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CORRESPONDENCE
Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 1003-1005

Neoadjuvant chemotherapy in advanced sinonasal teratocarcinosarcoma with intracranial extension: Report of two cases with literature review


1 Department of Medical Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India
2 Department of Radio Diagnosis, Tata Memorial Hospital, Mumbai, Maharashtra, India
3 Department of Head and Neck Surgical Oncology, Parel, Tata Memorial Hospital, Mumbai, Maharashtra, India

Date of Web Publication15-Feb-2016

Correspondence Address:
Kumar Prabhash
Department of Medical Oncology, Tata Memorial Hospital, Parel, Mumbai - 12, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.165878

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 > Abstract 

Sinonasal teratocarcinosarcoma (SNTCS) is a highly aggressive rare tumor of the nasal cavity. Surgery followed by concurrent chemoradiation is the mainstay of treatment in SNTCS. However, intracranial extension may complicate surgical resection, with difficulty in achieving R0 resection. Here we present two cases of SNTCS with intracranial extension; both patients were seen in skull base clinic of our hospital and deemed unsuitable for surgery. These patients then were offered neoadjuvant chemotherapy (NACT), both patients had a partial response with cisplatin and etoposide protocol; subsequently they underwent R0 resection (no macroscopic residual tumor at surgery with all margins were negative for tumor on microscopy). The present cases highlight the fact that NACT with cisplatin and etoposide protocol may be considered in technically unresectable SNTCS.

Keywords: Neoadjuvant chemotherapy, sinonasal tertocarcinosarcoma, unresectable


How to cite this article:
Joshi A, Noronha V, Sharma M, Dhumal S, Juvekar S, Patil VM, Pai P, Prabhash K. Neoadjuvant chemotherapy in advanced sinonasal teratocarcinosarcoma with intracranial extension: Report of two cases with literature review. J Can Res Ther 2015;11:1003-5

How to cite this URL:
Joshi A, Noronha V, Sharma M, Dhumal S, Juvekar S, Patil VM, Pai P, Prabhash K. Neoadjuvant chemotherapy in advanced sinonasal teratocarcinosarcoma with intracranial extension: Report of two cases with literature review. J Can Res Ther [serial online] 2015 [cited 2019 Jan 18];11:1003-5. Available from: http://www.cancerjournal.net/text.asp?2015/11/4/1003/165878


 > Introduction Top


Sinonasal teratocarcinosarcoma (SNTCS) are rare, aggressive neoplasm of the nasal cavity. Many cases are deemed inoperable, in view of intracranial extension. These patients are mainly treated with either radical chemoradiation or palliative radiation. Results of such nonsurgical treatments are unsatisfactory. 70% [1],[2] Misra et al., in a systematic review of this rare condition highlighted that very few patients (seven patients) were treated by radiation alone or with chemoradiation. In the limited follow-up reported, only 50% of patients had survived as opposed to 70% survivals seen in series involving surgery. [2] Neoadjuvant chemotherapy (NACT) may be used in these cases initially and later reassessment for possible surgery. We present two cases where NACT has led to R0 resection (no macroscopic residual tumor at surgery with all margins were negative for tumor on microscopy) of cases initially labeled inoperable.


 > Case Reports Top


Case I

A 38-year-old, male, smoker, presented with complaints of nasal blockage, initially right-sided, which became bilateral (B/L), associated with epistaxis, headache, and vomiting. He had diplopia on looking right. On examination, there was a reddish fleshy mass in left side nasal cavity. Computed tomography (CT) showed an enhancing polypoidal soft tissue mass measuring 6.8 × 4.8 × 6.7 cm involving B/L ethmoid, sphenoid, and frontal sinuses as well as B/L nasal cavities, with erosion of cribriform plate and intracranial extension. There was no intraorbital extension. Magnetic resonance imaging ( MRI) brain with contrast done confirmed the CT findings. It revealed enhancing lesion within B/L ethmoid sinuses and nasal cavities extending into basifrontal extra-axial region through cribriform plates with intracranial extension. The biopsy from the mass was consistent with SNTCS.

