Successful use of three-dimensional conformal radiotherapy as adjuvant treatment for alveolar soft part sarcoma. A case report / Uso exitoso de la radioterapia conformada tridimensional como tratamiento adyuvante del sarcoma alveolar de partes blandas. Reporte de un caso

Abstract Introduction: Alveolar soft part sarcoma is a very rare and aggressive type of sarcoma. Although its histology and genetic characteristics have been identified, the benefits of adjuvant radiotherapy for its treatment are still being studied. Case presentation: In November 2007, a 21-year-old woman presented with a primary tumor in the right thigh, with histological and immunohistochemical confirmation of an alveolar soft part sarcoma, which was totally resected in December 2007. Also, the large size of the mass suggested an unfavorable evolution. Two years after the first surgery, two metastatic tumors were detected in the right lung, which were completely resected separately. Two years later, the patient had two independent relapse events, five months apart: a mass in the right tight, and a metastatic tumor in the adrenal gland, together with a relapse in the tight. All tumors were successfully resected. In June 2014, after the last local relapse, adjuvant radiotherapy was started because of the risk of thigh amputation. At the end of treatment, the patient's general condition was good. Currently, at age 34, the patient is monitored through periodic evaluations, showing disease regression and stabilization. Conclusions: Currently, it is known that radiation not only produces cytotoxic effects on the target region but also induces an immune system-mediated systemic response with potential antimetastatic properties. Emerging radiobiological paradigms should be considered, particularly since they could explain some encouraging and unexpected results, such as those described in this case.

Resumen Introducción. El sarcoma alveolar de partes blandas es un raro y agresivo tipo de sarcoma. Aunque se han identificado sus características histológicas y genéticas, todavía se están estudiando los beneficios de la radioterapia adyuvante en su tratamiento. Presentación del caso. En noviembre de 2007, una mujer de 21 años se presentó con un tumor primario en el muslo derecho, con confirmación histológica e inmunohistoquímica de sarcoma alveolar de partes blandas y que fue completamente removido en diciembre de 2007. La masa mostró un gran tamaño, sugiriendo una evolución desfavorable. Dos años después de la primera cirugía, se detectaron dos tumores metastásicos en el pulmón derecho, que también fueron removidos, de forma separada. Dos años después, la paciente tuvo dos relapsos, separados por cinco meses: una masa en el muslo derecho, y un tumor metastásico en la glándula suprarrenal junto con una recaída en el muslo. Todos los tumores fueron extirpados con éxito. En junio de 2014, después de la última recaída local, el muslo estaba en riesgo de ser amputado, por lo que se decidió iniciar radioterapia adyuvante. Al final del tratamiento, la condición general de la paciente fue buena. Actualmente, ya con 34 años, es evaluada periódicamente, mostrando regresión y estabilización de la enfermedad. Conclusiones. Actualmente, se sabe que la radiación no solo produce efectos citotóxicos en la región objetivo, sino que también induce una respuesta sistémica mediada por el sistema inmune, con propiedades potencialmente antimetastásicas. En este sentido, se sugiere considerar los paradigmas radiobiológicos emergentes, ya que estos podrían explicar algunos resultados alentadores e inesperados como los descritos en este caso.

Alveolar soft part sarcoma of extremity

Alveolar soft-part sarcoma (ASPS) is an extremely rare connective tissue tumor, predominantly seen in adolescents and young adults, with a female preponderance. Alveolar soft-part sarcoma (ASPS) is a slow growing tumor, but with high likelihood of metastasis, leading to high mortality. A classical histopathological feature of an alveolar pattern from the biopsy of the lesion favors the diagnosis. We report a case of 14 years old male patient who presented with a history of single painless swelling over thigh for which surgical excision was done. Histopathology was suggestive of Alveolar soft-part sarcoma (ASPS). There was no evidence of distant metastases. He was treated with external beam radiotherapy in view of vascular invasion.

Progress of primary intracranial alveolar soft part sarcoma / 肿瘤研究与临床

Alveolar soft part sarcoma (ASPS) is a kind of extremely rare malignant soft tissue sarcoma, which was first discovered and defined by American scholars in 1952. Its main manifestation is painless and slow growth mass, and distant metastasis often occurs in lung, bone, brain, liver with poor prognosis. In recent years, with the accumulation of the number of cases and the development of related technology, the diagnosis and treatment of ASPS have been improved greatly. However, the primary intracranial ASPS is still rare, this paper reviews the progress of primary intracranial ASPS, in order to provide help for its clinical diagnosis and treatment.

