Sarcoma

Primary osteogenic sarcoma of the maxilla

September 30, 2010     Raafat Makary, MD, Sania Shuja, MD, Ruis Fernandes, DMD, MD, Robert Malyapa, MD, and Nelson Goldman, MD
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Imaging characterization of follicular dendritic cell sarcoma of the neck

June 30, 2010     Adam D. Singer, BS, Paul M. Weinberger, MD, Melanie W. Seybt, MD, Lana L. Jackson, MD, Daniel J. Teague, MD, and Ramon E. Figueroa, MD, FACR
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Follicular dendritic cell sarcoma of the neck: Case report and review of current diagnostic and management strategies

June 30, 2010     Laleh Amiri-Kordestani, MD, Dennis Priebat, MD, and Stanley H. Chia, MD
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Abstract

Follicular dendritic cell sarcoma (FDCS) is a rare malignant neoplasm that can clinically mimic a number of other tumors. FDCS can follow either an indolent or aggressive course. The prognosis tends to be worse for patients with large or high-grade tumors and/or an intra-abdominal tumor site. For optimal management, complete surgical excision is recommended. Radiation therapy and/or chemotherapy may be considered for incompletely resected tumors and for tumors with poor prognostic features, but the exact role of adjuvant therapy is unknown. We report a case of cervical FDCS in a 39-year-old black man, and we review the presentation and management of this disorder, with emphasis on the differential diagnosis.

Synovial sarcoma of the head and neck: A review of its diagnosis and management and a report of a rare case of orbital involvement

May 31, 2010     Shashidhar Kusuma, MD, David J. Skarupa, MD, Kim A. Ely, MD, Anthony J. Cmelak, MD, and Brian B. Burkey, MD
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Abstract

Synovial sarcoma is typically an aggressive malignant tumor of the soft tissues, usually in the extremities, that affects young adults. Tumors of the head and neck are rare. Reported head and neck sites have included the hypopharynx (the most common site), the oropharynx, the larynx, and the soft tissues of the neck; only 4 cases of orbital involvement have been previously reported. We describe a case of synovial sarcoma of the medial canthus, which we discovered during a review of a tumor registry. The patient, an 18-year-old woman, underwent conservative excision and postoperative radiation therapy. Long-term follow-up detected no evidence of recurrence. Because there is no established, consistent approach to the treatment of synovial sarcoma of the head and neck, we also present a consensus management plan based on our review of the literature.

Granulocytic sarcoma of the nasal cavity: A case report

October 31, 2009     Mary Gorman, MD, Khwaja Asif Ahmed, MD, Arnel Pallera, MD, and Sandeep Samant, MD
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Abstract

Granulocytic sarcoma of the nasal cavity is exceedingly rare. We describe the case of a 55-year-old man who presented with an intranasal mass that was later diagnosed as a granulocytic sarcoma. The mass was treated with rapid initiation of chemotherapy, which produced a substantial decrease in tumor bulk. Granulocytic sarcoma is often mistaken for lymphoma or other poorly differentiated malignancies, and the correct diagnosis requires a high index of clinical suspicion.

Atypical lipomatous tumor/well-differentiated liposarcoma of the parotid gland: Case report and literature review

September 30, 2009     Usama Mahmood, MD, John D. Nguyen, MD, John Chang, MD, Mai Gu, MD, PhD, and Brian J.F. Wong, MD, PhD
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Abstract

Although liposarcoma is the second most common soft-tissue sarcoma in adults, it is exceedingly rare in the head and neck. According to our MEDLINE search, only 11 cases of liposarcoma of the parotid gland have been reported since 1968. We report a new case of primary atypical lipomatous tumor/well-differentiated liposarcoma of the parotid gland in a 77-year-old man. Because only a very limited number of case reports and small series have been published on liposarcoma in the head and neck, we also provide a review of the literature on this uncommon disease entity.

Cricoid chondrosarcoma presenting as breathy dysphonia

September 30, 2009     Scott Rickert, MD, Robert Buckmire, MD, and Lucian Sulica, MD
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Synovial sarcoma of the cheek

September 30, 2009     Amy Mettman, MD, Larry L. Myers, MD, and Kelley Carrick, MD
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Alveolar soft-part sarcoma of the tongue in a 17-month-old

September 30, 2009     Shahab Hillyer, MD, Jose C. Vicens, DMD, Howard Levinson, MD, Rajendra Bhayani, MD, Lilia Mesea, MD, Rashid Chaudhry, MD, Edgar Fayans, DDS, and Richard Fogler, MD
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Abstract

Alveolar soft-part sarcoma (ASPS) is a rare and often fatal tumor. Overall survival rates have been reported to be 62% after 5 years, 42% after 10 years, and 18% after 20 years. ASPS accounts for 5% of all pediatric soft-tissue sarcomas other than rhabdomyosarcomas. In children, ASPS rarely occurs in the oral cavity, and to the best of our knowledge, only 12 cases of ASPS of the tongue occurring during the first decade of life have been previously reported in the literature. Because of the rarity of lingual ASPS in children, no specific treatment protocols have been developed, which makes its management difficult. We report a new case of lingual ASPS in a young child. Our patient was a 17-month-old girl whose tumor was located at the base of the tongue. She was successfully treated with surgical excision without adjuvant radiation or chemotherapy. Postoperatively, she has remained disease-free during 4 years of follow-up.

Chondrosarcoma of the nasal septum

January 1, 2009     Akram Rahal, MD, FRCSC, Joseph R. Durio, PA-C, and Michael L. Hinni, MD
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Abstract

Chondrosarcoma of the nasal septum is a rarely encountered malignancy. When it does occur, early diagnosis is difficult because patients generally present with common, nonspecific sinonasal complaints. We describe a case of chondrosarcoma of the nasal septum in a 38-year-old Hispanic man. The lesion was discovered on computed tomography during a workup for symptoms of chronic sinus disease. The tumor was removed in its entirety and identified as a low-grade lesion that required no further treatment. The patient remained disease-free 4 years postoperatively. We review the diagnosis of this neoplasm and its treatment and follow-up.

Chondrosarcoma of the greater cornu of the hyoid: A case report and literature review

September 25, 2008     Sanjay Verma, MBBCh, MA, FRCS (ORL-HNS), PhD, Perumal Chokkalingam, BSc, FRCS, and Duncan McRae, FRCS, FRCS (ORL-HNS)
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Abstract

We report an unusual case of a young woman with chondrosarcoma affecting the greater cornu of the hyoid bone. Only a handful of reports of primary chondrosarcoma involving the hyoid exist in the world literature. The case described presented as a swelling in the submandibular triangle, and as such posed a particular clinical and radiologic diagnostic challenge. Our subsequent surgical management of this rare entity comprised local excision followed by close observation, and is defined in the context of the existing literature. Two years after surgery, the patient remained free of tumor recurrence; she was subsequently lost to follow-up.

Leiomyosarcoma of the larynx: Case report and review of the literature

April 30, 2008     Jayant Sastri Goda, MD, DNB, Karuppiah Saravanan, MS, DNB, R.K. Vashistha, MD, FRCPath, Vinay Kumar, MD, and Ashok K. Gupta, MS
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Abstract

Leiomyosarcoma of the larynx is an extremely rare entity. This tumor is aggressive and is associated with dismal treatment results. We present a case of leiomyosarcoma of the larynx treated with surgery and postoperative radiotherapy. In this article we also discuss laryngeal leiomyosarcoma's clinical manifestations, associated diagnostic dilemmas, and optimal management, as well as a review of the literature.

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