Gastric Malignant Peripheral Nerve Sheath Tumor in Type 1 Neurofibromatosis / 대한소화기학회지

Gastric malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare spindle cell sarcomas that arise within the peripheral nerves of the gastrointestinal tract. MPNST can present as a mass that may or may not be accompanied by obstruction or bleeding.Type 1 neurofibromatosis (NF) is an autosomal dominant genetic disorder with an incidence of 1 in 2,500-3,000. Plexiform neurofibromas in Type 1 NF can undergo a malignant transformation to MPNSTs. Approximately half of the incidence of MPNST is associated with the NF-1 gene. MPNST behaves aggressively, and radical excisional surgery is important for treatment. Recurrence and metastasis are significant, even after a radical excision. Despite multidisciplinary treatment, the five-year survival rate is only 30-50%. This paper reports the case of a 47-year-old man with Type 1 NF who presented with hemorrhage of a gastric subepithelial lesion. He underwent surgery under the suspicion of a gastrointestinal stromal tumor, but it was diagnosed as MPNST after confirming the histopathological appearance and immunohistochemical profiles. In addition, the large mass invaded the spleen and diaphragm. Radical surgery was performed, and additional chemotherapy was administered. This paper reports the experience of a patient with NF 1 with advanced MPNST discovered due to a subepithelial lesion.

A Huge Malignant Gastric Gastrointestinal Stromal Tumor in a Young Patient / 대한소화기학회지

Malignant gastrointestinal stromal tumors (GISTs) are rare neoplasms originating from the gastrointestinal tract that rarely occur in patients below 40 years of age. To our knowledge, there have been no previous reports of satellite and metastatic nodules in GIST.We present a case of a young patient with a huge malignant gastric GIST accompanied by spontaneous bleeding and satellite and metastatic nodules, successfully treated surgically, without preoperative chemotherapy administration. A 28-year-old man was admitted to Haeundae Paik Hospital with melena. A huge bulging gastric mass with ulceration and bleeding was observed on endoscopy. A subepithelial lesion on the stomach body, abutting the pancreatic body and tail, with regional lymph node enlargement was confirmed by EUS and CT. Radical total gastrectomy was performed, the invasion surrounding the pancreatic tail and spleen were surgically dissected, and enlarged lymph nodes around the celiac trunk and the common hepatic artery were removed. The pathology results showed a malignant GIST with two satellite nodules and a metastatic tumor nodule at the left paracardial lymph node site. After complete resection of the malignant GIST, adjuvant chemotherapy with imatinib was initiated. Follow-up CT and endoscopy performed 6 months after surgery confirmed no recurrence of the disease.