Louisiana State University Health Sciences Center Shreveport, LA, United States
Award: Presidential Poster Award
Subhash Garikipati, MD1, Shachi Patel, MD1, Brian Morris, MD2, James Morris, MD1 1Louisiana State University Health Sciences Center, Shreveport, LA; 2Ochsner Medical Center, New Orleans, LA
Introduction: Soft tissue sarcomas are a group of malignant tumors originating from primitive mesenchymal tissue. Rhabdomyosarcomas account for 4% of these tumors while the remaining types account for 3% of these tumors in children. Undifferentiated pleomorphic sarcoma is one of the many types of non-rhabdomyosarcoma malignancies and accounts for 2-6% of all childhood soft tissue sarcomas. They most commonly arise in the extremities. We present a pediatric patient with an undifferentiated pleomorphic sarcoma arising from the cecum.
Case Description/Methods: A 12-year-old girl with a past medical history of obesity presented with intermittent abdominal pain, diarrhea, weight loss, and vomiting over a 4–5 month period. She was taking naproxen daily for the abdominal pain. She was also found to have iron deficiency anemia and anemia with a hemoglobin of 5.3 gm/dl. EGD was unremarkable but colonoscopy revealed a 7 cm fungating non-obstructing large mass in the cecum as well as 2 five mm pedunculated polyps in the rectum. Imaging revealed peri-colonic, mesenteric, and mediastinal lymph nodes as well as a 1.2 cm solid nodule in the left lower lobe. Then she underwent surgery revealing a colo-colonic intussusception and a right hemicolectomy for the malignancy. Later on, the patient had a video assisted thoracoscopic surgery with wedge resection of the pulmonary nodule. Colonic pathology revealed an undifferentiated pleomorphic sarcoma while lung pathology revealed histoplasmosis.
Discussion: While rare pediatric malignancies such as this have a scarcity of literature presented, this appears to be the first reported case of an undifferentiated pleomorphic sarcoma arising from the cecum in a pediatric patient. Patients commonly present in the second decade of life with a painless mass but since this patient’s mass is in the intestines, it went undetected until it grew quite large ( > 7 cm). She did have typical symptoms of gastrointestinal malignancy such as vague abdominal pain, weight loss, bowel habit changes, and iron deficiency anemia but a malignancy is oftentimes lower on the differential for the pediatric age group. At the time of diagnosis, it is important perform staging via imaging including CT/MRI and PET. In our patient, when found, it had already metastasized to distant lymph nodes. Surgical resection of local disease can possibly lead to a relapse free life and possibly a cure but oftentimes adjuvant chemotherapy is also needed if there is metastasis.
Figure: A and B: Colonoscopy revealed a fungating non-obstructing, non-circumferential large mass in the cecum. The mass measured seven cm in length and 5 mm in diameter.
Disclosures: Subhash Garikipati indicated no relevant financial relationships. Shachi Patel indicated no relevant financial relationships. Brian Morris indicated no relevant financial relationships. James Morris indicated no relevant financial relationships.
Subhash Garikipati, MD1, Shachi Patel, MD1, Brian Morris, MD2, James Morris, MD1. P2260 - A Rare Case of a Primary Colonic Undifferentiated Pleomorphic Sarcoma in a Pediatric Patient, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.