|Year : 2022 | Volume
| Issue : 1 | Page : 99-101
Alveolar soft part sarcoma: A rare diagnosis
Sarita Asotra, Ankita Sharma, Lexmi Vijayamohanan
Department of Pathology, Indira Gandhi Medical College and Hospital, Shimla, Himachal Pradesh, India
|Date of Submission||19-Oct-2021|
|Date of Decision||12-Nov-2021|
|Date of Acceptance||17-Nov-2021|
|Date of Web Publication||23-Jun-2022|
Dr. Sarita Asotra
Department of Pathology, Indira Gandhi Medical College and Hospital, New Shimla - 171 001, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Alveolar soft part sarcoma (ASPS) is an extremely rare sarcoma. It is a malignant soft tissue tumor that arises in the muscles, commonly seen in the age group between 15 and 35 years of age with slight increased predilection in young women. Clinically, it presents as a painless, slow-growing lesion principally around the lower limb or limb–girdle with a propensity for recurrence and metastasis. Clinically, it can be confused with a hemangioma or arteriovenous malformations. We report a case of ASPS in a 36-year-old female with pain and swelling over the anterior aspect of the right thigh without any features of metastasis, who has been treated with excision of the symptomatic lesion followed by chemotherapy.
Keywords: Alveolar soft part sarcoma, chemotherapy, excision
|How to cite this article:|
Asotra S, Sharma A, Vijayamohanan L. Alveolar soft part sarcoma: A rare diagnosis. Arch Med Health Sci 2022;10:99-101
| Introduction|| |
Alveolar soft part sarcoma (ASPS) is a rare tumor accounting for approximately 0.5%–1% of all soft tissue sarcomas. Taking into consideration the rarity of the disease, complete and thorough knowledge of its clinical behavior, pathology, and optimal treatment is still obscure. Majority of previous reports have suggested that complete surgical excision of the primary tumor in its early stages is the mainstay of treatment, as it is resistant to conventional cytotoxic chemotherapy. However, in advanced cases, with multiple metastatic involvement and recurrent cases, only palliative treatment is preferred due to its bad prognosis. Most patients succumb eventually to metastases, especially to the lung; older age at presentation and tumor size >10 cm seem to be indicators of poor prognosis, whereas cases in children appear generally to have a better outcome.
| Case Report|| |
A 36-year-old woman reported to the outpatient Department of Indira Gandhi Medical College, Shimla, with pain and swelling over the anterior aspect of the right thigh. The patient gave a history of swelling over the anterior aspect of the thigh, which gradually grew into a mass of size 14 cm × 8 cm × 7 cm. Medical history was unremarkable. Magnetic resonance imaging revealed a well-defined heterogeneous elliptical mass measuring 8.1 cm × 8.7 cm × 14.7 cm, predominantly arising from the left rectus femoris muscle [Figure 1]a and [Figure 1]b. The mass was excised and sent to the histopathology department; we received an encapsulated soft tissue mass of 15 cm × 13 cm × 8 cm with an area of necrosis [Figure 2]. Histopathology revealed sheets and nests of large polygonal tumor cells with uniform vesicular nuclei, prominent nucleoli, and abundant granular eosinophilic cytoplasm separated by thin vascular channels. At places showing discohesive cells with pseudoalveolar pattern&few atypical cells with occasional mitotic activity [Figure 3]a, [Figure 3]b, [Figure 3]c. Periodic acid–Schiff (PAS) Distastase resistant (DR) came out to be positive [Figure 4]. Immunohistochemistry: Cells showed positive nuclear staining for TFE3 [Figure 5] and cells were nonimmunoreactive to S100, MyoD1 [Figure 6], desmin, synaptophysin, and myogenin. A histopathological diagnosis of ASPS with vascular invasion was made. Following surgery, the patient was referred to the radiotherapy department and was put on a chemotherapy regimen. There is a mild reduction in symptoms without any features of metastasis.
|Figure 1: (a and b) Magnetic resonance imaging revealed a well-defined heterogeneous elliptical mass arising from the left rectus femoris muscle|
Click here to view
|Figure 2: Gross photograph showing an encapsulated soft tissue mass with an area of necrosis|
Click here to view
|Figure 3: (a) Microscopic examination shows sheets and nests of large polygonal tumor cells with vesicular nuclei, prominent nucleoli, and abundant granular eosinophilic cytoplasm separated by thin vascular channels. (b) Microscopic examination shows occasional cells showing vacuolations. (c) Microscopic examination shows dyscohesive cells with pseudoalveolar pattern|
Click here to view
|Figure 4: Periodic acid–Schiff (DR) came out to be positive. Immunohistochemistry|
Click here to view
|Figure 5: Immunohistochemistry showing cells showing positive nuclear staining for TFE3|
Click here to view
|Figure 6: (a) On immunohistochemistry cells – nonimmunoreactive to S100. (b) On immunohistochemistry cells – nonimmunoreactive to MyoD1|
Click here to view
| Discussion|| |
ASPS is an uncommon tumor and accounts for 0.5%–1% of all soft tissue sarcomas.
