|Year : 2016 | Volume
| Issue : 2 | Page : 48-50
Nonleukemic myeloid sarcoma presenting as intussusception of small bowel in a male patient: An unusual presentation
Sonmez Ocak1, Huseyin Alakus2, Mehmet Esat Duymus2, Mustafa Kaya2, Kursat Karadayi2, Nese Yeldir3, Hatice Ozer3, Elif Colak1
1 Department of General Surgery, Samsun Training and Research Hospital, Samsun, Turkey
2 Department of Surgical Oncology, Cumhuriyet University Hospital, Sivas, Turkey
3 Department of Pathology, Cumhuriyet University Hospital, Sivas, Turkey
|Date of Submission||19-Jan-2016|
|Date of Acceptance||29-Mar-2016|
|Date of Web Publication||1-Jul-2016|
Department of General Surgery, Samsun Training and Research, Baris Bulvari, No. 199, Ilyaskoy, Samsun 55300
Source of Support: None, Conflict of Interest: None
Myeloid sarcoma (MS) is an extramedullary tumor of the myeloid cells and usually develops in lymphoid organs, bones, skin, adipose tissue, and the central nervous system. Gastrointestinal or isolated mesenteric involvement is an extremely rare without bone marrow (BM) involvement. Only a few cases had reported before. In this paper, we report a case of a 37-year-old male who presented with small bowel intussusception due to mesenteric MS. Abdominal computed tomography findings revealed intussusception of the small bowel in the left lower quadrant of the abdomen and mesenteric masses. The patient underwent operation, and masses were excised with segmental small bowel and segmental colon resection because the masses were adjacent to the bowel wall. Histopathology and immunohistochemistry showed MS in mesentery. Patient received 2 cycles of cytosine arabinoside combined with idarubicin chemotherapy in the postoperative period and was doing well at 6 months of follow-up. MS in mesentery presenting as intussusception of small bowel without BM involvement is very rare and it should be considered in the differential diagnosis of intestinal obstruction.
Keywords: Acute myeloid leukemia, chemotherapy, intussusception, myeloid sarcoma
|How to cite this article:|
Ocak S, Alakus H, Duymus ME, Kaya M, Karadayi K, Yeldir N, Ozer H, Colak E. Nonleukemic myeloid sarcoma presenting as intussusception of small bowel in a male patient: An unusual presentation. Transl Surg 2016;1:48-50
|How to cite this URL:|
Ocak S, Alakus H, Duymus ME, Kaya M, Karadayi K, Yeldir N, Ozer H, Colak E. Nonleukemic myeloid sarcoma presenting as intussusception of small bowel in a male patient: An unusual presentation. Transl Surg [serial online] 2016 [cited 2019 Aug 17];1:48-50. Available from: http://www.translsurg.com/text.asp?2016/1/2/48/185200
| Introduction|| |
Myeloid sarcoma (MS), also called "granulocytic sarcoma or chloroma," was first described by Burns  in 1811.  It is a rare extramedullary tumor of myeloblasts or immature myeloid cells.  For surgeons this is not a customary situation because the disease usually occurs in connective tissues such as skin, bone, adipose tissue, and lymph nodes. , It can be seen during the treatment or in relapse in acute leukemia, especially in acute myeloid leukemia (AML). Moreover, it can be associated with myelodysplastic syndrome, chronic myeloid leukemia, and other myeloproliferative diseases. , Some cases can be nonleukemic and present an isolated mass that called primary or an isolated MS. , It is an extremely rare condition and the incidence of the disease is about 2/1,000,000 in adults.  Gastrointestinal MS commonly involves small bowel and mass in bowel usually infiltrates adjacent mesentery, omentum, or peritoneum. ,,,, However, gastrointestinal MS without AML involvement is rare.  Here, we report a rare case of isolated mesenteric MS presenting as intussusception of small bowel without bone marrow (BM) involvement.
