Sarcoma

AIDS-related primary Kaposi sarcoma of the nasopharynx

June 13, 2011     Fatih Çelenk, MD, Metin Yilmaz, MD, Korhan Asal, MD, Özgür Ekinci, MD, and Nil Tokgöz, MD
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Abstract

Primary nasopharyngeal Kaposi sarcoma is extremely rare, as only 1 case has been previously reported in the literature. We report a new case, which occurred in a 37-year-old man with a known history of acquired immune deficiency syndrome (AIDS). The patient presented with complaints of recurrent epistaxis and postnasal hemorrhage. Endoscopic examination detected a bluish, smooth, firm, nonpulsatile mass in the nasopharyngeal wall. Histopathologic findings on biopsy were consistent with Kaposi sarcoma. The tumor was successfully treated with radiotherapy. Kaposi sarcoma should be considered in the differential diagnosis of any AIDS patient who presents with recurrent unilateral nasal bleeding.

Nasopharyngeal dendritic cell sarcoma, not otherwise specified, in a 34-year-old man

June 13, 2011     William B. Horton, BS, David A. Joyner, MD, William P. Daley, MD, Karen T. Pitman, MD, and Majid A. Khan, MD
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Abstract

Dendritic cell sarcoma, not otherwise specified (NOS), is an entity that is poorly defined because of its rarity and poorly understood pathogenesis. It is characterized by positive immunohistochemical staining for S-100 and CD1a, along with an absence of cytoplasmic Birbeck granules on electron microscopy. We report the case of a surgically inaccessible nasopharyngeal dendritic cell sarcoma, NOS, in a 34-year-old man. Treatment with chemotherapy along with adjuvant radiation therapy was successful in decreasing the size of the nasopharyngeal mass, and the patient remained free of any evidence of recurrence nearly 5 years after treatment.

Abrupt tonsillar hemorrhage from a metastatic hemangiosarcoma of the breast: Case report and literature review

March 1, 2011     Ronen Bar, MD, Aviram Netzer, MD, Dmitry Ostrovsky, MD, Marcelo Daitzchman, MD, and Avishay Golz, MD
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Abstract

We describe a case of primary breast hemangiosarcoma with tonsillar metastasis that manifested as a profuse oropharyngeal hemorrhage. The patient was a 54-year-old woman who presented to the emergency department with oral bleeding. A thorough examination revealed a distorted morphology of the left tonsil and a hemorrhage originating from it. Conservative methods to control the hemorrhage failed, and tonsillectomy was carried out. Histopathologic analysis revealed that the left tonsil contained hemangiosarcoma cells of the same kind that had been removed from the breast. Primary hemangiosarcomas of the breast are rare; when they do occur, they usually recur locally and spread hematogenously. To the best of our knowledge, this is the first case of a primary breast hemangiosarcoma with tonsillar metastasis to be reported in the literature. We present a detailed analysis of this case, and we review the relevant literature on metastatic tonsillar tumors, with attention to the role of therapeutic embolization.

Laryngeal alveolar rhabdomyosarcoma involving the true vocal fold in an adult: Case report

December 17, 2010     Douglas D. Leventhal, MD, Joseph Spiegel, MD, and William Keane, MD
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Abstract

Rhabdomyosarcoma of the larynx is extremely rare in adults, as only 17 well-documented cases have been previously reported in the English-language literature. Of these, only 2 cases (both male) involved the alveolar subtype of rhabdomyosarcoma, and neither involved the true vocal folds. We report a case of alveolar rhabdomyosarcoma of the true vocal fold in 54-year-old woman. She was successfully treated with conservative surgery, radiation, and chemotherapy. Management of head and neck rhabdomyosarcoma has evolved from radical surgery to less morbid procedures supplemented with radiation and chemotherapy. However, because this tumor is so rare in the adult population, no adult-specific treatment regimen has emerged. Nevertheless, the success of treatment in the pediatric population supports its use in adults.

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

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

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.

Synovial sarcoma of the cheek

September 30, 2009     Amy Mettman, MD, Larry L. Myers, MD, and Kelley Carrick, MD

Cricoid chondrosarcoma presenting as breathy dysphonia

September 30, 2009     Scott Rickert, MD, Robert Buckmire, MD, and Lucian Sulica, MD

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.

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