scholarly journals Sinonasal teratocarcinosarcoma: a case report

Author(s):  
Thottukadavil S. Priya ◽  
Chakramakal Joseph Andrews ◽  
S. Suresh Kumar ◽  
S. Divya

<p class="abstract">Sinonasal teratocarcinosarcoma (TCS) is a very rare malignant neoplasm of sinonasal tract with intermixed teratomatous, carcinomatous and sarcomatous elements. While the diagnosis is largely based on tissue analysis and immunohistochemistry, the mode of management demands further study. Surgical resection with or without chemotherapy and radiation therapy is currently the most accepted treatment regimen. Locally aggressive, while also associated with metastatic lesions, SNTCS is not easily resectable owing to its location and possible intracranial extension. Due to its aggressive nature over one-third of TCS tend to recur leading to treatment failure with a mean survival time of 1.9 years. Possible differentials include squamous cell carcinoma, olfactory neuroblastoma, adenocarcinoma, malignant mixed tumor of salivary gland type, undifferentiated carcinoma, malignant craniopharyngioma, mucoepidermoid carcinoma, transitional carcinoma of Schneiderian type and adenosquamous carcinoma. In this report, we present a case of TCS in a 55 years old male patient who presented to us with complaints of hyposmia, blurring of vision, diplopia and epiphora.</p>

Neurographics ◽  
2019 ◽  
Vol 9 (6) ◽  
pp. 405-408
Author(s):  
A. Rizvi ◽  
H. Mehta ◽  
S. Bobra ◽  
G. M. Kleinman ◽  
D. N. Jourdy ◽  
...  

Sinonasal teratocarcinosarcoma is a rare, malignant neoplasm of the head and neck, and a locally aggressive tumor with a poor prognosis. Few case reports have been published. We present a case of recurrent sinonasal teratocarcinosarcoma in a 39-year-old man with extension into the intracranial compartment. Here we discuss the histopathologic characteristics and nonspecific imaging features, with key differential considerations, including squamous cell carcinoma, adenocarcinoma, sinonasal undifferentiated carcinoma, esthesioneuroblastoma, and lymphoma. Treatment is ultimately composed of surgical resection and chemoradiation therapy.


1998 ◽  
Vol 88 (4) ◽  
pp. 753-756 ◽  
Author(s):  
Shunsuke Terasaka ◽  
Max B. Medary ◽  
Donald M. Whiting ◽  
Takanori Fukushima ◽  
Evalynne J. Espejo ◽  
...  

✓ Sinonasal teratocarcinosarcoma is a rare malignant neoplasm characterized by the combined histological features of carcinosarcoma and teratoma. The primary symptoms of this tumor are usually nasal obstruction and epistaxis, and a nasal cavity mass is the most common clinical finding. The authors describe an exceptionally rare case in which the patient presented with massive intracranial extension and exhibited confusion as an initial symptom. He subsequently underwent combined radical surgery and radiation therapy and has remained free of disease for 31 months. The surgical approach to the lesion, histological features, and clinical course are detailed.


1998 ◽  
Vol 112 (7) ◽  
pp. 682-686 ◽  
Author(s):  
Hari Shankar Sharma ◽  
Jafri Malin Abdullah ◽  
Nor Hayati Othman ◽  
Mahayidin Muhamad

AbstractSinonasal teratocarcinosarcoma is very unusual malignant neoplasm histologically consisting of an epithelial element and one or more mesenchymal components. This is a report of teratocarcinosarcoma, in a 74-year-old male, involving the right nasal cavity and ethmoids with intracranial extension. The tumour was totally resected via the craniofacial approach and the patient was given post-operative chemotherapy. Extensive tumour necrosis, rapid growth and local destruction are the prominent features of this tumour. The clinical presentation, pathological features and clinical course of this rare malignancy are discussed with a review of the literature.


2019 ◽  
Vol 12 ◽  
pp. 117955141982583 ◽  
Author(s):  
Cristina Familiar ◽  
Ane Azcutia

Olfactory neuroblastoma (ONB) is an unusual malignant neoplasm originating from the olfactory neuroepithelium. Secretion of adrenocorticotropic hormone (ACTH) from this tumor has been exceptionally reported. We describe a young man with resistant hypertension and a cushingoid phenotype. After hormonal confirmation of an ACTH-dependent Cushing syndrome, non-invasive dynamic tests were carried out to evaluate the cause of the ACTH source. Plasma cortisol decrease after a high-dose dexamethasone suppression test and cortisol increase after a desmopressin (DDAVP) stimulation test suggested a Cushing disease. A magnetic resonance image (MRI) of the brain and an Indium-111 octreotide scan revealed a large mass centered in the sphenoid sinus with lateral and posterior extension. An ACTH secreting ONB was confirmed with a trasnasal biopsy. Patient was offered a combined therapy with surgical resection and radiotherapy but refused surgery. The neoplasm was treated with neoadjuvant cisplatin-based chemotherapy followed by fractionated radiotherapy. Hypercortisolism initially improved with metyrapone but normocortisolism was only achieved after local control of the tumor with radiotherapy. Clinical presentation of ONB is usually related to local symptoms (as nasal obstruction and epistaxis) dependent on its ubication and extension. Cushing syndrome from ACTH production is a rare manifestation of ONB. This case also underlies the difficulties related to the interpretation of dynamic endocrine tests in Cushing syndrome.


