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Nasal Chondromesenchymal Hamartoma in a Child

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Stefanos Finitsis at Aristotle University of Thessaloniki
Stefanos Finitsis
Constantinos Giavroglou at Aristotle University of Thessaloniki
Constantinos Giavroglou
Stamatia Potsi at Interbalkan European Medical Center
Stamatia Potsi
Jannis Constantinidis at Papageorgiou Hospital
Jannis Constantinidis
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Nasal chondromesenchymal hamartoma (NCMH) is a benign tumor that was described in 1998. The occurrence of this lesion in the nasal cavity of infants and children is especially rare, with only 21 cases reported in the international literature. We report a 12-month-old boy with respiratory distress due to nasal obstruction. Computed tomographic scan and magnetic resonance imaging examination demonstrated a soft-tissue mass obstructing the left nasal cavity. Digital subtraction angiography and preoperative superselective embolization with microparticles were also performed. The tumor was completely resected surgically. Histopathology and immunohistochemical analyses of the tumor disclosed a NCMH. The imaging characteristics of the tumor are described and the radiology literature is reviewed.
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CASE REPORT
Nasal Chondromesenchymal Hamartoma in a Child
Stefanos Finitsis ÆConstantinos Giavroglou Æ
Stamatia Potsi ÆIoannis Constantinidis ÆAngelos Mpaltatzidis Æ
Dimitrios Rachovitsas ÆValentini Tzioufa
Received: 29 May 2008 / Accepted: 6 October 2008
ÓSpringer Science+Business Media, LLC 2008
Abstract Nasal chondromesenchymal hamartoma
(NCMH) is a benign tumor that was described in 1998. The
occurrence of this lesion in the nasal cavity of infants and
children is especially rare, with only 21 cases reported in
the international literature. We report a 12-month-old boy
with respiratory distress due to nasal obstruction. Com-
puted tomographic scan and magnetic resonance imaging
examination demonstrated a soft-tissue mass obstructing
the left nasal cavity. Digital subtraction angiography and
preoperative superselective embolization with microparti-
cles were also performed. The tumor was completely
resected surgically. Histopathology and immunohisto-
chemical analyses of the tumor disclosed a NCMH. The
imaging characteristics of the tumor are described and the
radiology literature is reviewed.
Keywords Chondromesenchymal hamartoma
Nasal cavity CT MRI Digital subtraction angiography
imaging Transcatheter embolization Newborn infants
Introduction
Nasal chondromesenchymal hamartomas (NCMHs) are
extremely rare tumors [1,2]. Only 21 cases have been
reported in the international literature. They usually present
as polypoid lesions shortly after birth. They are benign
tumors, and complete resection is the treatment of choice
[1,3,4]. NCMH has also been called ‘‘chondroid hamar-
toma,’’ ‘‘mesenchymoma,’’ ‘‘nasal hamartoma,’’ and other
names in the literature [5]. McDermott et al. first suggested
the term ‘‘nasal chondromesenchymal hamartoma’’ in 1998
as a distinct pathologic entity [6]. The tumor contains a
mixture of mesenchymal elements. Histopathologic and
computed tomography (CT) and magnetic resonance
imaging (MRI) characteristics have been well described.
Digital subtraction angiography features have only been
reported in three cases in the radiology literature.
We report a 12-month-old boy with NCMH. To our
knowledge, this is the first case described in the interna-
tional literature that was preoperatively embolized. We
describe the imaging findings and review the literature.
Case Report
A 12-month-old boy was admitted to our hospital with
respiratory distress. The symptoms started 3 months ago.
Nasal endoscopy revealed a large polypoid mass that filled
the left nasal cavity and caused deviation of the lateral
nasal wall. Ophthalmologic examination was normal. The
patient had no other significant medical history. Laboratory
studies were within normal limits.
A contrast CT scan demonstrated a lobulated, hetero-
geneous mass in the left nasal cavity measuring
494.2 cm
2
. The mass filled the left nasal cavity,
S. Finitsis C. Giavroglou S. Potsi (&)A. Mpaltatzidis
Department of Radiology, AHEPA Hospital, Aristotle
University of Thessaloniki, Thessaloniki, Greece
e-mail: matinapotsi@hotmail.com
I. Constantinidis D. Rachovitsas
Department of Otorhinolaryngology, AHEPA Hospital, Aristotle
University of Thessaloniki, Thessaloniki, Greece
V. Tzioufa
Department of Pathology, School of Medicine, Aristotle
University of Thessaloniki, Thessaloniki, Greece
123
Cardiovasc Intervent Radiol
DOI 10.1007/s00270-008-9458-6
displaced the nasal septum, and extended into the maxillary
sinus and nasopharynx. Remodeling and expansion of the
adjacent bony structures were seen (Fig. 1). The medial
wall of the orbit was preserved, and there was no intra-
cranial extension.
MRI revealed an ovoid encapsulated mass occupying
the left nasal cavity, extending into the left maxillary sinus
and nasopharynx. Coronal, sagittal, and axial contrast-
enhanced T1-weighted images revealed heterogeneous
enhancement. Axial T2-weighted magnetic resonance
images revealed a homogeneous high signal (Fig. 2). Dif-
ferential diagnosis included a nasal angiomatous tumor.
