Gastroenterol Res

Gastroenterology Research, ISSN 1918-2805 print, 1918-2813 online, Open Access

Article copyright, the authors; Journal compilation copyright, Gastroenterol Res and Elmer Press Inc

Journal website https://www.gastrores.org

Case Report

Volume 15, Number 1, February 2022, pages 39-46

Isolated Enteric Myeloid Sarcoma as a Rare Etiology of Small Bowel Obstruction in a Young Female Patient

Figure 1. Axial (a) and coronal (b) images of CT of abdomen revealing mildly dilated small bowel loops with a transition point to the right of the midline (red arrow in a) in an area of a spiculated mass in the distal ileum simulating carcinoid tumors (red stars in a and b). CT: computed tomography.

Figure 2. Hematoxylin and eosin-stained sections of the small bowel mass. (a) Diffuse transmural infiltration with a dense population of polymorphous medium and large-sized immature cells with scant eosinophilic cytoplasm (× 2). (b, c) Immature neoplastic cells with rounded hyperchromatic nuclei and scattered eosinophils (× 200).

Figure 3. Immunohistochemistry staining of myeloid cells (× 200). Positive staining with myeloperoxidase (a) and CD34 (b).

**Table 1.** Summary of Characteristics of Reported Cases of Small Bowel Myeloid Sarcoma (MS) From 2003 to 2021
Number	Authors/publication year	Age (years)/gender	Clinical presentation	Anatomical site(s)	Concurrent AML	Management and outcome
AML: acute myeloid leukemia; BMT: bone marrow transplant; CT: computed tomography.
1	Aslan et al, 2019 [1]	57/female	Abdominal pain, nausea, vomiting, and constipation. Differential diagnosis included small bowel carcinoma and lymphoma on imaging.	Distal ileum	No	Surgical resection. Further management and outcome not described.
2	Cicilet et al, 2017 [2]	45/female	Lower abdominal pain and vomiting. Differential diagnosis on imaging included small bowel carcinoma and lymphoma.	Distal ileum	No	Surgical resection followed by chemotherapy.
3	Gajendra et al, 2014 [3]	35/male	Abdominal pain, distension, vomiting, and constipation for 3 months. Ileo-ileal intussusception on imaging.	Proximal jejunum	Concurrent AML with inv(16) and trisomy 22	Surgical resection followed chemotherapy. Achieved bone marrow remission post-induction chemotherapy.
4	Girelli et al, 2014 [4]	64/male	Chronic watery diarrhea and unintentional weight loss for 6 weeks. Strictures at distal ileum.	Distal ileum	Concurrent de novo AML	Surgical resection followed by chemotherapy. Stem cell mobilization was considered.
5	Hotta et al, 2013 [5]	56/male	Recurrent vomiting. Obstructing mass on imaging. Biopsy obtained through double-balloon endoscopy.	Proximal jejunum	No	Surgical resection followed by adjuvant chemotherapy. Achieved remission without recurrence up to 54 months.
6	Mizumoto et al, 2020 [6]	54/male	Abdominal pain and vomiting, misdiagnosed initially as colitis. Recurrence of symptoms and laparoscopic revealed stenotic jejunal mass.	Mid-jejunum	No	Surgical resection followed by systemic chemotherapy with complete remission.
7	Jung et al, 2007 [7]	48/male	Post-prandial pain and discomfort for 9 months.	Distal ileum with mesenteric disease	Concurrent AML diagnosed 4 weeks post-surgery	Surgical resection followed by systemic chemotherapy for AML and a complete remission achieved. Bone marrow transplant performed 6 months post-remission.
8	Kitagawa et al, 2008 [8]	33/female	Abdominal pain, vomiting and constipation. Imaging revealed two ileal masses.	Two distal ileal mases with mesenteric lymphadenopathy	No	Surgical resection followed by systemic chemotherapy. Bone marrow transplant with complete remission for 50 months.
9	Mrad et al, 2004 [9]	13/female (first pediatric case)	Abdominal swelling. Two mesenteric masses on imaging with bowel obstruction.	Unspecific small bowel site with mesenteric disease	No	Surgical resection followed by systemic chemotherapy with complete remission.
10	Kwan et al, 2011 [10]	39/female	Lower abdominal pain, nausea and vomiting for 2 months, initially misdiagnosed as Crohn’s disease, and received several steroids courses. Ileal biopsies through colonoscopy revealed MS.	Distal ileum	No	Systemic chemotherapy followed by surgical resection. Disease-free survival for at least 48 months.
