A Vasculitis-Associated Neuromuscular and Vascular Hamartoma Presenting as a Fatal Form of Abdominal Cocoon

Abstract

Neuromuscular and vascular hamartoma (NMVH), also known as neuromesenchymal hamartoma, is a rare hamartomatous condition of the intestine. It usually presents with submucosal humps protruding in the intestinal lumen causing obstructive features. The other clinical manifestations are hematochezia or melena and protein-losing enteropathy. The etiopathogenesis of these lesions is not well known, although an association with small bowel Cröhn's disease and diaphragm disease had been postulated, the latter related to chronic nonsteroidal anti-inflammatory drug intake. Only 24 cases of NMVH are reported in the English literature and all of them could be adequately cured by resection of the affected part of the bowel. Moreover, none of these cases presented with abdominal cocoon or showed any evidence of vasculitis. We describe a peculiar case of NMVH in a 45-year-old male who presented with abdominal cocoon with symptomatic recurrence and fatal outcome within a month of surgery. Histopathology revealed classical histomorphology of NMVH with evidence of vasculitis. This appears to be the first case of a fatal form of NMVH, presenting with abdominal cocoon and associated with vasculitis.

An extremely rare case of neuromuscular and vascular hamartoma of the appendix

“…Most of the patients complained of abdominal pain, and 3 patients had Crohn’s disease as a comorbidity. NMVH originated in the small intestine in all cases except one in which it originated in the cecum [ 1 – 20 ]. Therefore, this report appears to be the first case of NMVH occurring in the appendix.…”

“…First described by Fernando and McGovern in 1982, this lesion consists of an aberrant proliferation of neural, muscular, and vascular elements in the small intestine [1]. Only 26 cases have been reported since its initial description, and NMVH occurred in the small intestine in all cases except one, in which it was found in the cecum [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]. To our knowledge, NMVH occurring in the appendix has not been reported in the English literature until now.…”

Histopathologic Spectrum of Neuromuscular and Vascular Hamartoma With Special Reference to the Vasculopathic Phenomenon

“…Notably, one of these cases had previously been reported (case 4). 14 The medical records of all these patients were retrieved. The detailed histomorphology had been evaluated by routine hematoxylin and eosin stain.…”

“…The spectrum of NMVH has expanded in the past three decades with anecdotal case reports. [7][8][9][10][11][12][13][14][15][16] The nomenclature has also recently been changed to "NMVH of the intestine" following the report of an individual case with cecal involvement. 7 The cause of NMVH remains elusive despite the advances of histochemical and molecular techniques although many authors have associated this entity with DD, chronic "burnt-out phase" of Crӧhn's disease, ischemic and radiation enteritis, and various chronic inflammatory diseases.…”

“…2,10,19 We have reported a unique case of NMVH in the recent past, which augured fatally due to an associated vasculitic illness, unlike the other reported cases of NMVH in the literature that were cured only by the surgical resection of the strictured segment. 14 This prompted us to look into the archival specimens of intestinal resection performed due to intestinal obstruction, and we found three additional cases of NMVH in this retrospective analysis.…”