Granulocytic Sarcoma Involving the Gynecologic Tract: One Case Report and Review of the Literature

Case Report

Ann Hematol Oncol. 2015; 2(8): 1058.

Granulocytic Sarcoma Involving the Gynecologic Tract: One Case Report and Review of the Literature

Quintela A¹, Ducreux S¹, Lequeu H¹, Chassagne C², Plesa A¹, Le Jeune C¹ and Thomas X¹*

¹Department of Hematology, Lyon-Sud Hospital, France

²Centre Léon Bérard, Laboratory of Anatomopathology, France

*Corresponding author: Xavier Thomas, Department of Hematology, Lyon-Sud Hospital, Bat. 1G, 165 chemin du Grand Revoyet, 69495 Pierre Bénite Cedex, France

Received: August 24, 2015; Accepted: November 10, 2015; Published: November 15, 2015


Granulocytic sarcoma is an extramedullary tumor associated with acute myeloid leukemia (AML). It is rarely seen in the female tract. We report an unusual case of granulocytic sarcoma of the uterus in an AML patient who relapses after 14 years of first complete remission. A mixed-lineage leukemia (MLL) gene rearrangement supports the association of that malignancy with prior chemotherapy. Second line therapy consisted in chemotherapy, radiation, and allogeneic stem cell transplantation.

Keywords: Acute myeloid leukemia; Granulocytic sarcoma; Prognosis; Treatment; Gynecologic tract


Myeloid sarcomas are rare extramedullary tumors of immature myeloid cells. They comprise two major subtypes: granulocytic sarcomas composed of granulocytic precursors at various stages of differentiation and monoblastic sarcoma which consist of monoblasts and immature monocytes [1]. In the largest published series of myeloid sarcomas, 50% were of granulocytic type, 43.5% either monoblastic or myelomonoblastic, and 6.5% corresponded to different histotypes [2]. The lesion was first described by Burns in 1811 [3]. It was named ‘chloroma’ because it displays a greenish colour due to the presence of myeloperoxidase (or verdoperoxydase) [4] and was then found to be associated with acute myeloid leukemia (AML) [5,6]. The preferred term of ‘granulocytic sarcoma’ was coined later [7]. Myeloid sarcoma resembles a solid tumor and should not be confused with diffuse extramedullary leukemic infiltrates. It is most commonly diagnosed as part of the systemic manifestations of AML, and develops in 2-8% of patients with AML [8,9]. On rare occasions, it may also be the presenting symptom. Myeloid sarcoma may also be the sole manifestation of relapse of previously treated myeloid leukemia. Granulocytic sarcoma may occur at almost any body site. Involvement of the female genital tract is uncommon [10]. The most commonly involved organs are the ovary, the uterine cervix and the uterus corpus [11].

We report here one case of granulocytic sarcoma of the uterine cervix with extension to the left parametrium concomitant to bone marrow involvement in one patient treated 14 years earlier for AML.

Case Presentation

A 44-year old woman (gravida 2, para 2) (with a past history of surgery for adenofibroma of the left breast in 1985) presented to our institution in October 2000 with fatigue, dyspnea, gingival hemorrhage, and menorrhagia. Clinical examination was normal. Blood test displayed white blood cell count at 2.4 x 109/L with 18% neutrophils and 60% circulating blasts, anemia, and thrombocytopenia. Bone marrow aspirate confirmed the diagnosis of FAB M1-AML with medullary infiltration by 40% of blastic cells of which 25% with Auer rods. Cytogenetic studies showed 46, XX [17]/46, XX, add (21) (q21-22) [3]. Molecular analyses were negative for mixed-lineage leukemia (MLL) rearrangement; CEBPa, FLT3, and NPM1 mutations; and WT1 overexpression. After given written informed consent, she was included into the ALFA 9802 trial [12]. Induction chemotherapy regimen consisted of a timed sequential chemotherapy with a first sequence combining daunorubicin (80 mg/ m²/d on days 1-3) and cytarabine (500 mg/m²/d over the same period) and a second sequence, administered after a 4-day free interval, with mitoxantrone (12 mg/m²/d on days 8 and 9) and cytarabine (500 mg/ m²/12h on days 8-10). After complete remission (CR) achievement, she was assigned to consolidation chemotherapy, despite one HLAcompatible sibling, in the absence of risk factors. She received 4 cycles of high-dose cytarabine (3 g/m²/12h on days 1, 3, and 5) followed by 4 additional maintenance courses (daunorubicin, 45 mg/m² on day 1, and cytarabine, 100 mg/m²/12h on days 1-5). During chemotherapy, she received granulocyte-macrophage colony-stimulating factor as priming, and lynestrenol was prescribed once daily as prophylaxis against menorrhagia.

After 14 years without any symptoms, she presented again in January 2015 with lumbar pains and functional and obstructive urinary signs, suggesting symptoms in relationship with kidney stone. Sonographic evaluation of the pelvis revealed left ureteropyelocaliceal dilatation. Urinary tract double-J stent was positioned to facilitate the upper urinary tract drainage. CT and magnetic resonance imaging (MRI) scans performed by February 2015 showed an increase in size of the uterus cervix with a tumor of 4 cm of largest diameter and multifocal heterogeneous low signal intensity lesions from the upper vagina to the left parametrium, which suggested a malignant infiltrative process (Figure 1). A pelvic examination confirmed hypertrophy of the cervix. Histopathological examination of multiple colposcopic biopsies of the uterine cervix revealed an infiltration by proliferative, round, small immature myeloid cells among stromal cells (Figure 2). The immunophenotypic study showed that malignant cells expressed vimentin, CD56, CD117, myeloperoxidase, CD43, and CD34, and confirmed the diagnosis of granulocytic sarcoma. Positron emission tomography (PET) scan imaging performed on March 2015 showed hypermetabolism of the cervical mass contiguous with a large left parametrial mass that displaced the left ureter. This was associated with hypermetabolic tumor infiltration of the left iliac axis and hypermetabolic activity in right iliac, aortico-lumbar and intersaortico cave adenopathies.