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Sinonasal teratocarcinosarcoma in an adolescent male: A case report of an unusual neoplasm

1 Department of Pathology, Government Medical College, Thrissur, Kerala, India
2 Department of ENT, Government Medical College, Thrissur, Kerala, India

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Date of Submission28-Nov-2021
Date of Decision18-Mar-2022
Date of Acceptance19-Mar-2022
Date of Web Publication11-May-2023


Sinonasal teratocarcinosarcoma (SNTCS) is an extremely rare and aggressive malignant tumor arising in the sinonasal tract, having a combined clinicopathological feature of teratoma and carcinosarcoma. It shows a male predominance and affects adults with an age range of 18–79 years and a mean age of 60 years. Here, we report a case of SNTCS in a 14-year-old male patient who presented with swelling over the upper right alveolus and pain in the right jaw for 2 months. The tumor was completely removed by right total maxillectomy with orbital mess reconstruction, and postoperative radiotherapy with chemotherapy was given. The follow-up of the patient for 2 years has shown evidence of recurrence and is now on palliative care.

Keywords: Histopathological heterogeneity, immunohistochemistry, nasal symptoms, sinonasal teratocarcinosarcoma -SNTCS

How to cite this URL:
Soman A, Prasad P H, Ajayan P V. Sinonasal teratocarcinosarcoma in an adolescent male: A case report of an unusual neoplasm. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Jun 1]. Available from:

   Introduction Top

Sinonasal teratocarcinosarcoma (SNTCS) is an extremely rare neoplasm characterized by variegated histopathological features and has a combination of epithelial and mesenchymal components. It was first described by Shanmugaratnam in 1983 as teratoid carcinosarcoma.[1] Heffner and Hyams first coined the term “teratocarcinosarcoma” based on the clinicopathological study on 20 cases of sinonasal tract neoplasms.[2] To date there are <100 cases reported in the English literature. It is almost exclusively seen in adults with only three pediatric cases being reported.[1] Because of the infrequency and the complex phenotypic elements, these lesions are often misdiagnosed leading to management difficulties.

   Case History Top

A 14-year-old male child presented with cheek swelling of 2 months duration associated with difficulty in swallowing. Had a history of change in voice associated with 2–3 episodes of epistaxis.

O/E: Right cheek swelling – diffuse 10 × 8 cm soft and cystic. Oral–ulcero-proliferative growth approximately 5 × 6 cm over hard palate, soft friable approximately 0.5 cm from the incisors involving the right upper retro-molar trigone was seen [Figure 1]a, [Figure 1]b.
Figure 1: a and b: Right maxillary swelling with an ulcero-proliferative growth in the oral cavity. c: Multiple soft tissue bits aggregate measuring 7 × 5 × 3 cm. d: CT PNS showing Large enhancing mass in the right maxillary region. e to h [H&E]: Epithelial Components: e [10 ×] and f [40 ×]: Glandular elements with area of adenocarcinoma. g [40 ×] and 1h [40 ×]: Benign Squamous elements with keratin pearl and clear cell change. i -j [H&E]: Mesenchymal components. i [10 ×]: Area of spindle cell sarcoma. j [10 ×]: Areas of immature cartilage

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MRI/CT finding: Large enhancing mass was seen in the right maxillary region, eroding the lateral, medial, and anterior wall and floor of the right maxillary sinus. The mass was seen eroding the alveolar process laterally and extending into the infratemporal fossa cheek, deep lobe of the parotid, and right parapharyngeal space. Along the inferior and medial aspect, the lesion was seen involving the pterygopalatine fissure, entire soft palate, and reaching up to the hard palate. The lesion extended to involve the right ethmoidal sinus, fronto-ethmoidal recess, and partly the frontal sinus and right nasal cavity [Figure 1]d.

He underwent an incisional biopsy of the mass lesion, which was initially diagnosed as odontogenic keratocyst, and he was referred to our hospital. There was a rapid enlargement of the mass after the initial biopsy. A repeat biopsy of the same was done again after 2 weeks in our ENT department and received as multiple nodular grey white soft tissue bits aggregate measuring 7 × 5 × 3 cm. [Figure 1]c Paraffin-embedded sections were made and stained with hematoxylin and eosin [H & E].

