Intestinal Schwannoma: A Clinicopathological, Immunohistochemical, and Prognostic Study of 9 Cases

Abstract

Background. Intestinal schwannoma is a type of intestinal interstitial tumor with a very low incidence. At present, there are few studies on intestinal schwannoma. Methods. From January 2010 to January 2018, the patients diagnosed with intestinal schwannoma at the China-Japan Union Hospital of Jilin University were retrospectively reviewed. The patients’ clinicopathological features and prognosis were analyzed. Results. This study enrolled 9 patients with intestinal schwannoma, including 3 males and 6 females. The main symptoms of the patients were abdominal pain and melena. Abdominal computed tomography showed intussusception, slightly high-density shadowing in the intestine, thickening of the intestinal wall, and an intestinal mass. Colonoscopy and endoscopic ultrasonography showed submucosal masses without ulcer formation. Two patients underwent endoscopic biopsy, and the pathological results revealed inflammation and necrosis. One patient had increased neuron-specific enolase (NSE) levels. Immunohistochemical analysis showed that the tumor cells were positive for S-100 and negative for CD117, DOG-1, desmin, and smooth muscle actin. An average of 17 lymph nodes were found around the intestines in 4 patients, all of which demonstrated reactive hyperplasia. No recurrence or metastasis occurred during postoperative follow-up. Conclusions. Intestinal schwannoma is a rare tumor, and in our study its incidence was higher in women than in men. The main symptoms were abdominal pain and melena. Preoperative increases in NSE levels might contribute to a diagnosis. Complete surgical resection with free negative margins is the standard treatment for benign schwannoma. There was no recurrence or metastasis after complete surgical resection, suggesting that follow-up may not be required.

Clinical Characteristics and Surgical Management of Gastrointestinal Schwannomas

“…Schwannomas are tumors arising from Schwan cells of the neural sheath [1]. They are most commonly found in the central nervous system, spinal cord, and peripheral nerves in the extremities [2]. Gastrointestinal schwannomas (GS), which originate from Auerbach's nerve plexus in the muscularis propria, are extremely rare [3,4].…”

“…Because GS are rare and easily confused with a heterogeneous group of neuroectodermal or mesenchymal neoplasms such as gastrointestinal stromal tumors (GISTs), leiomyomas, neurofibromas, and glomus tumors, the preoperative diagnostic accuracy is quite low. Accurate diagnosis is established by postoperative pathology and immunohistochemistry [2,6,9].…”

Current status of the diagnosis and treatment of gastrointestinal schwannoma (Review)

“…Reports have shown that common serum tumor markers, such as fetoprotein, CEA and carcinoembryonic antigen, were within the normal range (42,43), but occasionally increased CA19-9 was observed (44). Notably, Shu et al (45) pointed out that one patient had elevated serum neuron-specific enolase (NSE) levels. NSE is an acid protease that is specific to neurons and neuroendocrine cells, and is highly concentrated in nerve cells, neuroendocrine cells and tumor cells (45).…”

Spindle cell proliferations of the sigmoid colon, rectum and anus: a review with emphasis on perineurioma

“…Nuclear palisading and well‐formed Verocay bodies are only occasionally seen . The presence of a peripheral cuff of lymphoid aggregates helps to distinguish from GIST . Myxoid stroma may be a helpful diagnostic clue.…”

“…Both soft tissue and colorectal schwannomas are diffusely positive for S100, but in contrast to their soft tissue counterpart, glial fibrillary acidic protein (GFAP) is positive in only a small proportion (5%) of colorectal schwannomas . Tumour cells are negative for muscle markers and CD117 . A positive cytokeratin stain should alert the pathologist to the possibility of an alternative diagnosis (e.g.…”