Sarcoma

Unusual maxillary chondrosarcoma

September 7, 2012     Daniel O. Rosenblatt, MD; Rosa B. Lipin, MD; Enrique Palacios, MD, FACR; Paul Friedlander, MD; Harold Neitzschman, MD, FACR
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Chondrosarcomas can be distinguished from osteosarcomas because they have the distinction of producing chondroid (cartilaginous matrix) but not osteoid. When they occur in the head and neck, they usually are found in the anterior maxilla and sinonasal structures 

Case report: Leiomyosarcoma of the parapharyngeal space

July 5, 2012     Pradipta Kumar Parida, MBBS, MS; Jaimanti Bakshi, MBBS, MS; Sanjeev Bhagat, MBBS, MS; Ramandeep Singh Virk, MBBS, MS
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Abstract

Leiomyosarcoma is usually found in the female genital tract, the retroperitoneum, the wall of the gastrointestinal tract, and subcutaneous tissues. An appearance of this malignant tumor in the parapharyngeal space is extremely rare and may be difficult to diagnose. Because of its rarity, little information exists on management and prognosis. We report the case of a 50-year-old man with a parapharyngeal space leiomyosarcoma who was treated with total excision of the tumor and postoperative radiotherapy. At follow-up 6 months postoperatively, he was well and free of disease. To the best of our knowledge, this is only the third case of a leiomyosarcoma in the parapharyngeal area to be reported in the literature. We discuss the diagnosis and treatment of leiomyosarcoma in this aspect.

Myxoid malignant fibrous histiocytoma presenting as a midline nasal mass

March 31, 2012     Jaimie DeRosa, MD and Jeffrey R. Smit, MD
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Abstract

Myxoid malignant fibrous histiocytoma is a rare type of pediatric non-rhabdomyosarcoma soft-tissue sarcoma. The case of a 5-year-old girl is presented, highlighting the potential for multiple pitfalls and aberrant differential diagnoses that need to be identified for successful treatment of pediatric myxofibrosarcomas. An awareness of these tumors and a call for standardized postsurgical treatment protocols is necessary in order to successfully treat children with this disease.

Myxoid/round cell liposarcoma of the supraclavicular fossa

February 18, 2012     Cagatay Oysu, MD, Celil Uslu, MD, Barıs Naiboglu, MD, and Evren Ay Ogredik, MD
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Abstract

Metastases from the tracheobronchial tree and the genitourinary and gastrointestinal systems are the most common malignant lesions of the supraclavicular fossa. Liposarcomas in this region, as primary tumors, are rarely encountered. In this article, we report a case of myxoid liposarcoma of the supraclavicular fossa with a fulminant course.

Suboccipital malignant solitary fibrous tumor: Report of a case

February 18, 2012     M. Geraldine Zuniga, MD, Brent A. Orr, MD, Steven S. Chang, MD, and Ralph P. Tufano, MD, FACS
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Abstract

Solitary fibrous tumors (SFTs) are rare soft-tissue sarcomas usually arising in the pleura of the thoracic cavity. Thirty percent of SFTs present in extrapleural locations, and infrequently in the head and neck region. While the majority of SFTs are benign, aggressive variants have been described. We report a case of a patient with a malignant SFT arising in the suboccipital soft tissues of the neck. We elucidate the diagnostic and therapeutic challenges in managing this tumor in this unusual location.

Recurrent myxoid liposarcoma of the buccal mucosa in a young boy: A case report and review of the literature

December 15, 2011     Elizabeth M. Nichols, MD, Ali Mirmiran, MD, Michael C. Garofalo, MD, Chen-Chih J. Sun, MD, Kyle Hatten, MD, and Jeffrey Wolf, MD, FACS
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Abstract

Liposarcomas are uncommon in the pediatric population. We present the case of a boy who had experienced multiple recurrences of liposarcoma in the buccal space before he reached the age of 13 years. We also provide a review of the literature and a discussion of adjuvant therapy, which are important to understanding the nature of this disease.

Osteosarcoma of the skull base in a 15-year-old boy

October 26, 2011     Gretchen M. Oakley, BA, Dary J. Costa, MD, Ron B. Mitchell, MD, and Cirilo Sotelo, MD
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External ear sarcoma: A review of the Surveillance Epidemiology and End Result (SEER 17) database

August 15, 2011     Stephanie A. Austin, MD, Mary J. Hawkshaw, BSN, RN, CORLN, and Robert T. Sataloff, MD, DMA, FACS
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Abstract

Soft-tissue sarcomas arising in the skin of the external ear have a better prognosis than might be expected based on the behavior of other sarcomas. Currently there are a few major case series analyzing soft-tissue sarcomas and the factors related to their prognosis. The purpose of this study was to determine the behavior of these tumors and attempt to define factors affecting prognosis. We extracted data from the Surveillance Epidemiology and End Result (SEER) 17 Registry and identified a total of 297 patients with sarcoma of the external ear who were included in the registry from 1973 to 2004. Of these, 176 (59.3%) patients were diagnosed with malignant fibrous histiocytoma. Dermatofibrosarcoma was the second most prevalent soft-tissue sarcoma (56 patients; 18.9%), followed by leiomyosarcoma (27 patients; 9.1%). The overall 5-year survival rate of patients with all histologic types of STS of the external ear was 78.5%.

Bilateral tonsillar and esophageal Kaposi sarcoma in an HIV-negative patient

July 13, 2011     Irem Hicran Ozbudak, MD, Kenan Guney, MD, Derya Mutlu, MD, Tekinalp Gelen, MD, and Gulay Ozbilim, MD
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Abstract

Tonsillar involvement in Kaposi sarcoma is extremely rare, as only a few such cases have been reported; all but 1 of these previously reported cases occurred in patients with human immunodeficiency virus (HIV) infection. We describe what to the best of our knowledge is the first reported case of concurrent bilateral tonsillar and esophageal Kaposi sarcoma in an HIV-negative patient. A 68-year-old man presented with sore throat and dysphagia. Clinical examination revealed the presence of bilateral and asymmetrical tonsillar masses, as well as generalized lymphadenopathy in the cervical chain. The masses were resected, and findings on histopathologic analysis were consistent with Kaposi sarcoma. In addition, human herpesvirus 8 was demonstrated on a tonsil specimen by polymerase chain reaction, and microinvasive squamous cell carcinoma was also detected. Later, another Kaposi sarcoma lesion was detected in the lower third of the esophagus. We recommend that clinicians not discount the possibility of oral classic Kaposi sarcoma in the workup of an immunocompetent patient with oral vascular lesions.

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.

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.

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.

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