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Indian Journal of Medical and Paediatric Oncology
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Year : 2019  |  Volume : 40  |  Issue : 2  |  Page : 309-310  

Alveolar soft-part sarcoma on the abdominal wall in an adult: A rare presentation with cytological and histological correlation

Department of Pathology, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, India

Date of Web Publication17-Oct-2019

Correspondence Address:
Rajeshwari K Muthusamy
Department of Pathology, Kovai Medical Center and Hospital, Coimbatore - 641 014, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijmpo.ijmpo_95_18

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How to cite this article:
Muthusamy RK, Mehta SS. Alveolar soft-part sarcoma on the abdominal wall in an adult: A rare presentation with cytological and histological correlation. Indian J Med Paediatr Oncol 2019;40:309-10

How to cite this URL:
Muthusamy RK, Mehta SS. Alveolar soft-part sarcoma on the abdominal wall in an adult: A rare presentation with cytological and histological correlation. Indian J Med Paediatr Oncol [serial online] 2019 [cited 2020 Mar 30];40:309-10. Available from: http://www.ijmpo.org/text.asp?2019/40/2/309/269472


We report a case of a male patient who presented with a painless swelling in the abdomen noticed 6 months back, with sudden increase in size over the past 3 months. On examination, there was a firm swelling of size 8 cm × 6 cm in the left hypochondrium, in the intramuscular plane. Contrast-enhanced computed tomography revealed a well-defined solid mass lesion with central hypodensity and heterogeneously intense postcontrast enhancement within the rectus abdominis muscle in the supraumbilical region. Imaging features suggested the possibility of a neurogenic tumor. Fine-needle aspiration revealed loose aggregate and dispersed large cells with eccentric nuclei, prominent nucleoli, and abundant granular cytoplasm [Figure 1]a and [Figure 1]b. Many bare nuclei were seen. With the differential diagnosis of alveolar soft-part sarcoma (ASPS) and granular cell tumor, a wide excision was done to remove the tumor.
Figure 1: (a) Smear showing loose clusters of large cells with abundant granular cytoplasm (H and E, ×10). (b) High-power image showing frayed cytoplasm and large nuclei with nucleoli (H and E, ×40). (c) histology showing large cells in nests with delicate septa (H and E, ×10); inset showing granular cytoplasm, large nuclei, and prominent nucleoli (H and E, ×40). (d) Intracytoplasmic rod-like crystals (Periodic acid–Schiff with diastase, ×40)

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The specimen showed a circumscribed soft reddish-brown tumor of size 6.2 cm × 5.7 cm × 3.3 cm. Histology revealed large polygonal cells in nests and organoid pattern with delicate septa. The cells had abundant eosinophilic granular cytoplasm, eccentric round nuclei, and prominent nucleoli [Figure 1]c. Nuclear atypia, tumor giant cells, multinucleation, and extensive lymphovascular emboli were present. Periodic acid–Schiff (PAS) and PAS with diastase revealed tumor cells with intracellular crystalline material [Figure 1]d. The tumor sections were subjected to a panel of immunohistochemical markers including vimentin, desmin, pancytokeratin, chromogranin, CD10, myogenin, NSE, S-100, and Ki-67. The tumor cells were positive to vimentin [Figure 2]a and desmin [Figure 2]b. The other immunohistochemical markers were negative. These morphological and immunohistochemical features favored the diagnosis of ASPS.
Figure 2: (a) Tumor cells showing positivity to vimentin (×40). (b) Tumor cells with desmin positivity (×40)

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  Discussion Top

ASPS is a slow-growing malignant soft-tissue tumor of uncertain origin and usually occurs in young adults. It accounts for 1% of all soft-tissue sarcomas [1],[2] with female preponderance. It is seen in the deep soft tissues of thigh or buttock in adults and the head and neck region, especially tongue and orbit, in children.[1],[2] Jia et al. described ASPS on the abdominal wall in a 2-year-old child.[3]

Aspiration smears will show large dyscohesive monotonous cells, having abundant fine vacuolated to granular cytoplasm and round nuclei with prominent nucleoli. Stripped nuclei and fraying of the cytoplasmic margins are quite common. ASPS shows distinctive histomorphology of organoid or nesting pattern at low magnification, separated by delicate vasculature. Loss of cellular cohesion of the centrally located cells in nests results in pseudoalveolar pattern.[1],[4] The neoplastic cells show distinct cell borders, granular cytoplasm, and central nuclei with prominent nucleoli. Multinucleation and atypia can be seen. Faint intracytoplasmic rod-shaped inclusions and granules seen in hematoxylin and eosin stain; highlighted by PAS stain after diastase digestion as rod like crystals is very characteristic.[1],[2],[4] The differentials to be considered are granular cell tumor, metastatic renal cell carcinoma, and clear-cell sarcoma of soft parts [Table 1].[5],[6]
Table 1: Diagnostic criteria to differentiate alveolar soft-part sarcoma and its mimics

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ASPS demonstrates consistent strong nuclear staining to Transcription factor binding to IGHM enhancer3 (TFE3), in addition to focal desmin and S-100 positivity. Electron microscopy and cytogenetics further helps confirm the diagnosis, with distinctive membrane-bound rhomboid or rectangular crystals, composed of a periodical lattice work of rigid fibrils and ASPSCR1-TFE3 fusion transcript due to specific translocation der(17)t(X; 17)(p11;25).[1]

Surgical resection with clear margins is the appropriate treatment plan for ASPS. Chemotherapy and radiotherapy are under consideration to prevent local recurrence or metastasis. Age at presentation, tumor size, and the presence of metastasis influence the prognosis.[1],[4]

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There are no conflicts of interest.

  References Top

Ordonez NG, Ladanyi M. Alveolar soft part sarcoma. In: Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F, editors. WHO Classification of Tumours of Soft tissue and Bone. 4th ed. Lyon: International Agency for Research on Cancer; 2013. p. 218-20.  Back to cited text no. 1
Weiss SW, Goldblum JR, editors. Malignant soft tissue tumors of uncertain type. In: Enzinger and Weiss's Soft Tissue Tumors. 4th ed. St. Louis: Mosby; 2001. p. 1509-21.  Back to cited text no. 2
Jia Y, Wu D, Shang C, Yu J, Zhang KR. Alveolar soft part sarcoma occurring on the abdominal wall of a 2-year-old child. J Pediatr Hematol Oncol 2011;33:e80-2.  Back to cited text no. 3
Folpe AL, Deyrup AT. Alveolar soft-part sarcoma: A review and update. J Clin Pathol 2006;59:1127-32.  Back to cited text no. 4
Majumdar K, Saran R, Tyagi I, Jain A, Jagetia A, Sinha S. Cytodiagnosis of alveolar soft part sarcoma: Report of two cases with special emphasis on the first orbital lesion diagnosed by aspiration cytology. J Cytol 2013;30:58-61.  Back to cited text no. 5
[PUBMED]  [Full text]  
Wakely PE Jr., McDermott JE, Ali SZ. Cytopathology of alveolar soft part sarcoma: A report of 10 cases. Cancer 2009;117:500-7.  Back to cited text no. 6


  [Figure 1], [Figure 2]

  [Table 1]


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