It was first described as a discrete clinical entity by Smetana and Scott in 1951, and in view of its unknown cellular origin, given the descriptive title of ASPS in the following year. ASPS is seen mostly in children and adolescents. However, its usual age range of presentation is from 15 to 35 years. Extremities are commonly involved in adults, particularly lower limbs. Most studies have found a female preponderance in adult patients. Lieberman et al. reviewed the clinical and histopathological features of 102 cases of ASPS. In their study, the median age at diagnosis was 22 years (range: 2–71 years), and the female-to-male ratio was 1.5:1, with a higher female-to-male ratio in childhood and adolescence. In accordance with the above facts, the patient who reported to our department was a 36-year-old woman with a swelling in the left lower limb. ASPS arises in association with skeletal muscles or musculofascial planes, a fact that explains the strong predilection of this tumor for the thighs, buttocks, and abdominal or chest walls. In children, a substantial percentage of cases occur in the head and neck, often in the orbit or tongue. It tends to grow slowly and insidiously, often with a long clinical history and a large mass at presentation, similar to our patient, who had a large swelling in the left leg. Despite the slow-growing nature of the primary tumor, metastases are common. Metastases are found most often in the lungs, followed in frequency by bone and brain. They are detected in about 20%–25% of patients at diagnosis. The prognosis for children with ASPS may be considerably better; lingual and orbital tumors also have very high survival rates, possibly reflecting a combination of small size at the time of diagnosis and younger patient age.
ASPS tumor cells exhibit characteristic PAS-positive, diastase-resistant, intracytoplasmic rhomboid crystals and act as a diagnostic marker for ASPS. In addition, tumor cells exhibit characteristic round, regular, eccentrically placed nuclei with vesicular chromatin and prominent nucleolus. These histopathological findings were present in our patient, confirming the diagnosis of ASPS. No histopathological features are predictive of prognosis in patients with ASPS.
Metastasis is common for lungs (42%–65%), brain, and bone. Lymph node involvement is unusual. Patients with localized form of the disease are in the mean age of 22 years and have a favorable prognosis with prolonged survival.
Accurate diagnosis and treatment of this unusual tumor requires clinical suspicion and clinicopathological correlation with appropriate radiological studies. Either alone or in combination, surgery and radiotherapy are the favored modes of therapy in such situations.
| Conclusion|| |
ASPS is a rare tumor, thus a high index of suspicion and clinicopathological correlation with radiological studies are required to make a definitive diagnosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
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.
Jaber OI, Kirby PA. Alveolar soft part sarcoma. Arch Pathol Lab Med 2015;139:1459-62.
Sarkar P, Mukherjee S, Saha ML, Biswas RS. Alveolar soft part sarcoma: A rare diagnosis. Indian J Dermatol 2013;58:244.
] [Full text]
Anbarasi K, Sathasivasubramanian S, Kuruvilla S, Susruthan. Alveolar soft-part sarcoma of tongue. Indian J Pathol Microbiol 2011;54:581-3.
] [Full text]
Lieberman PH, Brennan MF, Kimmel M, Erlandson RA, Garin-Chesa P, Flehinger BY. Alveolar soft-part sarcoma. A clinico-pathologic study of half a century. Cancer 1989;63:1-13.
Das KK, Singh RK, Jaiswal S, Agrawal V, Jaiswal AK, Behari S. Alveolar soft part sarcoma of the frontal calvarium and adjacent frontal lobe. J Pediatr Neurosci 2012;7:36-9. [Full text]
Argyris PP, Reed RC, Manivel JC, Lopez-Terrada D, Jakacky J, Cayci Z, et al.
Oral alveolar soft part sarcoma in childhood and adolescence: Report of two cases and review of literature. Head Neck Pathol 2013;7:40-9.
Fanburg-Smith JC, Miettinen M, Folpe AL, Weiss SW, Childers EL. Lingual alveolar soft part sarcoma; 14 cases: Novel clinical and morphological observations. Histopathology 2004;45:526-37.
Zamani F, Jabbari M, Alimohamadi SM, Shakeri R, Rostami Z, Abedi B, et al.
Primary alveolar soft part sarcoma of chest wall: A case report and review of the literature. MedGenMed 2006;8:2.
Sidi V, Fragandrea I, Hatzipantelis E, Kyriakopoulos C, Papanikolaou A, Bandouraki M, et al.
Alveolar soft-part sarcoma of the extremity: A case report. Hippokratia 2008;12:251-3.
Folpe AL, Deyrup AT. Alveolar soft-part sarcoma: A review and update. J Clin Pathol 2006;59:1127-32.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]