Soft tissue sarcomas. Case 2. Orbital alveolar soft part sarcoma in a child.
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Child, Preschool↗
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Results for “Sarcoma, Alveolar Soft Part”
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Alveolar soft part sarcoma of the tongue.
In this report a 13-year-old girl with characteristic clinical and pathological features of alveolar soft part sarcoma of the tongue is presented. Alveolar soft part sarcoma is a rare malignant tumor of unknown origin. Forty eight cases of alveolar soft part sarcoma have so far been reported in the head and neck region. Median survival reported for all sites of the body is 79 months following various modes of therapy. In this case, the treatment was surgical excision of the mass combined with radiotherapy and chemotherapy. The patient has been asymptomatic for the past 12 months.
Adolescent↗
Retroperitoneal sarcoma with features suggestive of alveolar soft part sarcoma.
A 72-year-old man presented with a 15 x 12 x 5 cm cystic and necrotic tumor involving the superior region of the stomach and the retroperitoneum down to the transverse colon and extending laterally from the left lobe of the liver to the spleen without involving either organ. Light microscopy showed the tumor cells to be arranged in an organoid pattern. Immunocytochemistry showed a strong reaction for desmin and vimentin. Electron microscopy demonstrated features suggestive of an alveolar soft part sarcoma.
Aged↗
Alveolar soft part sarcoma, granular cell tumor, and paraganglioma. An immunohistochemical comparative study.
Five cases of alveolar soft part sarcoma, 5 cases of granular cell tumor, and 6 cases of paraganglioma were investigated immunohistochemically to examine the expression of tissue-specific intermediate filaments (cytokeratin, vimentin, desmin, and glial fibrillary acidic protein (GFAP], actin, myoglobin, and nervous tissue markers (S-100 protein, neuron-specific enolase, and Leu-7). In alveolar soft part sarcomas, some of the tumor cells were positive for desmin, but negative for nervous tissue markers. The tumor cells of granular cell tumors were stained with anti-S-100 protein antibody, but not with anti-neuron-specific enolase antibody. In contrast, the tumor cells of paragangliomas were positive for neuron-specific enolase, but not for S-100 protein except for stellate cells surrounding the tumor cell nests. This immunohistochemical approach was valuable for the differential diagnosis of these three tumors. Furthermore, the complete absence of cytokeratin in all of the tumor cells may be helpful in distinguishing these three tumors from metastatic carcinoma in soft tissue. The histogenesis of alveolar soft part sarcoma is a matter of controversy. The result that besides desmin actin was also demonstrated in some of the tumor cells may support the myogenic origin of this tumor.
Adult↗
Alveolar soft part sarcoma metastatic to the breast.
Metastasis to the breast is uncommon, with an incidence of 0.5-3%. Alveolar soft part sarcoma is rare, accounting for < 1% of malignant soft tissue tumors, which are themselves unusual. Excluding contralateral breast and hematologic malignant disease, the primary lesion in most cases of metastasis to the breast is melanoma, small cell carcinoma of the lung, or ovarian carcinoma, although rhabdomyosarcoma is the most common primary tumor in children. We describe a 26-year-old woman with no history of malignant disease who presented with two masses in the right breast that clinical evaluations and ultrasonography indicated were fibroadenomas. Pathological studies after excisional biopsy, however, indicated alveolar soft part sarcoma. Subsequent computed tomography showed the primary tumor in the anterior left thigh and multiple bilateral lung metastases. Because of the presence of distant metastases, the patient was treated with chemotherapy.
Adult↗
Alveolar soft part sarcoma in children and adolescents: clinical features and outcome of 11 patients.
The clinical features and response to therapy of pediatric alveolar soft part sarcoma, a rare soft tissue sarcoma of uncertain histogenesis, have not been previously described in detail in the literature. We retrospectively reviewed the clinical characteristics of all patients with alveolar soft part sarcoma who were seen at our institution over a 32-year period. We found 11 patients with the diagnosis of alveolar soft part sarcoma. Their ages ranged from 2.8-16 years (median 9.8). Staging was determined using the Intergroup Rhabdomyosarcoma Study clinical grouping system and the UICC TNM system. Accordingly, there were six patients with grossly resected tumors (clinical groups I and II) and five with unresected or metastatic disease (clinical groups III and IV). Children with resected disease were more likely to have smaller noninvasive tumors. The main feature predictive of survival was tumor resectability, since chemotherapy in various combinations failed to produce significant tumor responses. Nine patients are disease-free with a median follow-up of 11.9 years. Surgical resection remains the mainstay of therapy for pediatric alveolar soft part sarcoma. Since active chemotherapy agents have not been identified, patients with unresected or metastatic disease may benefit from experimental agents. The survival rate of this cohort is superior to that seen in adults.