Secondary Malignancies Developing After Acute Lymphoblastic Leukemia And Its TreatmentWith Constitutional Mismatch Repair Deficiency Syndrome In Siblings.

Background & Objectives: Acute Lymphoblastic Leukemia (ALL) is the most common childhood malignancy, with an annual rate of 3 to 4 cases per 1,00,000 children. ALL patients are treated with chemotherapeutic agents and cranial irradiation. Long-term sequelae of treatment are impaired intellectual and psychomotor functioning, neuroendocrine abnormalitites, impaired reproductive capacity, cardiotoxicity and second malignant neoplasms are being reported with increased frequency in the survivors. Among second neoplasms observed after treatment of ALL, central nervous system (CNS) tumors in patients treated with cranial irradiation are the most common. Others are Lymphoma, Acute Myeloid Leukemia (AML) and Thyroid Cancer. Methodology & Results: We are reporting four cases ,who developed ALL at the age of 8,6,4 and 25 years respectively(among that case 1 & case 2 are siblings) and were treated with chemotherapy and cranial irradiation. They developed Astrocytoma Grade-4 , Alveolar Soft-Part Sarcoma(ASPS) , Anaplastic Large Cell Medulloblastoma & Ewing’s Sarcoma at, 12 , 15, 8, 12 & 26 years of age respectively. Conclusion: Oncologists should make sure about the radiation doses before starting treatment and they should keep CMMR-D as a possibility in pediatric patients with siblings having h/o malignancy.

Durable Response to Pazopanib in a Patient with Metastatic Alveolar Soft Part Sarcoma / 이화의대지

Alveolar soft part sarcoma (ASPS) is a rare form of soft tissue sarcoma, and frequently, metastases are found at diagnosis. In patients with metastatic or unresected ASPS, systemic treatment is extremely limited, because conventional chemotherapeutic agents have not been effective in most cases. A novel agent inhibiting angiogenesis, pazopanib, has been proven to be effective for metastatic soft tissue sarcoma in a second-line setting. However, the efficacy of pazopanib in ASPS has not yet been reported. A 22-year-old man presented with right calf ASPS and multiple lung metastases. Pazopanib as a second-line treatment showed significant tumor response. To the best of our knowledge, this is the first report of the effectiveness of pazopanib in ASPS.

Clinicopathological study of the alveolar soft part sarcoma with 16 cases reports / 临床与实验病理学杂志

Purpose To investigate the clinicopathological changes, immuniohistochemistry and molecular genetics phenotypic charac-teristics of the alveolar soft part sarcoma ( ASPS) . Methods 16 cases of ASPS were studied with clinicopathological, cytochemistry technique and immunohistochemical staining, two cases of ASPS were studied by FISH. Results There were 6 males and 10 females with the age 8~58 years (median age 31. 7 years). The tumors were located at limbs, shoulder and back, tongue, vocal cords, lung, cervix, and ureter. The clinical manifestations of the patients was a slowly growing mass. Histopathologically the tumor showed typical organ-like or acinar-like structure with sinus-like blood vessels and the fibrous septa formation. Sometime the clear or abundant eosino-philic granular cytoplasm of the tumors were obvious. The tumor cells had a crystalline substance formation by PAS staining. The tumor cells were positive for TFE3 and Cathepsin K by immunohistochemical staining. The ASPL-TFE3 gene fusion detection of tumor cells were present. Conclusion ASPS often located on the limbs of young patients. It may misdiagnosed as malignant epithelial tumors, primary or metastatic adenocarcinoma and paraganglioma when the tumor locate on a rare anatomical parts or an organs, such as tongue, vocal cords, cervix, ureter, etc. It is valueable that the typical alveolar-like structure of the tumor and the expression for TFE3 and Cathepsin K for the pathological diagnosis of ASPS. It is an important indicator that the ASPL-TFE3 gene fusion detection by FISH for the tumor.

Alveolar Soft Part Sarcoma Arising from the Kidney: Imaging and Clinical Features

Alveolar soft part sarcoma (ASPS) is an extremely rare malignant soft tissue sarcoma primarily affecting young patients. It usually occurs in the lower extremities, although it can occur in soft tissue anywhere in the body. However, to our knowledge, there has been no case of primary ASPS originating from the kidney in the literature. We herein present the imaging and clinical features of an ASPS which occurred in a 16-year-old male presented as a palpable mass in the left side of the abdomen.

