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Alveolar soft-part sarcoma of the cheek.

Alveolar soft-part sarcoma is a rare malignant tumour of uncertain histogenesis, the two main sites of which are the lower extremities in adults and the head and neck in children. We know of only three case reports of intraoral locations without tongue involvement. Here, we describe the first case of an alveolar soft-part sarcoma of the cheek which presented in a patient over 30 years old and had an extremely rapid and eventually fatal evolution. We have also reviewed hypotheses about its aetiology.

Biopsy↗

Alveolar soft-part sarcoma of the orbit.

Alveolar soft-part sarcoma is a malignant soft tissue neoplasm that involves mainly the deep soft tissues of the extremities, particularly the thighs and the buttock. Involvement of the orbit is uncommon. We describe a young child with such a tumor and illustrate the characteristic features.

Antineoplastic Combined Chemotherapy Protocols↗

Alveolar soft-part sarcoma: a new type of rhabdomyosarcoma?

Alveolar soft part sarcoma (ASPS) was described as a distinct entity in 1952. ASPS mainly affects young adults, with a slight prevalence of female patients. It arises in the extremities, trunk, and head and neck region. Metastases are frequent and are mainly localized to lungs, brain, and bone. Local recurrences are rare if the primary tumor is completely excised. Adjuvant chemotherapy or radiotherapy are not useful. Death eventually supervenes, after several years, in most of the patients. Histogenesis and differentiation of ASPS, since its first description, have remained a matter of controversy. Many hypotheses have been taken into consideration, among which neural and muscular differentiation are the most plausible. The finding that intracytoplasmic crystals, present in most of the cases, are composed of actin, and the immunocytochemical localization in ASPS of several muscular markers, such as actin (skeletal and smooth muscle actin), desmin, and MyoD1 are all features that point towards skeletal muscle differentiation.

Adolescent↗

[Alveolar soft tissue sarcoma].

Alveolar soft part sarcoma is a malignant soft part tumor the etiology of which has not yet been clarified. Histopathological examinations show an alveolar or pseudoglandular pattern. PAS-positive, diastase resistant intraplasmic inclusion bodies are characteristic for this tumor which occurs in the head and neck region; mainly the tongue and the orbita. Primary therapy includes total resection which can later be combined with radiotherapy or chemotherapy. The present study involves a 17 year old patient. We totally removed the tumor and then used radiotherapy. The postoperative examinations over 3 years showed neither local recurrence nor distant metastases.

Adolescent↗

Alveolar soft-part sarcoma: a review and update.

Alveolar soft-part sarcoma (ASPS) is a rare, distinctive sarcoma, typically occurring in young patients. Although it displays a relatively indolent clinical course, the ultimate prognosis is poor and is often characterised by late metastases. Recently, our understanding of the genetic events underlying the pathogenesis of ASPS has greatly increased. The historical, histopathological, ultrastructural, immunohistochemical and genetic aspects of ASPS are reviewed in this article.

Biomarkers, Tumor↗