11	Lee et al, 2008 [11]	45/male	Intermittent abdominal pain and projectile vomiting for 4 days. Small bowel intussusception on imaging.	Distal jejunum or proximal ileum	No	Surgical resection followed by systemic chemotherapy with complete remission. Disease-free survival at least for 12 months.
12	Lim et al, 2016 [12]	55/male	Abdominal fullness and dyspepsia. Obstructing mass on CT and colonoscopy.	Distal ileum	Unknown	Not described.
13	McCusker et al, 2016 [13]	22/female	Abdominal pain. Initially misdiagnosed as appendicitis. Ongoing small bowel obstruction and laparoscopy revealed multiple small bowel masses with extensive lymphadenopathy simulating lymphoma.	Unspecified small bowel site	No	Surgical resection followed by systemic chemotherapy with complete remission. Disease-free survival for at least 13 months.
14	McKenna et al, 2009 [14]	50/male	Abdominal pain, vomiting and weight loss for 6 weeks.	Mid-ileum	No	Surgical resection followed by systemic chemotherapy with complete remission. Disease-free survival at least for 48 months.
15	Ioannidis et al, 2009 [15]	48/male	Epigastric pain, distension, and vomiting.	Proximal jejunum with mesenteric disease	No	Surgical resection followed by systemic chemotherapy. Complete remission with no recurrence at a 6-month follow-up.
16	Kumar et al, 2009 [16]	55/female	Left lower abdominal pain, alternating bowel habits, and weight loss.	Unspecified small bowel site, multiple mesenteric lesions	No	Surgical resection followed by systemic chemotherapy with complete remission.
17	Nakanshi et al, 2021 [17]	40/male	Bloody diarrhea in a patient with a known AML. Endoscopy revealed ulcerative ileal lesions.	Distal ileum	Concurrent AML preceding MS diagnosis	Not described.
18	Palanivelu et al, 2009 [18]	52/male	Abdominal pain and vomiting for 5 days	Proximal jejunum	No	Surgical resection and scheduled surveillance for leukemia.
19	Plowman et al, 2016 [19]	41/male	Persistent abdominal pain and constipation. Mesenteric mass on imaging. Differentials included small bowel carcinoma, carcinoid tumor or lymphoma.	Unspecific small bowel site with mesenteric disease	No	Surgical resection followed by systemic chemotherapy.
20	Van de Voorde et al, 2017 [20]	46/male	Epigastric pain and nausea. Bowel obstruction by ileal mass on imaging.	Ileum with mesenteric disease	No	Surgical resection followed by systemic chemotherapy and scheduled for a stem cell transplant.
21	Wang et al, 2017 [21]	25/male	Intermittent upper abdominal pain for 6 months.	Jejunum with mesenteric, regional lymph nodes and kidney disease	Initially no AML. Relapsing small bowel MS with AML after 3 months	Surgical resection. Initially declined chemotherapy. Relapsing small bowel MS with concurrent AML after 3 months. Chemotherapy with complete remission.
22	Lai PB, 2005 [22]	36/male	Recurrent central abdominal pain for 6 months. Mid-ileal annular obstructing lesion on imaging.	Mid-ileum	No	Surgical resection followed by systemic chemotherapy. No recurrence for at least 1 year.
23	Yoshida et al, 2019 [23]	47/male	Abdominal pain, vomiting, and diarrhea for 4 days. Thickened masses at distal ileum and appendix.	Distal ileum and appendix	No	Surgical resection followed by systemic chemotherapy.
24	Kim et al, 2012 [24]	49/male	Abdominal pain for 2 weeks. Obstructing proximal jejunal mass.	Proximal jejunum	Unknown	Surgical resection. Multiple liver and lung suspicious lesions. Histopathological exploration declined by patient, who died after short time.
25	Alvarez et al, 2011 [25]	41/male	Intermittent abdominal and vomiting for 5 days. Multiple peritoneal masses and ascites on imaging.	Proximal jejunum. Diffuse omental and peritoneal disease	AML with inv(16) and CBF-MYH11	Surgical resection followed by systemic chemotherapy.
26	Xavier et al, 2004 [26]	36/male	Acute abdomen and recurrent intestinal obstruction. Initially misdiagnosed as non-Hodgkin’s lymphoma with extensive mesenteric disease on imaging.	Distal ileum with mesenteric involvement	No	Surgical resection followed by systemic chemotherapy to achieve bone marrow remission. Ileal recurrence and second surgery followed by autologous BMT.
27	Abou-Ghanem et al, 2021 (present case)	30/female	Abdominal pain and vomiting 3 days. Obstructing distal ileal mass simulating carcinoid tumors on imaging.	Distal ileum	No	Surgical resection followed by systemic chemotherapy.

