Infantile intestinal leiomyosarcoma is prognostically favorable despite histologic aggressiveness: case report and literature review.
Intestinal leiomyosarcomas in children are extremely rare. The authors present a case of infantile intestinal leiomyosarcoma of the colon that was resected and was classified histologically as grade 2 because of abundant mitoses. The tumor cells infiltrated the submucosa and the serosal surface. Immunohistochemically, these cells were positive for alpha-smooth muscle actin but not for CD34 or CD117, by which the tumor was differentiated from gastrointestinal stromal tumor (GIST). The patient underwent follow-up without adjuvant therapy and is now doing well with no evidence of the disease for 42 months. Based on the current case and a review of 18 reported cases, it is concluded that infantile intestinal leiomyosarcoma, which is differentiated from GIST, has a favorable prognosis even when the histologic appearance is suggestive of aggressive biological characteristics. Adjuvant therapy is unnecessary when tumors are completely resected.
Department of Pediatric Surgery, Dokkyo University School of Medicine, Koshigaya Hospital, Koshigaya, Japan.
Hideki
H
Yamamoto
Takayoshi
T
Tsuchiya
Yuki
Y
Ishimaru
Yoshiyuki
Y
Kisaki
Junko
J
Fujino
Hiroo
H
Uchida
Morikazu
M
Yoshida
Yoshiomi
Y
Mori
Hitoshi
H
Ikeda
D000199:Actins / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D003110:Colonic Neoplasms / Q000737:chemistry / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D003111:Colonic Polyps / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D005260:Female; D006801:Humans; D007223:Infant; D007443:Intussusception / Q000209:etiology; D007813:Laparotomy; D007890:Leiomyosarcoma / Q000737:chemistry / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D008551:Melena / Q000209:etiology; D008940:Mitotic Index; D009361:Neoplasm Invasiveness; D009363:Neoplasm Proteins / Q000032:analysis; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D011379:Prognosis; D014057:Tomography, X-Ray Computed
D000199:Actins; D014408:Biomarkers, Tumor; D009363:Neoplasm Proteins; D010751:Phosphopyruvate Hydratase