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Alveolar Soft Part Sarcoma: A Case Report with Immunohistochemical Study Koji YAMAGUCHI, 1,2 J u n i c h i SOEJ~Ma, 2'3 S h o g o MAEDA t and Katsutoshi KITAMURA 1 ABSTRACT: We report herein, a rare case of a 28 year old Japanese man with alveolar soft part sarcoma. The patient noticed a rapidly growing mass in the inner aspect of his left thigh and a smooth-surfaced, hard nodule revealing bruit on auscultation was found on physical examination. An angiogram showed dense neovascularity from the left profunda femoris artery. Histopathologically, the tumor was composed of nests of tumor cells with hyperchromatic nuclei and vesicular cytoplasm arranged in an alveolar fashion and a histopathologic diagnosis of alveolar soft part sarcoma was made. Immunohistochemically, the tumor cells had desmin and vimentin immunoreactants in the cytoplasm, suggesting muscle origin. KEY WORDS: alveolar soft part sarcoma, immunohistochemistry INTRODUCTION Alveolar soft part sarcoma (ASPS) is an uncommon soft tissue neoplasm. 1-~ It is a clinically and morphologically distinct soft tissue sarcoma first defined and named by Christopherson et al. in 1952. 4 The incidence of this tumor ranges from 0.4 per cent to 1 per cent of all soft tissue sarcomas 1,5 and a surgeon may scarcely experience one case during his profession. The histogenesis is still uncertain although Smetana and Scott 6 proposed that it was an unusual variant of paraganglioma. Different authors have sug- gested neuroectodermal or neural origin while others favor a myogenic origin. We 1The Department of Surgery, Shinkokura Hospital, Kitakyushu, Japan 2The First Department of Surgery, Faculty of Medi- cine, Kyushu University, Fukuoka, Japan 3The Department of Surgery, Kyushu Welfare Pen- sion Hospital, Kitakyushu, Japan Reprint requests to: Koji Yamaguchi, MD, The First Department of Surgery, Faculty of Medicine, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812, Japan report herein a typical case of ASPS experi- enced in a 28 year old Japanese man and present the results of immunohistochemistry done in an attempt to ascertain the origin of this rare tumor. ~ASE REPORT A 28 year old Japanese man noticed a mass in the inner aspect of his left thigh one month prior to admission, measuring 2 cm in its greates t diameter. The mass rapidly in- creased in size and the patient experienced neither pain nor functional disturbance. On admission to Shinkokura Hospital, the tumor was 10 cm in its greatest diameter and located in the subcutaneous tissue of the inner aspect of the left thigh. There was no redness, local heat, pain or tenderness but the tumor had pulsation and bruit on auscul- tation. An angiogram demonstrated man}, tumor vessels from the left profunda femoris artery in the arterial phase and no tumor staining in the venous phase. The patient underwent extirpation of the tumor which revealed it to be located mainly in the JAPANESE JOURNAL OF SURGERY, VOL. 20, No. 4 pp. 476-480, 1990

Alveolar soft part sarcoma: A case report with immunohistochemical study

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Page 1: Alveolar soft part sarcoma: A case report with immunohistochemical study

Alveolar Soft Part Sarcoma: A Case Report with Immunohistochemical Study

Koji YAMAGUCHI, 1,2 Junichi SOEJ~Ma, 2'3 Shogo MAEDA t and Katsutoshi KITAMURA 1

ABSTRACT: We report herein, a rare case of a 28 year old Japanese man with alveolar soft part sarcoma. The patient noticed a rapidly growing mass in the inner aspect of his left thigh and a smooth-surfaced, hard nodule revealing bruit on auscultation was found on physical examination. An angiogram showed dense neovascularity f rom the left p rofunda femoris artery. Histopathologically, the tumor was composed of nests o f tumor cells with hyperchromatic nuclei and vesicular cytoplasm arranged in an alveolar fashion and a histopathologic diagnosis of alveolar soft part sarcoma was made. Immunohistochemical ly, the tumor cells had desmin and vimentin immunoreactants in the cytoplasm, suggesting muscle origin.

