Trisomy 8 in alveolar soft part sarcoma.
Cytogenetic studies were performed on an alveolar soft part sarcoma of a 6-year-old boy. An extra chromosome 8 was present in 26 of 28 metaphases analyzed.
Arm↗
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[Three cases of alveolar soft part sarcoma and MRI findings].
Three cases of alveolar soft part sarcoma were reported with characteristic MRI findings. On MRI findings, slightly higher signal intensity than muscles on T1 weighted image and remarkably high signal intensity on T2 weighted image were thought to be characteristic of alveolar soft part sarcoma.
Adult↗
Ultrastructural studies of tubules, analogous to skeletal cell T-tubules, in alveolar soft part sarcoma.
Tumor tissues from six patients with alveolar soft part sarcoma (ASPS) were investigated ultrastructurally to determine the presence of smooth tubules associated with plasmalemmal invaginations. Two different types of smooth tubular structures were identified: an aggregated and a dispersed type. The aggregated type (found in one of the six ASPSs) showed marked aggregation of many elongated smooth tubules without ribosomes associated with the plasmalemma. In the dispersed type (observed in the remaining five ASPSs), there was a conspicious appearance of a few smooth tubules scattered throughout the cytoplasm. Smooth tubules were the main component in both types, suggesting that both types appeared to have the same origin, although there were some differences in number, appearance, and distribution of the tubules between the two types. The smooth tubules in both types were classified into three different subtypes on the basis of materials in the tubular lumen. Ultrastructual observation and a plasmalemmal tracer-method, showed smooth tubules in continuity with the plasmalemma in three of the six cases, indicating that the tubules originated from the plasmalemma. Since the tubular structures were found in the tumor tissues of all six patients, they are probably one of the characteristic features of ASPS.
Adolescent↗
Alveolar soft part sarcoma of the head and neck region.
Alveolar soft part sarcoma is a soft tissue malignancy most often found in the extremities of young adults; when these tumors arise in the head and neck area, they usually appear in the orbit or the tongue. Their initial behavior is relatively indolent, but over time a sizable number of these tumors recur locally and metastasize; as such, they are best regarded as fully malignant neoplasms. The derivation of these tumors remains uncertain: while some have suggested that these are tumors of muscle origin and others have postulated a neuroendocrine origin, the evidence accumulated to date is conflicting, and so these neoplasms continue to be regarded as tumors of uncertain origin. Surgical excision is the mainstay of therapy.
Adult↗
Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults.
PURPOSE: Alveolar soft part sarcoma is a rare soft tissue neoplasm that can affect children and adolescents. There are few reported series of these patients in the literature. To define the clinical presentation, treatment, and outcome of young people with this rare sarcoma, we reviewed our clinical experience. METHODS: After institutional review board approval, we examined the records of all patients younger than 25 years old who received treatment at our institution for alveolar soft part sarcoma in the past 30 years. Demographics, tumor sizes, sites and extent of disease, treatments used, progression-free survival, and overall follow-up were evaluated. RESULTS: Each of the 20 patients presented with a mass. Primary disease sites were thigh (n = 8), trunk (n = 6), retroperitoneum (n = 2), and scalp, neck, forearm, and calf (n = 1 each). Metastatic sites included lymph nodes, lung, and brain. Four patients presented to us with incomplete excision of the primary, and 1 had undergone embolization of what was thought to be a vascular malformation. Although wide local excision provided the best chance for a patient to remain free of disease, 14 (70%) of 20 patients exhibited metastases either at presentation or later. Thirty-one metastasectomies were performed. Chemotherapy was used in 11 patients; radiation was used in 8. Median overall follow-up was 36 months; median progression-free follow-up was 12.5 months. Younger patients tended to have Intergroup Rhabdomyosarcoma Study group I disease. Tumors smaller than 5 cm were associated with longer progression-free survival. CONCLUSIONS: Achievement of complete microscopic resection is critical in localized alveolar soft part sarcoma, but incomplete excision and misdiagnosis are often encountered. Despite the occurrence of metastases in 70% of patients, 5-year overall survival was 83%. We found an association between smaller tumor size and longer time to progression. We were not able to demonstrate any benefit from chemotherapy or radiation. Metastasectomies have been performed in multiple long-term survivors.
