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neuromuscular and vascular hamartoma

Friday 30 April 2010

Definition: Neuromuscular and vascular hamartoma is an extremely rare stricturing condition of the small bowel. It consists of abnormal mixtures of intestinal tissues: disorganized fascicles of smooth muscle derived from the submucosa, bundles of nonmyelinated nerve fibers with scattered abnormal ganglion cells and hemangiomatous vessels, occurring focally and causing recurrent obstructive symptoms or occult chronic gastrointestinal bleeding.

Neuromuscular and vascular hamartoma (NMVH) is a very rare stricturing condition of the small intestine, occurring focally and causing recurrent obstructive symptoms or occult chronic gastrointestinal bleeding.

It is a hyperplasia of varied tissue of the submucosa such as smooth muscle bundles, peripherals nerve tracts, vessels and ganglia.

The diffuse fatty infiltration of the submucosa shows the participation of mesenchymal tissues. It has been proposed the term of "Neuromesenchymal hamartoma" for the cases of NMVH with involvment of mesenchymal tissues.

The neuromuscular and vascular hamartoma of small bowel has been reported in less than 15 cases up to 2010. In all these cases similar gross and morphological findings were found.

Macroscopy

- stenosis
- long concentric stenosis
- thick serosa
- widely fatty submucosal infiltration
-  mucosa is brown, friable with superficial ulcerations and absence of plicae mainly at the centre of the stenosis.
- thick mesenterium with a nodular appearance
- possible submucosal extensive infiltration of the adipose tissue

Microscopy

- The submucosa is thick with extensive fatty infiltration.
- Mixing of abnormal intermingled non-myelinated nerve bundles, interspersed large ganglion cells, hyperplastic smooth muscle tissue (that originates mainly from the thickened muscularis mucosa and less from the muscularis propria), and hemangiomatous vessels.
- The vessels show muscular hypertrophy and locally ectasia and are observed also in the serosa. Small areas of fibrosis were seen everywhere.
- The mucosa is ulcerated and severe inflammed by lymphoplasma cells and less eosinophils.
- Foci of inflammation are seen in the subserosa.
- There is no transmural inflammation, granulomas or fissuring.
- No microorganism with histochemical stains is observed.
- Hyperaemic, thick wall vessels are observed in the mesenterium.

Localization

- small bowel (19943922, 11528149)
- cecum (11889608)

Variants

- Neuromuscular and vascular hamartoma arising in a Meckel’s diverticulum (16581660)

Differential diagnosis

Muscularization, hyperplasia of bundle nerves of the submucosa or hemangiomatous vessels can be also seen in Crohn’s disease, ischemia, enteritis by radiation injury. Therefore, the main differential diagnosis should be from Crohn disease.

See also

- neuromesenchymal hamartoma

Nota bene: Salas et al. (neuromesenchymal hamartoma of the small bowel. J Clin Gastroenterol. 1990, 12 (6): 705-9) proposed the term of "neuromesenchymal hamartoma" for the cases of NMVH with participation of mesenchymal tissues.

References

- Neuromesenchymal hamartoma of small bowel—an extremely rare entity: a case report. Theodosiou E, Voulalas G, Salveridis N, Pouggouras K, Manafis K, Christodoulidis K. World J Surg Oncol. 2009;7:92. PMID: 19943922

- Neuromuscular and vascular hamartoma arising in a Meckel’s diverticulum. Hemmings CT. Pathology. 2006 Apr;38(2):173-4. PMID: 16581660

- Neuromuscular and vascular hamartoma of the cecum. Shiomi T, Kameyama K, Kawano Y, Shimizu Y, Takabayashi T, Okada Y. Virchows Arch. 2002 Mar;440(3):338-40. PMID: 11889608

- Neuromuscular and vascular hamartoma of the small intestine. Scintu F, Giordano M, Mascia R, Comella D, Casula G. Dig Surg. 2001;18(4):331-3. PMID: 11528149

- Clinical and pathological overlap in nonsteroidal anti-inflammatory drug-related small bowel diaphragm disease and the neuromuscular and vascular hamartoma of the small bowel. de Sanctis S, Qureshi T, Stebbing JF. Am J Surg Pathol. 2001 Apr;25(4):539-41. PMID: 11257631

- Neuromuscular and vascular hamartoma of the small bowel: case report and review of the literature. Zolota V, Melachrinou M, Kakkos S, Spiliotis JD. Dig Dis Sci. 2000 Oct;45(10):2051-3. PMID: 11117582

- Clinical and pathologic overlap in nonsteroidal anti-inflammatory drug-related small bowel diaphragm disease and the neuromuscular and vascular hamartoma of the small bowel. Cortina G, Wren S, Armstrong B, Lewin K, Fajardo L. Am J Surg Pathol. 1999 Nov;23(11):1414-7. PMID: 10555011

- Neuromesenchymal hamartoma of the small bowel. Salas A, Casellas F, Sanz J, Garcia F, Margarit C, Malagelada JR. J Clin Gastroenterol. 1990 Dec;12(6):705-9. PMID: 2266253

- Neuromuscular and vascular hamartoma of the small bowel. Kwasnik EM, Tahan SR, Lowell JA, Weinstein B. Dig Dis Sci. 1989 Jan;34(1):108-10.
PMID: 2910666

- Salas A, Casellas F, Sanz J, Garcia F, Margarit C, Malagelada JR. Neuromesenchymal hamartoma of the small bowel. J Clin Gastroenterol. 1990;12(6):705–9.

- Scintu F, Giordano M, Mascia R, Comella D, Casula G. Neuromuscular and vascular hamartoma of the small intestine. Dig Surg. 2001;18(4):331–3. PubMed

- Shepherd NA, Jass JR. Neuromuscular and vascular hamartoma of the small intestine: is it Crohn’s disease? Gut. 1987;28(12):1663–8. PubMed

- Smith CE, Filipe MI, Owen WJ. Neuromuscular and vascular hamartoma of small bowel presenting as inflammatory bowel disease. Gut. 1986;27(8):964–9. PubMed

- Fernando SSE, McGovern VJ. Neuromuscular and vascular hamartoma of small bowel. Gut. 1982;23:1008–1012. PubMed

- Zolota V, Melachrinou M, Kakkos St, Spiliotis J. Neuromuscular and vascular hamartoma of the small bowel. Dig Dis Sci. 2000;45(10):2051–3. PubMed