Apollo Cancer Centre, Plot no, 251, Sainik School Rd, Bhubaneswar, Odisha

A Case Report Of Sinonasal Teratocarcinosarcoma In A 30 Year Old Male And Mini Review Of Literature

30year old male
– Presented with complaints of
– intermittent left nasal bleeding for 3months duration
– left nasal cavity block for 1 month duration
-On examination-
– ECOG-1
– Red coloured fleshy mass was seen in the left nasal cavity.

MRI Brain and PNS

Ill defined lobular hyperintense and avidly enhancing lesion measuring 75x17x62mm predominantly in the nasal cavity, left ethmoid and sphenoid sinuses. Superiorly it was eroding the cribriform plate with intracranial extension into left frontal lobe measuring 3x2x1.6cm. Laterally it was extending upto left orbital plate and posteriorly upto cavernous sinus without obvious infiltration.

Biopsy of the lesion – teratocarcinosarcoma.
IHC:
Synaptophysin, NSE, CD99 positive in primitive component, SALL 4 and GLYPICAN 3 positive in tubules, pan CK positive in glands, OCT ¾,WT1 and Desmin were negative.

– In view of huge disease with intracranial extension the patient was initially treated with neo adjuvant chemotherapy.
– Post chemotherapy imaging showed minimal reduction in the size of the lesion
The patient then underwent surgery with gross total excision of the lesion, left anterior skull base resection and reconstruction.

Post Op HPE :
– Biphasic tumour tissue composed of round to oval cells arranged in sheets and clusters having hyperchromatic nuclei and scanty cytoplasm. Other elements showed columnar to cuboidal cells arranged in glandular, acinar and pseudorosettes. Stroma showed desmoplasia and hemorrhage.
– Features were suggestive of teratocarcinosarcoma and there was no LVSI, PNI or bone invasion.
– The patient subsequently received Adjuvant radiotherapy (RT) to the tumour bed with IGRT technique to a dose of 60Gy in 30fractions.

Sinonasal Teratocarcinosarcoma
– Rare and highly aggressive tumour
– It was initially described by various terms like teratoid carcino sarcoma by Shanmugaratnam et al in the year 1983.
– However the term teratocarcinosarcoma was first described by Heffner and Hyams in 1984
– Nearly 100 cases have been reported in english literature so far
– It most commonly affects male gender with a male to female ratio of 8:1.
– The average age of presentation of this tumours is 52.8 yrs and most commonly it occurs in adults over the age of 35years.
– Less than 15 cases have been reported in English literature in the patients below 35years age.
-Usual presenting symptoms include nasal obstruction and epistaxis.
Pathology – The origin of SNTCS is thought to be from the pluripotent olfactory epithelial cells in the nasal cavity. Microscopically it usually shows a triphasic pattern of epithelial, mesenchymal and neuroectodermal components at various degrees of maturity
IHC – The diagnosis of SNTCS is usually confirmed only by IHC – Cytokeratin and EMA are positive in epithelial components. Vimentin and/or actin are positive in mesenchymal components Neuroepithelial components show positivity for neuron specific enolase, CD99, chromogranin, synaptophysin, glial fibrillary acidic protein and
S-100 protein.

Differential diagnosis:
– olfactory neuroblastoma
– synovial sarcoma
– rhabdomyosarcoma
– neuroendocrine carcinoma
– adenocarcinoma
– sinonasal undifferentiated carcinoma
– malignant mixed salivary gland tumours etc.

Treatment:
– The treatment of SNTCS includes radical surgery followed by radiotherapy.
– The role of chemotherapy is unclear.
– Most common surgical techniques include craniofacial resection, maxillectomy and rhinotomy.
– Due to its aggressive presentation many of the cases do require adjuvant treatment in the form of radiotherapy or concurrent chemoradiation.

Survival:
– The two year overall survival ranged from 40% to 46% in various case series
The average survival was 1.7years.
Local recurrence is the most common form of treatment failure.
– Distant metastasis is rare and usually occurs in lungs

References:

1. Shanmugaratnam K, Kunaratnam N, Chia KB, Chiang GSC, Sinniali R. Teratoid carcinosarcoma of the paranasal sinuses. Pathology 1983;15:413–19
2. Heffner DK, Hyams VJ. Teratocarcinosarcoma (malignant teratoma?) of the nasal cavity and paranasal sinuses. Cancer 1984;47:2140–54
3. 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.
4. Carrizo F, Pineda-Daboin K, Neto AG, Luna MA. Pharyngeal teratocarcinosarcoma: review of the literature and report of two cases. Ann Diagn Pathol 2006;10:339-42.
5. Salem F, Rosenblum MK, Jhanwar SC, et al. Teratocarcinosarcoma of the nasal cavity and paranasal sinuses: report of 3 cases with assessment for chromosome 12p status. Hum Pathol 2008;39:605–9.
6. 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.
7. Fatima SS, Minhas K, Din NU, Fatima S, Ahmed A, Ahmad Z. Sinonasal teratocarcinosarcoma: a clinicopathologic and immunohistochemical study of 6 cases. Ann Diagn Pathol 2013;17:313-8.
8. Agrawal N, Chintagumpala M, Hicks J, Eldin K, Paulino AC. Sinonasal teratocarcinosarcoma in an adolescent male. J Pediatr Hematol Oncol. 2012;34(7):e304–7.
9. Batsakis JG, El-Naggar AK, Luna MA. Teratomas of the head and neck with emphasis on malignancy. Ann OtolRhinolLaryngol 1995; 104(6): 496-500.
10.Yang S, Sun R, Liang J, Zhou Z, Zhou J, Rui J. Sinonasal teratocarcinosarcoma: A clinical and pathological analysis. Int J Surg Pathol 2013 ;21(1):37-43.
11.Thomas J, Adegboyega P, Iloabachie K, et al. Sinonasal teratocarcinosarcoma with yolk sac elements: a neoplasm of somatic or germ cell origin? Ann Diagn Pathol 2011;15:135–9.
12.Shaohua Chen, Ting Zhou, Ruxue Yang, Nan Li, Zhaogen Cai, Gengsheng Su: Sinonasalteratocarcinosarcoma: a report of two cases and clinicopathological analysis. Int J Clin Exp Pathol 2018;11(3):1581-1586
13.Terasaka S, Medary MB, Whiting DM, et al. Prolonged survival in a patientwith sinonasal teratocarcinosarcoma with cranial extension. Case report. J Neurosurg 1998;88:753–6.
14.Joshi A, Noronha V, Sharma M, et al. Neoadjuvant chemotherapy in advanced sinonasal teratocarcinosarcoma with intracranial extension: report of two cases with literature review. J Cancer Res Ther 2015;11:1003-5.
15.Tokunaga T, Sunaga H, Kimura Y, Tsuda G, Fujieda S. A case of sinonasal teratocarcinosarcoma treated with surgery and post-operative intensity-modulated radiotherapy (IMRT). Auris Nasus Larynx 2012;39:641-5.
16.Peng G, Ke Y, Wang T, Feng Y, Li Y, Wu G. Intensity-modulated radiotherapy for sinonasal teratocarcinosarcoma. J Huazhong Univ Sci Technolog Med Sci 2011;31:857-60.
17.Foong YC, Murdolo V, Naiman N, Hepner L, Awad R. Sinonasal teratocarcinosarcoma: a case report. J Med Case Rep 2017;11:167