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.
Yamamoto
Hideki
H
Department of Pediatric Surgery, Dokkyo University School of Medicine, Koshigaya Hospital, Koshigaya, Japan.
Tsuchiya
Takayoshi
T
Ishimaru
Yuki
Y
Kisaki
Yoshiyuki
Y
Fujino
Junko
J
Uchida
Hiroo
H
Yoshida
Morikazu
M
Mori
Yoshiomi
Y
Ikeda
Hitoshi
H
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