| Case Report|| |
A 37-year-old male was admitted to the emergency room complaining of vomiting and abdominal pain of 2-day duration. According to the patient history, he had suffered from celiac sprue for 5 years. He did not have history of any hematological malignancy. The abdominal examination revealed a mildly distended abdomen with moderate tenderness over the epigastrium. No rebound tenderness or guarding was present. Bowel sound was slightly increased. Neither cervical nor inguinal lymph nodes were palpated. In complete blood count, hemoglobin was 10.2 g/L, total leukocyte count was 10.2 × 10  /μL, and platelet count was 560 × 10  /μL. The leukocyte differential count revealed that the neutrophils were 79.4%, lymphocytes were 13.7%, monocytes were 4.7%, and eosinophils were detected to be 1.8%. Laboratory examinations including liver and renal function test were within normal limit. The standard abdominal X-ray imaging revealed a small bowel obstruction. A computed tomography (CT) scan of the abdomen showed intussusception of the small intestine in the left lower quadrant of the abdomen and 2 mesenteric masses [Figure 1]. On laparotomy, there was intussusception of jejunum due to the mesenteric mass of 5 cm × 5 cm. Another mass of 9 cm × 5 cm was located in the mesentery of descending colon. Segmental resection of jejunum and descending colon with mesenteric masses were performed with jejunojejunal and colocolonic anastomoses. There was no other abnormal finding in other organs or lymph nodes. The patient was allowed oral intake at the 5 th postoperative day, and he was discharged on the 7 th day after surgery.
|Figure 1: Computed tomography images of the patient. (a) White arrow shows the invagination of the small bowel. (b) White arrows show the mesenteric masses|
Click here to view
Histological findings showed diffuse infiltration by malignant cells with blastic features compatible with very immature myeloid cells. Immunohistochemical (IHC) stains performed on formalin-fixed, paraffin-embedded tissue sections demonstrated that the tumor cells expressed myeloperoxidase (MPO) and CD43 (strongly positive) and CD68 (weak). They were negative for CD3, CD5, CD117, and CD 34 [Figure 2]. A peripheral blood smear revealed no circulating blasts, and the BM aspirate showed no abnormal morphology. The patient received 2 cycles of cytarabine 334 mg per day for 7 days combined with idarubicin 20 mg for 3 days as a first-line treatment of AML. The patient was evaluated with positron emission tomography-CT scan 3 months after the treatment. Till date, the patient remained symptom-free and has shown no evidence of AML or relapse after 6 months.
|Figure 2: Photomicrographs of specimen. (a) atypic cells are infiltrating bowel serosa (H and E, ×10). (b) CD43 positivity can be seen as a dark brown on the cell surface (×20). (c) Myeloperoxidase is strongly positive in the cytoplasm|
Click here to view
| Discussion|| |
MSs are rare extramedullary tumors of immature myeloid cells and these tumors are often associated with AML, chronic myeloid leukemia, myeloproliferative, and myelodysplastic disorders either at the initial diagnosis or at relapse of those diseases. , MS can occur at every stage of AML, and some cases may be nonleukemic. In the latter cases, the diagnosis can be difficult and up to 55% cases misdiagnosed as lymphoma. ,,,,, The incidence of MS in AML is 3-8%. , It commonly occurs in skin, bone, and lymph nodes. ,, Gastrointestinal sarcoma is less common and mostly seen in small bowels. ,,,, Neiman et al. found that overall GI involvement occurred only in 7% of the cases. Similar to our case, most common symptoms of these patients were abdominal pain and vomiting. Mesenteric involvement is a rare, and a few cases are reported in the literature. ,, Isolated mesenteric MS occurring at initial diagnosis without BM involvement has been reported only twice. , At the time of diagnosis, the presence of an isolated MS with normal BM findings has been reported in only 30% of cases. In most cases, acute myelocytic leukemia is ultimately diagnosed after 1-49 months. Therefore, regarding the time of diagnosis of MS, the incidence and clinical course of the hematopoietic disorder are different. ,
Histochemical and immunoperoxidase stains are used for correct diagnosis. Standard hematoxylin and eosin stains are ineffective for differential diagnosis, so using some (IHC) markers such as MPO, CD117 is critical. ,,, MPO expression was strongly positive in our case similar to these reports.
Treatment modalities of MS include systemic chemotherapy, radiation therapy, and BM transplantation.  Chemotherapy is the main treatment, and surgery is indicated only in the event of complications, such as bowel obstruction, bleeding, or perforation.  The incidence of AML or extramedullary relapse was significantly higher in patients who were treated with surgery only.  A nonleukemic period longer than 2 months was observed in 19% of patients who received systemic treatment, and in only 5% of patients who were not treated systemically.  The rapid initial diagnosis of MS and immediate treatment with either chemotherapy, radiation, surgery, or a combination of any of these modalities are associated with a better prognosis, as misdiagnosis or treatment delays can result in disease progression to AML.  The median survival of MS patients without AML has been reported to be 36 months, whereas those progressing to AML have a poor prognosis with median survival 6-14 months. , Prognosis of isolated mesenteric MS is not well known due to lack of large case series; however, common belief that it has a poor prognosis. ,
As a consequence, MS of gastrointestinal system usually occurs in small bowel and isolated mesenteric MS without BM involvement is an extremely rare condition. It can be a diagnostic challenge, especially in patients with no previous leukemic disease. In the evaluation of an abdominal mass causing small bowel obstruction or intussusception, MS should be considered in the differential diagnosis.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Burns A, editor. Observations of surgical anatomy. In: Head and Neck. Edinburgh: Thomas Royce and Company; 1811. p. 364-6.