Therefore, it was decided to explore the lesion angio-
graphically under general anesthesia and eventually to
perform a preoperative embolization.
The right common femoral artery was catheterized, and
a 4-F catheter was inserted via the Seldinger technique.
Special care was taken in the amount of contrast media
that was used and the total amount of liquids provided.
The bilateral external carotid angiography, which was
performed under systemic heparinization, showed that
both infraorbital arteries supplied the lesion. Branches of
the right facial artery also contributed to the tumor blood
supply (Fig. 3A). Although the tumor showed a mild
angiographic blush, we decided to embolize it to reduce
the perioperative blood loss and facilitate complete
removal.
With a coaxial technique (Rapid Transit microcatheter,
Cordis), both infraorbital arteries were sequentially cathe-
terized, and the tip of the catheter was placed as close to
the lesion as possible. Then the vessels were embolized
with microparticles 250–350 lm in size (Embospheres,
Boston Scientific). A follow-up angiography showed
complete devascularization of the tumor (Fig. 3B).
Immediately after embolization, the patient was trans-
ferred to the operating suite of the ear, nose, and throat
clinic. Access of the mass was conducted after midfacial
degloving. A mass of considerable size, with a smooth
external surface and distinct capsule, was revealed. It was
displacing, without infiltration, the adjacent structures (left
maxillary sinus, ethmoid sinuses, medial wall of left orbit
and nasal septum). The mass was soft and pedunculated,
Fig. 1 A Postcontrast axial CT
scan revealing a lobulated mass
in the left nasal cavity
displacing the nasal septum and
the lateral wall of the nasal
cavity. Hyperintense foci may
be observed in the mass. BThe
mass also displaces and erodes
the bony margins of the medial
wall of the left orbit
Fig. 2 A Axial contrast-
enhanced T1-weighted images
revealed a large
heterogeneously enhancing
mass in the left nasal cavity. B
Axial T2-weighted MRI scan
demonstrating a diffuse high
signal
S. Finitsis et al.: Nasal Chondromesenchymal Hamartoma in a Child
123
with the pedicle attached to the lateral nasal wall without any
infiltration of adjacent structures. No significant bleeding
occurred during the operation, and complete resection of the
tumor mass was achieved. The recovery of the patient was
uneventful, and he was discharged a week later.
Histological examination revealed that the specimen
was composed of lobules of benign hyaline cartilage
with small areas of spindle cells stroma and prominent
vessels between them. The cytologically benign cartilage
was mildly hypercellular, with occasional binucleate
chondrocytes without appreciable cytologic atypia or
pleomorphism. Mitotic figures were not present. Tiny bony
trabeculae were present locally. Stromal and cartilage cells
were positive for vimentin. S-100 immunoreactivity was
evident in the lobules of mature cartilage and in scattered
stromal cells. Some spindle cells were positive for SMA.
No cells were positive for cytokeratins and EMA (Fig. 4).
Histopathologic examination and immunohistochemical
analyses of the tumor disclosed a NCMH.
Discussion
NCMH is a rare benign tumor that occurs predominantly in
infants [5,7]. They usually manifest as polypoid lesions
shortly after birth. The neoplasm is composed of mesen-
chymal–stromal and chondroid tissue in varying
proportions [2]. Most documented cases with characteristic
histologic features occurred in infants, especially in the first
year of life [3,4,6–9]. However, there are a few reported
cases in adolescents [1,2,5,6,10,11] and even adults,
with the oldest patient being 69 years old [5]. The NCMH
demonstrates characteristic clinicopathological features
and is presumed to be a benign tumor that usually occurs in
the nasal cavity in the newborns and infants, occasionally
showing paranasal and intracranial extension. NCMH may
be analogous to a similar entity involving the chest wall of
neonates and children, the so-called mesenchymal hamar-
toma of chest wall [2,6]. The pathogenesis of hamartomas
of the sinonasal tract is unknown, but they may have an
embryologic origin [1,5]. They are apt to be misdiagnosed
because of overlapping histologic features shared with a
number of benign and malignant soft tissue tumors.
Awareness of this entity is essential for correct diagnosis
and adequate therapy.
NCMH manifestation and its symptoms depend on the
size and location of the tumor as well as involvement of
surrounding structures. Symptoms associated with NCMH
include respiratory and feeding difficulties, rhinorrhea,
epistaxis, visual disturbances, and otitis media. Orbital
involvement of the tumor can result in proptosis, enoph-
thalmos, or impairment of eye movement [1,4].
Concerning diagnostic tools, CT and MRI are the
dominant modalities in the noninvasive preoperative
evaluation of NCMH. They both provide useful informa-
tion not only about the tumor itself, but also about
paranasal sinus involvement, bony remodeling (thinning or
erosion), and intracranial extension. Even though they are
Fig. 3 A Superselective
catheterization of the left
external carotid artery shows a
faint angiographic blush
vascularized by the left
infraorbital artery (arrow). B
Postembolization angiography
shows occlusion of the
supplying vessel (arrow)
Fig. 4 S-100 stains cartilaginous component and spindle cells
(magnification 2009)
S. Finitsis et al.: Nasal Chondromesenchymal Hamartoma in a Child
123
benign tumors, their radiological appearance can raise
suspicion for malignancy as a result of erosion of the
surrounding bone, cortical thinning, displacement, and
intracranial extension. The differential diagnosis for a
sinonasal mass in the child is broad, with all entities being
rare. Benign pediatric tumors with imaging features that
can mimic NCMH include hemangioma, angiofibroma,
nasal glioma, inverted papilloma, giant cell reparative
granuloma, ossifying fibroma, chondro-osseous respiratory
adenomatoid hamartoma, and aneurysmal bone cyst.
