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Adult Langerhans cell histiocytosis with caecal and regional lymph node involvement

  • Case Report
  • Published:
Hellenic Journal of Surgery

Abstract

Background

Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized bone marrow-derived Langerhans cells and mature eosinophils. Involvement of the gastrointestinal tract is very rare in LCH and has only been described in case reports and small series.

Case description

A 58-year-old woman who presented with recurrent and progressive right lower quadrant pain, bloating and change in bowel habits was submitted to surgical treatment. A computed tomography scan of the abdomen revealed circumferential caecal wall thickening as well as multiple mesenteric lymph nodes which were suspicious for involvement by a caecal tumour. A right hemicolectomy was performed.

Results

Upon opening the caecum, a soft, nodular, poorly circumscribed, grey-white to yellow mass measuring 4 cm in greatest dimension was observed close to the ileocaecal valve. Multiple lymph nodes were also identified in the surrounding mesenteric fat. Histology revealed a mucosal lesion of the caecum and terminal ileum characterized by proliferation of histiocytic cells and accompanied by a moderate number of eosinophils and lymphoid cells. Some of the regional mesenteric lymph nodes exhibited at least focal involvement with numerous histiocytes. Immunoperoxidase studies showed that the histiocytic cells were reactive to langerin, CD1a, S-100 and CD68 antigens.

Based on these findings, a diagnosis was reached of Langerhans cell histiocytosis with lymph node involvement.

Conclusions

LCH is rare in adults, and gastrointestinal tract involvement even rarer. Our case presented as a mass lesion located in the mucosa of the caecum and terminal ileum. With the increasing number of colonoscopies being performed, gastroenterologists and pathologists should be aware of this rare cause of colon polyps.

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Abbreviations

LCH:

Langerhans Cell Histiocytosis

GI:

Gastro Intestinal

RDD:

Rosai - Dorfman Disease

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Authors and Affiliations

  1. Department of Pathology, General Hospital “Elpis”, 15 Dimitsanas str., 11522, Athens, Greece

    A. Androulaki & A. Klimis

Authors
  1. A. Androulaki
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  2. A. Klimis
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Correspondence to A. Androulaki.

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Androulaki, A., Klimis, A. Adult Langerhans cell histiocytosis with caecal and regional lymph node involvement. Hellenic J Surg 88, 201–204 (2016). https://doi.org/10.1007/s13126-016-0316-8

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  • DOI: https://doi.org/10.1007/s13126-016-0316-8

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