Granulocytic sarcoma (chloroma) is a mass of malignant early myeloid precursor cells in an extramedullary location [1, 2]. Rarely, the ovary may be the first site for clinical manifestation of granulocytic sarcoma. The estimated incidence of acute myelocytic leukemia (AML) in pregnancy is 1 in 75,000 [3]. Granulocytic sarcoma of the ovary in association with AML has been rarely reported in the literature. However, only a few such cases have been reported in association with pregnancy [2].
A 20-year-old primigravida presented to our obstetric department with severe pallor, lethargy, epistaxis, inability to walk, and unilateral leg pain. She had a single live fetus with cephalic presentation of 30-weeks 4 days gestation from her last menstrual period (LMP). She was a carrier of beta-thalassemia. On examination, she had severe pallor. Her blood pressure was 100/60 mmHg and pulse rate was 94/min. On admission, her hemoglobin level was 6.2 g%. Peripheral blood smear showed hypochromic, microcytic red blood cells, target cells, and plenty of immature blast cells. Her platelet count was 22,000/mm3 and blast cells were 75 %. The bone marrow was markedly hypercellular with 40 % of blast cells. These cells showed few nuclear chromatins, prominent nucleoli, and basophilic cytoplasm (Fig. 1); flow cytometry showed CD13, CD33, CD 34 positivity. Cytogenetic study showed a normal XX pattern. Based on the clinical features and laboratory findings, a diagnosis of AML FAB M2 Subtype (acute myeloblastic leukemia with granulocytic maturation) was made. Ultrasonography showed single live fetus with cephalic presentation with gestational age of 32 weeks 5 days; the placenta was situated in the anterior part of upper segment of the uterus with grade II maturity, and liquor amnii was adequate in volume. She was transfused 10 U of packed RBC and 24 U of platelet concentrates. Her pregnancy was termi- nated at 33 weeks by lower segment cesarean section (as per advice of hematologist) in order to start chemotherapy. Corticosteroid was administered for lung maturity of the fetus 4 days before termination. The patient delivered a preterm female baby with 1.7 kg birth weight and good Apgar scores.
Per-operatively, we noticed two bilateral reniform ovarian masses each measuring 12.5 9 12.5 cm (Fig. 2). The masses were removed and sent for histopathological examination. Histopathology revealed that there was complete replacement of ovarian parenchyma by eosino- philic cells of myeloid origin (Fig. 3) and a diagnosis of granulocytic sarcoma was made. After an uneventful postoperative recovery, she was transferred to a regional cancer hospital for chemotherapy where she was treated with cytarabine and daunorubicin (7 ? 3 days regimen). Cytarabine 100 mg/m2 daily from day 1 to 7 and daunorubicin 60 mg/m2 daily iv from day 1 to 3 were given.
However, the patient suffered from severe chest infections leading to septicemia which was non-responsive to intravenous ceftriaxone, metronidazole and amikacin antibiotics, and the patient died on the 8th day of completion of chemotherapy.
Granulocytic sarcoma was also known as chloroma [1, 2]. This name was derived from the Greek word ‘‘chloros’’ (green) as these tumors often have a greenish tint due to presence of myeloperoxidase. Rappaport renamed it gran- ulocytic sarcoma in 1967. Currently, any extramedullary manifestation of acute myeloid leukemia may be termed as granulocytic sarcoma or chloroma.
It occurs only in 32.3 % of patients with granulocytic leukemia, being clinically evident in less than 1 % of the patients. Granulocytic sarcoma may involve virtually any organ or tissue. The bone is the commonest site of involvement [2]. Involvement of the ovaries may occur rarely. Biopsy of the lesion followed by histopathological examination formed the basis of the diagnosis.
Association of granulocytic sarcoma in a patient with AML indicates poor response to treatment and poor prog- nosis [1]. The tumor is quite sensitive to antileukemic chemotherapy. If sarcoma is persistent even after comple- tion of induction chemotherapy, local surgery or radiation therapy is often considered. In our patient, the tumor was found during cesarean section and in order to establish a diagnosis, excisional biopsy was considered.
