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Nasal Chondromesenchymal Hamartoma in Children.

Report of 2 Cases With Review of the Literature

Nasal masses in infancy are infrequently encountered, and most of them are developmental anomalies, such as encephalocele, nasal glioma, hamartoma, and nasolacrimal duct cyst. The rest are neoplasms, primarily composed of teratoma and dermoid cyst and occasionally a variety of benign and malignant soft tissue tumors.[1,2] The overall incidence of these disorders has been reported to be around 1 per 20 000 to 1 per 40 000 live births.[2] Hamartoma, teratoma, and dermoid cysts share common histologic features composed of an admixture of tissues of diverse derivation. Hamartoma is a tumorlike malformation that results from excessive growth of tissues indigenous to the site of origin, whereas teratoma and dermoid cyst are considered neoplasms that arise from pluripotential cells that are foreign to the anatomical region where they occur. Teratoma typically contains tissues derived from all 3 germinal layers, whereas dermoid cyst presumably originates from ectoderm and mesoderm. Hamartomas are common in skin, lung, liver, chest wall, kidney, and gastrointestinal tract. In the region of the head and neck, hamartomas are uncommon but have been described in the oral cavity, nasal cavity, nasopharynx, hypopharynx, tongue, and eustachian tube.[3] The occurrence of hamartoma in the nasal cavity of infants and children is especially rare. Most previous examples in the literature were single case reports. The largest series was recorded by McDermott et al[4] and comprised 7 cases. Herein, we present 2 such cases with documentation of gross, microscopic, immunohistochemical, and electron microscopic findings, along with a review of the literature.


Case 1

A full-term male newborn was born to a gravida 1, para 1 mother at 41 gestational weeks via cesarean section. At birth, swelling of the left face and a polyp in the left nasal cavity were found. There was cyanosis with difficult breathing during feedings. The newborn was then transferred to Chang Gung Children's Hospital. Physical examination showed an alert newborn, evincing mucoid nasal discharge, and teary eyes. Pulse rate was 130/min and respiratory rate was 48/min. A fungating mass protruding through the left nostril from the left nasal cavity was noted, and the nasal septum was deviated to the right. The physical findings were otherwise unremarkable. Maternal history was remarkable for anemia and gestational diabetes. Both [Alpha]-fetoprotein and [Beta]-human chorionic gonadotropin levels were within normal limits. Magnetic resonance imaging (MRI) demonstrated a round mass in the left nasal cavity, involving the sphenoid sinus and ethmoid sinus and compressing the left orbit and nasal septum, but without intracranial component (Figure 1). Computed tomography (CT) revealed a mixed solid and cystic mass with foci of calcification located in the left ethmoid sinus, approaching the anterior skull base and the medial aspect of the left optic canal. Remodeling and expansion of the adjacent bony structure were seen. The left orbit was medially compressed, and exophthalmos had occurred. An excisional biopsy specimen was suggestive of angiofibroma. The mass became slowly and progressively enlarged, and proptosis of the left eye appeared at 1 month of age. A Weber-Fergusson lateral rhinotomy with total excision of the mass by craniofacial approach was performed 3 months after biopsy. At operation, the tumor was found to involve the left ethmoid sinus, left sphenoid sinus, and left orbital region and compressed the optic nerve. The left nasal cavity was dilated but not invaded by the tumor. The tumor was called extracranial fibromatous meningioma. The patient was free of recurrence for 5 years.


Case 2

A 9-month-old male infant was noted to have impaired right eye movement and asymmetric face since birth. He was brought to an ophthalmologist for persistent impairment of right eye movement. Hypotropia, enophthalmos, and ophthalmoplegia of the right eye were found. No epistaxis, rhinorrhea, or facial swelling was noted. Congenital orbital adherens syndrome was suspected, and MRI of orbits revealed retraction of the right eyeball with shortening of the right medial rectus and left deviation of the nasal septum. A soft tissue mass in the right maxillary sinus was found. The mass was not visualized at routine ear, nose, and throat examination. A CT scan of the head and neck showed a large oval-shaped, low-density, nonenhancing mass in the right anterior nasal cavity. A cyst of the nasolacrimal duct was suspected. Sinoscopy revealed an intact lateral wall of the nasal cavity. The inferior turbinate was incised, and a bulging tumor in the right maxillary sinus was taken out piecemeal through the maxillary sinus ostium. Frozen section was thought to be suggestive of fibrous dysplasia. On thorough study of permanent sections, the diagnosis of nasal chondromesenchymal hamartoma was made. The patient was well 6 months after operation.


