Clinical and pathologic features of primary gastric rhabdomyosarcoma

Abstract

The authors report a case of a collision tumor composed of a primary gastric rhabdomyosarcoma intermingled with an adjacent infiltrating gastric adenocarcinoma. Only eight cases of gastric rhabdomyosarcoma have been reported previously and little information is recorded about the behavior of this tumor. Gastric rhabdomyosarcoma has several distinctive clinical features. It is a highly aggressive tumor (median survival, 2.5 months) occurring in both children and adults and frequently presents as metastatic disease to lung or cervical lymph node. The initial diagnosis is often difficult to establish, especially on biopsy material. In several instances, the correct diagnosis was established only at autopsy. The authors' patient was correctly diagnosed during life and received considerable benefit from cyclophosphamide, doxorubicin, and vincristine (CAV) therapy and survived for 26 months after diagnosis. Two of the previously reported eight cases of primary gastric rhabdomyosarcoma and this case have adjacent admixed glandular gastric adenocarcinomas. Since three of the nine known cases of primary gastric rhabdomyosarcoma have an adjacent admixed gastric adenocarcinoma, there appears to be a greater than chance association between these two tumors.

Primary gastric tumors of infancy and childhood: 54-year experience at a single institution

“…Because it is rare to find this tumor in the stomach, the treatment must be based on the treatment of other primary sites. Although palliative resection followed by postoperative chemotherapy and radiotherapy is most commonly used, some recommend radical excision if the tumor is detected early [59]. Chemotherapy remains a mainstay of treatment, and several clinical trials are currently underway.…”

Sarcomes gastriques

“…Les sarcomes peuvent sié ger à tous les niveaux avec une nette pré dominance des localisations au niveau de la paroi posté -rieure du corps gastrique et de la grande courbure [4,15]. Ils sont gé né ralement volumineux (de 5 à 30 cm) et ont souvent un dé veloppement exogastrique (54 à 85 %) [1,4].…”

“…Les sarcomes gastriques, comme les sarcomes du tube digestif et ceux des tissus mous, avaient une ré putation mé rité e de chimioré sistance à l'exception des rhabdomyosarcomes embryonnaires qui sont rares dans cette localisation [15,35]. La chimiothé rapie est utilisé e essentiellement à visé e palliative, dans le cadre des sarcomes é volué s, dé couverts au stade de mé tastases synchrones ou d'apparition secondaire aprè s le traitement chirurgical [4,15,35,43].…”

“…La chimiothé rapie est utilisé e essentiellement à visé e palliative, dans le cadre des sarcomes é volué s, dé couverts au stade de mé tastases synchrones ou d'apparition secondaire aprè s le traitement chirurgical [4,15,35,43]. Les 3 agents les plus utilisé s seuls ou en association dans les sarcomes digestifs sont la doxorubicine, l'ifosfamide et la dacrobazine.…”

“…l'envahissement locoré gional des organes de voisinage au moment du diagnostic, considé ré comme un critè re majeur de malignité [5,16,26] ; la qualité de la ré section chirurgicale complè te ou incomplè te, la rupture tumorale, et la pré sence ou non de mé tastases à distance [5,16,26,37,48,50] ; le type histologique : les histiocytofibromes malins et les angiosarcomes gastriques semblent avoir un comportement plus agressif que les autres sarcomes [15,51]. Quant aux tumeurs musculaires lisses, il n'existe pas de diffé rence pronostique significative relative au type histologique [3,5].…”