1989 ◽  
Vol 29 (10) ◽  
pp. 902-907 ◽  
Author(s):  
Masaharu ICHIKAWA ◽  
Takuya NAKAZAWA ◽  
Hirofumi NIOKA ◽  
Masayuki MATSUDA ◽  
Jyoji HANDA

2018 ◽  
Vol 15 (6) ◽  
pp. 100-109 ◽  
Author(s):  
Julia R Schneider ◽  
Deborah R Shatzkes ◽  
Stephen C Scharf ◽  
Tristan M Tham ◽  
Kay O Kulason ◽  
...  

Abstract BACKGROUND AND IMPORTANCE Olfactory neuroblastoma, also known as esthesioneuroblastoma (ENB), is a malignant neoplasm with an unpredictable behavior. Currently, the widely accepted treatment is inductive chemotherapy, with or without surgery, followed by radiotherapy. Since data on genetics and molecular alterations of ENB are lacking, there is no standard molecularly targeted therapy. However, ENB commonly expresses the somatostatin receptor (SSTR) that is also expressed by neuroendocrine tumors. Peptide receptor radionuclide therapy (PRRT) using radiolabeled somatostatin analogues, such as 177Lu-octreotate, is an effective treatment for the latter. We present the complex neuroradiological and neuropathological changes associated with 177Lu-octreotate treatment of a patient with a highly treatment-resistant ENB. CLINICAL PRESENTATION A 60-yr-old male presented with an ENB that recurred after chemotherapy, surgery, stereotactic radiosurgery, and immunotherapy. Pathology revealed a Hyams grade 3 ENB and the tumor had metastasized to lymph nodes. Tumor SSTR expression was seen on 68Ga-octreotate positron emission tomography (PET)/computed tomography (CT), suggesting that PRRT may be an option. He received 4 cycles of 177Lu-octreotate over 6 mo, with a partial response of all lesions and symptomatic improvement. Four months after the last PRRT cycle, 2 of the lesions rapidly relapsed and were successfully resected. Three months later, 68Ga-octreotate PET/CT and magnetic resonance imaging indicate no progression of the disease. CONCLUSION We describe imaging changes associated with 177Lu-octreotate PRRT of relapsing ENB. To our knowledge, this is the first report describing neuropathological changes associated with this treatment. PRRT is a promising therapeutic option to improve the disease control, and potentially, the survival of patients with refractory ENB.


2019 ◽  
Vol 131 (1) ◽  
pp. 238-244 ◽  
Author(s):  
Gary L. Gallia ◽  
Anthony O. Asemota ◽  
Ari M. Blitz ◽  
Andrew P. Lane ◽  
Wayne Koch ◽  
...  

OBJECTIVEOlfactory neuroblastoma (ONB) is a rare malignant neoplasm of the sinonasal cavity. Surgery has been and remains a mainstay of treatment for patients with this tumor. Open craniofacial resections have been the treatment of choice for many decades. More recently, experience has been growing with endoscopic approaches in the management of patients with ONB. The object of this study is to report the authors’ experience over the past 11 years with ONB patients treated with purely endonasal endoscopic techniques.METHODSThe authors performed a retrospective chart review of 20 consecutive patients with ONB who underwent a completely endonasal endoscopic approach for an oncological tumor resection at their institution between January 2006 and January 2017. Patient demographics, tumor stage, pathological grade, frozen section analysis, permanent margin assessment, perioperative complications, postoperative therapy, length of follow-up, and outcomes at last follow-up were collected and analyzed.RESULTSEighteen patients presented with newly diagnosed disease, with a modified Kadish stage of A in 2 cases, B in 3, C in 11, and D in 2. Two patients presented with recurrent tumors. An average of 25.3 specimens per patient were examined by frozen section analysis. Although analysis of intraoperative frozen section margins was negative in all but 1 case, microscopic foci of tumor were found in 7 cases (35%) on permanent histopathological analysis. Perioperative complications occurred in 7 patients (35%) including 1 patient who developed a cerebrospinal fluid leak; there were no episodes of meningitis. All but 1 patient received postoperative radiotherapy, and 5 patients received postoperative chemotherapy. With a mean follow-up of over 5 years, 19 patients were alive and 1 patient died from an unrelated cause. There were 2 cases of tumor recurrence. The 5-year overall, disease-specific, and recurrence-free survival rates were 92.9%, 100%, and 92.9%, respectively.CONCLUSIONSThe current results provide additional evidence for the continued use of endoscopic procedures in the management of this malignancy.


1996 ◽  
Vol 89 (9) ◽  
pp. 1089-1095
Author(s):  
Akiko OKUYAMA ◽  
Yusuke WATANABE ◽  
Ritsu SEO ◽  
Osamu SEMBA

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