The tissues were fixed in 10% buffered formalin and embedded in paraffin. Sections were stained with hematoxylin-eosin. Immunohistochemical studies were performed on formalin-fixed and paraffin-embedded tissues using the avidin-biotin-peroxidase complex method. The antibodies used included monoclonal antibodies against cytokeratin (AE1/AE3) and cytokeratin 14 (BioGenex, San Ramon, Calif), epithelial membrane antigen, vimentin, desmin, actin (HHF35), smooth muscle actin, glial fibrillary acidic protein, and CD34 (Dakopatts a/s, Glostrup, Denmark). The polyclonal antibodies used were against neuron-specific enolase (Dakopatts) and S100 protein (Dakopatts). CD34 and cytokeratin 14 were used only on case 2. The specimen of case 1 was fixed in 4% cocadylate-buffered glutaraldehyde and processed for ultrastructural examination with a Goel-1200EX electron microscope.


The nasal tumor of case 1 was light tan and rubbery and measured 6 x 4 x 4 cm. The external surface was smooth, except at its root. Cut surface showed a mucoid appearance and multiple cystic spaces that ranged from 0.1 to 1.6 cm in diameter. Foci of hemorrhage and calcification were seen. The specimen of case 2 consisted of multiple pieces of gray-to-tan soft tissue fragments that measured 0.7 x 0.6 x 0.4 cm in aggregate. 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 (Figure 2). The cellularity varied from loose to compact. There were fascicles of spindle cells sometimes arranged in a whorled pattern or admixed with thick collagen fibers. Scattered areas with myxoid change, cystic formation, and erythrocyte-filled spaces that resembled aneurysmal bone cyst were present. Also seen were areas rich in small thick-walled vessels in a spindle cell background that mimicked angiofibroma and reactive bone formation within a spindle cell stroma reminiscent of fibrous dysplasia (Figure 3). Mild pleomorphism and rare mitosis were noted in both cases.


Ultrastructural examination of case 1 at low magnification showed spindle to oval-shaped cells that contained abundant, well-developed rough endoplasmic reticulum and Golgi complex. Rich collagenous matrix existed adjacent to tumor cells. Higher magnification of the cells revealed bundles of microfilaments with condensed aggregates suggestive of myofibroblastic differentiation (Figure 4). No primitive cell junctions, desmosomes, basal laminae, or interdigitating cytoplasmic extensions were found.


Immunohistochemical study showed consistent immunoreactivity to vimentin and S100 protein in both cases. Smooth muscle actin and neuron-specific enolase were expressed only in case 2. Cytokeratin, epithelial membrane antigen, desmin, actin (HHF35), and glial fibrillary acidic protein were negative in both cases. CD34 and cytokeratin 14 were also negative in case 2.


Nasal hamartomas may be predominantly composed of mesenchymal or epithelial tissues. The mesenchymal hamartomas are more common and have been named chondroid, chondromesenchymal, angiomatous, or lipomatous, depending on the preponderant tissue.[3-7] Lesions with a rich vascular component tend to be classified as hemangiomas. The epithelial hamartomas comprise such lesions as respiratory epithelial adenomatoid hamartoma, seromucinous hamartoma, or salivary gland Anlage tumor of the nasopharynx, considered a possible hamartoma of accessory salivary gland origin.[8-10] Rare instances of respiratory epithelial adenomatoid hamartoma with additional chondro-osseous component have been described[11] and may arise in a child.[4] However, hamartoma of pure epithelial composition has not been recorded in children.

