WCRJ 2019; 6: e1429 MYELOID SARCOMA OF THE SPLEEN: REPORT OF A RARE CASE WITH REVIEW OF LITERATURE M. RAMEZANI1, K. SEIDI2, F. AMIRIAN1, M. SADEGHI3 1Molecular Pathology Research Center, Imam Reza Hospital, Kermanshah University of Medical Sciences, Kermanshah, Iran 2Students Research Committee, Kermanshah University of Medical Sciences, Kermanshah, Iran 3Medical Biology Research Center, Kermanshah University of Medical Sciences, Kermanshah, Iran Abstract – Objective: Myeloid sarcomas (MSs) are rare tumors of myeloid blasts and immature cells. Rare cases of splenic involvement are on record. We report a case of MS in a young female which is confirmed on pathologic examination of the spleen. Patients and Methods: A 20-year-old female was admitted with the chief complaints of ab- dominal pain, fever, anorexia, and dyspnea. She was a known case of acute myelogenous leukemia (AML). The spiral computed tomography scan without contrast showed a massive splenic enlarge- ment. The ultrasound examination showed liver and spleen were larger than normally measured 190 and 210 mm, respectively, with normal parenchymal echo. Results: The patient underwent surgery with clinical suspicion of MS. The specimen consisted of the red spleen with several infarcted areas totally measuring 22x14x10 cm and weighing 1885 g. The pathology report was splenic infarction with myeloid sarcoma. Conclusions: The risk of misdiagnosis is high in the pathologic exam, especially in non-leukemic forms and low clinical suspicion. It is important to consider this entity in the differential diagnosis of splenomegaly or splenic mass in any age group. KEYWORDS: Myeloid sarcoma, Chloroma, Granulocyic sarcoma, Spleen, Case report. INTRODUCTION CASE REPORT Granulocytic sarcomas, chloromas or myeloid sar- A 20-year-old female was admitted on 19th January comas (MS) are rare tumors originating from my- 2017 at the Oncology Department with the chief eloid blasts and immature cells and most data in the complaints of abdominal pain, fever, anorexia, and literature are from case reports and case series1-3. It dyspnea. She was a known case of AML since 2 is estimated with an incidence of up to 9% of acute months before. On 6th November 2016, bone mar- myelogenous leukemias (AMLs)4. Some research- row aspiration and trephine biopsy were carried ers stated an incidence of as low as 0.8% of AMLs5. out and the pathologist reported: Hyper cellular This tumor is reported in bone, periosteum, soft marrow (cellularity 100%) containing 50% atypi- tissue, lymph node, skin, and other organs6. The cal monocytoid cells suggestive for acute myelog- most common site of presentation is demonstrated enous leukemia (FAB-M5). At the same time, a in connective/soft tissue followed by skin/breast5. computed tomography (CT) scan of the lung and The involvement of the liver, spleen, brain, and oth- mediastinum demonstrated massive effusion with er organs is also on the report7. Herein, we report a collapse and consolidation in the left hemitho- case of MS in a young female which is confirmed rax and shift of the heart and mediastinum to the on splenectomy pathologic diagnosis according to right. There was segmental atelectasis in the left morphology and clinical data. para cardiac region. The spiral CT scan without Corresponding Author: Masoud Sadeghi, MSc; e-mail: [email protected] 1 contrast showed a moderate amount of fluid in the Li8 reported 39 cases of myeloid sarcoma with a left pleural space and parenchymal opacity in the mean age of 33.4 years; one of them was originating basal segment of the left lower lobe and lower seg- from the spleen with clinical expression of massive ment of lingual suggestive for the infectious pro- splenomegaly and traumatic rupture. Most of the cess. The massive splenic enlargement was noted. cases showed poor prognosis. Goyal et al5 reported The patient was diagnosed with left pulmonary 746 cases of myeloid sarcoma with a median age of emboli and chest tube indwelled. The ultrasound 59 years. They considered cases of lymph node and examination at the same time reported: gall blad- splenic involvement in one prognostic group and der, kidneys, urinary bladder, endometrium, and considered them as poor prognosis along with cases ovaries were within normal limits. Few lymph of the nervous system, soft tissue, mediastinum, and nodes in porta hepatis measuring 20 x 9 mm were bone. Patients with less than 70 years had a better noted. The liver and spleen were larger than nor- prognosis than the older ones. Median overall sur- mally, measuring 190 and 210 mm, respectively, vival was worse in men, blacks, and poor prognostic with normal parenchymal echo. The patient re- sites5. Reports of cases with unusual age and pre- ceived chemotherapy. Bone marrow aspiration and sentation are on record. Kim and Kim9 reported trephine biopsy on 19th December 2016 were nor- myeloid sarcoma of the breast with hamartoma-like mocellular marrow with 5-10% monocytoid cells features in ultrasound