland and 2 from the submandibular gland. The patients' ages ranged from 22 to 63 years, with an average of 38.1 years. One patient was lost to follow-up, 2 patients died of metastatic disease, and the remaining 9 patients were all alive and disease free at last follow-up. The actuarial 5-year survival rate was 79.8%. In situ hybridization demonstrated EBER1 in 9 of the undifferentiated carcinomas with lymphoid stroma, but EBER1 was not demonstrated in the other 2 tumors without lymphoid stroma.
Undifferentiated carcinoma with lymphoid stroma of the major salivary glands is closely associated with EBV. The mainstay of treatment is surgery and postoperative radiotherapy. The outcomes in this series were good except for two elderly patients who died of rapid and progressive distant metastases.
Clear cell hidradenoma was diagnosed in a girl at 3
½ years of age. Only one case of hidradenoma has been documented in the first decade of life, although it remains unclear whether it was clear cell hidradenoma. This case demonstrates that clear cell hidradenoma is a rare differential diagnosis of cutaneous tumors even in young children, which is of special interest, because malignant transformations can occur. (J Am Acad Dermatol 2000;42:693-5.)
Clear cell hidradenoma is a benign skin appendage tumor that may mimic conventional-type renal cell carcinoma. Histologically, clear cell hidradenoma contains small ductular lumens, focal apocrine and squamoid change, and a less prominent vascular pattern than renal cell carcinoma. Furthermore, immunohistochemical studies can aid in distinguishing the 2 tumors. Knowing the cytologic features of primary skin adnexal neoplasms helps distinguish them from cutaneous metastases, which are more commonly referred for fine-needle aspiration biopsy evaluation. Detailed clinical history, physical findings, and ancillary studies are essential for correct diagnosis and categorization of these tumors. We report the rare case of a patient with renal cell carcinoma who underwent excision of an axillary clear cell hidradenoma, which was clinically suggestive of cutaneous metastatic disease.
The salivary glands, despite their relatively simple morphology, give rise to more than 30 histologically distinct benign and malignant tumors. Salivary gland neoplasms comprise less than 2% of all tumors in humans and 3% of all head and neck tumors. They arise in the parotid gland in 80% of cases, and approximately 80% are benign and 20% are malignant. Among them are lymphoepithelial lesions, rare lesions of the salivary glands and especially of the parotid gland that are characterized by lymphocytic infiltration associated with an epithelial proliferation. They are divided into benign, which is considered as a tumorlike condition, and malignant, which is a rare carcinoma of the salivary glands. This article provides a review of the current knowledge on lymphoepithelial carcinoma with a look at its association with benign lesions and on the importance of making the correct diagnosis for the appropriate treatment.
- Faulhaber
Epstein-Barr virus in nasopharyngeal and salivary gland carcinomas in Greenland Eskimos
- A K Salmundsen
- H Albeck
- Jph Hause
Salmundsen AK, Albeck H, Hause JPH, et al. Epstein-Barr virus in
nasopharyngeal and salivary gland carcinomas in Greenland Eskimos. Br
J Cancer 1982;46:721-728