|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 3 | Page : 398-400
Recurrent sinonasal teratocarcinosarcoma with intracranial extension: Case report
A Joshi1, SB Dhumal1, DR Manickam1, V Noronha1, M Bal2, VM Patil1, K Prabhash1
1 Department of Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
2 Department of Pathology, Tata Memorial Hospital, Mumbai, Maharashtra, India
|Date of Web Publication||10-Dec-2014|
Dr. K Prabhash
Department of Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Joshi A, Dhumal S B, Manickam D R, Noronha V, Bal M, Patil V M, Prabhash K. Recurrent sinonasal teratocarcinosarcoma with intracranial extension: Case report. Indian J Cancer 2014;51:398-400
|How to cite this URL:|
Joshi A, Dhumal S B, Manickam D R, Noronha V, Bal M, Patil V M, Prabhash K. Recurrent sinonasal teratocarcinosarcoma with intracranial extension: Case report. Indian J Cancer [serial online] 2014 [cited 2019 Sep 19];51:398-400. Available from: http://www.indianjcancer.com/text.asp?2014/51/3/398/146785
Sinonasal teratocarcinosarcoma (SNTCS) is a rare and aggressive malignant tumor. As the name implies, it arises from the sinonasal tract and is characterized by the presence of benign and malignant epithelial, mesenchymal and neural components. Common sites of involvement of SNTCS are nasal cavities and paranasal sinuses. The tumor is often misdiagnosed due to rarity, the complex phenotypic composition and inadequate biopsy specimen.
First described by Shanmugaratnam et al.  in 1983 as teratoid carcinosarcoma the term "Teratocarcinosarcoma" was given by Heffner and Hyams in 1984.  The cornerstones for the management of SNTCS include local treatment with surgical resection or radiation therapy with 45-70 Gy with or without concurrent chemotherapy or a combination of both.  Chemotherapy is rarely effective in this condition, but adjuvant multi-drug chemotherapy with cisplatin, etoposide and ifosfamide had shown good results.  Despite aggressive efforts, the prognosis is still poor. The local recurrence after excision is high with reported 3 and 5 year survival rates at about 30% and 20%, respectively. 
In an analysis by Sobani et al., recurrence was found in 42.68% of the cases with a mean recurrence time of 21.3 months in patients followed-up for a mean period of 76.1 months. 
A 44-year-old male patient presented with the complaints of epistaxis, nasal blockage for month duration. He underwent left craniotomy excision of the mass at local place 10-month back. The histopathology report was suggestive of a SNTCS. He also received adjuvant radiotherapy 60 Gy/5 weeks at local place. After a disease free interval of 5 months, he presented to our hospital with recurrence of nasal blockage and epistaxis and mass protruding from the left nostril for a month. On examination, there was a soft-tissue mass protruding out of the left nasal cavity [Figure 1]. Histopathology report was confirmed at our hospital and was compatible with SNTCS [Figure 2]. Whole body positron emission tomography-computed tomography scan was done, which showed an enhancing soft-tissue mass involving the nasal cavity, the ethmoid sinus, destroying the cribriform plate of ethmoid, posteriorly extending into the nasopharynx. The mass superiorly involved the frontal sinus, destroying the posterior wall, to enter into the frontal lobe. In view of extensive disease, which was declared unresectable by our surgical team, the patient was started on 3 weekly chemotherapy with ifosfamide and doxorubicin with a palliative intent. After two cycles of chemotherapy, there was a significant symptomatic benefit. The mass had shrunk; epistaxis and nasal blockage had resolved [Figure 3]. However, his disease was still unresectable. This highlights that this tumor may be chemoresponsive and chemotherapy can be integrated in the management of these patients in either neoadjuvant, adjuvant or palliative setting. A multi-modality treatment approach is required in these patients with aggressive therapy and close follow-up.
|Figure 2(a): Low magnification reveals nests of primitive neuro-ectodermal tumor cells (H and E, original magnification ×200). (b) Tumor cells showing extensive skeletal muscle differentiation evident as eosinophilic strap cells (H and E, original magnification ×200). (c) The primitive neuro - ectodermal component shows immunoreactivity for synaptophysin (indirect peroxidase, original magnification ×400). (d) The skeletal muscle differentiation is highlighted by immunoreactivity to desmin (indirect peroxidase, original magnification ×200)|
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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.
Fukuoka K, Hirokawa M, Shimizu M, Shirabe T, Manabe T, Hirai M, et al
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Sobani ZA, Akhtar S, Junaid M, Salahuddin I. Sinonasal teratocarcinosarcoma. J Pak Med Assoc 2012;62:633-5.
[Figure 1], [Figure 2], [Figure 3]