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Pancreatic metastasis of alveolar soft-part sarcoma: a case report and review of the literature.

Alveolar soft-part sarcoma is a rare tumour. Patients commonly present with distant metastases both at the time of diagnosis and late in the course of disease. We present a case of pancreatic metastasis, occurring more than six years after diagnosis. Treatment consisted in subtotal pancreatoduodenectomy with pylorus resection. Both specific patterns of relapse and treatment opportunities of this uncommon feature are discussed.

Adult↗

[Alveolar soft-part sarcomas in the skeleton (author's transl)].

The rare alveolar soft-part sarcomas, tumours with well defined histological pattern but uncertain histogenesis, are up to now described only in soft tissues, especially in those of the extremities of adolescents and young adults. We can report two cases of alveolar soft-part sarcomas in the sceleton, both in the right femur of 22 years old women. The delimitation of these tumours against others with similar histological pattern may be very difficult. The occurrence of these tumours in the sceleton does not require to substitute the term alveolar soft-part sarcoma by an other one.

Adult↗

Colic metastases of alveolar soft-part sarcoma: a case report and review of the literature.

Alveolar soft-part sarcoma is a rare soft tissue malignant tumor that commonly arises in skeletal muscles or musculofascial planes in children and young adults. Metastases of lung, brain, and bone are often present at the time of diagnosis or appear late in the course of disease. We present the first case, to our knowledge, of colic metastases in a 43-year-old woman who had a leg primary tumor more than 15 years ago and multiple lung and brain metastases. The treatment of these two caecal metastases, revealed by an anemia, consisted in laparoscopic right colectomy.

Adult↗

Primary alveolar soft-part sarcoma of stomach.

A case of primary gastric alveolar soft-part sarcoma is presented. The tumor was found in the gastric remnant of a 67-year-old male who had undergone partial gastrectomy due to hemorrhagic gastric ulcer 13 years before. It was located mostly in the submucosa arising from the muscularis propria. The large eosinophilic cells showed the characteristic alveolar compartmentalization and contained intracytoplasmic periodic acid-Schiff-positive granules and typical crystals. Numerous electron-opaque secretory granules in the tumor cell cytoplasm, in addition to crystals of 9 nm periodicity, were confirmed at the ultrastructural levels. Immunostaining failed to detect muscle-related antigens. In contrast, methionine-enkephalin and neuropeptide Y appeared positive in the tumor cells. Interstitial spindle cells showed an occasional positivity to S-100. This is the first case of such a tumor occurring in the gastrointestinal tract, and the findings suggest that gastric alveolar soft-part sarcomas may have a different origin from those arising in the skeleton.

Aged↗

Cytologic presentation of alveolar soft-part sarcoma of the vagina. A case report.

Alveolar soft-part sarcoma primary in the vagina is an exceptionally rare neoplasm. The cytologic features in a case of this rare sarcoma are described, with a discussion of the differential diagnosis. Structures suggestive of the characteristic PAS-positive, diastase-resistant cytoplasmic crystals were seen in the cytologic specimen; the crystals were well-demonstrated in the histologic material and at the electron microscopic level.

Adult↗

Radiation therapy for alveolar soft-part sarcoma.

A clinical experience with radiotherapy in 18 patients with alveolar soft-part sarcoma is presented. Adjuvant radiotherapy was associated with prolonged local control in six of six patients without metastatic disease at diagnosis; later one patient relapsed systematically. Meaningful palliation was achieved in all patients with extra-skeletal (and possibly skeletal) metastatic disease. Radiation therapy may be beneficial for patients with alveolar soft-part sarcoma by enhancing local control achieved with limited surgery, by retarding progression of metastatic deposits, and by providing meaningful palliation.

Adult↗

Surgery for multiple lung metastases from alveolar soft-part sarcoma.

Between 1985 and 1993, six patients were surgically treated for alveolar soft-part sarcoma (ASPS) arising from the thighs or buttocks, four of whom underwent aggressive excision of multiple metastases using a neodymium:yttrium-aluminum garnet (Nd:YAG) laser. In total, 333 tumors were removed from these four patients during eight pulmonary operations. In patients 1, 3, and 4, uncontrollable extrapulmonary involvement and/or local recurrence at the primary site were noted during their treatment course, and they died of tumor progression 40, 68, and 46 months after excision of the primary lesion, respectively. In patient 1, a 37-year-old woman, prolonged survival with adequate lung function was achieved after the excision of metastases, including one bulky metastatic tumor located adjacent to the mediastinum, which might have led to a lethal complication. Patient 3, a 25-year-old woman who underwent aggressive metastasectomies for both pulmonary and extrapulmonary metastases combined with intermittent chemotherapy, died of widespread metastases to multiple organs. On the other hand, patient 2, a 23-year-old woman who underwent excision of 130 pulmonary and three brain metastases during four thoracotomies and two craniotomies, is still alive without any symptoms 98 months after excision of the primary lesion. These data suggest that repeated excisions of lung metastases from ASPS may influence long-term survival or maintenance of good performance status in patients in whom extrapulmonary metastasis and recurrence are either absent or controlled.

Adult↗

Alveolar soft-part sarcoma: a review of the pathology and histogenesis.

The descriptive designation, alveolar soft-part sarcoma, continues to be used for this uncommon soft-tissue malignancy because an acceptable hypothesis for its histogenesis has not been advanced, despite studies with electron microscopy and immunohistochemistry. These techniques have, nevertheless, provided significant information that is useful in the differential diagnosis of the tumor and pertinent in speculation concerning its nature. The most intriguing ultrastructural feature is the secretory process that culminates in the formation of the distinctive cytoplasmic crystals. Myogenic differentiation has been favored in a number of recent reports on the basis of immunohistochemical findings and the presence of the crystals does not rule out the possibility, but accounts of immunoreactivity for the myogenic regulatory protein MyoD1 have not been confirmed in subsequent studies or in the authors' own staining of six cases.

Adolescent↗