Adolescent↗
An unusual organoid tumor. Alveolar soft part sarcoma or paraganglioma?
The histogenesis of alveolar soft part sarcoma (ASPS) is a subject of continued debate. Although many recent reports suggest a muscle origin, others advocate a neuroendocrine derivation. A tumor in the chest wall of a 16-year-old woman was diagnosed and treated as ASPS. The light microscopic, electron microscopic, and immunohistochemical findings showed features of both ASPS and paraganglioma. In addition, this lesion was positive for antibody to glucagon, a characteristic of neither ASPS nor paraganglioma, although seen in a few gangliocytic paragangliomas. This case demonstrates the need for continued inquiry into the histogenesis of ASPS.
Adolescent↗
[Alveolar soft part sarcoma--case report].
A case of alveolar soft part sarcoma has been described in a 12-year from old girl. Immunohistochemical reactions on paraffin sections from the tumour were directed towards the presence of desmin , vimentin and neurospecific enolase. Positive reaction to desmin and neurospecific enolase was found in neoplastic cells.
Child↗
[Pulmonary manifestations of alveolar soft part sarcoma; bronchial arterial embolization for hemoptysis].
Alveolar soft part sarcoma is a rare soft tissue tumor arising in extremities. The universal tendency for late widespread metastasis to lung, bone and brain, make the treatment of this tumor a particular challenge. This time, we performed the intra-arterial infusion of MMC and bronchial arterial embolization by spongel for hemoptysis of this metastatic lung tumor. Rosenbaum has reported the characteristics of angiogram of the metastatic brain tumor. In our case, bronchial arteriography showed the manifest hypervascular pattern "rosary form," "pooling" and "B-P shunt." These findings should yield benefits for the diagnosis and treatment of this metastatic lung tumor.
Adult↗
An important role for chromosome 17, band q25, in the histogenesis of alveolar soft part sarcoma.
A cytogenetic study of two cases of alveolar soft part sarcoma showed near-diploid karyotypes with multiple chromosomal rearrangements. An abnormality of the long arm of chromosome 17, involving band q25, is present in both cases and in 2 of 4 cases in the literature. This recurrent structural abnormality probably plays an important role in the histogenesis of this unusual neoplasm and therefore is important for further molecular investigation.
Adult↗
Alveolar soft part sarcoma.
We present a rare case of alveolar soft-tissue sarcoma, with extensive bone invasion. The differential diagnosis with bone metastasis and primary bone tumors is discussed.
Adolescent↗
TFE3 immunoreactivity in alveolar soft part sarcoma of the uterine cervix: case report.
SUMMARY: : Alveolar soft part sarcoma (ASPS) is an uncommon neoplasm that has only rarely been reported in the female genital tract. We report a case of incidentally discovered ASPS in the uterine cervix of a 39-year-old woman that exhibited immunoreactivity for TFE3, a recently described marker of ASPS. Strong nuclear immunoreactivity for TFE3 in tumors characterized by a chromosomal translocation involving the TFE3 gene has been reported in ASPS and a subset of pediatric renal cell carcinomas. This translocation, t(X;17)(p11;q25), fuses the TFE3 transcription factor gene on Xp11 to a novel gene on 17q25, designated ASPL. In our case, the neoplastic nuclei were strongly positive for TFE3. To the best of our knowledge, this is the first report of ASPS of the female genital tract with immunoreactivity for TFE3. Other interesting findings in our case were the small size of the tumor and the absence of clinical symptoms.
Adult↗
Alveolar soft part sarcoma: an unusually long interval between presentation and brain metastasis.
While alveolar soft part sarcoma is an uncommon soft tissue tumor known for late metastases to lung, bone, and brain, and interval of 33 years between primary presentation and development of brain metastasis has not been described. We document a patient with a removal of an alveolar soft part sarcoma from the pectoralis major muscle at the age of 10 years, a lung metastasis at the age of 31 years, and brain and renal masses at the age of 43 years. The patient received surgical resections each time, but never radiotherapy or chemotherapy. He is currently alive and well. Immunohistochemistry, karyotypic analysis, flow cytometry, and gene expression were analyzed on primary tumor and established cell cultures in the hopes of further elucidating the histogenesis of this unusual neoplasm.
