Indian Journal of Dermatology
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E–CASE REPORT
Year : 2013  |  Volume : 58  |  Issue : 3  |  Page : 244
Alveolar soft part sarcoma: A rare diagnosis


1 Department of Dermatology, Leprology and Venereology, College of Medicine and Sagore Dutta Hospital, Kamarhati, Kolkata, India
2 I. P. G. M. E and R, Kolkata, India

Date of Web Publication20-Apr-2013

Correspondence Address:
Priyanka Sarkar
Department of Dermatology, Venereology and Venereology, College of Medicine and Sagore Dutta Hospital, Kamarhati, Kolkata 58, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.110873

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   Abstract 

Alveolar soft-part sarcoma (ASPS) is an extremely rare disease arising from connective tissues with a propensity for recurrence and metastasis. Clinically, it can be confused with hemangioma or arterio-venous malformations. Thus, a high index of suspicion and histopathological examination are required to make a definitive diagnosis. We report a case of recurrent ASPS in a young female with multiple sites involvement without any features of metastasis who has been treated with excision of the symptomatic lesions followed by chemotherapy.


Keywords: Alveolar soft-part sarcoma, alveolar pattern in histopathology, surgical excision, chemotherapy


How to cite this article:
Sarkar P, Mukherjee S, Saha ML, Biswas RS. Alveolar soft part sarcoma: A rare diagnosis. Indian J Dermatol 2013;58:244

How to cite this URL:
Sarkar P, Mukherjee S, Saha ML, Biswas RS. Alveolar soft part sarcoma: A rare diagnosis. Indian J Dermatol [serial online] 2013 [cited 2019 Aug 21];58:244. Available from: http://www.e-ijd.org/text.asp?2013/58/3/244/110873

What was known? Alveolar soft part sarcoma is a rare type of soft tissue sarcoma affecting female more than male and clinically mimics hemangioma or arterio-venous malformation



   Introduction Top


Alveolar soft-part sarcoma (ASPS) is an extremely rare disease arising from connective tissues. ASPS predominately affects adolescents and young adults with a predilection for women. [1] It occurs commonly in the lower extremities (44%) and the head and neck (27%), of which 25% occur in the tongue. [2] Although the origin of ASPS is still unknown, recent cytogenetic studies revealed some chromosomal translocation, which may have a role. [3] The tumor runs an indolent course, but may recur and metastasize, leading to high mortality. We describe such a rare case.


   Case Report Top


A 32-years-old female from a remote village presented with multiple painless soft globular swellings all over the body including face, tongue, neck, chest, abdomen, back, genitalia, and all the limbs for a duration of 3 months [Figure 1] and [Figure 2]. Most of the lesions were skin-colored, but few of the lesions over forehead, neck, back, and genitalia showed reddish to bluish discoloration while few showed crusting over the surface. The patient gave history of similar type of lesion in right thigh 3 years back, for which she underwent surgical excision, but no histopathological examination, was done at that time. There was associated history of anorexia, weight loss, and fatigue. On examination, apart from the aforesaid lesions, a nodule was palpable in the posterior fornix of vagina and another in the anterior rectal wall 6 cm from anal verge. Other systems were within normal limit. After thorough investigations, all the blood parameters were found to be normal, except moderate degree of anemia. Fortunately, her chest X-ray, USG of whole abdomen, CT scan of brain revealed no abnormality. The most symptomatic swelling which were present over right thigh (at the site of previous excision) and back were excised followed by biopsy. Histopathology showed large polygonal cells with eccentrically-placed prominent nucleus and abundant eosinophilic cytoplasm arranged in an alveolar pattern separated by fibrous septae and occasional mitotic figures [Figure 3]. Cytopathology revealed the tumors cells expressing vimentin. Following surgery, the patient was referred to radiotherapy department and was put on chemotherapy regimen comprising of vincristine (2 mg), adriamycin (60 mg), and cyclophosphamide (90 mg), given at an interval of 3 weeks. She received 6 such cycles and is under close follow-up. There is mild reduction of the lesions with moderate symptomatic improvement and without any features of metastasis.
Figure 1: Multiple globular swellings over the back of trunk

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Figure 2: Similar swellings over face and neck

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Figure 3: Characterisistic histopathology of ASPS

