• Humans
• Rectal Neoplasms/surgery
• Rectal Neoplasms/pathology
• Lymph Node Excision/methods
• Middle Aged
• Female
• Male
• Prospective Studies
• Aged
• Neuroendocrine Tumors/surgery
• Neuroendocrine Tumors/pathology
• Pelvis
• Lymphatic Metastasis
• Laparoscopy/methods
• Lymph Nodes/pathology
• Lymph Nodes/surgery
• Proctectomy/methods

• Local excision
• Lymph node metastasis
• Quality of life
• Radical resection
• Rectal neuroendocrine tumors

• Lymph node metastasis
• Rectal multiple neuroendocrine neoplasms
• Rectal neuroendocrine neoplasm

• adults
• constipation
• dolichocolon
• ganglioneuromaintestinal
• ganglioneuromatosis

Multiple neuroendocrine tumors (M-NETs) are rare in the rectum and there is no consensus on their characteristics and treatments. Here, we report 15 cases of rectal M-NETs and review the previous literature. We discuss the clinical characteristics, endoscopic features and pathological features of rectal M-NETs, aiming to analyze the treatments and follow-up strategies in combination with these characteristics. We retrospectively reviewed and analyzed the data of 15 patients with rectal M-NETs who were diagnosed and treated at Beijing Friendship Hospital, Capital Medical University. Their clinical data, endoscopic findings, pathological features and treatments were analyzed. Follow-up evaluations and literature review were performed. In all, 14 male (93.3%) and 1 female (6.7%) were recruited. The average age at diagnosis was 55.7 years. The clinical manifestations include asymptomatic in 9 patients (60.0%), defecation habits changes in 2 patients (13.3%), anal distension in 2 patients (13.3%), and abdominal distension in 2 patient (13.3%). The largest tumor diameter ≤10mm was found in 13 patients (86.7%) and >10mm in 2 patients (13.3%). All of the lesions originated from the mucous or submucosa layer. WHO grades were all NET G1. The number of tumors diagnosed by pathology in 13 patients was consistent with that observed by endoscopy, while more lesions were observed by pathology than endoscopy in two patients. Lymph node metastasis occurred in 1 patient (6.7%), and vascular or lymphatic invasion occurred in 9 patients (60.0%). Among the 13 patients with the largest tumor diameter being ≤10mm, lymphovascular invasion occurred in 8 patients (61.5%). And among the 2 patients with the largest tumor diameter of >10mm, lymphovascular invasion occurred in 1 patient (50.0%). 14 patients underwent endoscopic resection and 1 underwent surgical excision. Postoperative follow-up was achieved in 13 patients and no recurrence or metastasis was found. The true number of rectal M-NETs may be more than seen under endoscopy. Rectal M-NETs is associated with a high risk of metastasis; therefore, treatment and surveillance strategies should be more radical than single lesion.

• Digestive Medical Coordinated Development Center of Beijing Hospitals Authority

• Ganglioneuromatosis
• genetics
• pediatrics

• ESMR-L
• endocrine cell micronests
• rectal NET

• Endocrine Cells/pathology
• Humans
• Male
• Middle Aged
• Neuroendocrine Tumors/pathology
• Neuroendocrine Tumors/surgery
• Rectal Neoplasms/pathology
• Rectal Neoplasms/surgery
• Tomography, X-Ray Computed

Neurofibromatosis type 1 (NF-1) is commonly associated with benign or malignant tumors in both the central and peripheral nervous systems. However, rare cases of NF-1-associated multiple rectal neuroendocrine tumors have been reported. This report describes a case of a 39 year old female with NF-1 and intermittent hematochezia as a primary symptom. Physical examination showed multiple subcutaneous nodules and café au lait spots with obvious scoliosis of the back. Imaging examinations and colonoscopy found malformation of the left external iliac vein and multiple gray-yellow nodules with varying sizes and shapes in the rectal submucosal layer. Histological and immunohistochemical results suggested multiple rectal neuroendocrine tumors, a rare disease with few appreciable symptoms and a particularly poor prognosis. The patient with NF-1 presented here had not only multiple rectal neuroendocrine neoplasms but also vascular malformations, scoliosis and other multiple system lesions. This case therefore contributes to improving clinical understanding, diagnosis and treatment of related complications for patients with NF-1 who present with associated medical conditions.

