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

Girelli CM, Carsenzuola V, Latargia M, Aguzzi A, Serio G - Int J Surg Case Rep (2014)

Bottom Line: The diarrhoea promptly resolved after surgery, and the patient is now undergoing chemotherapy.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Gastroenterology and Digestive Endoscopy Unit, Hospital of Busto Arsizio, Busto Arsizio, VA, Italy. Electronic address: cargirel@vodafone.it.

No MeSH data available.


Related in: MedlinePlus

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).
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fig0015: 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).

Mentions: A previously healthy 64-year-old woman was admitted to our gastrointestinal unit for unexplained chronic diarrhoea and a loss of more than 10% of her usual body weight. Watery, non-bloody diarrhoea had started six weeks before admission. Blood analyses performed four weeks before admission showed mild normocytic anaemia, low serum albumin, and hypokalaemia. Total serum immunoglobulin A was normal. Anti-transglutaminase antibodies were negative, along with stool examination (including culture and a search for ova and parasites), oesophagogastroduodenoscopy with duodenal biopsies, and ileocolonoscopy with biopsies performed two weeks before admission. The patient appeared pale and thin, with dry skin and mucous membranes. Her abdomen was flat and soft, and deep palpation did not arouse pain or guarding. Her liver and spleen were not felt, and bowel sounds were present. A blood cell count showed 11.2 × 109/L white blood cells, with 22% of immature cells in a peripheral smear. After fluid and lytes replacement, a bone marrow biopsy was performed, which disclosed immature cells: namely, 30% of blasts, CD45+, CD34+, CD117+, CD33+, CD13+, and HLADR+; normal karyotype (46XX); FLT3 0; and NPM: absence of mutations. A diagnosis of de novo AML, FAB M1 was made. As chemotherapy was not possible due to the persisting large volume of diarrhoea, small-bowel capsule endoscopy (SBCE) was performed (PillCam SB3, Given Imaging). One hour and twenty-two minutes after ingestion, the capsule did not pass through an ileal stricture next to the dilated lumen, filled with luminal debris. The mucosa was thickened and pale, with short and swollen villi (Fig. 1). The following day, vomiting, abdominal pain, and distension ensued, and abdominal CT disclosed a stricture of the distal ileum next to the retained capsule, with proximal bowel dilation. On the 10th day of the hospital stay, laparotomy was performed. When the peritoneum was opened, a marked enlargement of the small bowel adjacent to an annular stenosis, 15 cm proximal to the ileocaecal valve, was found (Fig. 2). Segmental ileal resection with manual latero-lateral anastomosis was performed. The still-flashing capsule was retrieved within the resected bowel. The postoperative course was uneventful, with early oral feeding, bowel canalisation, and full resolution of the diarrhoea. Pathologic examination of the resected specimen disclosed diffuse infiltration by round, small- to medium-sized cells with moderate basophilic cytoplasm. The cells had round or oval folded nuclei containing dispersed chromatin. The blastic features were compatible with very immature myeloid cells, and the cells were strongly positive for CD34, CD117, and myeloperoxidase, a pathologic picture diagnostic of MS (Fig. 3). At the time of writing, the patient was being treated with a combination of cytarabine and anthracyclines (3-plus-5 scheduled regimen), and stem cell mobilisation was obtained for eventual rescue treatment.


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

Girelli CM, Carsenzuola V, Latargia M, Aguzzi A, Serio G - Int J Surg Case Rep (2014)

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).
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4200883&req=5

fig0015: 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).
Mentions: A previously healthy 64-year-old woman was admitted to our gastrointestinal unit for unexplained chronic diarrhoea and a loss of more than 10% of her usual body weight. Watery, non-bloody diarrhoea had started six weeks before admission. Blood analyses performed four weeks before admission showed mild normocytic anaemia, low serum albumin, and hypokalaemia. Total serum immunoglobulin A was normal. Anti-transglutaminase antibodies were negative, along with stool examination (including culture and a search for ova and parasites), oesophagogastroduodenoscopy with duodenal biopsies, and ileocolonoscopy with biopsies performed two weeks before admission. The patient appeared pale and thin, with dry skin and mucous membranes. Her abdomen was flat and soft, and deep palpation did not arouse pain or guarding. Her liver and spleen were not felt, and bowel sounds were present. A blood cell count showed 11.2 × 109/L white blood cells, with 22% of immature cells in a peripheral smear. After fluid and lytes replacement, a bone marrow biopsy was performed, which disclosed immature cells: namely, 30% of blasts, CD45+, CD34+, CD117+, CD33+, CD13+, and HLADR+; normal karyotype (46XX); FLT3 0; and NPM: absence of mutations. A diagnosis of de novo AML, FAB M1 was made. As chemotherapy was not possible due to the persisting large volume of diarrhoea, small-bowel capsule endoscopy (SBCE) was performed (PillCam SB3, Given Imaging). One hour and twenty-two minutes after ingestion, the capsule did not pass through an ileal stricture next to the dilated lumen, filled with luminal debris. The mucosa was thickened and pale, with short and swollen villi (Fig. 1). The following day, vomiting, abdominal pain, and distension ensued, and abdominal CT disclosed a stricture of the distal ileum next to the retained capsule, with proximal bowel dilation. On the 10th day of the hospital stay, laparotomy was performed. When the peritoneum was opened, a marked enlargement of the small bowel adjacent to an annular stenosis, 15 cm proximal to the ileocaecal valve, was found (Fig. 2). Segmental ileal resection with manual latero-lateral anastomosis was performed. The still-flashing capsule was retrieved within the resected bowel. The postoperative course was uneventful, with early oral feeding, bowel canalisation, and full resolution of the diarrhoea. Pathologic examination of the resected specimen disclosed diffuse infiltration by round, small- to medium-sized cells with moderate basophilic cytoplasm. The cells had round or oval folded nuclei containing dispersed chromatin. The blastic features were compatible with very immature myeloid cells, and the cells were strongly positive for CD34, CD117, and myeloperoxidase, a pathologic picture diagnostic of MS (Fig. 3). At the time of writing, the patient was being treated with a combination of cytarabine and anthracyclines (3-plus-5 scheduled regimen), and stem cell mobilisation was obtained for eventual rescue treatment.

Bottom Line: The diarrhoea promptly resolved after surgery, and the patient is now undergoing chemotherapy.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.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Gastroenterology and Digestive Endoscopy Unit, Hospital of Busto Arsizio, Busto Arsizio, VA, Italy. Electronic address: cargirel@vodafone.it.

No MeSH data available.


Related in: MedlinePlus