Alveolar Soft Part Sarcoma: Clinical Presentation, Treatment and Outcome in a Series of 19 Patients

BACKGROUND: Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor that usually affects young patients. Because of the rarity of the disease, most reports relating to ASPS are in the form of case reports or small series. METHODS: We performed a retrospective study to evaluate the clinicopathologic features, treatment, outcome and pattern of treatment failure in a consecutive series of patients with localized or metastatic ASPS between 1996 and 2011. Demographics, tumor sizes, sites and extent of disease, treatments provided, progression-free survival, and overall survival were evaluated. RESULTS: A total of 19 patients were identified. The clinical assumptive diagnosis of the first medical examination doctor was benign soft tissue tumor in 5 cases (26%) and benign hemangioma in 4 cases (21%), delaying treatment. The most common location of primary tumor was the thigh. The median diameter of the mass was 55 mm (range, 10 to 130 mm). An R0 resection was obtained in 11 cases. Adjuvant radiotherapy was delivered in 8 cases; postoperative systemic chemotherapy was delivered in 10 cases. Eight out of 15 patients (53%) exhibited metastases either at presentation or later. Median overall follow-up was 54 months. CONCLUSIONS: The treatment principle for alveolar soft tissue sarcoma is massive resection, and when the surgical margin is questionable, radiation treatment can be added for prevention of local recurrence. Also, due to discovery of metastases and local recurrence, even after 5 years of general treatment, outpatient department follow-up is needed, and we need to keep in mind that lung, intracranial, and bone metastases are common.

A Case of Alveolar Soft Part Sarcoma of the Pleura

Alveolar soft part sarcoma (ASPS) is a rare malignant soft-tissue neoplasm of unknown histogenesis. The two main sites of occurrence are the lower extremities in adults and the head and neck in children. We report the first case of pleural ASPS occurring in a 58-yr-old man who presented with progressive dyspnea. A computed tomographic scan of the thorax revealed a large enhancing pleural mass with pleural effusion in the left hemithorax. Wide excision of the pleural mass was performed. Histologically, the tumor consisted of organoid nests of large polygonal cells, the cytoplasm of which had eosinophilic and D-PAS positive granules. Immunohistochemical staining showed that the tumor cell nuclei were positive for transcription factor 3 (TFE3). The pleural ASPS with multiple bone metastases recurred 1 yr after surgery and the patient died of acute pulmonary embolism 1.5 yr after diagnosis.

Alveolar Soft Part Sarcoma Metastasized to Both the Skull and the Brain

Alveolar soft part sarcoma (ASPS) with skull and brain metastases is extremely rare. A 53-year-old patient diagnosed as skull metastasis of ASPS visited our clinic complaining of an outgrowing scalp mass in spite of radiation therapy. Past medical history revealed that the patient had been diagnosed and treated for ASPS of the thigh 4 years ago. Magnetic resonance imaging revealed a hyperintense ovoid mass on the T2-weighted image, an isointense on the T1-weighted image, and a homogeneous enhanced mass with gadolinium. Another small-sized enhanced mass with mild peritumoral swelling was found at the deep white matter of the left frontal lobe. A gross total resection of the skull lesion with cranioplasty was performed for the surgical defect. A histologic examination of the specimens revealed metastatic ASPS involving the skull. Surgery with a total removal of the lesions may be effective for improving a patient's symptoms especially from neurological dysfunction.

Alveolar soft-part sarcoma of tongue.

Alveolar soft-part sarcoma is a clinically and morphologically distinct soft-tissue sarcoma of adolescent and young adult patients. Though immunohistochemical stains implicate a myogenic origin, the histogenesis of this tumor has not yet been established. Its high vascular nature leads to dissemination of the tumor cells into the bloodstream and metastasis. It comprises less than 1% of all soft-tissue sarcomas and less than 0.1% of sarcomas of the head and neck, preferably involving the orbit (48%) and tongue (25%). Lingual involvement is very rare and only about 31 cases have been reported in English literature. Their deceivingly indolent clinical courses often lead to misdiagnosis and delayed treatment. The reported case indicates its asymptomatic nature and microscopic similarity to granular cell tumor, which is the common benign tumor of the tongue.

