Chronic myeloid leukemia presenting as a gynecological emergency

An unusual case of chronic myeloid leukemia presenting to the gynecological unit as a case of septic abortion is presented. Though history and clinical examination suggested the possibility of double pathology of septic abortion and chronic myeloid leukemia, histology of the products removed from the uterus showed it to be leukemic deposits. The diagnosis of leukemia was confirmed by marrow aspiration (trephine biopsy). Careful clinical examination and systematic investigation helped in diagnosing the pathology and to treat her appropriately.     

Acute abdomen as first symptom of acute leukemia

Case report. The authors presented a rare case of acute abdomen syndrome caused by the rupture of the corpus rubrum as the first symptom in a 35-years-old woman with the acute lymphatic leukemia. During the laparotomy is notice diffuse bleeding from under skin blood vessels and muscles. The blood was electrocoagulated and was sewn with catgut sutures. The right ruptured corpus rubrum was found from which fresh blood was leaking. The right ovary was carefully resected and sutured, and each ligature was bleeding. At the beginning of the surgery laboratory analysis results arrived which showed a high leukocytosis (28.0×10⁹/l) with sever thrombocytopenia (10×10⁹/l) and afibrinogenemia (0.1 g/l) with anemia (1.9×10¹²/l erythrocyte, haematocrit 0.24), which indicated leukemia with disseminated intravascular coagulopathy (antithrombin III levels 0.9 g/l, D-dimers 1989 μg/l). Result. A year later she died with the picture of severe disseminated intravascular coagulopathy, agranulocytosis and septic condition with multiorganic failure.     

Abnormal vaginal bleeding as first symptom of acute leukemia

We report a case of a 37-year-old woman, presenting with vaginal hemorrhage, together with severe anemia and thrombocytopenia (hematocrit: 10.6%; platelets: 6 G/l) caused by previously unknown acute lymphocytic leukemia. Received: 15 May 2000 / Accepted: 27 August 2000     

Aggressive angiomyxoma of the vulva presenting as a huge pedunculated tumor

In 1983, Steeper and Rosai described aggressive angiomyxoma as a rare, slow growing, locally infiltrative, soft tissue tumor that occurs mainly in the genital, perineal and pelvic region, mostly in women. Usually this tumor is nonmetastasing. Recurrence is frequent in about 30%-72% cases. Angiomyxoma demonstrates estrogen and progesterone receptor positivity. Primary surgical resection is the treatment of choice. Hormonal therapy with tamoxifen, raloxifene, and gonadotropin-releasing hormone analogs (GnRH agonists) and vascular embolization are used as the treatment of recurrence and may help to make complete excision feasible in large tumors. We report a case of a 43-year-old woman with huge pedunculated aggressive angiomyxoma of the vulva treated with primary excision.     

Cyclosporine-induced encephalopathy in a patient with relapsed acute myeloid leukemia treated with unrelated allogeneic bone marrow transplantation.

Cyclosporine (CSP) is the most frequently used immunosuppressive agent for prevention of graft versus host disease (GVHD) in allogeneic bone marrow transplantation (BMT). Some adverse effects such as hepatic and renal toxicity have been frequently encountered, but central nervous system (CNS) toxicity caused by CSP is rare. We report an adult male patient with acute myeloid leukemia who developed CSP-induced encephalopathy under treatment for allogeneic BMT from an unrelated donor. Methotrexate and CSP were used for GVHD prophylaxis. Leukocyte and platelet engraftment were successfully achieved on days 21 and 24 after BMT, respectively. Abrupt onset of mental confusion and disorientation occurred on day 25, followed by a generalized tonic clonic seizure and consciousness disturbance. The whole blood CSP level was 160.65 ng/mL. Magnetic resonance (MR) imaging revealed high signal intensities in the bilateral occipital lobes with predominant involvement of the cortical areas. The patient recovered from the CNS toxicity, but with slight memory impairment, 6 days after CSP was discontinued. When patients receiving CSP treatment for allogeneic BMT develop mental confusion, consciousness disturbance, or seizure, CSP-induced CNS toxicity should be taken into consideration.     

