Archives of Medicine and Health Sciences

CASE REPORT
Year
: 2022  |  Volume : 10  |  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

Correspondence Address:
Dr. Sarita Asotra
Department of Pathology, Indira Gandhi Medical College and Hospital, New Shimla - 171 001, Himachal Pradesh
India

Abstract

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.



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


How to cite this URL:
Asotra S, Sharma A, Vijayamohanan L. Alveolar soft part sarcoma: A rare diagnosis. Arch Med Health Sci [serial online] 2022 [cited 2022 Dec 5 ];10:99-101
Available from: https://www.amhsjournal.org/text.asp?2022/10/1/99/347959


Full Text

 Introduction



Alveolar soft part sarcoma (ASPS) is a rare tumor accounting for approximately 0.5%–1% of all soft tissue sarcomas.[1] Taking into consideration the rarity of the disease, complete and thorough knowledge of its clinical behavior, pathology, and optimal treatment is still obscure.[2] 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.[3] 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}{Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}

 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.[4] 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.[5] 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.[6] 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.[7] 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.[8]

ASPS tumor cells exhibit characteristic PAS-positive, diastase-resistant, intracytoplasmic rhomboid crystals and act as a diagnostic marker for ASPS.[9] 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.[10]

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.[11]

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

Nil.

Conflicts of interest

There are no conflicts of interest.

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