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. 2014;5(9):613-6.
doi: 10.1016/j.ijscr.2014.07.002. Epub 2014 Jul 10.

Small-bowel myeloid sarcoma: Report of a case with atypical presentation

Carlo M Girelli  1 , Valentina Carsenzuola  2 , Marilù Latargia  3 , Alessandra Aguzzi  4 , Giovanni Serio  4
Affiliations

Small-bowel myeloid sarcoma: Report of a case with atypical presentation

Carlo M Girelli et al. Int J Surg Case Rep. 2014.

Abstract

Introduction: Small-bowel myeloid sarcoma is rare. Acute bowel obstruction is its usual clinical presentation.

Presentation of case: We report a case of small-bowel myeloid sarcoma that occurred in a 64-year-old woman who presented chronic secretory diarrhoea, hypokalaemia, and weight loss. Immature white blood cells in a peripheral smear and small-bowel capsule endoscopic features were the main diagnostic clues. The patient experienced capsule retention and developed acute bowel obstruction. Urgent laparotomy showed a stricturing ileal mass and pathology of the resected bowel specimen unveiled a CD34+, CD117+, and myeloperoxidase-positive myeloid sarcoma. The diarrhoea promptly resolved after surgery, and the patient is now undergoing chemotherapy.

Discussion: Secretory diarrhoea can be the first manifestation of small-bowel myeloid sarcoma. Capsule endoscopy may provide a diagnostic clue, but it can trigger an acute bowel obstruction. Differential diagnosis of the pathologic specimen may be difficult and a high suspicion index of is mandatory to perform immunophenotyping to determine the correct management.

Conclusion: Chronic diarrhoea with alarm features can be the first manifestation of small-bowel myeloid sarcoma.

Keywords: Acute leukaemia; Capsule endoscopy; Chloroma; Granulocytic sarcoma; Myeloid sarcoma; Small-bowel.

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Figures

Fig. 1
Fig. 1
Small-bowel capsule endoscopy frame showing a luminal stricture with thickened mucosa and short, swollen villi.
Fig. 2
Fig. 2
Laparotomic finding of an annular, stricturing mass lesion of the ileum (arrow).
Fig. 3
Fig. 3
Histopathology of the resected bowel specimen. (A) Diffuse infiltration by round, small- to medium-sized cells with moderate basophilic cytoplasm. The cells had round or oval folded nuclei containing dispersed chromatin and exhibited strongly positive staining for CD34 (B), CD117 (C), and myeloperoxidase (D).
See this image and copyright information in PMC

References

    1. Swerdlow S.H., Campo E., Harris N.L., Jaffe E.S., Pileri S.A., Stein H. 4th ed. IARC Press; Lyon: 2008. WHO classification of tumours of haematopoietic and lymphoid tissues.
    1. Zekry N., Klooster N.J., Ragavhan R., Wang Y. A 7-year-old child with a history of acute myeloid leukemia presenting with multiple gastrointestinal polyps. Extramedullary myeloid sarcoma. Arch Pathol Lab Med. 2006;130:e3–e4. - PubMed
    1. Pileri S.A., Ascani S., Cox M.C., Campidelli C., Bacci F., Piccioli M. Myeloid sarcoma: clinico-pathologic, phenotypic and cytogenetic analysis of 92 adult patients. Leukemia. 2007;21:340–350. - PubMed
    1. Dores G.M., Devesa S.S., Curtis R.E., Linet M.S., Morton L.M. Acute leukemia incidence and patient survival among children and adults in the United States, 2001–2007. Blood. 2012;119:34–43. - PMC - PubMed
    1. Kohl S.K., Aoum P. Granulocytic sarcoma of the small intestine. Arch Pathol Lab Med. 2006;130:1570–1574. - PubMed