Malignant pediatric tumors that can mimic NCMH include
rhabdomyosarcoma, esthesioneuroblastoma, and chondro-
sarcoma, with the latter two usually seen only in the
adolescent age group [1]. It is essential that the correct
diagnosis is made so that potentially harmful therapies can
be avoided.
Imaging typically reveals a nonencapsulated, poorly
defined mass, often with both solid and cystic components
[2]. The size of the reported nasal mass ranged from 1.4 to
5.5 cm. CT findings in 23 patients have been reported in the
literature [1–11]. Fourteen patients (60.8%) demonstrated
paranasal sinus involvement, 12 (52%) demonstrated bony
remodeling, thinning, or erosion, and 10 (43.4%) demon-
strated intracranial extension through the cribriform plate. In
10 patients (45.4%), the tumor had internal calcifications,
and in 7 (30.4%), there was a cystic component. In 11
patients with suspected orbital extension, surgery was
positive for tumor involvement in 9 (81.8%). Postcontrast
enhancement was present in 75% of patients in a postcon-
trast study (Fig. 5). In our case, the mass was encapsulated
and well defined, and it showed remodeling of adjacent bony
structures and heterogeneous enhancement. This raised the
suspicion of a angiomatous mass. MRI characteristics have
been reported in four patients. All revealed a heterogeneous
nasal mass on T1-weighted images. T2-weighted images
reveal the coexistence of cystic components [4,6,7,10]. In
our case, the T1-weighted images showed an encapsulated
mass with heterogeneous enhancement, while the T2-
weighted images demonstrated a high signal. To shorten the
time of the MRI examination, which was desirable because
the child had to be sedated, we decided to take only the
sequences with the highest diagnostic yield. Therefore, we
did not perform a precontrast T1-weighted sequence. The fat
suppression sequence was not available at that time for
technical reasons. We considered the absence of frank fatty
tissue in the tumor to be information we had already
obtained from the CT images.
In the literature, angiographic examination has been
performed in only three patients, and the tumors were
characterized as avascular in all cases [3,5,6]. In our case,
preoperative angiography was performed because we
considered it as an angiomatous tumor. Although the tumor
proved to be poorly vascularized, we decided to embolize it
because we considered the procedure to entail a low risk
and would allow a safer surgical excision.
The treatment of choice of NCMH is complete resection.
There are no recurrences as long as the tumor is completely
removed [6]. Recurrence develops after incomplete resec-
tion or microscopic deposits of residual tumor [4,6].
Residual tumor or unresectable lesions may present a ther-
apeutic challenge.
In summary, we report an additional case of this benign
nasal tumor usually occurring in infancy. It is essential that
the correct diagnosis be made so that potentially harmful
therapies can be avoided. Awareness of the imaging char-
acteristics will lead to appropriate close inspection of
commonly involved surrounding structures. CT scanning
and MRI are helpful in both characterization and description
of the anatomic extent or invasion. Angiographic examina-
tion provides useful information in cases of angiomatous
tumors. Superselective embolization and devascularization
of such tumors may play a role in the preoperative man-
agement, making the complete resection easier. Moreover,
the operation time becomes shorter and safer, and less blood
is lost.
References
1. Johnson C, Nagaraj U, Esguerra J et al (2007) Nasal chondro-
mesenchymal hamartoma: radiographic and histopathologic
analysis of a rare pediatric tumor. Pediatr Radiol 37:101–104
2. Norman ES, Bergman S, Trupiano JK et al (2004) Nasal chon-
dromesenchymal hamartoma: report of a case and review of the
literature. Pediatr Dev Pathol 7:517–520
3. Kato K, Ijiri R, Tanaka Y et al (1999) Nasal chondromesenchy-
mal hamartoma of infancy: the first Japanese case report. Pathol
Int 49:731–736
4. Hsueh C, Hsueh S, Gonzalez-Crussi F et al (2001) Nasal chon-
dromesenchymal hamartoma in children: report of 2 cases with
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5. Ozolek JA, Carrau R, Barnes EL et al (2005) Nasal chondro-
mesenchymal hamartoma in older children and adults: series and
immunohistochemical analysis. Arch Pathol Lab Med 129:1444–
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Fig. 5 Computed tomography characteristics of NCMH in 23
patients
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6. McDermott MB, Ponder TB, Dehner LP et al (1998) Nasal
chondromesenchymal hamartoma: an upper respiratory tract
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Supplementary resource (1)

Nasal Chondromesechymal Hamartoma in a Child
Data
September 2013
Stefanos Finitsis · Constantinos Giavroglou · Stamatia Potsi · Jannis Constantinidis · Valentini Tzioufa
... [1][2][3][4][5] We described the combined computed tomography (CT) and magnetic resonance imaging (MRI) features of NCMH and its recurrence in a young boy with a pathological review. [3,[5][6][7] ...