Adolescent↗
Alveolar soft part sarcoma of the nasal cavity: report of a case and a review of the literature.
A case of alveolar soft part sarcoma of the nasal cavity is presented. Alveolar soft part sarcoma is a rare malignant neoplasm, which often affects females (ratio 2 to 1) in their second decade. It occurs most frequently in the extremities, with an unusual predilection to involve the right side of the body. Excluding the orbit, only a few cases have been reported in the head and neck area. We present the first reported case of alveolar soft part sarcoma limited to the nasal chamber.
Adolescent↗
Alveolar soft part sarcoma: report of a case occurring in the sinonasal region.
A primary alveolar soft part sarcoma arising in the nasal-paranasal sinuses region is quite rare. In this report, a 14-year-old boy with typical features of alveolar soft part sarcoma of the nasal and paranasal sinuses is presented. Treatment was surgical excision of the mass combined with radiotherapy. There has been no recurrence or metastasis as of 2 years after treatment.
Adolescent↗
Alveolar soft part sarcoma of the tongue. Report of a case.
The clinicopathologic, immunohistological, and ultrastructural features of an alveolar soft part sarcoma of the tongue occurring in a 2-year-old girl are described. A primary alveolar soft part sarcoma arising in the dorsum part of the tongue is quite rare. There has been no recurrence or metastasis as of 7 years postoperatively.
Biopsy↗
Infratemporal alveolar soft part sarcoma: CT, MRI and angiographic findings.
The imaging features of a rare alveolar soft part sarcoma found in a 44-year-old female are presented. Although the tumor showed hypervascularity by angiography, CT and MRI suggested slow growth. Despite this relatively benign appearance, alveolar soft part sarcoma is one of the most malignant sarcomas.
Adult↗
Alveolar soft part sarcoma of the vagina. A case report.
We present a case of alveolar soft part sarcoma of the vagina in an 18-year-old woman. The presenting symptoms were menometrorrhagia and a painless vaginal mass. The tumor was in the vaginal stroma with intact mucosa. Periodic acid-Schiff stain showed positive diastase-resistant crystals in some of the tumor cells. Electron microscopy demonstrated characteristic intracytoplasmic crystals. Formaldehyde-induced fluorescence stain showed no catecholamines, and Grimelius stain showed no argyrophilic granules. Black pigment was found within some tumor cells; it proved to be melanin pigment by Masson-Fontana stain. The tumor mass recurred four months after local excision. Following the second surgical removal, the patient received adjuvant radiotherapy to the whole pelvis, including the vagina. At this writing she was currently alive, without evidence of recurrence, five years after the primary diagnosis.
Adolescent↗
Alveolar soft part sarcoma. A clinicopathologic and immunohistochemical study.
The histogenesis of alveolar soft part sarcoma (ASPS) has been investigated since its description. Twenty ASPS cases were analyzed for immunohistochemical content, with emphasis directed toward the paraganglial, Schwann cell, and muscle theories of histogenesis. In addition, the cases were examined for possible prognostic clinical features. The clinical characteristics of the patients were similar to those reported previously concerning average age (23 years); male:female ratio (1:1); and predominant primary site (lower extremity, nine cases). Despite a local recurrence rate of 20% and a metastatic rate of 68% (including four at presentation), the natural history was often indolent and relapse commonly occurred very late. The average follow-up period was 10.1 years. While the overall 5-year survival was 67%, only seven of 18 patients were alive without disease at last follow-up (1.7-32 years), and one patient died of tumor after a 28-year disease-free interval. Neither tumor size nor site appeared to affect prognosis. The tumors were analyzed immunohistochemically for neurofilament, S-100 protein, met-enkephalin, leu-enkephalin, acetylcholinesterase, alpha 1-antichymotrypsin, Factor VIII-related antigen, serotonin, lysozyme, neuron-specific enolase, myoglobin, cytokeratins, desmin, and vimentin. Except for weak vimentin immunoreactivity, no other antigenic expression was detected despite multiple repeated experiments with several antibodies. S-100 protein which is present in virtually all granular cell tumors was absent in the cases of ASPS. The lack of detectable expression of neurofilament, met-enkephalin and leu-enkephalin, and neuron-specific enolase is interpreted as evidence against the paraganglial theory of histogenesis. Similarly, the repeated absence of the muscle proteins, desmin and myoglobin, in contrast to a previous report, is interpreted as evidence against a myogenic origin.
