Colorectal sarcoma: more than a gastrointestinal stromal tumor

Abstract

Primary colorectal sarcomas have been defined as a rare and diverse group of mesenchymal cancers distinct from gastrointestinal stromal tumors (GISTs). Primary colorectal sarcomas have been recognized as a distinct entity from GISTs due to the dramatically worse prognosis these sarcomas carry. Also, primary colorectal sarcomas when compared to the more common colorectal adenocarcinoma, demonstrate more aggressive biology, present at a younger age and carry worse outcomes. At this time, surgery remains the mainstay of treatment and adjuvant chemotherapy has an unclear role in treatment of primary colorectal sarcoma. This paper attempts to review the available data regarding primary colorectal sarcomas.

Mesenteric Lymph Node Recurrence of a Primary Colorectal Leiomyosarcoma

“…It is unclear if these guidelines could also be applied to soft tissue sarcomas of the colon, because lymph node involvement in these cases is extremely rare. For the same reason, extended lymphadenectomy is not routinely recommended [10,12]. In the case described here, in which standard guidelines for lymphadenectomy in classic adenocarcinoma were followed, none of the 15 lymph nodes removed during the initial surgery were positive.…”

“…The most widely accepted and performed treatment with colonic leiomyosarcomas is surgical excision [6,10,11]. The goal of surgery is to achieve complete microscopic removal of tumor with negative margins [11].…”

“…It is most commonly diagnosed during the 5 th or 6 th decades of life and usually presents with nonspecific symptoms, such as abdominal pain and hematochezia [9]. The standard treatment of colorectal leio-myosarcoma consists of surgical resection [6,10,11]. The potential role of systemic treatment remains highly controversial, namely, when a nodal isolated recurrence is present [4].…”

Leiomyosarcoma of the Rectum as a Radiation-Induced Second Malignancy after Cervical Cancer Treatment: Case Report with Review of the Literature

“…However, pooled data from a majority of recently published case series suggest that primary colorectal sarcomas are characterized by both rapid progression and very poor oncological outcomes with a median of survival ranging from 30 to 53 months and a local recurrence rate of up to 85% [3–6]. Overall five-year survival rate of patients with primary colorectal sarcomas is significantly lower comparing to those with colorectal cancer—43% and 52%, respectively [7]. Tumor grade of primary colorectal sarcomas is the most important prognostic factor for overall five-year survival—38% for high grade and 61% for low grade [8].…”

Gastrointestinal carcinoma and sarcoma surgery

“…Dr. Grethlein has written a current review of chemotherapy choices for patients with liposarcoma (9). Additionally, articles on the management of solitary fibrous tumors (10), sarcomas of the colon (11), and specific focused problems in the management of patients with GIST are also provided (12,13). Finally, Spera and coworkers provide a useful overview of treatment of tissue defects that arise following complex sarcoma resections (14).…”