... NCMH is most commonly seen in the nasal cavity of children <3 months old with male predilection, but may be seen up-to-the second decade of life, and occasionally in adults. [1][2][3][4][5][6][7][8] Studies have shown genetic as well as non-genetic components to NCMH pathogenesis. [9] e NCMH usually manifests as a polypoid expansile mass lesion occasionally showing paranasal, orbital, and intracranial extension without frank bone destruction. ...
... Intracranial extension through the cribriform plate is not infrequent. [6,7] However, frank bony destruction as seen in malignant tumors is not a feature of NCMH. [10] NCMH on CT is typically seen as a non-encapsulated and poorly defined mass often with or without cystic components; internal calcifications/chondroid matrix, and very occasionally as non-enhancing mass. ...
Chondromesenchymal hamartoma of sinonasal region in a young child: Computed tomography, MR imaging, and pathological findings with brief review
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  • Mar 2023
This report documents computed tomography and magnetic resonance imaging (MRI) findings for a case of sinonasal chondromesenchymal hamartoma and its recurrence occurring in an 11-year-old boy. We have described MRI dynamic contrast enhancement patterns in the recurrence of pathology, which, to the best of our knowledge, is the first documentation of such features. Sinonasal chondromesenchymal hamartoma is a rare pediatric benign hamartoma that can mimic aggressive inflammatory lesions or malignancies in the sinonasal region. A correct diagnosis is imperative to avoid unnecessary adjuvant therapy.
View
... On CT, NCMHs display presence of calcification in ~ 70% and osseous remodeling in majority of cases. Bony erosion may be seen, most commonly involving the cribriform plate and the lamina papyracea [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19], although frank bony destruction is usually absent [3,5]. Osseous changes are generally better appreciated on CT [19], but areas of mineralization within the cartilaginous matrix may also be suggested on MRI, specifically by hypointense T2 signal [19], or with the use of SWI. ...
... T2-weighted images most commonly show heterogeneous mass, with signal characteristics ranging from predominantly hyperintense in approximately 50% to predominantly hypointense in ~ 25% of cases [5-9, 11, 13, 14, 18-25]. Most of NCMHs show intermediate to hypointense T1 signal [5,7,8,10,22,23,25], with heterogeneous contrast enhancement present in ~ 75% of cases [5,7,9,13,15,19,23,24]. Of note, enhancement may only be peripheral in about 15% of cases [8,25]. ...
Pediatric nasal chondromesenchymal hamartomas: a case series
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Full-text available
  • Jan 2024
  • NEURORADIOLOGY
Purpose Nasal chondromesenchymal hamartomas (NCMH) are rare, predominantly benign tumors of the sinonasal tract. The distinction from higher grade malignancy may be challenging based on imaging features alone. To increase the awareness of this entity among radiologists, we present a multi-institutional case series of pediatric NCMH patients showing the varied imaging presentation. Methods Descriptive assessment of imaging appearances of the lesions on computed tomography (CT) and magnetic resonance imaging (MRI) was performed. In addition, we reviewed demographic information, clinical data, results of genetic testing, management, and follow-up data. Results Our case series consisted of 10 patients, with a median age of 0.5 months. Intraorbital and intracranial extensions were both observed in two cases. Common CT findings included bony remodeling, calcifications, and bony erosions. MRI showed heterogeneous expansile lesion with predominantly hyperintense T2 signal and heterogenous post-contrast enhancement in the majority of cases. Most lesions exhibited increased diffusivity on diffusion weighted imaging and showed signal drop-out on susceptibility weighted images in the areas of calcifications. Genetic testing was conducted in 4 patients, revealing the presence of DICER1 pathogenic variant in three cases. Surgery was performed in all cases, with one recurrence in two cases and two recurrences in one case on follow-up. Conclusion NCMHs are predominantly benign tumors of the sinonasal tract, typically associated with DICER1 pathogenic variants and most commonly affecting pediatric population. They may mimic aggressive behavior on imaging; therefore, awareness of this pathology is important. MRI and CT have complementary roles in the diagnosis of this entity.
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... In immunohistochemical study, the cells of the cartilage component are strongly positive for surface antigens such as vimentin and S100 protein. The cells of the mesenchymal components are positive for vimentin (1,11). Immunohistochemical study in our case revealed stromal vimentin positive staining, and cartilage islands were positive for S100 protein. ...
... For intracranial extension a combined intranasalneurosurgical approach is required (2,14). Although there is a case report of pre-operative embolization (11) and some recommendations for using adjuvant chemoradiotherapy for residual tumors (15), their validity cannot be accurately determined. After surgery, we did not use any complementary treatments. ...
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Article
Full-text available
  • Sep 2021
Background: Nasal chondromesenchymal hamartoma (NCMH) is a rare benign tumor almost always found in children. NCMH was first described by McDermott in 1998. To the best our knowledge, about 50 NCMH cases have been reported worldwide with very few adult cases and no long-term follow-ups. Case Presentation: Here, we report a 43-year-old woman who referred to the Otolaryngology Clinic of Payambar Azam Hospital, Bandar Abbas, Iran, in 2008, with a one-year history of progressive left-sided nasal obstruction that had not relieved with topical nasal decongestants and steroid sprays. A unilateral left-sided nasal soft-tissue mass was found upon examination. Histological and immunohistochemical analyses were consistent with NCMH. Trans-nasal endoscopic excision showed good results and no sign of recurrence has been observed after 12 years of regular follow-up. Conclusion: Awareness of imaging and immunohistochemical characteristics of NCMH combined with the probability of presentation in the adult population are necessary for correct diagnosis and adequate therapy.