KEY WORDS: alveolar soft part sarcoma, immunohistochemistry

INTRODUCTION

A l v e o l a r soft part sarcoma (ASPS) is an u n c o m m o n soft tissue neoplasm. 1-~ It is a clinically and morphologically distinct soft tissue sarcoma first defined and named by Chris topherson et al. in 1952. 4 The incidence of this tumor ranges f rom 0.4 per cent to 1 per cent of all soft tissue sarcomas 1,5 and a surgeon may scarcely experience one case during his profession. The histogenesis is still uncertain al though Smetana and Scott 6 proposed that it was an unusual variant o f paraganglioma. Different authors have sug- gested neuroectodermal or neural origin while o thers favor a myogenic origin. We

1The Department of Surgery, Shinkokura Hospital, Kitakyushu, Japan

2The First Department of Surgery, Faculty of Medi- cine, Kyushu University, Fukuoka, Japan

3The Department of Surgery, Kyushu Welfare Pen- sion Hospital, Kitakyushu, Japan

Reprint requests to: Koji Yamaguchi, MD, The First Department of Surgery, Faculty of Medicine, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812, Japan

report herein a typical case of ASPS experi- enced in a 28 year old Japanese man and present the results of immunohistochemistry done in an attempt to ascertain the origin of this rare tumor.

~ASE REPORT

A 28 year old J apanese man noticed a mass in the inner aspect of his left thigh one month prior to admission, measuring 2 cm in its greates t diameter. The mass rapidly in- creased in size and the patient experienced nei ther pain nor functional disturbance. On admission to Shinkokura Hospital, the tumor was 10 cm in its greatest diameter and located in the subcutaneous tissue of the inner aspect of the left thigh. There was no redness, local heat, pain or tenderness but the tumor had pulsation and bruit on auscul- tation. An angiogram demonstrated man}, tumor vessels f rom the left p rofunda femoris artery in the arterial phase and no tumor staining in the venous phase. The patient underwent extirpation of the tumor which revealed it to be located mainly in the

JAPANESE JOURNAL OF SURGERY, VOL. 20, No. 4 pp. 476-480, 1990

Page 2: Alveolar soft part sarcoma: A case report with immunohistochemical study

Volume 20 Alveolar soft part sarcoma 477 Number 4

subcutaneous tissue and partially invading the muscle tissue. The margin was well- defined and the tumor easily dissected from the surrounding tissue. The surgical speci- men was a smooth-surfaced and hard tumor, measuring 11 X 9 X 8 cm and weighing 118 g. The cut-surface exhibited yellowish-white solid tissue with necrotic loci (Fig. 1). Micro- scopically, the tumor was composed of nests of tumor cells forming alveolar structures (Fig. 2). The tumor cells were polygonal and had centrally placed hyperchromatic nuclei with prominent nucleoli and vesicular cyto- plasm (Fig. 3). Mitosis was scarcely seen and at the periphery, the tumor invaded the

Fig. 3. Nests of large tumor cells with central loss of cellular cohesion resulting in a pseudoalveolar pattern. The cell nests are separated by thin-walled sinu- soidal vascular spaces lined with flat- tened endothelial cells (HE, X160).

Fig. 1. Cut-surface of the tumor yields yellow-white soft and friable tissue with necrosis.

Fig. 2. Organoid or nest-like arrange- ment of tumor cells (HE, X73).

Fig. 4. Venous invasion of tumor cells at the periphery of the main tumor (HE, X39).

adjacent striated muscle and permeated the vascular spaces (Fig. 4). Periodic acid Schiff (PAS) stain showed a granular PAS positive substance in the cytoplasm and failed to reveal typical crystalline materials. A histo- pathologic diagnosis of alveolar soft part sarcoma was made. An immunohistochemi- cal study was done with the antibodies listed in Table 1, using the avidin-biotin complex peroxidase method (ABC method). Cytokera- tin and epithelial membrane antigen (EMA) were the epithel ial markers , v iment in a

Page 3: Alveolar soft part sarcoma: A case report with immunohistochemical study

478 Yamaguchi et al. JPn)uJy Surg. 1990

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mesenchymal marker, desmin, actin and myoglobin myogenic markers, and neuron specific enolase (NSE) and S-100 protein neurogenic markers. The tumor cells were positive for vimentin and desmin and nega- tive for EMA, carcinoembryonic antigen (CEA), Factor VIII (F VIII) related antigen, NSE and actin. The immunoreactants of vimentin and desmin were diffusely present in the cytoplasm of the tumor cells (Fig. 5).

