Sarcoma

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
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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.

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.

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.

Extraosseous Ewing sarcoma and peripheral primitive neuroectodermal tumor of the thyroid gland: Case report and review

April 17, 2013     Magdalena Chirila, MD, PhD; Mihaela Muresan, MD; Elisabeta Ciuleanu, MD, PhD; and Marcel Cosgarea, MD, PhD
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Abstract

The Ewing family of tumors and peripheral primitive neuroectodermal tumor (pPNET) represent different manifestations of the same entity. Immunohistochemical and cytogenetic studies suggest that these tumors have a common origin. Ewing sarcoma is more common in bone, while pPNET is more common in soft tissues. Extraosseous Ewing sarcoma (EoES) is rare. We present the case of a 48-year-old man who presented with acute obstructive respiratory failure secondary to a large thyroid swelling. The patient was initially diagnosed with giant B-cell non-Hodgkin lymphoma and treated with chemotherapy. However, subsequent immunohistochemical staining of biopsy specimens revealed that the patient actually had EoES/pPNET of the thyroid gland. We performed a nearly complete surgical resection of the tumor plus a total laryngectomy and resection of five tracheal rings. However, the patient died of a cerebral metastasis 1 month later after he had completed one cycle of postoperative chemotherapy.

Adult case of large sinonasal embryonal rhabdomyosarcoma with intracranial extension

April 17, 2013     Enrique Palacios, MD; Alexander Quiroz-Casian, MD; Lorena Garza Garcia, MD; Philip J. Daroca, MD; Harold R. Neitzschman, MD
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Rhabdomyosarcomas often present with nonspecific symptoms such as headache, nasal congestion, proptosis, epistaxis, and cranial nerve palsies, reflecting extension to the base of the skull.

Radiation-induced osteosarcoma of the larynx: Case report and literature review

October 8, 2012     Murat Ulusan, MD; Rasim Yilmazer, MD; Yasemin Ozluk, MD; Murat Enoz, MD; Yusufhan Suoglu, MD
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Abstract

Laryngeal osteosarcoma is an extremely rare disease. Only 23 cases have been published in the literature. Radiation-induced laryngeal osteosarcoma is even rarer; this is only the third such case to be reported. A 59-year-old man underwent radiotherapy for an in situ laryngeal squamous cell carcinoma at another institution. Five years later he developed a laryngeal osteosarcoma, and a total laryngectomy was performed. Although previous reports showed a poor prognosis, our patient was without disease at the 8-year follow-up. To the best of our knowledge, this is the longest disease-free follow-up to be reported in the literature. We also present a review of the world’s literature.

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.

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.

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