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... The term: "Nasal chondromesenchymal hamartoma" was first suggested by McDermott et al. in 1998 [3] and has been used to date. NCHMs are mostly seen within the first 6 months of life [4]. The presentation and symptoms depend on the location and size of the tumor as well as involvement of surrounding structures. ...
... NCMH is an uncommon chondro-stromal proliferation of the nasal and sinus cavities, which is composed of one or more tissue components with mature diffentiation and disordered structure, showing circumscribed growth and no tendency of malignant degeneration. NCMH is an exceedingly rare tumor and mostly presents as an intranasal mass in infants in the first few weeks of life [4]. Neonatal nasal masses are rare, the overall incidence of these disorders has been reported to be around 1:20,000-1:40,000 live births [6]. ...
Transnasal endoscopic resection of nasal chondromesenchymal hamartoma in infancy: an analysis of 5 cases
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  • Jan 2022
  • BMC Pediatr
Background Nasal chondromesenchymal hamartomas (NCMHs) are extremely rare benign tumors that most commonly affect children in the first year of life. The purpose of this study was to investigate and summarize the characteristics of NCMH cases and the efficacy of transnasal endoscopic resection of NCMHs. Methods This is a retrospective study including 5 cases of infant diagnosed as NCMH between April 2016 and April 2020. Diagnostic techniques include nasoendoscopy, computerized tomography (CT) scan, magnetic resonance imaging (MRI) with contrast and microscopic and immunohistologic studies. Data collected included patient demographics, patient symptoms, radiographic findings, characteristics of tumor growth, follow-up time, recurrence, and postoperative complications. Results In 5 cases, 3 were males and 2 were females who aged 1, 2, 3, 6 months and 1 year, respectively. The size of the mass measured 1.6 cm*1.9 cm*1.8 cm at its smallest and largest was 4.0 cm*3.5 cm*3.0 cm. All five patients underwent tumor resection via transnasal endoscopic approach. Four tumors were completely removed, and one underwent partial resection, which was completely resected by midfacial degloving operation 13 months after the first surgery. There was no postoperative complication. The current postoperative follow-up period was 1 to 4 years, and no recurrence has been observed. Conclusions Complete surgical resection of NCHM is necessary to resolve the symptoms and prevent recurrence. Transnasal endoscopic approach is a safe and effective choice for pediatric NCMH patients.
View
... In all the cases, the lesions were masses protruding from the nasal cavity or paranasal sinuses to the nasopharynx. [5][6][7][8] In this work, we report 2 new cases of nasopharyngeal CMH in adult patients, and to our knowledge, those are the only cases of CMH completely situated in the nasopharyngeal space. ...
... Nasopharyngeal involvement of CMH takes place through the protrusion of this lesion from the nasal cavity, paranasal sinuses, and skull base to the nasopharyngeal space, and only 4 such cases have been reported in the world literature. [5][6][7][8] We report the first 2 cases of CHM completely situated in the nasopharynx. In the first patient, the origin of the tumor was the posterior edge of the nasal septum and, in the second one, the posterolateral wall of the nasopharynx, near the pharyngeal part of the Eustachian tube. ...
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Chondromesenchymal hamartoma (CMH) is a rare, benign lesion of the nasal cavity, paranasal sinuses, and skull base, composed of islands of hyaline cartilage in a myxoid background. The vast majority of CMH cases are infants and young children. According to the world literature, nasopharyngeal involvement of CMH is extremely rare. In all cases, the lesions were masses protruding from the nasal cavity or paranasal sinuses to the nasopharynx. We hereby report 2 adult male patients with masses completely situated in the nasopharyngeal space. In the first patient, the tumor originated from the posterior edge of the nasal septum and in the second one, from the posterolateral wall of the nasopharynx, adjacent to the pharyngeal orifice of the Eustachian tube. In both patients, the lesion was excised endoscopically, and histopathological analyses were consistent with a diagnosis of CMH. To our knowledge, those are the only cases of CMH completely situated in the nasopharynx.
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A Systematic Review of Nasal Chondromesenchymal Hamartoma (NCMH) with a New Case Report
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Nasal chondromesenchymal hamartoma (NCMH) is a very rare, benign sinonasal tract tumor commonly affecting infants. In this paper, in addition to presenting a systematic review of the literature on NCMH, we also report an unusual case of NCMH in an adolescent patient. A systematic review conducted following the PRISMA guidelines. PubMed, EMBASE and manual search through references of relevant publication were utilised to gather all published case-reports of NCMH. Data collected from each case-report for patient demographics, site and size of NCMH, clinical presentation, co-morbidities, diagnostic methods, treatment options and follow-up methods. The systemic review collected sixty-two case-reports of NCMH (including our case) affecting 42 men and 21 women (2:1 male to female ratio). Mean average age was 5.1 years (age range: 1 day to 70 years). The anatomical sites of the tumor were: nasal cavity (n = 17), paranasal sinuses (n = 30), orbital region (n = 17), and the base of the skull (n = 16). The reported clinical manifestations were nasal obstruction or congestion (n = 29), nasal mass (n = 27), epistaxis (n = 6), orbital symptoms (n = 14). NCMH is a very rare cause of nasal masses in infants and toddlers. Our case and previous case reports confirm that NCMH can mimic other benign and malignant tumors, therefore we should be vigilant for rare pathologies that lead to nasal masses. Recently the link between DIECR1 mutation with NCMH has been established, so NCMH should be considered in any patient with nasal or orbital symptoms with a history of DICER1-related tumor spectrum.