Adult↗
Evidence supporting neural crest origin of an alveolar soft part sarcoma: an ultrastructural study.
A case of an alveolar soft part sarcoma was studied electron microscopically. This showed tumor cells separated by external lamina that incorporated desmosome-like structures. Well defined basal lamina separated several cells from underlying endothelial cells by a zone of tissue composed predominantly of fibrous long-spacing collagen. A unique finding was the presence within several tumor cells of well defined myelin sheaths and myelinated axons. Apart from various other intracellular organelles some of the cells contained closely aggregated fine filamentous structures: the so-called "angulate bodies." All these findings are interpreted as evidence for a tumor showing marked Schwann cell differentiation, and therefore, it is suggested that alveolar soft part sarcoma is histogenetically a neoplasm of the neural crest.
Adult↗
Alveolar soft part sarcoma involving the ilium. A case report.
A report of alveolar soft part sarcoma of the hip region with extension to the ilium is presented to highlight an unusual presentation for this sarcoma and to discuss the treatment options for limb salvage for large malignant neoplasma involving the periacetabular region of the pelvis. A review of the literature for reports of alveolar soft part sarcoma extending to bone is presented.
Adult↗
Alveolar soft part sarcoma of the tongue.
We report a case of alveolar soft part sarcoma--a rare malignancy that presented as a swelling at the base of the tongue in a 5-year-old child. Only about one-quarter of the few reported cases arise within the head and neck, the tongue and orbit being the favoured sites.
Child, Preschool↗
[A calvalial metastasis of alveolar soft part sarcoma (author's transl)].
The authors report and discuss a calvalial metastasis of alveolar soft part sarcoma which primarily developed in the left arm. A 13-year-old girl noted a painless tumor in the left upper arm at the age of 7 years. The tumor was excised and histologically alveolar soft part sarcoma. 5 years later, a rapid growing tumor was noted in the parietal area. The craniograms showed the left parietal bone defect. The chest X-ray showed a few large oval metastatic shadows. The left common carotid angiograms demonostrated high vascularities with irregular vessels in the arterial phase and a homogenous blush or stain in the venous phase. It was thought likely to meningioma. In selective external carotid angiograms, early arterial phase showed a few of vascular streaks in beads, fine tortuous vessels and early conspicuous filling of enlarged vein. Paradoxically, despite evidence of rapid shunting of blood from the lesion. "Wash out" of contrast media from the lesion was slow. Above mentioned angiographic appearance was consistent with that of cases reported by Rosenbaum, A.E., and was considered characteristic features of alveolar soft part sarcoma, meningiosarcoma and malignant melanoma. It must be emphasized that the slective angiography is important to differentiate angiographically alveolar soft part sarcoma, meningiosarcoma, malignant melanoma from meningioma or angioma. Radiotherapy is not effective to the pulmonary metastatic lesion. Selective angiography should be emphazied to be essential for differentiation of alveolar soft part sarcoma from angioma or meningioma.
Adolescent↗
Alveolar soft part sarcoma: a report of two cases with some histochemical and ultrastructural observations.
In a survey of all malignant soft tissue tumors in the extremities and limb girdles in Finland between 1960 and 1969, only one alveolar soft part sarcoma was found among 246 tumors (0.4%). Another alveolar soft part sarcoma, diagnosed in 1976, was more thoroughly studied. There was evidence that the characteristic crystals of alveolar soft part sarcoma are formed from the dense granules. Both were PASM-positive at ultrastructural level. No monoamines were detected in the cells by formaldehyde-induced fluorescence. This is a further fact to nullify the theory of the paraganglionic origin of alveolar soft part sarcoma, but the question of the histogenesis of the tumor still remains open.
Adult↗
Soft tissue sarcomas. Case 2. Orbital alveolar soft part sarcoma in a child.
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Child, Preschool↗