Adolescent↗
Alveolar soft part sarcoma: immunological evidence of rhabdomyoblastic differentiation.
Two cases of alveolar soft part sarcoma have been studied immunocytochemically using antisera against epithelial membrane antigen, lysozyme, keratins, S-100 protein, desmin, vimentin, fetal myosin, slow myosin, alpha-skeletal muscle actin, alpha-smooth muscle actin and myoglobin. The neoplastic cells were negative with all antisera employed with the exception of the alpha-skeletal muscle actin antiserum which stained the cytoplasm of numerous neoplastic elements, including the crystalloid rods, typical cytoplasmic inclusions of these tumours. It is suggested that the presence of this protein indicates rhabdomyoblastic differentiation of these tumours.
Actins↗
The role of MR imaging in the diagnosis of alveolar soft part sarcoma: a report of 10 cases.
OBJECTIVE: The objective of this study was to analyze the characteristics of alveolar soft part sarcoma using magnetic resonance imaging (MRI). DESIGN: MRI studies of pathologically proven alveolar soft part sarcomas (ASPS) in ten patients were reviewed and compared with computed tomographic (CT) studies and angiograms. PATIENTS: Ten patients presented with a soft tissue mass of the extremities, neck, axilla, or buttocks. MR images were obtained in all patients prior to surgical intervention, chemotherapy, or irradiation. RESULTS AND CONCLUSION: Although most soft tissue sarcomas are isointense relative to muscle or MRI T1-weighted images (T1WI), nine of the ten alveolar ASPS in the present study demonstrated high signal intensity on both T2 and T1WI. Flow voids were observed both at the core and at the margins of the tumors studied. Recognition of these characteristic MRI findings may lead to the early diagnosis of ASPS, especially when the clinical presentation is that of a slow-growing soft tissue mass in a young adult patient.
Adult↗
Alveolar soft part sarcoma following radiotherapy for a spinal hemangioma. A case report.
A case of alveolar soft part sarcoma arising some 20 years later in a site previously irradiated as a treatment for a spinal cord hemangioma is described. This is the first known case of radiation-associated alveolar soft part sarcoma, and it fulfills the criteria for a tumor to be radiation-induced. The coincidental finding of "viral-like" particles within some of the tumor cells was noted.
Head and Neck Neoplasms↗
Crystals of alveolar soft part sarcoma in a fine needle aspiration biopsy cytology smear. A case report.
BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor. It has characteristic histomorphology, with typical ultrastructural features demonstrating unique crystalloids. It occurs predominantly in adolescents and young adults, in whom the most common location is within the fascial planes of skeletal muscle of the lower extremity. CASE: We present fine needle aspiration biopsy (FNAB) findings along with histopathologic features and ultrastructural appearance of a large gluteal mass in a 29-year-old female. FNAB cytology smears showed single and small groups of polyhedral malignant cells with granular cytoplasm, anisokaryosis and prominent nucleoli. The delicate cytoplasm had a tendency to rupture, with the presence of many bare nuclei. The characteristic crystals were observed in Papanicolaou-stained smears within the cytoplasm and in the background near the tumor cells. This consolidated the radiologic suspicion of ASPS and facilitated the application of relevant ancillary tests. Biopsy of the mass showed the characteristic histologic pattern. Electron microscopy confirmed the diagnosis with demonstration of membrane-bound, rhomboid crystalloids with a latticelike ultrastructure. CONCLUSION: Detection of characteristic crystalloids in Papanicolaou-stained FNAB smears facilitated a proper evaluation and correct diagnosis of ASPS.
Adult↗
[Paraganglial nature of tumors of organoid-endocrine structure of the skeletal muscles; with reference to the claim of new oncological entity as alveolar soft part sarcoma].
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Humans↗