• Neurofibromatosis type 1
• Multiple rectal neuroendocrine tumors
• Vascular malformations
• Scoliosis

• Adult
• Colonoscopy
• Female
• Gastrointestinal Hemorrhage/etiology
• Humans
• Magnetic Resonance Imaging
• Neoplasms, Multiple Primary/diagnosis
• Neoplasms, Multiple Primary/etiology
• Neoplasms, Multiple Primary/pathology
• Neuroendocrine Tumors/diagnosis
• Neuroendocrine Tumors/etiology
• Neuroendocrine Tumors/pathology
• Neurofibromatosis 1/complications
• Rectal Neoplasms/diagnosis
• Rectal Neoplasms/etiology
• Rectal Neoplasms/pathology
• Rectum/diagnostic imaging
• Rectum/pathology
• Scoliosis/diagnosis
• Scoliosis/etiology
• Skin Neoplasms/complications
• Tomography, X-Ray Computed
• Vascular Malformations/diagnosis
• Vascular Malformations/etiology
• Watchful Waiting

Multiple rectal carcinoids are rare. Due to the unreliability of endoscopic polypectomy in treating these submucosal lesions, a laparotomy is usually performed. We present a case report on multiple rectal carcinoids with three carcinoid foci < 10 mm in diameter located in the mid-rectum. Preoperative examination showed the lesions to be confined to the submucosal layer with no perirectal nodal involvement. A transanal endoscopic microsurgery was successfully performed to remove the three lesions with accurate full-thickness resection followed by secured suture closure. The postoperative pathology revealed neuroendocrine tumors G1 (carcinoids) located within the submucosal layer without lymphatic or vessel infiltration. Both the deep and lateral surgical margins were completely free of tumor cells. The patient recovered quickly and uneventfully. No tumor recurrence was observed at the six-month follow-up. For the multiple small rectal carcinoids without muscularis propria or lymphatic invasion, transanal endoscopic microsurgery offers a reliable and efficient alternative approach to traditional laparotomy for select patients, with the added advantages of minimally invasive surgery.

• Colorectal
• Gastrointestinal surgery
• Oncological surgery
• Technical
• Minimally invasive surgery

• Biopsy
• Carcinoid Tumor/pathology
• Carcinoid Tumor/surgery
• Endosonography
• Humans
• Male
• Microsurgery/methods
• Middle Aged
• Neoplasms, Multiple Primary/pathology
• Neoplasms, Multiple Primary/surgery
• Rectal Neoplasms/pathology
• Rectal Neoplasms/surgery
• Sigmoidoscopy/methods
• Time Factors
• Treatment Outcome
• Tumor Burden

• diffuse ganglioneuromatosis
• ganglioneuromas
• neurofibromatosis
• schwannoma

Minute (<5 mm) and small (5–10 mm) rectal carcinoids discovered during colonoscopy are generally considered to be non-aggressive, and the management and surveillance of patients with this entity are usually limited. We present the case of a 61-year-old Chinese female with multiple sub-5 mm carcinoid tumours in the rectum without any computed tomography (CT) evidence of lymph node or distant metastases. She underwent an ultra-low anterior resection for a sessile rectal polyp with the histological appearance of a moderately differentiated adenocarcinoma. Seven foci of minute carcinoids in the rectum and perirectal lymph node metastastic spread from the carcinoid tumours were also discovered on histopathology. There were no lymph node metastases originating from adenocarcinoma. This case report and review of the literature suggests that minute rectal carcinoids are at risk of metastasizing and that these patients should be investigated for lymph node and distant metastatic spread with CT and somatostatin receptor scintigraphy or its equivalent, as this would influence prognosis and surgical management of these patients. Findings relating to lymphovascular invasion, perineural invasion, high Ki-67, mitotic rate, depth of tumour invasion, central ulceration, multifocal tumours and size are useful in predicting metastases and may be used in scoring tools. Size alone is not a good predictor of metastastic spread.

• lymph node metastases
• rectal carcinoid
• tumour size

Rectal and perianal pain is a common problem. Most people have experienced it at least once in their lifetime. It usually manifests as mild discomfort, but sometimes the pain can be so severe that it is incapacitating. A 59-year-old woman admitted with a 2-year history of paroxysmal perianal pain underwent a full work-up including proctoscopy, sigmoidoscopy, full colonoscopy, and barium enema that were unremarkable. Lumbosacral magnetic resonance imaging with and without gadolinium showed an intradural-extramedullary lesion at the level of L5. The pathologic diagnosis was a neurofibroma. She underwent surgery, and after a few weeks she felt well and medication was no longer needed for her paroxysmal pain. Although one should consider the usual causes of colorectal pain such as hemorrhoids, anal fissure, proctalgia fugax, and chronic perianal pain syndrome, we should keep in mind that some referral pain may mimic local pathologies and should be evaluated properly.

• neurofibroma
• perianal pain
• proctalgia
• referral pain