Ten days after the operation, a chest X-ray film showed bilateral multiple coin lesions in the lung fields and although the patient was given chemotherapy using vincristine, he died of local recurrence and distant meta- stases about 10 m o n t h s after his initial operation.

D I S C U S S I O N

ASPS is a rare soft tissue sarcoma occur- ring in adolescents and young adults) -s,~ There are two main sites of preponderance, being the lower extremities, especially the anterior aspect of the thigh in adults, and the head and neck in children. 7 This tumor usually presents as a slow-growing painless mass without functional impairment which is highly vascular, causing pulsation and/or distinct bruit. Histologically, the tumor is composed of nests of large tumor cells with abundant granular eosinophilic cytoplasm, and the tumor ceils have PAS positive,

Page 4: Alveolar soft part sarcoma: A case report with immunohistochemical study

Volume 20 Alveolar soft part sarcoma 479 Number 4

diastase-resistant crystals in the cytoplasm, although about 20 per cent o f cases fail to reveal such typical crystals. The ultimate prognosis is poor despite the relatively slow growth of the tumor and in most cases, metastasis occurs early in the course of the disease. The principal metastatic sites are the lungs followed by the brain and bone, how- ever, metastasis to the lymph nodes is infre- quent. Evans 8 ment ioned that the survival of patients and likelihood of metastasis in ASPS were related to tumor size, but Suerbach and Brooks 9 denied this relationship. The p r e - sent case was a 28 year old Japanese man with a mass in the left thigh present ing with bruit on auscul ta t ion whose t u m o r had metastasized to the lungs by 10 days after the operation and who died 10 months later. This patient showed the characteristic clini- cal course and histologic features of ASPS.

There is still controversy regarding its histogenesis, involving a variant o f paragan- glioma, a mal ignant counterpart o f benign granular cell tumor, Schwann cell differen- tiation, rhabdomyob las t i c differentiat ion, and a renin-producing tumor. Smetana and Scott 6 were the first to emphasize the close morphological resemblance of ASPS and paragangl ioma t h r o u g h e lec t ron micro- scopy, l~ while Shiphey 1~ first described the rhomboid crystals demonstrated by electron microscopy. Some authors ment ioned that the cytoplasmic granules were similar to those of granular cell tumors in histochemis- try and proposed that ASPS was a mal ignant counterpart o f benign granular cell tumorJ 4 On the o t h e r h a n d , d i f f e r e n t a u t h o r s pointed out the morphological resemblance between the crystals in ASPS and Z-band derived rod-like structures found in rhab- domyoma and nem a l i ne myopathy , sup- porting the myogenic origin? 5 DeSchryver- Kerskemati et al. ~6 reported the cytoplasmic granules possessing the ultrastructural ap- pearance and s ta ining character is t ics o f renin, and proposed the name of angioreni- noma. Mathew 17 reported so-called angulate bodies in the tumor cells, suggesting a neural

crest origin. Mukai et al. ls-~~ immunohisto- chemically demonstrated desmin and actin in the tumor cells and revealed structural similarities to actin filaments using digital image analysis. The immunohistochemical results reported hithertofore are not con- stant because of the different methods em- ployed and sources of antibodies used.m1 Most o f the results reported, however, sug- gest the myogenic origin, ~-~4 as supported by the present case.

ACKNOWLEDGMENTS

We thank Dr. Mune tomo Enjoji, Professor of the Second Depar tment of Pathology, Faculty of Medicine, Kyushu University, Fukuoka, Japan, for histopathologic and immunohistochemical comments and Dr. Fumio Nakayama, Professor o f the First De- pa r tment of Surgery, Kyushu University Faculty of Medicine, Fukuoka, J apan for his continuous encouragement. This study was supported in part by a Grant f rom the Fukuoka Cancer Society, Fukuoka,Japan and a Grant from the Federation of National Public Service and Affiliated Personne l Mutual Aid Associations, Tokyo, Japan .

(Received for publication on Aug. 24, 1989)

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