Multiple myeloma (MM) is a clonal proliferation of plasma cells with multiple osteolytic lesions. Extramedullary dissemination of multiple myeloma in ovary is relatively uncommon. Here, we presented a 54-year-old female patient with MM and plasmacytoma of ovary.
A 54-year-old female patient was admitted to the Department of Hemotology, Beijing Chaoyang Hospital with complaints of intermittent abdominal pain for three days and loss of appetite for three-month duration. The pain was moderate in intensity, constant, and localized primarily to the lower part of the abdominal. She had been diagnosed as MM for three years. Three years ago, the patient experienced lower back pain without evident cause and the symptoms progressively aggravated. She was treated in a local hospital and clearly diagnosed with MM IgA-κ type III stage A (DS) by bone marrow puncture and M protein identification. She received one cycle of vincristine, doxorubicin, and dexamethasone (VAD) treatment, seven cycles of combined bortezomib and VAD chemotherapy, and one cycle of melphalan and prednisone (MP) chemotherapy, which achieved a partial response.
Six months ago, the body pain was significantly aggravated and she received bone marrow puncture and M protein identification for the evaluation of disease progression. We found a bulge in the right arm and the pathological exam and immunohistological staining indicated plasmacytoma. The patient received one cycle of VAD chemotherapy, one cycle of cyclophosphamide, thalidomide, and dexamethasone (CTD) chemotherapy, and one cycle of cyclophosphamide, doxorubicin thalidomide, and dexamethasone (CATD) chemotherapy three months ago. The tumor significantly got shrunk in size during the treatments above but relapsed because the patient failed to follow up further chemotherapy due to personal reasons.
Three days before the current admission to hospitalization, the patient experienced interrupted lower abdominal cramp. The symptoms spontaneously relieved after 10 to 15 minutes. She had no fever, nausea, vomiting, or constipation.
The physical examination at admission showed normal consciousness and conversation, pale conjunctive no touchable enlargement of superficial lymph nodes, no icterus or bleeding under the skin or sclera, even increased bulge in the right upper arm than before, no abnormal cardiopulmonary signs, full and soft abdomen, tenderness under the abdominal cesarean section area, no tenderness or rebound tenderness at Michael's point, and no lower limbs swelling. Routine investigation showed white blood cell (WBC) count of 3.7×109/L, hemoglobin (Hb) count of 80 g/L, and platelet (PLT) count of 323×109/L, and serum creatinine 1.9 mg/dl. The anteroposterior abdomen radiograph showed no fluid level or free gas below the diaphragm. The abdomen ultrasound showed fatty liver, gallbladder stones, and a small amount of liver fluid. The vaginal gynecological ultrasound showed multiple uterine fibroids, an heterogeneous hypoechoic area of 13.4 cm × 12.9 cm × 9.3 cm in the uterus above the lower abdomen with still clear boundary, deformed structure, and a small amount of blood flow signals inside, as well as a very small amount of free liquid dark area with the deep diameter of 0.7 cm in the left iliac fossa. Four days later, the patient received a complete review. The WBC count was 6.3×109/L, Hb 51 g/L, and PLT 281×109/L. The anemia was significantly worse than before. The abdomen was still soft and no tenderness or rebound tenderness was found. The vaginal gynecological ultrasound showed celiac solid mass, which was considered extramedullary lesion. The ascites kept increasing during the inspection, probably due to blood flows. Under ultrasound guide, a 5 ml syringe was inserted into the lower right abdomen and extracted about 5 ml of non-coagulated dark red blood.
To treat the pelvic tumor rupture, the bleeding was immediately stopped and transfusion of fresh frozen plasma and red blood cell suspension was administered. The patient was then transferred into the Department of Obstetrics for emergent laparotomy, which showed a significantly enlarged right cystic ovary (12 cm × 12 cm × 10 cm). The tumor capsule was still intact. The gray colored tumor had thick walls as well as rich blood vessels and was not attached to the surrounding tissue. The tumor surface showed papillae, ruptured blood vessels, and active bleeding. The intra-abdominal hemorrhage was about 2000 ml. Blood clots were observed in rectal fossa and the right-side oophorectomy was performed.
The postoperative recovery was good. Pathological reviews showed ovarian plasmacytoma and immunohistochemistry results revealed positive CD38, CD138, leukocyte-common antigen (LCA, CD45), and CD99 staining. The Ki-67 staining was about 90%. Therefore the final diagnosis of this patient was ovarian extramedullary plasmacytoma rupture and bleeding.
In MM, bone marrow is infiltrated with aggregates of abnormal plasma cells and that leading to multifocal destructive bone lesions. Its incidence ranks the second of all hematological malignancies. The malignant plasma cells mainly affects the bone marrow, but may also spread to all the other important organs and tissues, resulting in extramedullary plasmacytoma. With the continuous development of the treatment of myeloma, the patient survival duration is significantly prolonged. While the bone marrow plasma cells and urine M protein are getting better control, the incidence of extramedullary plasmacytoma is gradually escalating in recent years. Autopsy studies have shown extra-skeletal involvement in 70% of patients with MM.1
It happens most commonly in the upper respiratory tract,2–4 such as the nasopharynx, paranasal sinuses, and other submucosal regions. It can also affect the stomach intestine, lung, lymph nodes, skin, mediastinum, and spleen, although the incidence is much less. It has seldom involved the female reproductive organs, even more rarely in the ovary.5 Despite advances in the diagnosis of MM, it remains an incurable disease, because the disease follows a relapsing course in majority of patients, regardless of the treatment regimen or initial response to treatment.
Newly diagnosed patients with good performance status are best treated with autologous stem cell transplantation. These patients are treated with high dose chemotherapy (HDCT) with vincristine, melphalan, cyclophosphamide and prednisone (VMCP) alternating with vincristine, carmustine, doxorubicin and prednisone (BVAP) combined with bone marrow transplantation, it improves the response rate, even free survival and overall survival in multiple myeloma. Induction therapy in patients ineligible for transplantation (old age, coexisting conditions, poor physical condition) includes thalidomide in combination with melphalan and prednisone or melphalan and prednisolone. Recently, the management of patients with MM has been transformed by introduction of three novel agents: thalidomide, lenalidomide, and bortezomib.6
The occurrence of extramedullary plasma cell tumors is an independent factor for poor prognosis in multiple myeloma patients and should receive widespread attention.7 The present patient was diagnosed as a rare case of multiple myeloma with ovarian plasmacytoma, which was surgically resected due to the rupture and bleeding. We proposed postoperative treatment with combined bortezomib and dexamethasone, etoposide, cyclophosphamide, and cisplatin (DECP) chemotherapy to control the disease progression.
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