An unusual presentation of neuroendocrine tumor of the small bowel

A previously well 37-year-old woman attended the emergency assessment unit with symptoms of lethargy, breathlessness and peripheral oedema, whereby initial basic investigations revealed an iron deficiency anaemia and serum hypoalbuminaemia. The patient subsequently had multiple admissions to secondary care over a 2-year period due to worsening peripheral and central oedema. Investigations ruled out non-gastrointestinal causes of serum hypoalbuminaemia, such as renal, cardiac and hepatic failures. Gastrointestinal investigations later revealed raised faecal alpha-1 antitrypsin and small bowel ulceration on capsule endoscopy, with a histological diagnosis of Crohn’s disease made after a small bowel wedge resection. This case describes the unusual presentation of Crohn’s disease displaying symptoms primarily of protein-losing enteropathy, an uncommon and under-recognised consequence of inflammatory bowel disease. A review of current literature and the underlying pathophysiology for this rare condition are discussed, particularly in relation to Crohn’s disease.

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ASO Author Reflections: Indolent Growth and Small Bowel Neuroendocrine Tumor Management

Annals of Surgical Oncology ◽

10.1245/s10434-021-09641-4 ◽

2021 ◽

Author(s):

S. K. Sherman ◽

C. G. Tran ◽

J. R. Howe

Keyword(s):

Small Bowel ◽

Neuroendocrine Tumor ◽

Tumor Management

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Radiological and histological findings of an asymptomatic ever-increasing neoplasm: The small bowel neuroendocrine tumor (NET)

Current Problems in Cancer ◽

10.1016/j.currproblcancer.2019.07.001 ◽

2020 ◽

Vol 44 (1) ◽

pp. 100495

Author(s):

Simona Picchia ◽

Monica Terlizzo ◽

Maria Antonietta Bali

Keyword(s):

Small Bowel ◽

Neuroendocrine Tumor ◽

Histological Findings

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Small bowel intussusception: An unusual presentation of angioedema

Allergy and Asthma Proceedings ◽

10.2500/aap.2010.31.3288 ◽

2010 ◽

Vol 31 (1) ◽

pp. 84-87 ◽

Cited By ~ 1

Author(s):

Kusum Sharma ◽

Timothy J. Craig ◽

Tracy B. Fausnight

Keyword(s):

Small Bowel ◽

Unusual Presentation ◽

Small Bowel Intussusception

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Transanal Small Bowel Evisceration: An Unusual Presentation of Rectal Impalement

European Journal of Trauma and Emergency Surgery ◽

10.1007/s00068-008-7097-x ◽

2008 ◽

Vol 34 (6) ◽

Cited By ~ 2

Author(s):

Rodrigo Vincenzi ◽

Ruy J. Cruz

Keyword(s):

Small Bowel ◽

Unusual Presentation

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Liver and Bone Metastases From Small Bowel Neuroendocrine Tumor Respond to 177Lu-DOTATATE Induction and Maintenance Therapies

Clinical Nuclear Medicine ◽

10.1097/rlu.0000000000000569 ◽

2015 ◽

Vol 40 (2) ◽

pp. 162-165 ◽

Cited By ~ 1

Author(s):

William Makis ◽

Karey McCann ◽

Francois A. Buteau ◽

Alexander J. B. McEwan

Keyword(s):

Small Bowel ◽

Bone Metastases ◽

Neuroendocrine Tumor

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Management of Small Bowel Neuroendocrine Tumors

Cancers ◽

10.3390/cancers11091395 ◽

2019 ◽

Vol 11 (9) ◽

pp. 1395 ◽

Cited By ~ 4

Author(s):

Larouche ◽

Akirov ◽

Alshehri ◽

Ezzat

Keyword(s):