Our 2 examples are mesenchymal hamartomas with histologic features consistent with those described by McDermott et al[4] in 1998. Their series consisted of 7 infantile nasal tumors diagnosed before 3 months of age, except one that was diagnosed at the age of 7 years. The characteristic histologic features of mixed stromal and chondroid tissue in various proportions were reminiscent of the mesenchymal hamartoma of the chest wall, which is a lesion almost exclusively of infants. Thus, the term nasal chondromesenchymal hamartoma (NCMH) was originally proposed because of the clinical and pathologic similarities of the chest wall and nasal lesions. Four additional single case reports were found in the English-language literature, of which 3 were reported before the series of McDermott et al. These cases were designated "chondroid hamartoma," "nasal hamartoma," and "mesenchymoma," respectively.[3,12,13] Along with our 2 patients, altogether we identified 13 cases documented in children, as summarized in the Table. Nine were male and 4 were female. The age at diagnosis ranged from 1 day to 7 years, with a mean age of 13.9 months. Nine patients were 3 months or younger, and 5 patients had their lesions diagnosed in the first 2 weeks of life. Most nasal masses were nonencapsulated and ill defined, containing both solid and cystic portions, with calcifications frequently seen on CT or MRI. Before the present report, the gross appearance of NCMH had not been well illustrated as an entire mass (Figure 2), because resection is usually carried out piecemeal. The adjacent paranasal sinuses were frequently involved, especially the ethmoid sinus. Erosion of the surrounding bone and extension to the skull base and orbital region are not uncommon, but aggressive bony destruction was not encountered. Intracranial extension was found in 6 patients, and the usual mode was through the cribriform plate to the anterior cranial fossa. Respiratory distress, feeding difficulties, and otitis media were the usual clinical presentations in infants. Some patients may manifest oculomotor disturbance when the orbit and optic nerve are compressed.


Five of the cases were most likely congenital, but only 2 of them were diagnosed at birth (cases 9 and 12) and 3 in the first 2 weeks of life (cases 1, 5, and 6). Case 5 had prenatal hydrocephalus diagnosed by ultrasonography. The histologic features of case 9, called congenital mesenchymoma, were indistinguishable from those of NCMH. Traditionally, the term mesenchymoma is reserved for a tumor, whether neoplastic or hamartomatous, composed of 2 or more mesenchymal elements other than fibrous tissue. Therefore, some overlapping histologic features may exist between hamartoma and mesenchymoma.

In view of the shared histologic features of NCMH and certain soft tissue tumors, it is reasonable to assume that NCMHs have been reported under different names. One possible example is a case reported as mesenchymal chondrosarcoma with intracranial extension in a male newborn.[14] The tumor was composed of small, round to spindle-shaped mesenchymal cells admixed with irregular islands of benign-appearing cartilage, but the pleomorphism was mild and the mitoses were few. The clinical and microscopic findings would seem more compatible with NCMH than mesenchymal chondrosarcoma. Bone lesions uncommon in neonates have been reported under such designations as fibrous histiocytoma, chondromyxoid fibroma, or ossifying fibroma. These diagnoses should not be uncritically accepted, because they may well represent NCMH.[15-17] In practice, a number of entities may occur in the sinonasal region and show some degree of morphologic overlap with NCMH. These include extracranial fibrous meningioma, fibro-osseous lesions, nasopharyngeal angiofibroma, Schwannoma, neurofibroma, spindle cell myoepithelioma, and solitary fibrous tumor. Our experience with case 1 brings into relief the possibility of misdiagnosis, because extracranial meningioma may display foci of chondroid or osseous metaplasia, and it was only after our experience with case 2 that we were compelled to revise our original diagnosis.

The immunohistochemical profile and ultrastructural findings in our 2 cases are consistent with previous reports. It is believed that fibroblasts and myofibroblasts are the major component cells in NCMH. However, the histogenesis is not completely understood, since the embryonic development of the nose, and especially of the paranasal sinuses, is not yet fully elucidated.[18]

The growth potential of hamartoma is limited and thus the tumor is benign. Recurrence develops after incomplete resection or microscopic deposits of residual tumor. No malignant transformation of NMCH has been found. Local resection through functional endoscopic sinus surgery is recommended when the tumor is localized in nasal cavity. No adjuvant therapy is necessary. A correct diagnosis is imperative to avoid potentially harmful therapies.


[1.] Reilly JR, Koopman CF, Cotton R. Nasal mass in a pediatric patient. Head Neck. 1992;14:415-418.

[2.] Harley EH. Pediatric congenital nasal masses. Ear Nose Throat J. 1991;70: 28-32.

[3.] Kim DW, Low W, Billman G, Wickersham J, Kearns D. Chondroid hamartoma presenting as a neonatal nasal mass. Int J Pediatr Otorhinolaryngol. 1999; 47:253-259.