King A. A case of chloroma. Mon J Med
Brunning RD, Matutes E, Flandrin G, Vardiman J, Bennett J, Head D, et al
. Acute myeloid leukemia not otherwise categorized: Myeloid sarcoma. In: Jaffe ES, Harris NL, Stein H, Vardiman JW, editors. World Health Organization Classification of Tumours: Pathology and Genetics of Tumours of Hematopoietic and Lymphoid Tissues. Lyon, France: IARC Press; 2001. p. 104-5.
Yamauchi K, Yasuda M. Comparison in treatments of nonleukemic granulocytic sarcoma: Report of two cases and a review of 72 cases in the literature. Cancer
Billio A, Pianezze G, Amato B, Fabris P. Isolated peritoneal granulocytic sarcoma with molecular and chromosomal bone marrow involvement. Haematologica
Breccia M, Mandelli F, Petti MC, D′Andrea M, Pescarmona E, Pileri SA, Carmosino I, Russo E, De Fabritiis P, Alimena G. Clinico-pathological characteristics of myeloid sarcoma at diagnosis and during follow-up: Report of 12 cases from a single institution. Leuk Res
Kohl SK, Aoun P. Granulocytic sarcoma of the small intestine. Arch Pathol Lab Med
Neiman RS, Barcos M, Berard C, Bonner H, Mann R, Rydell RE, Bennett JM. Nonleukemic granulocytic sarcoma presenting as intussusception of small bowel. Int J Clin Oncol
Neiman RS, Barcos M, Berard C, Bonner H, Mann R, Rydell RE, Bennett JM. Granulocytic sarcoma: A clinicopathologic study of 61 biopsied cases. Cancer
Sivan-Hoffmann R, Waksman I, Cohen HI, Eitan A. Small bowel obstruction as a presenting sign of granulocytic sarcoma. Isr Med Assoc J
Narayan P, Murthy V, Su M, Woel R, Grossman IR, Chamberlain RS. Primary myeloid sarcoma masquerading as an obstructing duodenal carcinoma. Case Rep Hematol
Fujieda A, Nishii K, Tamaru T, Otsuki S, Kobayashi K, Monma F, Ohishi K, Nakase K, Katayama N, Shiku H. Granulocytic sarcoma of mesentery in acute myeloid leukemia with CBFB/MYH11 fusion gene but not inv (16) chromosome: Case report and review of literature. Leuk Res
Göhring G, Erlacher M, van Buiren M, Jüttner E, Niemeyer CM, Schlegelberger B. Mesenteric chloroma with t (16;16) followed by acute myelomonocytic leukemia with clonal evolution. Cancer Genet Cytogenet
Ohanian M, Huang RS, Yakoushina TV, Estrov Z, Juneja H, Chen L, Idowu M, Abruzzo LV. Isolated mesenteric CD20-positive myeloid sarcoma. Clin Lymphoma Myeloma Leuk
Jung SH, Kim HC, Yu CS, Kim JC. Solitary preleukemic granulocytic sarcoma as a cause of small bowel obstruction. Gut Liver
Antic D, Elezovic I, Bogdanovic A, Vukovic NS, Pavlovic A, Jovanovic MP, Jakovic L, Kraguljac N. Isolated myeloid sarcoma of the gastrointestinal tract. Intern Med
Antic D, Elezovic I, Milic N, Suvajdzic N, Vidovic A, Perunicic M, Djunic I, Mitrovic M, Tomin D. Is there a "gold" standard treatment for patients with isolated myeloid sarcoma? Biomed Pharmacother
Imrie KR, Kovacs MJ, Selby D, Lipton J, Patterson BJ, Pantalony D, Poldre P, Ngan BY, Keating A. Isolated chloroma: The effect of early antileukemic therapy. Ann Intern Med
Yilmaz AF, Saydam G, Sahin F, Baran Y. Granulocytic sarcoma: A systematic review. Am J Blood Res
[Figure 1], [Figure 2]