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Nasal chondromesenchymal hamartoma: an update
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Objective: Nasal chondromesenchymal hamartoma is a rare pathology that can present with a myriad of symptoms to ENT, maxillofacial and ophthalmology departments. This study reviewed the literature on nasal chondromesenchymal hamartoma as well as adding three new cases to the reported literature. Method: This study was an up-to-date review of the world literature with the addition of three new cases to provide the most comprehensive review to date. Results: A total of 56 patients with nasal chondromesenchymal hamartoma were identified, ranging from children to adults. Nasal symptoms and ocular symptoms were most common, and surgical resection was the most frequent treatment modality. Conclusion: This study advocates for increased awareness of the condition associations for nasal chondromesenchymal hamartoma, multi-specialty treatment and the role for the ENT surgeon in treatment of the condition.
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Article
  • Feb 2021
Introduction: Nasal chondromesenchymal tumors (NCMT) are rare benign neoplasms that usually present in children <1 year of age. They can display rapid growth and significant local bony remodeling that can mimic a malignant process. Of the ∼50 published cases to date, few have documented the need for neurosurgical intervention. We herein report a NCMT in an infant treated with a staged cranial and transnasal approach, as well as summarize the available literature on this pathology. Case report: A newborn male with a compromised airway was noted to have a large sinonasal lesion. After stabilization, MRI demonstrated a 4-cm enhancing mass with diffuse sinus involvement and significant extension into the anterior cranial fossa, with displacement of the optic apparatus and hypothalamic pituitary axis. After an initial biopsy, the patient underwent a bifrontal craniofacial approach at 2 months of age, followed by a second-stage transnasal endoscopic approach at 15 months which resulted in a complete resection. There were no neurosurgical complications. Pathology was consistent with a NCMT. Discussion: Although rare, neurosurgical involvement is critical for the treatment of NCMTs with intracranial extension. Staged cranial and endonasal endoscopic approaches may be needed for complete resection of such lesions.
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Nasal Chondromesenchymal Hamartoma: Rare Case Report in an Elderly Patient and Brief Review of Literature
Article
Full-text available
  • Oct 2018
Hamartomas are considered a mixture of nonneoplastic tissue, which may be indigenous to a different location in the body. As such, they may be epithelial, mesenchymal, or mixed. In the sinonasal region, the following hamartomatous lesions are considered to lie on a spectrum and include respiratory epithelial adenomatoid hamartoma (REAH), chondro-osseous respiratory epithelial adenomatoid hamartoma (COREAH), and nasal chondromesenchymal hamartoma (NCMH). To our knowledge, less than 50 cases of sinonasal hamartomas have been reported in the English literature so far with NCMH being very rare and primarily a tumor in infancy, with only 2 cases reported in individuals older than 16 years of age. We report a highly unusual case of a NCMH in the right maxillary sinus of a 70-year-old female.
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Hamartomas
Chapter
  • Jan 2014
A hamartoma is a nonneoplastic heterotopia composed of an overgrowth of cytologically normal, mature cells in an abnormal, disorganized architectural pattern. Pathophysiological outcomes and differential diagnoses will depend upon area of involvement. It is a low-incidence disorder, more commonly found in young children and men. Diagnosis is based upon histopathological findings. Treatment is typically complete excision when possible, though it will depend upon the site of the tumor and the associated effects.
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Nasal Chondromesenchymal Hamartoma in Older Children and Adults: Series and Immunohistochemical Analysis
Article
  • Nov 2005
Context.—Nasal chondromesenchymal hamartoma is a benign mass lesion of the nasal cavity predominantly described in young infants. These unusual lesions are composed of a proliferation of mesenchymal and cartilaginous elements. Their pathogenesis is unknown, but they may be derived from embryologic rests. To our knowledge, only 1 case in an older child has been reported, and no cases have been reported in adults. Objective.—To report 4 cases of nasal chondromesenchymal hamartoma occurring in older children and adults, including immunohistochemical analysis of these unusual lesions. Design.—Cases identified from our archives were examined to confirm the diagnosis of nasal chondromesenchymal hamartoma. Immunohistochemical analysis was performed using a panel of antibodies (epithelial membrane antigen, smooth muscle actin, all muscle actin, cytokeratin, S100, and KP1) to evaluate for epithelial, smooth muscle, neural, chondroid, and histiocytic differentiation. Results.—Four cases of nasal chondromesenchymal hamartoma in patients of 11, 69, 17, and 25 years of age demonstrated histologic evidence of mesenchymal and cartilaginous elements underlying a chronically inflamed respiratory mucosa. Bony and adipose elements and rare glandular elements were interspersed. Cartilaginous elements stained strongly with S100, whereas mesenchymal regions showed variable and weaker staining. Smooth muscle differentiation was seen primarily in the mesenchymal areas. Epithelial membrane antigen was focally positive in all cases. Conclusions.—Nasal chondromesenchymal hamartomas can rarely occur in the older child and adult. Mesenchymal areas show both myofibroblastic and cartilaginous differentiation. We speculate that inflammation or a recapitulation of developmental signals may be components in the pathogenesis of these lesions.
