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   Table of Contents - Current issue
January-June 2019
Volume 2 | Issue 1
Page Nos. 1-23

Online since Wednesday, October 28, 2020

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Pleomorphic adenoma of hard palate: An usual tumor in an unusual location with brief review of literature p. 1

Pleomorphic adenoma (PA) is the most common benign tumor affecting the major salivary glands, though infrequently it may arise from the minor salivary glands as well. Majority of tumors affecting the minor salivary gland are malignant. We report a rare case of minor salivary gland PA of the hard palate in a 40-year-old female patient who presented with painless swelling in the palatal region for 2 years affecting swallowing and deglutition. A brief review of literature is also presented here.
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Biphenotypic sinonasal sarcoma: An infrequent neoplasm with classical clinical presentation and mimetic histology. Report of two cases p. 5

Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade sarcoma that occurs mainly in the upper sinonasal tract of middle-aged women. It exhibits a dual immunophenotype characterized by the expression of neural and myogenic markers. It is characterized by PAX3 rearrangement with various fusion partners, being MAML3 the most frequent. BSNS is a locally aggressive neoplasm, with frequent local recurrences but no metastasis reported. Here, we present the clinical, histological, immunohistochemical, and molecular findings of two new cases of BSNS that fulfill the canonical demographic, clinical, morphologic, and molecular criteria described for this infrequent entity.
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A case of well-differentiated liposarcoma of the larynx histologically indistinguishable from spindle cell lipoma p. 9

Correctly identifying atypical lipomatous tumors/well-differentiated liposarcomas (WDLs) can be exceedingly difficult, as they account for <0.5% of all laryngeal neoplasms. Diagnosis is further complicated by their morphologic similarity with other more benign lipogenic tumors, such as spindle cell lipoma. We describe a 63-year-old Caucasian man who presented with a left aryepiglottic mass clinically suspicious for a simple cyst. On excision, a frozen section diagnosis of benign fibrolipomatous lesion was reported. Permanent sections revealed an adipocytic neoplasm comprising cellular fibrosis and monomorphic spindle cells, histologically suggestive of a spindle cell/pleomorphic lipoma. Immunostains were positive for CD34 and negative for smooth muscle actin, desmin, and S-100 protein, also consistent with spindle cell/pleomorphic lipoma. However, amplification of MDM2 by fluorescence in situ hybridization confirmed the diagnosis of WDL. The case was reviewed by two soft-tissue pathologists, and they both favored the diagnosis of spindle cell lipoma until reviewing the relevant MDM2 result. The morphology and immunophenotype of WDL have previously been reported as closely related to that of spindle cell lipoma, and this case provides a striking example of how unmistakable these two entities can be on histologic examination alone.
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Compound odontoma associated with a dentigerous cyst p. 12

Dentigerous cysts (DCs) and odontomas are lesions of odontogenic origin relatively common in the oral cavity with a rare recurrence after treatment. The purpose of this study is to present a case of association of odontoma with DC in the mandible of a young patient and to describe all cases published in English literature to date. A 16-year-old male patient presented with a well-defined unilocular radiolucent lesion associated with impacted mandibular right canine. Mesially, there was another lesion, mixed with small radiopaque structures, similar to dental structures surrounded by a radiolucent halo. The diagnosis of DC associated with compound odontoma was made. This case report associated with the literature review of other reports highlights that the association of DC with odontoma is rare and further studies are needed to investigate the probable etiology of this association.
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Malignant extrarenal rhabdoid tumor of parapharyngeal space: A rare presentation p. 15

Malignant extrarenal rhabdoid tumors (ERRTs) are very rare, highly aggressive tumors and are associated with a poor prognosis. They usually occur in young children. Diligent histopathological examination along with the use of judicious immunohistochemistry (IHC) panel is essential to diagnose this rare entity with distinct therapeutic implications. We report a rare case of ERRT with extension up to the skull base involving the right jugular fossa with thrombus in the right internal jugular vein.
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Monophasic (spindle cell) synovial sarcoma of mandible p. 18

Synovial sarcoma (SS) is a malignant soft-tissue tumor of uncertain histogenesis. It is a malignant mesenchymal tumor commonly affecting the extremities. Rarely, it is seen in areas without any relationship to synovial structures. Only 6%–7% of cases have been reported in the head-and-neck region. In head-and-neck region, it usually involves the hypopharynx, parapharyngeal space, and posterior pharyngeal region. A few cases in mandible have been described in literature. Hereby, we report a rare case of monophasic (spindle cell) SS of the mandible in a 17-year-old male with a previous history of tooth extraction along with an unhealed socket.
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Cervical neuroblastoma in a 2-month-old child p. 22

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