Lymphangiomatosis of the ileum with perforation: A case report and review of the literature

Giuliani, Antonio; Romano, Lucia; Coletti, Gino; Fatayer, Mohammad Walid A; Calvisi, Giuseppe; Maffione, Francesco; Muolo, Chiara; Vicentini, Vincenzo; Schietroma, M; Carlei, F

doi:10.1016/j.amsu.2019.03.010

Cited by 25 publications

(21 citation statements)

References 19 publications

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“…Incidence of NECs is 1000 cases annually; 11% within the GI tract [27], with a poor prognosis and commonly arising in the oesophagus and large bowel [28–35]. According to the Surveillance, Epidemiology, and End Results (SEER) database, colorectal NECs has an incidence of 0.2 per 100,000 inhabitants [36].…”

Section: Discussionmentioning

confidence: 99%

Poorly differentiated neuroendocrine rectal carcinoma with uncommon immune-histochemical features and clinical presentation with a subcutaneous metastasis, treated with first line intensive triplet chemotherapy plus bevacizumab FIr-B/FOx regimen: an experience of multidisciplinary management in clinical practice

et al. 2019

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BackgroundNeuroendocrine tumors (NETs) are heterogeneous, widely distributed tumors arising from neuroendocrine cells. Gastrointestinal (GI)-NETs are the most common and NETs of the rectum represent 15, 2% of gastrointestinal malignancies. Poorly differentiated neuroendocrine carcinomas of the GI tract are uncommon. We report a rare case of poorly differentiated locally advanced rectal neuroendocrine carcinoma with nodal and a subcutaneous metastasis, with a cytoplasmic staining positive for Synaptophysin and Thyroid Transcription Factor-1.Case presentationA 72-year-old male presented to hospital, due to lumbar, abdominal, perineal pain, and severe constipation. A whole-body computed tomography scan showed a mass of the right lateral wall of the rectum, determining significant reduction of lumen caliber. It also showed a subcutaneous metastasis of the posterior abdominal wall. Patient underwent a multidisciplinary evaluation, diagnostic and therapeutic plan was shared and defined. The pathological examination of rectal biopsy and subcutaneous nodule revealed features consistent with small-cell poorly differentiated neuroendocrine carcinoma. First line medical treatment with triplet chemotherapy and bevacizumab, according to FIr-B/FOx intensive regimen, administered for the first time in this young elderly patient affected by metastatic rectal NEC was highly active and tolerable, as previously reported in metastatic colo-rectal carcinoma (MCRC). A consistent rapid improvement in clinical conditions were observed during treatment. After 6 cycles of treatment, CT scan and endoscopic evaluation showed clinical complete response of rectal mass and lymph nodes; patient underwent curative surgery confirming the pathologic complete response at PFS 9 months.Discussion and conclusionsThis case report of a locally advanced rectal NEC with an unusual subcutaneous metastasis deserves further investigation of triplet chemotherapy-based intensive regimens in metastatic GEP NEC.

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Section: Discussionmentioning

confidence: 99%

Poorly differentiated neuroendocrine rectal carcinoma with uncommon immune-histochemical features and clinical presentation with a subcutaneous metastasis, treated with first line intensive triplet chemotherapy plus bevacizumab FIr-B/FOx regimen: an experience of multidisciplinary management in clinical practice

et al. 2019

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“…16 Reported cases describe complications of mesenteric volvulus and perforation. 7,8,17 Our patient presented with intussusception and upstream bowel distention, emesis, and gastrointestinal bleeding. Only 5% of intussusceptions occur in adults, commonly because of adhesions, metastatic or primary neoplasm, feeding tubes, or idiopathic etiology.…”

Section: Discussionmentioning

confidence: 87%

Primary Intestinal Lymphangiectasia Causing Intussusception and Small Bowel Obstruction

et al. 2019

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Abdominal lymphangiectasia is a rare disease manifestation with a variety of anatomic locations and clinical presentations. The gastrointestinal tract may be affected, and lymphangiectasia originating in the wall of the intestine has rarely been described. We present a case of primary small bowel lymphangiectasia causing intussusception in a 30-year-old woman who presented with emesis and gastrointestinal bleeding. This case emphasizes the clinical presentation, diagnosis, and management in adults with abdominal lymphangiectasia. We highlight the importance of a high clinical suspicion for lymphangiectasia in an adult with acute abdomen to avoid catastrophic morbidity.

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“…A very limited number of pancreatic tumors are reported in association with agenesis of the dorsal pancreas, including a malignant IPMN [ 22 ] and cystic lesions [ 23 ]. The association is unclear, but chronic pancreatitis is considered one of the risk factors for cancer.…”

Section: Discussionmentioning

confidence: 99%

Partial agenesis of dorsal pancreas. Report of two cases

Valiyeva

Romano

Schietroma

et al. 2020

International Journal of Surgery Case Reports

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Highlights Agenesis of the dorsal pancreas is a rare congenital anomaly. The possibility of finding this disorder is increasing with the use of advanced radiological techniques. Some patients experience no symptoms. Rarely, they may also develop pancreatic exocrine insufficiency or pancreatic adenocarcinoma. This diagnosis could be missed by US, due to difficulty on visualization of body and tail of pancreas.

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Lymphangiomatosis of the ileum with perforation: A case report and review of the literature

Cited by 25 publications

References 19 publications

Primary Intestinal Lymphangiectasia Causing Intussusception and Small Bowel Obstruction

Partial agenesis of dorsal pancreas. Report of two cases

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