Alveolar soft part sarcoma: a clinicopathological analysis of 21 cases / 临床与实验病理学杂志

Purpose To investigate the clinical and pathological features and differential diagnosis of alveolar soft part sarcoma (ASPS).Methods The clinical data were retrospectively analyzed in 21 cases of ASPS. Histopathological and immunohistochemical changes of the tumors were also observed in the paraffin-embedded tissue samples.Results In 21 cases, 11 were male and 10 females. Ages of the patients ranged from 4 to 56 years old,with mean 25.9 years old.The lesions were mainly located in the deep soft tissues of legs.Microscopically,tumor cells with granular cytoplasm arranged in alveolar or solid structures,and were separated by sinusoidal vessels. There existed crystals in the cytoplasm of tumor cells after PAS staining.Immunohistochemically,10 cases were positive for MyoD1, 4 positive for desmin,9 positive for S-100,11 positive for NSE, and 11 positive for Vim. All of ASPS were negative for AE1/AE3,CK, EMA, SMA, MSA and Syn.Conclusions ASPS is a rare malignant neoplasm in adolescents and young adults. It is prone to early hematogenous metastases and a high recurrence rate after conservative surgical excision. Therefore, the prognosis of ASPS is poor. Careful analysis of the clinicopathological features and immunohistochemisitry will be helpful to reach an accurate diagnosis.

Recurrent Alveolar Soft-Part Sarcoma With Concurrent Involvement of the Orbit and Multiple Sites of the Body

PURPOSE: To report a rare case of recurrent alveolar soft-part sarcoma (ASPS) with concurrent involvement of the orbit and multiple sites of the body that was removed successfully by surgery. CASE SUMMARY: A 37-year-old woman presented with diplopia at the left lateral gaze and proptosis of the left eye. Two years earlier, the patient had a mass excision of the right gluteus maximus and the left orbit, and ASPS was histopathologically diagnosed at that time. In addition, the patient had been treated with chemotherapy and radiation therapy. On radiologic examination, recurrent tumor of the left orbit was found and surgically removed. The histopathologic examination showed that tumor cells were arranged in an alveolar pattern divided by fibrous septa and contained abundant granules in the cytoplasm, typically consistent with ASPS. Postoperatively, the symptoms of diplopia and proptosis improved. CONCLUSIONS: ASPS can occur and recur in the orbit as well as systemically. In addition, at the time of surgical removal of ASPS in the orbit, the surgeon should be particularly cautious of massive bleeding.

Clinical features and prognostic factors of alveolar soft part sarcoma in 15 patients / 中国癌症杂志

Background and purpose: Alveolar soft part sarcoma (ASPS) is a rare malignant neoplasm characterized by slow growth and indolent behavior, and most of the patients had metastases in diagnoses. This study was to define clinical features and assess prognostic factors of ASTS. Methods: Between January 2003 and December 2008, there were 15 patients with ASTS who received treatment and were followed up in the Department of Orthopedic Ontology of Peking University People's Hospital. We retrospectively analyzed the clinical factors of the disease related to prognosis: gender, age, tumor size, primary tumor site, metastatic sites, local recurrence, the roles of postoperative radiotherapy and postoperative chemotherapy. Resnlts: At a follow-up from 7 to 76 months, only 2 patients had local recurrences after excision, but there was a high incidence of pulmonary metastases. Only 6 patients achieved disease free survival, 5 patients died of metastases or other complications. The five-year overall survival rates was 41.7%, and the median survival time was 42.0 months. Univariate analysis and Cox regression analysis revealed that tumor size, tumor primary site and metastases at diagnoses were prognostic factors, but not gender and age, Conclusion: Alveolar soft part sarcoma is a high grade malignant soft tissue neoplasm with frequent metastases to lung, bone or brain. Tumor size, primary tumor site and metastases at diagnoses are some of the prognostic factors. Chemotherapy or radiotherapy after surgery have no benefit to the survival in our analysis. Further prospective studies are needed to clarify the best strategy for the treatment of ASPS.

Alveolar soft-part sarcoma of the tongue: report of a case

Alveolar soft-part sarcoma(ASPS) is a rare, aggressive malignancy of uncertain histologic origin with a propensity for vascular invasion and distant metastasis. ASPS may mimic benign vascular neoplams of malformation but careful evaluation of the unique imaging features on CT scans, MR images, and angiograms lead to the correct diagnosis. ASPS of the tongue is slow-growing, painless mass, especially ASPS of the base the tongue is difficult to be noticed by patient, dentists or oral and maxillofacial surgeons on oral examintion because of its location and clinical resemblance to a benign lesion. And it leads to delayed or inadequate diagnosis. We report radiologic and clinical features of an ASPS of the basal portion of the tongue in a 17-year-old boy, showing normal appearance, but palpation of the tongue and floor of the mouth reveals the tumor. Among the 23 cases of a primary ASPS of tongue reported, 7 cases occured on the basal region of the tongue, inculding the present one. There has been no recurrence or metastasis as of 3 years postoperatively.