Table 1. Summary of Characteristics of Reported Cases of Small Bowel Myeloid Sarcoma (MS) From 2003 to 2021

Number

Authors/publication year

Age (years)/gender

Clinical presentation

Anatomical site(s)

Concurrent AML

Management and outcome

AML: acute myeloid leukemia; BMT: bone marrow transplant; CT: computed tomography.

Aslan et al, 2019 [1]

57/female

Abdominal pain, nausea, vomiting, and constipation. Differential diagnosis included small bowel carcinoma and lymphoma on imaging.

Distal ileum

Surgical resection. Further management and outcome not described.

Cicilet et al, 2017 [2]

45/female

Lower abdominal pain and vomiting. Differential diagnosis on imaging included small bowel carcinoma and lymphoma.

Distal ileum

Surgical resection followed by chemotherapy.

Gajendra et al, 2014 [3]

35/male

Abdominal pain, distension, vomiting, and constipation for 3 months. Ileo-ileal intussusception on imaging.

Proximal jejunum

Concurrent AML with inv(16) and trisomy 22

Surgical resection followed chemotherapy. Achieved bone marrow remission post-induction chemotherapy.

Girelli et al, 2014 [4]

64/male

Chronic watery diarrhea and unintentional weight loss for 6 weeks. Strictures at distal ileum.

Distal ileum

Concurrent de novo AML

Surgical resection followed by chemotherapy. Stem cell mobilization was considered.

Hotta et al, 2013 [5]

56/male

Recurrent vomiting. Obstructing mass on imaging. Biopsy obtained through double-balloon endoscopy.

Proximal jejunum

Surgical resection followed by adjuvant chemotherapy. Achieved remission without recurrence up to 54 months.

Mizumoto et al, 2020 [6]

54/male

Abdominal pain and vomiting, misdiagnosed initially as colitis. Recurrence of symptoms and laparoscopic revealed stenotic jejunal mass.

Mid-jejunum

Surgical resection followed by systemic chemotherapy with complete remission.

Jung et al, 2007 [7]

48/male

Post-prandial pain and discomfort for 9 months.

Distal ileum with mesenteric disease

Concurrent AML diagnosed 4 weeks post-surgery

Surgical resection followed by systemic chemotherapy for AML and a complete remission achieved. Bone marrow transplant performed 6 months post-remission.

Kitagawa et al, 2008 [8]

33/female

Abdominal pain, vomiting and constipation. Imaging revealed two ileal masses.

Two distal ileal mases with mesenteric lymphadenopathy

Surgical resection followed by systemic chemotherapy. Bone marrow transplant with complete remission for 50 months.

Mrad et al, 2004 [9]

13/female (first pediatric case)

Abdominal swelling. Two mesenteric masses on imaging with bowel obstruction.

Unspecific small bowel site with mesenteric disease

Surgical resection followed by systemic chemotherapy with complete remission.

Kwan et al, 2011 [10]

39/female

Lower abdominal pain, nausea and vomiting for 2 months, initially misdiagnosed as Crohn’s disease, and received several steroids courses. Ileal biopsies through colonoscopy revealed MS.

Distal ileum

Systemic chemotherapy followed by surgical resection. Disease-free survival for at least 48 months.

Lee et al, 2008 [11]

45/male

Intermittent abdominal pain and projectile vomiting for 4 days. Small bowel intussusception on imaging.

Distal jejunum or proximal ileum

Surgical resection followed by systemic chemotherapy with complete remission. Disease-free survival at least for 12 months.