The patient was seen in our hospital's skull base clinic. He was planned for, and received NACT with cisplatin (100 mg/m 2 on day 1) and etoposide (100 mg/m 2 day 1-3), repeated every 21 days, in view of intracranial extension. After three cycles of NACT, his nasal blockage was completely relieved, diplopia resolved, and there were no further episodes of epistaxis. Response evaluation magnetic resonance imaging after NACT showed residual enhancing lesion measuring 3.6 × 1.6 × 4.8 cm involving B/L nasoethmoid regions, extending up to the right medial wall of orbit; however, there was no intraorbital extension, suggestive of partial response as per Response Evaluation Criteria In Solid Tumors (RECIST) criteria. He underwent surgical exploration with combined endoscopic and open craniofacial resection (CFR) approach. Histopathology report showed tumor infiltrating the underlying bone with intracranial extension. All margins were free. Immunohistochemistry showed major portion of residual viable SNTCS showing post chemotherapy neuroglial maturation highlighted by glial fibrillary acidic protein (GFAP), synaptophysin, and S100. In addition, fetal squamous elements highlighted by p63 along with benign glands were seen; however, obvious malignant elements, that is, carcinoma, sarcoma, and primitive neuroectodermal tissue were absent. Postoperatively, the patient received concurrent chemoradiotherapy in view of residual viable tumor. He received external beam radiotherapy to tumor bed to a dose of 60 Gy/30 # with 6 MV photons with intensity-modulated radiotherapy (IMRT) technique, concurrent with weekly cisplatin 30mg/m2 six cycles. After 2 months of completion of chemotherapy and radiotherapy (CTRT), patient remains asymptomatic.

Case II

A 48-year-old male, presented to us with complaints of epistaxis since 1 month. His biopsy was consistent with SNTCS. His positron emission tomography (PET) showed metabolically active, enhancing, ill-defined, soft tissue mass lesion involving the posterior part of B/L nasal cavity with intracranial extension and erosion of cribriform plate of ethmoid representing the site of primary, no distant metastasis. His MRI [Figure 1] showed an ill-defined heterogeneous lesion with epicenter in the left anterior and middle ethmoid sinuses extending to right side. It measures 3.0 × 2.2 × 3.0 cm in AP, TS, and CC dimensions. Superiorly the lesion involved the cribriform plate of ethmoid with intracranial extension along the basifrontal region abutting adjacent brain parenchyma; however, there was no altered signal intensity in the brain. Inferiorly it extended up to the superior turbinate on left side. There was no extension into the nasal cavity or nasopharynx. Laterally the lesion abuts the lamina papyracea; however, there was no intraorbital extension. Mucosal thickening was noted in the left maxillary and posterior ethmoid sinuses. The patient was seen in Skull Base Clinic, and in view of cribriform plate involvement he had was planned for NACT with cisplatin 100 mg/m 2 on day 1 and etoposide 100 mg/m 2 day 1-3, repeated every 21 days. He received two cycles of the same; post two cycles, his MRI [Figure 2] showed a lesion in the ethmoid sinuses measuring 1.8 × 1.9 × 2.3 cm. He had partial response as per RECIST criteria. Subsequently the patient underwent endonasal endoscopic resection of residual tumor. R0 resection was achieved. Post-surgery histopathology report showed residual viable SNTCS. The patient was further planned for weekly cisplatin-based CTRT. Patient received external beam radiation to face region using 6 MV photons using image-guided radiotherapy (IGRT) to a total dose of 60 Gy/30 # F along with concurrent chemotherapy as cisplatin 30mg/m2 for first 3 weeks. As he developed persistent neutropenia after 3 weeks, his chemotherapy was withheld. Post 3 months of completion of CTRT, patient remains asymptomatic.
Figure 1: Coronal, sagittal, and axial post-contrast MRI reveals a lobulated, enhancing lesion in the nasoethmoid region on either side of midline with erosion of cribriform plate and intracranial extension. MRI = Magnetic resonance imaging

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Figure 2: Post neoadjuvant chemotherapy MRI in coronal and sagittal plane reveals significant regression of mass in the nasoethmoid region and the intracranial portion of the lesion

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 > Discussion Top


SNTCS is a rare, aggressive tumor of nasal cavity, paranasal sinuses. It is defined as complex arrangement of malignant sinonasal tumors, having characteristics of teratoma and carcinosarcoma. [1],[3] This tumor shows a blend of epithelial elements, glandular elements, and neural elements; diagnosis is difficult in small biopsies. In this site, tumor needs to be differentiated from esthesioneuroblastoma, sinonasal tumors with poor differentiation, sinonasal tumors with neuroendocrine differentiation, craniopharyngiomas, and germ cell tumors. [4] A histomorphology consisting of all three distinct elements is very specific for the diagnosis of teratocarcinosarcoma. [4],[5],[6]