Microscopic examination of the sections showed variegated appearance having an intimate admixture of carcinomatous, sarcomatous, primitive neuroectodermal, and teratoid elements. Carcinomatous elements included foci of moderately differentiated adenocarcinoma [Figure 1]e, [Figure 1]f and squamous cell carcinoma with keratin pearl formation [Figure 1]g, [Figure 1]h. The sarcomatous component was a focus of spindle cell sarcoma along with immature cartilage [Figure 1]i, [Figure 1]j. Islands of primitive neuroectodermal elements with true and pseudo-rosettes were also seen [Figure 2]. These microscopic findings clearly suggested the diagnosis of SNTCS.
Figure 2: [H & E]: Teratoid elements. a [10 ×] and b [40 ×]: Primitive neuroepithelial elements. c [40 ×]: Pseudo rosette formation. d [40 ×]: Areas of glial differentiation

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Immunohistochemistry (IHC) showed the expression of vimentin and desmin in the mesenchymal component and neuron-specific enolase (NSE), synaptophysin, and CD 99 in the primitive neuroepithelial component, and CK in the epithelial component [Figure 3]. Hence, a final diagnosis of sino-nasal teratocarcinosarcoma was made.
Figure 3: [IHC]: Immunohistochemistry. a [10 ×]: CK positive in epithelial elements. b [10X]: Vimentin positive in spindle cell areas. c [10 ×]: Desmin positive focally in spindle cells. d [10 ×], e [10 ×], and f [40 ×]: Synaptophysin, NSE, and CD 99 positive in primitive neuroepithelial elements

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He was then referred to a higher center for further management. He underwent right total maxillectomy with orbital mess reconstruction with free fibular flap reconstruction under general anesthesia (GA). It was reported as sinonasal teratocarcinosarcoma right maxilla, stage pT4aN0M0 with an additional component of immature squamous lobules with clear cytoplasm and in nests with plugged keratin, glial-like areas, areas of hemorrhage, necrosis, and hemosiderophages.

On further follow-up, he presented with respiratory distress, restlessness and on examination, a proliferative lesion was found in the oropharynx extending to the nasopharynx noted. A wide local excision of the lesion with tracheostomy was done. The excised lesion was consistent with recurrence of teratocarcinosarcoma on histopathology examination.

   Discussion Top

SNTCS is an aggressive and highly malignant neoplasm of the sino-nasal region which is a combination of a teratoma and a carcinosarcoma comprising benign to malignant epithelial components, mesenchymal components, and neuroectodermal components. It shows a male predominance and affects adults with an age range of 18 to 79 years and a mean age of 60 years.[2],[3],[4],[5]

The symptoms at presentation include nasal obstruction, epistaxis, pain, and proptosis. Radiologic studies generally show a nasal mass with bone destruction and extension into the ethmoid or maxillary sinus. A review of the literature showed that these symptoms presented within a short mean duration of 102 days, which could be attributed to the aggressive nature of the tumor.[6]

SNTCS is thought to arise from the pluripotent olfactory epithelial cells in the nasal cavity.

Grossly the tumors may be polypoid, friable, and hemorrhagic. Histologically, they are characterized by a heterogeneous combination of epithelial and mesenchymal elements.[2],[4] The epithelial components are composed of a mixture of clear cells, nonkeratinizing epithelium, squamous epithelium without clear cell elements, squamous cell carcinoma, and benign, atypical, or clearly malignant glandular elements. Immature neuroepithelial tissue with rosette formation, ganglion cells, and glial differentiation are also present.[7] The mesenchymal tissues also have a variable appearance with areas of nonspecific myxomatous tissue, cellular areas of benign and malignant appearing fibroblasts, and smooth muscle cells.[2],[3],[4] Rhabdomyoblastic and chondroblastic differentiation with areas of rhabdomyosarcoma and chondrosarcoma or fetal cartilage may be seen.[8]

The differential diagnosis of SNTCS is broad and includes squamous cell carcinoma, sarcamatoid carcinoma, adenocarcinoma, olfactory neuroblastoma (ONB), craniopharyngioma, and other sarcomas. The histological complexity of teratocarcinosarcoma and the presence of mixed epithelial and mesenchymal elements should suggest the diagnosis.