[4.] McDermott MB, Ponder TB, Dehner LP. Nasal chondromesenchymal hamartoma: an upper respiratory tract analogue of the chest wall mesenchymal hamartoma. Am J Surg Pathol. 1998;22:425-433.

[5.] Kato K, Ijiri R, Tanaka Y, Hara M, Sekido K. Nasal chondromesenchymal hamartoma of infancy: the first Japanese case report. Pathol Int. 1999;49:731-736.

[6.] Mahindra S, Daljit R, Sohail MA, Malik GB. Hamartomas of the nose. J Laryngol Otol. 1978;92:57-60.

[7.] Majumder NK, Venkataramaniah NK, Gupta KR, Gopalakrishnan S. Hamartoma of nasopharynx. J Laryngol Otol. 1977;91:723-727.

[8.] Baillie EE, Batsakis JG. Glandular (seromucinous) hamartoma of the nasopharynx. Oral Surg Oral Med Oral Pathol. 1974;38:760-762.

[9.] Dehner LP, Valbuena L, Perez-Atayde A, Redick RL, Askin FB, Rosai J. Salivary gland anlage tumor. Am J Surg Pathol. 1994;18:25-36.

[10.] Wenig BM, Heffner DK. Respiratory epithelial adenomatoid hamartomas of the sinonasal tract and nasopharynx: a clinicopathologic study of 31 cases. Ann Otol Rhinol Laryngol. 1995;104:639-645.

[11.] Adair CF, Thompson LDR, Wenig BM, Heffner DK. Chondroosseous and respiratory epithelial hamartomas of the sinonasal tract and nasopharynx [abstract]. Mod Pathol. 1996;9:100.

[12.] Terris MH, Billman GF, Pransky SM. Nasal hamartoma: case report and review of the literature. Int J Pediatr Otorhinolaryngol. 1993;28:83-88.

[13.] Ludemann JP, Tewfik TL, Meagher-Villemure K, Bernard C. Congenital mesenchymoma transgressing the cribriform plate. J Otolaryngol. 1997;26:270-272.

[14.] Roland NJ, Khine MM, Clarke R, Van Velzen D. A rare congenital intranasal polyp: mesenchymal chondrosarcoma of the nasal region. J Laryngol Otol. 1992;106:1081-1083.

[15.] Shrier DA, Wang AR, Patel U, Monajati A, Numaguchi Y. Benign fibrous histiocytoma of the nasal cavity in a newborn: MR and CT findings. AJNR Am J Neuroradiol. 1998;19:1166-1168.

[16.] Nazeer T, Ro JY, Varma DG, de la Hermosa JR, Ayala AG. Chondromyxoid fibroma of paranasal sinuses: report of two cases presenting with nasal obstruction. Skeletal Radiol. 1996;25:779-782.

[17.] Engelbrecht V, Preis S, Hassler W, Lenard HG. CT and MRI of congenital sinonasal ossifying fibroma. Neuroradiology. 1999;41:526-529.

[18.] Wang RG, Jiang SC, Gu R. The cartilaginous nasal capsule and embryonic development of human paranasal sinuses. J Otolaryngol. 1994;23:239-243.

Accepted for publication August 2, 2000.

From the Department of Pathology, Chang Gung Children's Hospital, Tao Yuan, Taiwan, Republic of China (Dr C. Hsueh); the Departments of Pathology (Dr S. Hsueh) and Ear, Nose, and Throat (Drs Lee and Su), Chang Gung Memorial Hospital, Tao Yuan, Taiwan, Republic of China; and the Department of Pathology, Children's Memorial Hospital, Chicago, Ill (Dr Gonzalez-Crussi).

Reprints: Chuen Hsueh, MD, Department of Pathology, Chang Gung Children's Hospital, 5 Fu-Shin St, Kuei Shan 333, Tao Yuan, Taiwan, Republic of China (e-mail:
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Article Details
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Author:Hsueh, Chuen; Hsueh, Swei; Gonzalez-Crussi, Frank; Lee, Ta-jen; Su, Jen-liang
Publication:Archives of Pathology & Laboratory Medicine
Geographic Code:9TAIW
Date:Mar 1, 2001
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