Sarcoma

A rare case of malignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma NOS) of the vocal fold

July 20, 2015     Jin Pyeong Kim, MD; Jin Yong Kim, MD; Gyung Hyuck Ko, MD; Seung Hoon Woo, MD
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Abstract

Malignant fibrous histiocytoma, also known as pleomorphic undifferentiated sarcoma not otherwise specified, arises in numerous organs. The first-line treatment is complete excision of the mass, but in some cases postoperative chemo- and/or radiotherapy is recommended. Only a few cases of malignant fibrous histiocytoma of the vocal fold have been previously reported in the literature. We report a new case, which originated in the true vocal fold of a 65-year-old man. The mass was removed via CO2 laser excision with preservation of the vocal fold and without the need for further treatment. At 3 years of follow-up, the patient remained recurrence-free.

Biphasic synovial sarcoma of the hypopharynx

April 27, 2015     Ustun Osma, MD; Hülya Eyigor, MD; Dinc Suren, MD; Cem Sezer, MD; Mustafa Deniz Yilmaz, MD
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Abstract

Synovial sarcoma is a malignant mesenchymal neoplasm usually involving the extremities of young adults. Localization in the head and neck region is rare. Histologically, these lesions have biphasic and monophasic variants, the latter being more rare and difficult to identify. Immunohistochemistry plays a crucial role in the diagnosis. We report a case of a biphasic synovial sarcoma arising in the hypopharynx with a review of the literature concerning this rare neoplasm.

Temporal bone chondrosarcoma: Presentation of 4 cases and review of the literature

December 19, 2014     Duoduo Tao, MD; Matthew R. Hoffman, MD; Bing Chen, MD, PhD
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We describe 4 cases of chondrosarcoma of the temporal bone, which occurred in a 66-year-old man and in 3 women aged 34, 37, and 47 years. One of these patients was originally diagnosed with an epithelial cyst and another with a middle ear neoplasm. Three patients underwent surgical removal of their tumor, while the other patient declined (a craniotomy for biopsy was performed in this case). An accurate diagnosis in all 4 cases was not made until histopathologic examination was performed. None of the patients received postoperative radiotherapy for various reasons. However, the male patient experienced a tumor recurrence, which necessitated a second surgery, and he did receive radiotherapy after the second operation. All 3 operated patients were doing well at the most recent follow-up, and the other patient was soon lost to follow-up. Because of its rarity and potential for severe complications, temporal bone chondrosarcoma remains a difficult disease to diagnose and manage.

Mesenchymal chondrosarcoma of the mandible: A rare malignant tumor

September 17, 2014     Rakesh Kumar Singh, MS; Saurabh Varshney, MS; Sampan Singh Bist, MS; Meena Harsh, MD; Nitin Gupta, MD
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Abstract

Mesenchymal chondrosarcoma (MCHS) is a highly aggressive, malignant, cartilaginous tumor that represents about 1% of all chondrosarcomas. Only about 50 cases of MCHS of the jaw have been reported in the English-language literature; maxillary tumors are much more common than mandibular tumors. We present a new case of MCHS of the mandible that arose in a 22-year-old woman whose initial two biopsies did not reveal the features of the tumor. The diagnosis was made only after an incisional biopsy.

Pediatric rhabdomyosarcoma

July 13, 2014     Rosemary Ojo, MD; Si Chen, MD; Liset Pelaez, MD; Ramzi Younis, MD
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All children with rhabdomyosarcoma require multimodality therapy to maximize local tumor control. This can involve different combinations of chemotherapy, surgery, and radiation therapy.

Leiomyosarcoma of the parapharyngeal space: A very rare entity

July 13, 2014     Haim Gavriel, MD; Eyal Yeheskeli, MD; Gratiana Hermann, MD; Ephraim Eviatar, MD
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Abstract

Leiomyosarcoma of the head and neck is very rare, as only about 100 cases have been reported; of these, only 3 cases have been previously reported in the parapharyngeal space. We report the fourth such case, and we review the features of this entity. The patient was an 84-year-old woman who presented to the emergency department for treatment of an 18-month history of right-sided headache, a 6-month history of right-sided hearing loss and nasal obstruction, and a 2-month history of dysphagia. Physical examination revealed a bulge in the right side of the soft palate and the right lateral nasopharyngeal wall and complete obstruction of the right eustachian tube. Indirect laryngoscopy detected a bulge in the right lateral hypopharyngeal wall. Otoscopy revealed otitis media with effusion in the right ear. Imaging demonstrated a space-occupying lesion in the right parapharyngeal space that extended from the base of the skull to the level of the hypopharynx. Biopsy and histology identified the mass as a leiomyosarcoma. Metastasis to the brain was discovered shortly thereafter, and the patient died 10 months later. The unusual presentation of head and neck leiomyosarcoma requires a high index of suspicion and appropriate diagnostic imaging. Surgical excision is the recommended treatment when feasible; chemoradiotherapy does not appear to affect disease progression.