A literature review was done with PubMed search engine. The following words were used for searching "Malignant Teratocarcinosarcoma"[Supplementary Concept] OR "Malignant Teratocarcinosarcoma" [All Fields] OR "SNTCS" [All Fields]. Forty eight articles were obtained. Out of these 48 articles, 10 articles were review articles, 31 were case reports, and seven were case series. The most common form of treatment utilized was surgical resection followed by RT. In a case series by Smith, patients treated with surgery with or without RT, showed a recurrence rate of 37% and a mortality rate of 30%. [7] Similar aggressive nature of this disease was shown in our previous experience where disease recurred in 11 out of 14 patients, with a median time to recurrence of 7 months. [8] However, intracranial extension makes surgical resection difficult. [7]

Unfortunately there is no series dealing with use of induction chemotherapy or use of radical radiation ± chemotherapy or palliative RT reported. In a systematic review by Misra et al., intracranial extension, cribriform plate, and anterior cranial fossa involvement occurred in 20.9% patients. Combined treatment with radiation, surgery, and chemotherapy provided patients with the highest survival (88.8%), with no metastasis and the lowest recurrence rate (22.2%). With use of surgery and RT (no chemotherapy); 26.1% of cases recurred, 10.9% metastasized, and 8.7% reported recurrence and metastasis. In patients who were treated with either only CTRT or radiation (four cases), 50% of them recurred. [2]

So it seems that in sinonasal teratocarcinomas combined modality treatment inclusive of surgery, radiation, and chemotherapy has best results. Hence, in patients who are potentially unresectable, it may be useful to make them resectable with the use of chemotherapy. We have used this approach in head and neck cancer earlier. [9] Our case report suggests that teratocarcinosarcoma is a chemosensitive tumor, and cisplatin and etoposide are effective drugs to help downstage the tumor. It will be useful to integrate this modality in this aggressive malignancy to improve outcome in patients.

 
 > References Top

1.
Waal I, Lamovec J, Knuutila S, Kleihues P, Sobin LH. World Health Organization classification of tumours. Pathology and genetic of head and neck tumours. entjournal.com; 2005; Available from: http://www.entjournal.com/category/keyword-topics/tumor?page=9 [Last accessed on 2014 Dec 12].  Back to cited text no. 1
    
2.
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. 2
    
3.
Vranic S, Caughron SK, Djuricic S, Bilalovic N, Zaman S, Suljevic I, et al. Hamartomas, teratomas and teratocarcinosarcomas of the head and neck: Report of 3 new cases with clinico-pathologic correlation, cytogenetic analysis, and review of the literature. BMC Ear Nose Throat Disord 2008;8:8.  Back to cited text no. 3
    
4.
Fernández PL, Cardesa A, Alós L, Pinto J, Traserra J. Sinonasal teratocarcinosarcoma: An unusual neoplasm. Pathol Res Pract 1995;191:166-71.  Back to cited text no. 4
    
5.
Kane SV, Karpate AA, Bal M, Juvekar SL, Pai PS. Chemotherapy-induced neuronal maturation in sinonasal teratocarcinosarcoma--a unique observation. Head Neck Pathol 2009;3:31-6.  Back to cited text no. 5
    
6.
Wei S, Carroll W, Lazenby A, Bell W, Lopez R, Said-Al-Naief N. Sinonasal teratocarcinosarcoma: Report of a case with review of literature and treatment outcome. Ann Diagn Pathol 2008;12:415-25.  Back to cited text no. 6
    
7.
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. 7
    
8.
Budrukkar A, Agarwal JP, Kane S, Siddha M, Laskar SG, Pai P, et al. Management and clinical outcome of sinonasal teratocarcinosarcoma: Single institution experience. The Journal of [Internet]. Cambridge Univ Press; 2010; Available from: http://journals.cambridge.org/abstract_S0022215109992866 [Last  accessed on 2014 Dec 12].  Back to cited text no. 8
    
9.
Patil VM, Prabhash K, Noronha V, Joshi A, Muddu V, Dhumal S, et al. Neoadjuvant chemotherapy followed by surgery in very locally advanced technically unresectable oral cavity cancers. Oral Oncol 2014;50:1000-4.  Back to cited text no. 9
    


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