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Year : 1999  |  Volume : 9  |  Issue : 1  |  Page : 32-33
Alveolar soft part sarcoma of the elbow


1 Department of Imageology, Nizam's Institute of Medical Sciences, Panjagutta, Hyderabad, India
2 Department of Pathology, Nizam's Institute of Medical Sciences, Panjagutta, Hyderabad, India
3 Department of Orthopedics, Nizam's Institute of Medical Sciences, Panjagutta, Hyderabad, India

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How to cite this article:
Sreedevi I, Naidu G S, Sunderam C, Narendranath L. Alveolar soft part sarcoma of the elbow. Indian J Radiol Imaging 1999;9:32-3

How to cite this URL:
Sreedevi I, Naidu G S, Sunderam C, Narendranath L. Alveolar soft part sarcoma of the elbow. Indian J Radiol Imaging [serial online] 1999 [cited 2019 Oct 22];9:32-3. Available from: http://www.ijri.org/text.asp?1999/9/1/32/28371
Sir,

We wish to report a case of an alveolar soft part sarcoma of the right elbow.

A forty-five years old woman presented with a profusely bleeding wound and a swelling of the right elbow. This swelling started six years ago, with a little pain and gradual increase in size. Two days prior to the presentation, fine needle aspiration cytology was attempted and the patient developed continuous bleeding. The patient was then referred to us for further management.

Radiographs of the right elbow showed an expansile osteolytic lesion involving the proximal ulna with a large soft tissue component [Figure - 1]. Posteriorly, the proximal radius showed cortical thinning. The humerus showed a permeative pattern. A radiograph of the chest showed multiple, bilateral nodular opacities of varying size, suggesting metastases. A diagnosis of a soft tissue malignant neoplasm with metastases was made. An above-elbow amputation was performed. Histopathology of the lesion revealed an alveolar soft part sarcoma.

Alveolar soft part sarcoma (ASPS) is a clinically morphologically distinct soft part sarcoma that was defined and named by Christopherson et al in 1952 [1]. Its incidence has been reported to be less than 1% of all primary soft tissue malignant neoplasms [2]. It is common in adolescents and young adults, especially in the age group of 15-35 years. It is more common in women, and children also can be affected. It occurs predominantly in the lower extremities. More often, the right side of body is involved. The tumor usually grows as a painless mass without any functional impairment. In most cases, metastases in the lungs or brain are the first manifestation. The incidence of metastases seems to be highest in the lungs (38%) followed by the brain and bones (33%) [3]. When the tumor is deep in the soft tissues and close to the bone it may produce secondary destruction. Sometimes the destruction is so extensive that it is difficult to determine whether the primary lesion is in the bone or in the soft tissues. On MR, most of the alveolar soft part sarcomas demonstrate increased signal intensity on both T1W and T2W images with flow voids in the center and at the margins of the tumor [4]. The recognition of these characteristic MR findings may lead to the early diagnosis of ASPS, especially when the clinical presentation is that of a slow growing soft tissue mass in young adult patients.

Alveolar soft part sarcoma shows a characteristic histological pattern with uncertain histogenesis. Microscopic pictures show dense fibrous trabeculae dividing the tumor into compact groups of compartments of irregular sizes. These compartments are further divided into sharply defined walls or islands of tumor cells that are separated from one another by thin-walled vascular channels. The cellular aggregates show central degeneration and loss of cohesion resulting in pseudo-alveolar patterns [Figure - 2]. The PAS preparation reveals varying amounts of extra-cellular glycogen and characteristically PAS-positive, diastase resistant crystalline material. The crystals are diagnostic of the tumor [2].

 
   References Top

1.Cristopherson, Foote WM, Jr Stewart, FW. Alveolar soft part sarcoma, structurally characteristic tumours of uncertain histogenesis. Cancer 1952; 5: 100.   Back to cited text no. 1    
2.Sreedevi, Sreenivas Rao, Bhaskar Reddy. Alveolar soft part sarcoma of knee joint. Ind J Radiol Imag 1991; Suppl to Nov. Part II: 628-631.   Back to cited text no. 2    
3.Kagei T, Kannuki S, Hondo H, Maisumoto K, Naramura H, Yamashita S. Two cases metastatic intracerebral alveolar soft part carcinoma. No-Shinkei-Geka 1995; 23: 627-32.   Back to cited text no. 3    
4.Twamoto Y, Morimoto N, Chuman H, Shinohara N, Sugioka Y. The role of MR Imaging in the diagnosis alveolar soft part sarcoma. A report of 10 cases. Skel Radiol 1995; 24: 267-70.  Back to cited text no. 4    

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Correspondence Address:
I Sreedevi
Department of Imageology, Nizam's Institute of Medical Sciences, Panjagutta, Hyderabad
India
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Source of Support: None, Conflict of Interest: None


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