Fine Needle Aspiration Cytology of Alveolar Soft Part Sarcoma: A Case Report / 대한세포병리학회지

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma, which occurs predominantly in adolescents and young adults. The cytological characteristics of this condition have been described only rarely in the literature. Here, we report a case of alveolar soft part sarcoma. A 28-year-old man presented with a mass in his right buttock, which had persisted for three years. The mass was subjected to a fine needle aspiration cytology (FNAC). The smears were cellular. The observed tumor cells were round or polygonal, and exhibited vesicular nuclei with prominent nucleoli and finely granular cytoplasm. Naked nuclei were frequently detected. Tumor cells were arranged singularly, but occasionally in a pseudoalveolar pattern.

A Case of Alveolar Soft Part Sarcoma in a Child / 대한소아혈액종양학회지

Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor that accounts for 5% of pediatric non-rhabdomyosarcoma soft tissue sarcomas. The disease presents as a indolent asymptomatic mass and affects primarily younger patients. The clinical course is often indolent and late metastases can develop even after prolonged disease-free intervals. Radiotherapy and chemotherapy play a very limited role in the treatment. We report a pediatric case of ASPS with a brief review of the literature.

Surgical Treatment for the Alveolar Soft Part Sarcoma

PURPOSE: The aim of this study was to evaluate the clinicopathologic characteristics and the results of surgical treatment for alveolar soft part sarcoma (ASPS). METHODS: The clinicopathologic characteristics and surgical treatment results were analyzed retrospectively by reviewing the medical records of 11 patients and interviewing them. Overall survival (OS) was calculated using the Kaplan-Meier method. RESULTS: Of the 11 patients, 5 men and 6 women, the median age at diagnosis was 27 years and the mean tumor diameter was 6.0+/-1.9 cm. Six patients without distant metastasis at the time of diagnosis were treated with radical resection, and the other 5 with distant metastasis underwent palliative surgery or biopsy only, followed by chemo-radiation therapy. The onset of distant metastasis (40.0+/-13.5 months) was similar to the onset of local recurrence (43.3+/-22.4 months) after a radical operation. The most common site of metastasis was the lung, followed by the brain and bone. The median survival was 52 months and the 5-year OS was 27.2%. The 2-year OS in stage IV disease was 27.7%. The 5-year OS and the disease free survival among the patients with localized disease were 53.3% and 0%, respectively (P=0.043). CONCLUSION: Complete resection is the primary therapeutic option for ASPS. Close, long-term follow-up is recommended in order to detect recurrence, particularly in the lung, bone and brain. Long term survival can be achieved with aggressive surgery for local recurrence and even distant metastasis.

Clinicopathologic study and review of alveolar soft part sarcoma / 中国医师杂志

Objective To investigate the clinicopathologic features and differential diagnosis of alveolar soft part sarcoma(ASPS).Methods A case of ASPS was studied by HE stain,light microscopic observation,histochemical stain and immunohistochemical stain,and discussed the disease synthetically with the literature.Results The patient was female,23 years old.The lesion was located in the deep skeletal muscles of left buttocks,microscopically,which consisted of round to polygonal tumor cells,showing alveolus and nest arrangement,arounded by stroma containing sinusoidal capillaries,and there were abundant crystals in cytoplasm of the tumor cells,prominent nucleoli and fewer nuclear fission images in the tumor cells.PAS stain was stongly positive.Immunohistochemical stain showed positive expression of myoglobin in tumor cells,and negative expression of Myosin,Desmin,SMA,Vimentin,S-100 protein,EMA and Syn in them.Conclusion ASPS is a rare malignant soft tissue neoplasm,its histogenesis is uncertain,and diagnosed by pathological examine and treated mainly by operation.

Alveolar Soft Part Sarcoma Metastatic to the Brain

Alveolar Soft Part Sarcoma(ASPS) is a rare entity that invariably ends in death from the disseminated disease. Although the most common site of metastasis is the lung, the central nervous system is also the third common site. Its histogenesis remains to be unknown and the gold standard treatment is radically surgical removal of the mass. However, adjuvant chemotherapy and radiotherapy are known to be less effective. The authors present a 24-year-old man who was admitted with headache and neck discomfort. Magnetic resonance(MR) imaging scans demonstrated multiple masses in the left frontal lobe, parietal lobe, and right cerebellum. The patient underwent surgery to remove multiple masses in the staged fashion. The postoperative course was uneventful, but the patient committed suicide 5 months later. The authors reviewed the pertinent literature and discussed this clinical entity with references.