Rhabdomyosarcoma of the supraglottis in an adult

June 8, 2014     Alex Fernandez, MS; Reena Gupta, MD; Hootan Zandifar, MD
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Primary laryngeal rhabdomyosarcoma is an extremely uncommon malignant neoplasm in adults, accounting for relatively few documented cases compared with squamous cell carcinoma.

Conservative cricoid surgery for chondrosarcoma: A case report

February 12, 2014     Elena Gaio, MD; Giandomenico Maggiore, MD; Alessandra Canesso, MD; and Riccardo Artico, MD
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Abstract

We present the case of a 39-year-old man who presented with hoarseness and progressively worsening dyspnea. Findings on laryngoscopy and computed tomography strongly suggested the presence of a chondrosarcoma. The patient underwent open surgery for removal of the lesion with wide margins. Reconstruction was carried out with two segments of costal cartilage. Laryngeal chondrosarcomas are rare, malignant, usually well-differentiated neoplasms that should be treated with conservative surgery. Recurrences should be treated more aggressively.

Synovial sarcoma of the parapharyngeal space

December 20, 2013     Jagdeep Singh Virk, MA, MRCS; Dhafir Al-Okati, FRCPath; Hesham Kaddour, FRCS ORL-HNS

Osteosarcoma

July 21, 2013     Lester D.R. Thompson, MD
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Osteosarcoma affects the mandible and the maxilla differently, with mandibular tumors tending to arise from the body of the mandible while maxillary tumors arise from the alveolar ridge and sinus.

Tonsillar Kaposi sarcoma in a patient with membranous glomerulonephritis on immunosuppressive therapy

July 21, 2013     Nabeel Al-Brahim, FRCPC; Ashraf H. Zaki, MD; Khaled El-Merhi, MD; Mahmoud S. Ahmad, MD
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Abstract

Kaposi sarcoma is a malignant vascular neoplasm uncommonly seen in immunosuppressed patients. Herein we report an unusual case of tonsillar Kaposi sarcoma in a patient with membranous glomerulonephritis treated with prednisolone and cyclosporine. The patient presented after 10 months of starting the treatment with a tonsillar mass. Histological examination was typical of monomorphic spindle cell proliferation with slit-like vascular channels. The tumor cells expressed CD34, D2-40 and positive nuclear stain for HHV-8. Kaposi sarcoma is associated with immunosuppression and rarely occurs in the tonsil. Clinicians should be aware of this rare presentation of Kaposi sarcoma.

Synovial sarcoma of the larynx treated by supraglottic laryngectomy: Case report and literature review

July 21, 2013     Kuauhyama Luna-Ortiz, MD; Ana Maria Cano-Valdez, MD; Isabela Werneck da Cunha, MD; Adalberto Mosqueda-Taylor, DDS
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Abstract

We describe a case of synovial sarcoma of the larynx, and we discuss the use of fluorescence in situ hybridization (FISH) in confirming the diagnosis. The patient was a 21-year-old woman who presented with a recurrence of a previously resected supraglottic tumor of the aryepiglottic folds. A horizontal supraglottic laryngectomy was performed, and the 0.5-cm tumor was resected. Histopathologic study suggested that it was a biphasic malignant tumor compatible with a synovial sarcoma. The diagnosis of synovial sarcoma was confirmed by FISH immunohistochemistry with the use of an SYT break-apart probe. The patient recovered satisfactorily, but at follow-up 5 years and 4 months later, tumoral activity was evident in the left side of the neck. A biopsy found that 5 lymph nodes contained a metastasis of the synovial sarcoma. Again, a bilateral neck dissection was performed, and it revealed that 16 of 16 right-side nodes and 36 of 36 left-side nodes were negative. Two months later, the patient received 46 Gy of radiotherapy in 23 sessions. She remained free of disease during 2 more years of follow-up. Synovial sarcoma of the larynx is a rare entity. Organ preservation seems to be indicated in these cases. The histologic diagnosis may be difficult. In this case, the identification of a genetic mutation corroborated the diagnosis.

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