199 Q 1990 The Japanese Society of Pathology

Alveolar Soft Part Sarcoma A Clinicopathologic and Immunohistochemical Study of 12 Cases

Yoshihiro Matsuno', Kiyoshi Mukai', Masayuki Ita bashi', Yuko Yamauchi', Teruyuki Hirota', Takashi Nakajima1,2, and Yukio Shimosato'

Twelve cases of alveolar soft part sarcoma (ASPS) were reviewed. Seven of them arose primarily in the lower extremities, three i n the head and neck region, and two in other parts. ASPSs in the head and neck region occurred in children before 10 years of age, whereas ASPSs i n the other regions occurred i n rather older patients. Moreover, ASPSs of the head and neck were relatively small in size, and were diagnosed earlier than those i n other regions. Histologically, six cases (including all the head and neck cases) contained considerable area of small and indistinct alveolar structures. Four cases showed remarkable cellular pleomorphism. lmmunohistochemical demonstration of vimentin, desmin, the p-subunit of enolase and the MM isozyme of creatine kinase, together with the absence of immunoreactive cytokeratin, supported the myogenic nature of this rare tumor. A small number of S-100 protein-positive tumor cells were also observed. Followup data for these cases disclosed that the tumors containing considerable area of small alveoli and uniform small tumor cells formed distant metastases at an early stage. Acta Pathol Jpn 40: 199-205, 1990.

patients (1, 2). After relatively slow growth at the primary site, it frequently forms blood-borne metastases in distant organs. However, in certain cases, multiple metastases occur very early in the clinical course. Although histological variations of ASPS have been described (2-4), no correlation between the histological features and biological behavior have been suggested. Histogenetically, Mukai et a/. (5-8) and other investigators (4, 9-12) have presented data suggesting a myogenic nature of ASPS on the basis of immunohistochemical and ultrastructural studies. However, the histogenesis of ASPS is still controversial (13). In the present study we reviewed 12 cases of ASPS and tried to find any clinicopathologic factors that were relevant to the degree of biological malignancy of this tumor. The histogenetic nature of ASPS was also investigated immunohistochemically.

MATERIALS AND METHODS Alveolar soft part sarcoma, Soft tissue tumor, lmmunohistochemistry, Desmin, S-1 O O a

Key words:

INTRODUCTION Alveolar soft part sarcoma (ASPS) is one of the rare

soft tissue tumors, and arises most frequently in the lower extremities or head and neck region of young Received August 4, 1989. Accepted for publication November 4, 1989. 'Pathology Division, National Cancer Center Research Institute, Tokyo. 2Present address : Department of Pathology, Gunma University School of Medicine, Maebashi. Mailing address: Yoshihiro Matsuno, M.D. ([email protected]% Pathology Division, National Cancer Center Research Institute, 5-1-1 Tsukiji, Chuo-ku, Tokyo 104, Japan. Presented in part a t the 77th annual meeting of the Japanese Society of Pathology, May 25 to 27, 1988.


There were 12 cases of ASPS in the pathology file of the National Cancer Center Hospital registered during the period from 1965 to 1988 (Table l), including one consultation case (case 6). Histology slides for case 12 were not available. The histology of 11 cases (primary tumor and/or metastasis) was reviewed in detail and evaluated according to the size of the alveolar structures (large, medium, small) which were predominant in area, and the prominence of cellular pleomorphism (+, promiinconspicuous). Roughly, each nent ; f, focal; -, ''large'' alveolus contained more than 20 tumor cells, each "medium"-sized alveolus five to 20 tumor cells, and each "small" alveolus less than five tumor cells. Alveolar size of the second frequency was additionally described if it occupied approximately more than 30% area of the tumor. Formalin-fixed paraffin-embedded tumor tissue sections from 10 cases were available for special


Alveolar Soft Part Sarcoma (Matsuno et a/.) Table 1. Clinical and Histological Features of Cases



Period ._ between onset and Therapy diagnosis ~

Site & size of tumor tongue approx. 3 cm rt. orbit approx. 2 cm


rt. thigh approx. 6 c m rt. thigh fist size







14 M


22 M


15 F




32 M



It. leg ?


29 M



15 F

It. leg approx. 1 0 c m It. thigh approx. 10 c m


24 M

It. upper arm approx. 8 c m It. chest wall 12 14 M quail egg size ~ _ _ _ _______

rt. thigh apple size It. orbit approx. 3 c m rt. thigh hen egg size


ly 4y ly


Period between onset and metastasis

Alveolar Cellular Crystalloid FO'lOw-Up structure DleomorDhism bodies 2y3m











lung, bone, scalp

Alveolar soft part sarcoma. A clinicopathologic and immunohistochemical study of 12 cases.

Twelve cases of alveolar soft part sarcoma (ASPS) were reviewed. Seven of them arose primarily in the lower extremities, three in the head and neck re...
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