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   Discussion Top


ASPS was first described by Christopherson, et al. in 1952. [4] ASPS comprises only 1% of all soft tissue sarcomas. [2] The tumor occurs most commonly in the soft tissues of the extremities, rarely in arm, chest, and retro-peritoneal tissues, even stomach and female genital tract has been reported. [5],[6] The tumor occurs predominantly in females, with a male:female ratio of 1:2. [1] The most probable genetic theory of origin is chromosomal re-arrangements at t (X;17)(p11;q25), resulting in the ASPL-TFE3 fusion gene. [3] Since women have an extra X-chromosome, they are likely to develop an X autosomal translocation, which is theoretically doubles that of men. [3] ASPS have a close clinical resemblance to hemangioma. Histo-pathologically, ASPS needs to be differentiated from paraganglioma, rhabdomyosarcoma, metastatic adrenal carcinoma and melanoma by demonstrating cytological uniformity, lack of nuclear atypia, and paucity of mitotic figures in ASPS. The outcomes depend on prognostic factors such as a patient's age, tumor size, recurrence (which has been reported to range from 11-50% [7] ), and the presence of metastasis at the time of diagnosis. It usually metastasizes late to the lungs, bones, lymph nodes, and brain. [7],[8] Accurate diagnosis and treatment of this unusual tumor requires clinical suspicion and clinico-pathological correlation with appropriate radiological studies. Wide excision is the therapy of choice for localized disease. [9] While surgery may improve outcomes, traditional chemotherapeutic agents and/or radiotherapy have limited role. [10] As treatment options are limited when lesions are multiple, surgical resection of the symptomatic lesions followed by chemotherapy was given in our patient. In spite of extensive involvement, metastasis did not occur in this patient till her last visit.


   Conclusion Top


ASPS is a rare tumor. To the best of our knowledge, very few cases of ASPS has been reported from India. Moreover, this female with multiple lesions involving unusual sites but without any features of metastasis is uncommon of ASPS. However, a classical histopathological feature from the biopsy of the lesions favors the diagnosis. Because of the rarity of occurrence of the tumor with uncommon presentation, the case has been reported.

 
   References Top

1.Stockwin LH, Vistica DT, Kenney S, Schrump DS, Butcher DO, Raffeld M, Shoemaker RH: Gene expression profiling of alveolar soft-part sarcoma (ASPS). BMC Cancer 2009;9:22.  Back to cited text no. 1
    
2.Rosai J, Ackerman LV: Soft tissue sarcoma. In: Ackerman's surgical pathology. 2 nd ed. St. Louis: Mosby Company; 1989. p. 1547-618.  Back to cited text no. 2
    
3.Ladanyi M, Lui MY, Antonescu CR, Krause-Boehm A, Meindl A, Argani P, et al. The der (17) t (X;17)(p11;q25) of human alveolar soft part sarcoma fuses the TFE3 transcription factor gene to ASPL, a novel gene at 17q25. Oncogene 2001;20:48-57.  Back to cited text no. 3
    
4.Christopherson WM, Foote FW Jr, Stewart FW. Alveolar soft part sarcoma: Structurally characteristic tumors of uncertain histogenesis. Cancer 1952;5:100-11.  Back to cited text no. 4
    
5.Yaziji H, Ranaldi R, Verdolini R, Morroni M, Haggitt R, Bearzi I. Primary alveolar soft part sarcoma of the stomach: A case report and review. Pathol Res Pract 2000;196:519-25.  Back to cited text no. 5
    
6.Nielsen GP, Oliva E, Young RH, Rosenberg AE, Dickersin GR, Scully RE. Alveolar soft-part sarcoma of the female genital tract: A report of nine cases and review of the literature. Int J Gynecol Pathol 1995;14:283-92.  Back to cited text no. 6
    
7.Portera CA Jr, Ho V, Patel SR, Hunt KK, Feig BW, Respondek PM, et al. Alveolar soft part sarcoma: Clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer 2001;91:585-91.  Back to cited text no. 7
    
8.Folpe AL, Deyrup AT. Alveolar soft-part sarcoma: A review and update. J Clin Pathol 2006;59:1127-32.  Back to cited text no. 8
    
9.Kayton ML, Meyers P, Wexler LH, Gerald WL, LaQuaglia MP. Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults. J Pediatr Surg 2006;41:187-93.  Back to cited text no. 9
    
10.Reichardt P, Lindner T, Pink D, Thuss-Patience PC, Kretzschmar A, Dorken B. Chemotherapy in alveolar soft part sarcomas. What do we know? Eur J Cancer 2003;39:1511-6.  Back to cited text no. 10
    

What is new? 1. Occurrence of multiple lesions in ASPS, astonishingly without any metastasis in the present case. 2. When lesions are multiple, surgical excision of the symptomatic lesions followed by chemotherapy is a good treatment option


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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    Abstract
   Introduction
   Case Report
   Discussion
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    References
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