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Nasal Chondromesenchymal Hamartoma: An Upper Respiratory Tract Analogue of the Chest Wall Mesenchymal Hamartoma
Article
  • Apr 1998
  • AM J SURG PATHOL
Nasal chondromesenchymal hamartoma is the suggested appellation for a tumefactive process of the nasal passages and contiguous paranasal sinuses in seven children with a detectable mass in the nose. With the exception of one patient who was 7 years of age at diagnosis, the others were 3 months of age or less upon recognition of the mass. Two children were diagnosed in the first 2 weeks of life. Imaging studies showed a complex solid and cystic mass or masses filling the nasal cavity and extending into the ethmoid sinuses in most cases. Erosion of the surrounding bone, including the cribriform plate, resulted in an intracranial component in the four cases. Surgical resection was the treatment of choice despite its technical difficulties that often necessitated a combined intranasal and intracranial approach. Residual disease with continued growth in one case was the clinical outcome in two children, and the remaining five patients have not experienced any further difficulties. The piecemeal fragments of tissue disclosed a collage of histologic features, but the basic morphologic elements were well-demarcated nodules of cartilage with some variation in the cellular density and maturation of the chondrocytes, a myxoid to spindle cell stroma, focal osteoclastlike giant cells in the stroma, and erythrocyte-filled spaces resembling those of the aneurysmal bone cyst. Two of the tumors were less polymorphous or complex in their spectrum of histologic features. These nasal masses have similarities to the so-called chest wall hamartoma or mesenchymal hamartoma of the chest wall in terms of the clinical presentation in infancy and the basic cartilaginous character of both entities. There is a degree of presumption in the designation of these nasal and chest wall tumors as hamartomas because the pathogenesis has not been established for either entity.
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Nasal chondromesenchymal hamartoma of infancy: The first Japanese case report
Article
  • Sep 1999
The first Japanese case of nasal chondromesenchymal hamartoma, a rare infantile nasal neoplasm, is presented. A 4-month-old Japanese boy was referred to our Centre because of intranasal mass and oculomotor disturbance. Radiological images showed the intranasal tumor extending to the paranasal sinus, orbit, and anterior frontal fossa. Subtotal resection and radiation therapy to residual tumor were performed. There has been no recurrence of the tumor for 13 years. Histologically, the lesion demonstrated admixture of various mesenchymal elements, including cellular spindle cell stroma with occasional myxoid change, nodules of mature/immature cartilaginous tissue, focal osteoclast-like giant cells, and erythrocyte-filled spaces resembling aneurysmal bone cyst. The histology was consistent with the findings presented by McDermott et al. 1986. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin. Chondrocytes in the mature cartilaginous tissue were positive for S-100 and vimentin; chondrocytes in the immature cartilaginous tissue were positive for S-100, vimentin, and smooth muscle actin. Ultrastructurally, the spindle cells showed features of either fibroblast or myofibroblast.
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Nasal chondromesenchymal hamartoma in children: Report of 2 cases with review of the literature
Article
  • Apr 2001
Hamartoma in the nasal cavity of children is especially rare. Most documented cases occurred in infants, with characteristic histologic features of a mixture of various mesenchymal tissues. McDermott et al designated it nasal chondromesenchymal hamartoma in 1998, and it has since been considered a distinct clinicopathological entity. We report 2 such examples in a full-term male newborn and a 9-month-old boy, respectively. Histologically, both cases were characterized by a mixture of various mesenchymal elements, including spindle cells, collagen fibers, and irregular islands of osseous and chondroid tissue. Immunohistochemical study showed positivity to vimentin and S100 protein. Ultrastructural examination of case 1 demonstrated fibroblastic and myofibroblastic differentiation in tumor cells. There were 11 cases of nasal chondromesenchymal hamartoma in children published to date. The tumor has a benign biological behavior, and complete resection is the treatment of choice. It is apt to be misdiagnosed because of overlapping histologic features shared with a number of benign and malignant soft tissue tumors. Awareness of this entity is essential for correct diagnosis and adequate therapy.
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Nasal chondromesynchymal hamartoma presenting in an adolescent
Article
  • Jul 2003
  • Int J Pediatr Otorhinolaryngol
Nasal chondromesenchymal hamartoma (NCMH) is extremely uncommon primary benign cartilaginous growth of the nasal and paranasal sinuses. To date, it has been reported almost exclusively in infancy. We report a NCMH in a 16-year-old patient who presented with an asymptomatic, fixed swelling on the left side of the nose approximately 2x2 cm in size. Computed tomography (CT) and magnetic resonance imaging (MRI) of the nose, paranasal sinuses and neck confirmed a heterogeneous, calcified polypoidal mass protruding caudally into the left nasal cavity. After an initial inconclusive incisional biopsy, the patient underwent a complete radical resection, with staged reconstruction of the full nasal defect using septal mucosal flap, conchal cartilage graft and forehead skin flap. Histological examination of the resected specimen confirmed NCMH, which, we believe, had probably been present and undetected for many years. This report greatly extends the age at which NCMH may be entertained as part of the differential diagnosis of cartilaginous lesions of the nose and paranasal sinuses.
