Alveolar soft part sarcoma: clinical, histopathological, molecular, and ultrastructural aspects

Alveolar soft part sarcoma (ASPS) is a rare soft tissue tumor occurring mainly in the adolescents and young adults. Multimodality treatment has not been effective, and excision remains the mainstay of treatment. Histopathologically, it varies little from case to case. It is composed of organoid aggregates of large polygonal cells with vesicular nuclei and abundant granular, eosinophilic cytoplasm, separated by delicate vascular channels. The line of differentiation of this unique tumor is yet undetermined, although recent advances have led to a better understanding of the genetic events underlying the pathogenesis of this tumor. The histopathological, ultrastructural, immunohistochemical, and genetic aspects of ASPS are discussed.     

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. © 1996 Wiley-Liss, Inc.     

Alveolar soft part sarcoma occurring on the abdominal wall of a 2-year-old child

Alveolar soft part sarcoma (ASPS) is a rare soft tissue malignant neoplasm that affects young people. It can occur in any region of the body and at any stage of development. But ASPS on the abdominal wall is rarely reported. However, a few cases were reported in children under the age of 10 years. In this study we report a case of ASPS that occurred on the abdominal wall of a 2-year-old patient.     

Pilot trial of tumor-specific peptide vaccination and continuous infusion interleukin-2 in patients with recurrent Ewing sarcoma and alveolar rhabdomyosarcoma: an inter-institute NIH study

BACKGROUND: Patients with recurrent Ewing sarcoma and alveolar rhabdomyosarcoma have poor prognoses and limited therapeutic options. We have investigated the use of peptide pulsed vaccination in an attempt to immunologically target the breakpoint region of tumor specific fusion proteins expressed in these tumors. PROCEDURE: Sixteen patients with recurrent, translocation positive, Ewing sarcoma, and alveolar rhabdomyosarcoma underwent apheresis for collection of peripheral blood mononuclear cells. Following countercurrent centrifugal elutriation, an apheresis product comprised predominantly of monocytes but containing small numbers of circulating immature dendritic cells was pulsed with peptides derived from the breakpoint region of the fusion proteins. Vaccines were administered intravenously concomitant with continuous intravenous rhIL-2 at 9 x 10(6) IU/m(2)/day. RESULTS: Toxicity was limited to IL-2 related effects and was generally mild. Following vaccination, all patients showed progressive disease, most in a rapid fashion following the first vaccine. One patient showed evidence of an immunologic response and another showed a mixed clinical response. Patients enrolled on this tumor vaccine trial showed significant immunosuppression and large bulky tumors. CONCLUSIONS: Peptide vaccination as administered in this trial did not alter the dismal clinical outcome for patients with recurrent pediatric sarcomas. Future trials of tumor vaccines in this population should target patient populations with improved immune competence and smaller tumor burdens. Furthermore, optimization of the antigen presenting cell populations may be important for inducing immune responses to peptide antigens.     

Alveolar soft part sarcoma Sehr seltene Ursache eines langsam wachsenden indolenten subkutanen Knotens

Zusammenfassung Fragestellung: Langsam wachsende nicht schmerzhafte subkutane Knoten k?nnen durch unterschiedlichste gut- oder b?sartige mesenchymale Proliferationen verursacht sein. Da sich hinter diesem Zeichen oft ein Malignom verbirgt und bildgebende Analysen nur selten den Weg zur Diagnose weisen, ist eine bioptische Kl?rung dringend erforderlich. Methode: Wir berichten über 3 Patientinnen (Alter 9, 19¹/₁₂ und 23¹/₆ Jahre) bei denen sich hinter einem langsam wachsenden subkutanen Knoten ein nicht embryonales Sarkom (Alveolar soft part sarcoma) verbarg. Ergebnisse: Das Alveolar soft part sarcoma (ASPS) ist ein ausgesprochenes seltenes Malignom. Zum Diagnosezeitpunkt lag bei 2 Patientinnen bereits eine Metastasierung vor. Eine Patientin verstarb noch vor Behandlungsbeginn. Bei der 2. sind Lungenmetastasen trotz multimodaler Therapie 4¹/₂ Jahre nach Diagnosestellung langsam progredient. Die 3. Patientin erhielt nach Resektion eines lokalisierten ASPS am Oberarm postoperativ eine Polychemo- und lokale Strahlentherapie und ist 8 Monate nach der Diagnosestellung ohne Krankheitszeichen. Schlu?folgerung: Das ASPS ist ein langsam wachsender Tumor mit ausgepr?gter Metastasierungstendenz. Die Prognose ist im wesentlichen von der Operabilit?t des Prim?rtumors und dem Vorliegen von Metastasen abh?ngig.      

Kongenitaler melanotischer neuroektodermaler Tumor

Congenital melanotic tumors (MNT) are very rare tumors of childhood. Microscopic findings overlap with those of other small round cell tumors. The neoplasia is almost always benign and generally arises in the maxilla in the 1st month of life. The histogenesis is still controversial today. The idea of a neuroectodermal origin is the most frequently advocated hypothesis. The diagnosis is possible by the coexpression of epithelioid antigen containing cells and melanotic-like cells. The differential diagnosis includes other small round blue cell tumors such as Ewing’s sarcoma, neuroblastoma, PNET, or malignant lymphoma. Treatment of MNT has typically been surgical. We report a case of a 3-month-old infant with an unusual recurrent tumor. Molecular cytogenetic studies in our case did not show numeric or structural genetic changes. The expression of the growth factor VEGF in the neuroblastic tumor cells was high and the growth factor angiopoietin-1 was strongly expressed in the melanotic tumor cells.