Lim et al, 2016 [12]

55/male

Abdominal fullness and dyspepsia. Obstructing mass on CT and colonoscopy.

Distal ileum

Unknown

Not described.

McCusker et al, 2016 [13]

22/female

Abdominal pain. Initially misdiagnosed as appendicitis. Ongoing small bowel obstruction and laparoscopy revealed multiple small bowel masses with extensive lymphadenopathy simulating lymphoma.

Unspecified small bowel site

Surgical resection followed by systemic chemotherapy with complete remission. Disease-free survival for at least 13 months.

McKenna et al, 2009 [14]

50/male

Abdominal pain, vomiting and weight loss for 6 weeks.

Mid-ileum

Surgical resection followed by systemic chemotherapy with complete remission. Disease-free survival at least for 48 months.

Ioannidis et al, 2009 [15]

48/male

Epigastric pain, distension, and vomiting.

Proximal jejunum with mesenteric disease

Surgical resection followed by systemic chemotherapy. Complete remission with no recurrence at a 6-month follow-up.

Kumar et al, 2009 [16]

55/female

Left lower abdominal pain, alternating bowel habits, and weight loss.

Unspecified small bowel site, multiple mesenteric lesions

Surgical resection followed by systemic chemotherapy with complete remission.

Nakanshi et al, 2021 [17]

40/male

Bloody diarrhea in a patient with a known AML. Endoscopy revealed ulcerative ileal lesions.

Distal ileum

Concurrent AML preceding MS diagnosis

Not described.

Palanivelu et al, 2009 [18]

52/male

Abdominal pain and vomiting for 5 days

Proximal jejunum

Surgical resection and scheduled surveillance for leukemia.

Plowman et al, 2016 [19]

41/male

Persistent abdominal pain and constipation. Mesenteric mass on imaging. Differentials included small bowel carcinoma, carcinoid tumor or lymphoma.

Unspecific small bowel site with mesenteric disease

Surgical resection followed by systemic chemotherapy.

Van de Voorde et al, 2017 [20]

46/male

Epigastric pain and nausea. Bowel obstruction by ileal mass on imaging.

Ileum with mesenteric disease

Surgical resection followed by systemic chemotherapy and scheduled for a stem cell transplant.

Wang et al, 2017 [21]

25/male

Intermittent upper abdominal pain for 6 months.

Jejunum with mesenteric, regional lymph nodes and kidney disease

Initially no AML. Relapsing small bowel MS with AML after 3 months

Surgical resection. Initially declined chemotherapy. Relapsing small bowel MS with concurrent AML after 3 months. Chemotherapy with complete remission.

Lai PB, 2005 [22]

36/male

Recurrent central abdominal pain for 6 months. Mid-ileal annular obstructing lesion on imaging.

Mid-ileum

Surgical resection followed by systemic chemotherapy. No recurrence for at least 1 year.

Yoshida et al, 2019 [23]

47/male

Abdominal pain, vomiting, and diarrhea for 4 days. Thickened masses at distal ileum and appendix.

Distal ileum and appendix

Surgical resection followed by systemic chemotherapy.

Kim et al, 2012 [24]

49/male

Abdominal pain for 2 weeks. Obstructing proximal jejunal mass.

Proximal jejunum

Unknown

Surgical resection. Multiple liver and lung suspicious lesions. Histopathological exploration declined by patient, who died after short time.

Alvarez et al, 2011 [25]

41/male

Intermittent abdominal and vomiting for 5 days. Multiple peritoneal masses and ascites on imaging.

Proximal jejunum. Diffuse omental and peritoneal disease

AML with inv(16) and CBF-MYH11

Surgical resection followed by systemic chemotherapy.

Xavier et al, 2004 [26]

36/male

Acute abdomen and recurrent intestinal obstruction. Initially misdiagnosed as non-Hodgkin’s lymphoma with extensive mesenteric disease on imaging.

Distal ileum with mesenteric involvement

Surgical resection followed by systemic chemotherapy to achieve bone marrow remission. Ileal recurrence and second surgery followed by autologous BMT.

Abou-Ghanem et al, 2021 (present case)

30/female

Abdominal pain and vomiting 3 days. Obstructing distal ileal mass simulating carcinoid tumors on imaging.

Distal ileum

Surgical resection followed by systemic chemotherapy.