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Two unusual lesions in the nasal cavity of infants - A nasal chondromesenchymal hamartoma and an aneurysmal bone cyst like lesion - More closely related than we think?
Article
  • Apr 2004
  • Int J Pediatr Otorhinolaryngol
Benign reparative lesions in the head and neck region in infants are rare and often difficult to classify on histology. Discussed herein are two rare lesions in infants occurring at identical locations in the nasal cavity with striking histologic similarity but different histologic labels. One was a case of nasal chondromesenchymal hamartoma (NCMH) occurring in a 1-year-old child and the other an aneurysmal bone cyst (ABC) like lesion affecting a 4-month infant. Both these lesions were locally destructive and had nearly similar clinical presentation. Both on immunohistochemistry showed myofibroblastic nature and had similar histology except that the ABC like lesion lacked the cartilage component of the former. In view of great similarity in the two lesions, it was thought that the second lesion might also represent a reparative, non-cartilage-containing counterpart of the former.
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Nasal Chondromesenchymal Hamartoma of Infancy Clinically Mimicking Meningoencephalocele
Article
  • Sep 2004
We report a typical case of nasal chondromesenchymal hamartoma (NCMH) of infancy, which extended to the orbit and intracranium through the skull base. The nasal mass was incidentally found in a 5-month-old otherwise healthy boy who had recently developed ptosis of the left eye. On neuroimaging, there were defects in the left ethmoid bone of the anterior cranial fossa and medial orbital wall. A round heterogeneous mass was found in the left nasal cavity. Solid portions were strongly enhanced. The preoperative diagnosis was nasal glioma or encephalocele. After frontal craniotomy, the mass was separated from the cranial basal dura. Through the transnasal approach, the mass was dissected from the left orbital content and removed. NCMH is a rare benign lesion and histopathological analogue of the mesenchymal hamartoma of the chest wall, having unique pathology: islands of hyaline cartilage, somewhat calcified or ossified hyalinized collagenous trabeculae and abundant myxoid stroma. Its occurrence in the nasal cavity was identified in 1998. The recognition of this entity is necessary to avoid misdiagnosis of the frozen section and for appropriate surgery.
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Nasal Chondromesenchymal Hamartoma: Report of a Case and Review of the Literature
Article
  • Nov 2004
The sinonasal region is the site of several hamartomatous lesions, the majority of which are mesenchymal, with vascular hamartomas predominating. The occurrence of hamartomas in the nasal cavity of infants and children is especially rare. Nasal chondromesenchymal hamartoma (NCMH) is a rare lesion of the intranasal sinuses generally diagnosed in the newborn period, with the eldest reported patient presenting at 16 years of age. This neoplasm is composed of mesenchymal-stromal and chondroid tissue in varying proportions. It is felt to be analogous to the mesenchymal hamartoma of the chest wall, a lesion of similar histology generally involving the ribs and chest wall of neonates. To the best of our knowledge, only 14 cases of NCMH have been reported to date. We report a case of NCMH in an 11-year-old boy.
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Nasal chondromesenchymal hamartoma in older children and adults: Series and immunohistochemical analysis
Article
  • Dec 2005
  • ARCH PATHOL LAB MED
Nasal chondromesenchymal hamartoma is a benign mass lesion of the nasal cavity predominantly described in young infants. These unusual lesions are composed of a proliferation of mesenchymal and cartilaginous elements. Their pathogenesis is unknown, but they may be derived from embryologic rests. To our knowledge, only 1 case in an older child has been reported, and no cases have been reported in adults. To report 4 cases of nasal chondromesenchymal hamartoma occurring in older children and adults, including immunohistochemical analysis of these unusual lesions. Cases identified from our archives were examined to confirm the diagnosis of nasal chondromesenchymal hamartoma. Immunohistochemical analysis was performed using a panel of antibodies (epithelial membrane antigen, smooth muscle actin, all muscle actin, cytokeratin, S100, and KP1) to evaluate for epithelial, smooth muscle, neural, chondroid, and histiocytic differentiation. Four cases of nasal chondromesenchymal hamartoma in patients of 11, 69, 17, and 25 years of age demonstrated histologic evidence of mesenchymal and cartilaginous elements underlying a chronically inflamed respiratory mucosa. Bony and adipose elements and rare glandular elements were interspersed. Cartilaginous elements stained strongly with S100, whereas mesenchymal regions showed variable and weaker staining. Smooth muscle differentiation was seen primarily in the mesenchymal areas. Epithelial membrane antigen was focally positive in all cases. Nasal chondromesenchymal hamartomas can rarely occur in the older child and adult. Mesenchymal areas show both myofibroblastic and cartilaginous differentiation. We speculate that inflammation or a recapitulation of developmental signals may be components in the pathogenesis of these lesions.
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