Epithelioid sarcoma of the vulva

We report a case of a 23-year-old woman diagnosed as having an epithelioid sarcoma of the vulva. She was treated by a clitoris-sparing hemivulvectomy and lymph node sampling of the ipsilateral groin. Vulvar reconstruction was performed with a rectus abdominis myocutaneous flap. Four years after the operation there is no evidence of disease and the patient has a normal sex life. The English literature on this subject is reviewed with special attention to the biological behavior and therapeutic approach.     

Epithelioid sarcoma of the vulva

The authors present the third known case of an epithelioid sarcoma involving the vulva. This case illustrates the confusion that can occur in making a proper pathologic diagnosis. The histologic and electron microscopic characteristics of the tumor are presented. The natural history of the disease is discussed describing the propensity for local recurrence and the sites of metastases. Recommendations for management are discussed.     

Pseudoathetosis as a presenting symptom of spinal multiple sclerosis.

We report on a 38-year-old female patient with acute proprioceptive sensory impairment and pseudoathetosis in the four limbs, particularly in the fingers of both hands. She had great difficulty in buttoning, unbuttoning, using chopsticks and writing, because she was no longer able to feel her fingers in space. There was a profound loss of position and vibration sensation in all limbs, especially in both hands. Pseudoathetoid movement, a rare presentation of multiple sclerosis, was observed in the outstretched hands and extended fingers. T1- and T2-weighted magnetic resonance imaging (MRI) of the spinal cord revealed an inactive intramedullary lesion in the C2-C5 segments. Gadolinium-enhanced MRI revealed an active lesion in the posterior columns of the cervical cord at the C3 vertebral level, which is very likely responsible for pseudoathetoid movement. Based on the disseminated lesions in the spinal cord verified by MRI the four neurologic manifestations, and the abnormal somatosensory-evoked potentials, we made a definitive diagnosis of multiple sclerosis of the spinal cord.     

Low-grade myofibroblastic sarcoma of the vulva

BACKGROUND: Vulvar sarcomas are rare tumors. A distinctive low-grade myofibroblastic sarcoma is described. CASE: A 46-year-old female presented with a progressively enlarging vulvar mass. Pathological evaluation revealed a low-grade myofibroblastic sarcoma. The immunophenotype is outlined and ultrastructural features are highlighted. Tumor cells were positive for p53 protein, smooth muscle actin, steroid receptors, and showed myofibroblastic differentiation on electron microscopy. The patient has been followed for >14 months without evidence of recurrence. CONCLUSION: The tumor was positive for p53, mitotically active, but was categorized as a low-grade malignancy. Immunohistochemical and ultrastructural criteria were utilized to distinguish this tumor from other neoplasms.     

Primary epithelioid sarcoma of the vulva

Abstract. Kasamatsu T, Hasegawa T, Tsuda H, Okada S, Sawada M, Yamada T, Tsunematsu R, Ohmi K, Mizuguchi K, Kawana T. Primary epithelioid sarcoma of the vulva.
A case of a 31-year-old woman with epithelioid sarcoma of the vulva which metastasized to the regional lymph node 8 years after onset of the disease is reported here. The patient first noticed a painless subcutaneous mass of 5 mm in diameter in the right labium majus at age of 21. This was excised locally at age 23, but recurred 17 months later. Although local excision was again performed, the tumor recurred and continued to enlarge very slowly. At this stage, based on the pathology of both the initial and second tumors, the diagnosis was of a benign inflammatory process. However, local recurrence and inguinal lymph node swelling occurred at age 29, and biopsy was taken. The pathology report indicated benign granulomatous changes. The slides were reconsidered and re-interpreted as epithelioid sarcoma, whereupon radical vulvectomy was performed at age 31. Vulvar epithelioid sarcoma with inguinal lymph node metastasis was first diagnosed at that time. Epithelioid sarcoma of the vulva is an exceedingly rare tumor, and only 15 cases have been reported thus far in the literature. Early diagnosis and curative treatment of this tumor may be problematic for gynecologists because of its rarity and therefore little-known characteristic clinical behavior and histology. Radical vulvectomy or extensive local excision with inguinal lymphadenectomy at the time of diagnosis is recommended as the treatment of choice.