A definite diagnosis may not be possible in small biopsy specimens as it happened in the present case. IHC staining depends on the cell type.[3],[7] The primitive neuroepithelial tissues are positive for CD 99, NSE, synaptophysin, and chromogranin. Epithelial membrane antigen and keratin are seen in the epithelial elements. Mesenchymal components have positive results for vimentin and may be reactive for myogenic markers or smooth muscle actin depending on cell types.

Although there is no defined treatment protocol, recent reports confirm that multimodality treatment for SNTCS with maximal safe surgical resection followed by radiotherapy or chemotherapy is the most effective modality to date.[9] The average survival time is less than 2 years. Approximately 67% of patients with adequate follow-up developed uncontrollable local recurrence and 35% developed metastasis to cervical lymph nodes.

   Conclusion Top

SNTCS is a rare entity, which often possesses a diagnostic challenge, particularly on small biopsies or partially removed tumor specimens. Total excision of the tumor and aggressive sampling for histopathological examination are necessary to avoid erroneous diagnosis. Multimodality treatment in the form of chemotherapy with or without radiotherapy following surgery is mandatory for a good clinical outcome. Very aggressive follow-up is necessary to detect early recurrence. Early diagnosis and management before regional spread can give a better prognosis.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Agrawal N, Chintagumpala M, Hicks J, Eldin K, Paulino AC. Sinonasal teratocarcinosarcoma in an adolescent male. J Pediatr Hematol Oncol 2012;34:e304-7.  Back to cited text no. 1
Heffner DK, Hyams VJ. Teratocarcinosarcoma (malignant teratoma?) of the nasal cavity and paranasal sinuses A clinicopathologic study of 20 cases. Cancer 1984;53:2140-54.  Back to cited text no. 2
Fernández PL, Cardesa A, Alós L, Pinto J, Traserra J. Sinonasal teratocarcinosarcoma: An unusual neoplasm. Pathol Res Pract 1995;191:166-73.  Back to cited text no. 3
Shanmugaratnam K, Kunaratnam N, Chia KB, Chiang GS, Sinniah R. Teratoid carcinosarcoma of the paranasal sinuses. Pathology 1983;15:413-9.  Back to cited text no. 4
Smith SL, Hessel AC, Luna MA, Malpica A, Rosenthal DI, El-Naggar AK. Sinonasal teratocarcinosarcoma of the head and neck: A report of 10 patients treated at a single institution and comparison with reported series. Arch Otolaryngol Head Neck Surg 2008;134:592-5.  Back to cited text no. 5
Leelamma JP, Mohan BP, Srinivasan A. Sinonasal teratocarcinosarcoma- A rare tumour not so rarely misdiagnosed. Iran J Pathol 2018;13:85-8.  Back to cited text no. 6
Pai SA, Naresh KN, Masih K, Ramarao C, Borges AM. Teratocarcinosarcoma of the paranasal sinuses: A clinicopathologic and immunohistochemical study. Hum Pathol 1998;29:718-22.  Back to cited text no. 7
Shimazaki H, Aida S, Tamai S, Miyazawa T, Nakanobou M. Sinonasal teratocarcinosarcoma: Ultrastructural and immunohistochemical evidence of neuroectodermal origin. Ultrastruct Pathol 2000;24:115-22.  Back to cited text no. 8
Misra P, Husain Q, Svider PF, Sanghvi S, Liu JK, Eloy JA. Management of sinonasal teratocarcinosarcoma: A systematic review. Am J Otolaryngol 2014;35:5-11.  Back to cited text no. 9

Correspondence Address:
Aashna Soman,
Senior Resident, Department of Pathology, Government Medical College Thrissur, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_1164_21


  [Figure 1], [Figure 2], [Figure 3]


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