examination; finally biopsy and less than 5% of blasts. Finally, on 19th January confirmed the diagnosis. Marwah et al 10 reported 2017, the patient underwent splenectomy with clin- myeloid sarcoma as a large temporal bone mass in ical suspicion of MS. The specimen consisted of a child with Down syndrome as the initial manifes- the red spleen with several infarcted areas totally tation of the disease. They emphasized on diverse measuring 22x14x10 cm and weighing 1885 g. The sites of presentation and the need for rapid diagnosis pathologist reported: compatible with splenic in- to achieve better survival. Others11 confirmed this farction with leukemic involvement (granulocytic idea with a report of the mass in paranasal sinus sarcoma; Figures 1, 2, and 3). Isolated lymph node with presentation as epistaxis. Moreover, Mitkow- showed lymphoma–leukemia involvement. The pa- ski and Gil12 reported the eyelid of a 36 years old tient achieved relief of symptoms after splenecto- woman. Tokunaga et al13 reported a man with pan- my and discharged with a prescription. The patient creatic mass as the manifestation of Granulocytic was in good condition in the last admission for the sarcoma that showed complete remission after he- removal of the right porto-jugular catheter on 24th matopoietic stem cell transplantation. Rao et al 14 re- April 2018. ported a case of non-leukemic granulocytic sarcoma of the spleen in an infant and emphasized early di- agnosis. One study15 reported a 58 years old woman DISCUSSION with acute onset of fever and abdominal pain, which was diagnosed as acute myelogenous leukemia, MS can occur in a wide range of ages from 1 to 81 FAB M2 (French-American-British classification). years3. The most common presentation is compres- Core needle biopsy of the splenic mass confirmed sion effect, pain, and abnormal bleeding3. Wang and the diagnosis of granulocytic sarcoma; however, de- Fig. 1. Diffuse infiltration of tumor cells with high nucleo-cytoplasmic ratio in the spleen, myeloid sarcoma of the spleen. Hematoxylin- Eosin (HE) staining (X40 magnification). 2 MYELOID SARCOMA OF THE SPLEEN: REPORT OF A RARE CASE WITH REVIEW OF LITERATURE Fig. 2. Diffuse infiltration of tumor cells with high nucleo-cytoplasmic ratio in the spleen, myeloid sarcoma of the spleen. Hematoxylin- Eosin (HE) staining (X100 magnification). layed targeted therapy culminated in mortality. The apy, surgery, radiotherapy, hematopoietic stem cell authors emphasized on early diagnosis and treat- transplantation, and targeted therapy are different ment of solitary splenic mass in a patient with acute treatment modalities that are indicated according to leukemia. the patient’s condition, presentation and stage of the The risk of misdiagnosis is high in the patholog- disease20. ic examination, especially in nonleukemic forms and low clinical suspicion16. In the experience of Neiman et al6 on 61 biopsies, most tumors were CONCLUSIONS originally diagnosed as lymphoma due to poor differentiation. Histopathology and immunohisto- Myeloid sarcoma is rare in the spleen and the risk of chemistry studies are helpful for correct diagno- misdiagnosis is high in the pathologic exam, espe- sis17,18. A research19 reported 25 cases of myeloid cially in non-leukemic forms and low clinical sus- sarcoma. The most common histopathology pattern picion but it is important to consider this entity in was the diffuse arrangement of pleomorphic cells the differential diagnosis of splenomegaly or splenic with irregular nuclei, prominent nucleoli, and abun- mass in any age group. Early diagnosis and appro- dant eosinophilic cytoplasms. Systemic chemother- priate treatment may result in better survival. Fig. 3. Diffuse infiltration of tumor cells with high nucleo-cytoplasmic ratio in the spleen, myeloid sarcoma of the spleen. Hematoxylin- Eosin (HE) staining (X400 magnification). 3 DECLARATION OF PATIENT CONSENT: 7. Mei KD, Lin YS, Chang SL. Myeloid sarcoma of the cheek and the maxillary sinus regions. J Chin Med Assoc 2013; 76: 235-238. The authors certify that they have obtained all ap- 8. Wang HQ, Li J. Clinicopathological features of myeloid propriate patient consent forms. In the form the pa- sarcoma: Report of 39 cases and literature review. tient(s) has/have given his/her/their consent for his/ Pathol Res Pract 2016; 212: 817-824. her/their images and other clinical information to be 9. Kim SJ, Kim WG. Sonographic features of a myeloid reported in the journal. The patients understand that sarcoma of the breast as a relapse of acute myeloid leukemia after stem-cell transplantation: a case report. their names and initials will not be published and Am J Case Rep 2019; 20: 612-619. due efforts will be made to conceal their identity, 10. Marwah N, Bhutani N, Budhwar A, Sen R. Isolated but anonymity cannot be guaranteed. myeloid sarcoma of the temporal bone: As the first clinical manifestation of acute myeloid leukemia in a patient of down’s syndrome. Int J Surg Case Rep 2019; ACKNOWLEDGMENT: 58: 77-80.