Small Bowel ◽

Neuroendocrine Tumor ◽

Neuroendocrine Tumors ◽

Management Strategies ◽

Mammalian Target Of Rapamycin ◽

Peptide Receptor Radionuclide Therapy ◽

Somatostatin Analogue ◽

Important Landmark ◽

Cancer Network ◽

Compare And Contrast

Several important landmark trials have reshaped the landscape of non-surgical management of small bowel neuroendocrine tumors over the last few years, with the confirmation of the antitumor effect of somatostatin analogue therapy in PROMID and CLARINET trials as well as the advent of therapies with significant potential such as mammalian target of rapamycin inhibitor (mTor) everolimus (RADIANT trials) and peptide receptor radionuclide therapy (PRRT) with 177-Lutetium (NETTER-1 trial). This narrative summarizes the recommended management strategies of small bowel neuroendocrine tumors. We review the main evidence behind each recommendation as well as compare and contrast four major guidelines, namely the 2016 Canadian Consensus guidelines, the 2017 North American Neuroendocrine Tumor Society guidelines, the 2018 National Comprehensive Cancer Network guidelines, and the 2016 European Neuroendocrine Tumor Society guidelines. Different clinical situations will be addressed, from loco-regional therapy to metastatic unresectable disease. Carcinoid syndrome, which is mostly managed by somatostatin analogue therapy and the serotonin antagonist telotristat etiprate for refractory diarrhea, as well as neuroendocrine carcinoma will be reviewed. However, several questions remain unanswered, such as the optimal management of neuroendocrine carcinomas or the effect of combining and sequencing of the aforementioned modalities where more randomized controlled trials are needed.

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Defining the number of lymph nodes needed to accurately stage small bowel neuroendocrine tumors: An 8-institution study from the US neuroendocrine tumor study group.

Journal of Clinical Oncology ◽

10.1200/jco.2018.36.4_suppl.265 ◽

2018 ◽

Vol 36 (4_suppl) ◽

pp. 265-265

Author(s):

Mohammad Zaidi ◽

Alexandra G Lopez-Aguiar ◽

Mary Dillhoff ◽

Eliza W Beal ◽

George A. Poultsides ◽

...

Keyword(s):

Small Bowel ◽

Lymph Nodes ◽

Neuroendocrine Tumor ◽

Neuroendocrine Tumors ◽

Lymph Node Staging ◽

Study Group ◽

Curative Intent ◽

Tumor Study ◽

The Us ◽

Tumor Study Group

265 Background: Small bowel neuroendocrine tumors (SB-NETs) frequently involve regional lymph nodes (LNs). The prognostic value of LN positivity on recurrence of disease is not well defined. The number of LNs needed to accurately stage patients is unknown. Methods: All patients with primary SB-NETs who underwent curative-intent resection at 8 institutions in the US Neuroendocrine Tumor Study Group between 2000 and 2016 were identified. Patients with distant metastatic disease were excluded. The association of LN positivity with recurrence of disease and the extent of lymphadenectomy required were analyzed. Results: Of 2182 patients with resected NETs, 203 had SB-NETs. Median age was 60 yrs, 56% were male, and median follow-up was 39 months. 83.5% of patients (n = 157) had LN positive disease. There was no difference in 3-year recurrence free survival (3-yr RFS) among patients with 1 or 2 positive LNs compared to patients with negative LNs (p = 0.63). Patients who had 3 or more positive LNs had a worse 3-yr RFS compared to those with 0, 1, or 2 positive LNs (n = 92 vs n = 73; 3-yr RFS 82% vs 92%; p < 0.001). Retrieval of 8 or more LNs was associated with a higher positive LN count compared to less than 8 LNs (4.6 vs. 1.6; p = 0.002). However, an increasing LN ratio was not associated with 3-yr RFS. When examining patients who had less than 8 lymph nodes retrieved, there was no difference in 3-yr RFS in those patients with 3 or more positive LNs compared to those with 0, 1, or 2 positive LNs (3-yr RFS: 100% vs 91%; p = 0.37). Retrieval of more than 8 lymph nodes, however, accurately discriminated patients with 3 or more positive LNs compared to those with 0, 1, or 2 positive LNs (3-yr RFS: 79.7% vs 93.5%; p = 0.005). Conclusions: For patients undergoing curative-intent resection of small bowel NETs, accurate lymph node staging requires a minimum of 8 lymph nodes for examination. 3 or more positive LNs is associated with decreased 3-yr RFS compared to 0, 1, or 2 positive lymph nodes. A thorough regional lymphadenectomy is critical for accurate staging and management of patients with small bowel neuroendocrine tumors.

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