Embryonal rhabdomyosarcoma of the uterine cervix: case report and review of the literature

OBJECTIVES: Sarcoma botryoides rarely arises in the uterine cervix. It usually presents in the adolescent age group. The peak incidence is in the second decade. A combined modality approach to treating rhabdomyosarcoma (RMS) has markedly improved survival. We present two cases of cervical RMS. One of them had a very aggressive clinical course. CASE: Two cases of RMS in young women who presented with rapidly progressive cervical tumor are reported. They were treated with two-stage surgical procedure and chemotherapy. CONCLUSIONS: A combined modality approach to treating RMS using multidrug chemotherapy, radiotherapy, and surgery has markedly improved survival. One of our cases has been free of disease for more than 5 years.     

Severe hyperandrogenemia in postmenopausal woman as a presentation of ovarian hyperthecosis. Case report and mini review of the literature

Ovarian hyperthecosis (OH) is characterized by the presence of abundant luteinized theca cells in ovaries that secret androgen. It typically presents as severe hyperandrogenism and/or virilization in postmenopausal woman. Here we describe a 66-year old woman with presentation of severe hirsutism, alopecia, clitoromegaly and laboratory finding of significantly elevated serum total testosterone concentration and hyperinsulinemia. Performed imaging studies revealed normal sized, homogeneous ovaries, signs of endometrial hypertrophy and normal adrenal glands. Due to severe hyperandrogenemia and signs of endometrial hypertrophy, the total abdominal hysterectomy with bilateral salpingo-oophorectomy has been performed. Pathological examination revealed OH and endometrial hyperplasia. Androgenic activity of ovarian stromal cells has been confirmed using alpha-inhibin histochemical staining. Postmenopausal hyperandrogenemia is a diagnostic and therapeutic challenge and the imaging studies often may be misleading and require careful and critical consideration.     

Embryonal rhabdomyosarcoma of the uterine cervix in a 47-year-old woman

Embryonal rhabdomyosarcoma (RMS) of the female genital tract usually occurs in the vagina during childhood. The uterine cervix as a primary site is rare, but is more frequent until the second decade of life. It usually has a good prognosis and the treatment is based on multidrug chemotherapy, radiotherapy and surgery. RMS accounts for <5% of all adult soft tissue sarcomas. Previous reports that included all primary sites showed a poorer five-year disease specific survival for adults with RMS when compared to the pediatric population. This difference has been attributed to a higher proportion of adverse prognostic clinical and pathological factors, and to inadequate treatment given to adults with RMS. A total of 115 patients with cervical embryonal RMS have previously been described; however, only 10 cases were reported in women older than 40 years. We present a 47-year-old woman treated with radical hysterectomy followed by adjuvant chemotherapy and review the current literature.     

Primary retroperitoneal lymphangioleiomyomatosis in a postmenopausal woman: a case report and review of the literature

Lymphangioleiomyomatosis (LAM) is a rare progressive disease of unknown etiology that typically affects women of childbearing age. It is characterized by an abnormal proliferation of smooth muscle cells causing gradual obstruction of small airways, frequently resulting in respiratory failure and death. While LAM is predominantly a lung disorder, we report a case of retroperitoneal LAM in a patient who had no evidence of pulmonary involvement. A 59-year-old female presented with postmenopausal bleeding and no other complaints. She was found to have a low attenuation retroperitoneal mass on abdominal and pelvic computed tomography (CT) scan suspicious for lymphoma. CT-guided biopsy was nondiagnostic. Laparoscopic resection of some of the enlarged retroperitoneal lymph nodes confirmed the diagnosis of LAM. This case is an atypical presentation of LAM. The disease typically presents in premenopausal women, with the initial site of involvement being the lungs and mediastinum. In rare cases, such as this of extrapulmonary LAM, patients typically present with a palpable abdominal mass, abdominal pain, or chylous ascites. As in our case, radiographic findings can mimic malignancies such as lymphoma. Laparoscopic lymph node biopsy is a valuable tool in these situations of diagnostic dilemma.     

Osteosarcoma of the uterus: Case report and review of the literature

Osteosarcomas of the uterus, whether pure, or mixed with other heterologous or homologous sarcomas, are rare. We studied a case of mixed osteosarcoma and leiomyosarcoma and reviewed the literature to determine if uterine osteosarcomas possess distinct clinicopathologic features separating them from other heterologous sarcomas or mixed mullerian tumors (MMMT). Clinically, osteosarcomas behave similarly to rhabdomyosarcomas and chondrosarcomas, and have a poorer prognosis than MMMT. The aggressive nature of osteosarcomas, rhabdomyosarcomas, and chondrosarcomas is evidenced by their poor prognosis, even when apparently confined to the uterus at the time of diagnosis. The ultrastructural findings in osteosarcoma of the uterus parallel observations in osteosarcoma of bone, and suggest an origin from mesenchymal cells capable of fibroblastic, osteoblastic, and histiocytic differentiation. Provisionally, we recommend that osteosarcomas be approached as other heterologous sarcomas, with total abdominal hysterectomy and supplemental chemotherapy and radiotherapy.     

Hyperparathyroidism in a pregnant woman with previous thyroid carcinoma. Case report and review of the literature

A 29 year old woman, primipara, at 26 weeks' gestation had been undergone, five years before, a total thyroidectomy owing to a mixed papillary-follicular carcinoma of thyroid. After thyroidectomy the patient was placed on suppressive thyroxine treatment. Since she was 22 years old she suffered from recurrent renal colics and cholelithiasis. For these reasons she underwent, at the age of 27, an operation to remove bilateral renal calculi and a cholecystectomy owing to gall-stones. During her pregnancy biochemical determinations revealed slight and persistent hypercalcemia, hypophosphatemia, elevated urine calcium besides elevated serum parathyroid hormone (PTH) levels. Thus a diagnosis of primary hyperparathyroidism was taken into consideration. It was considered, but temporarily delayed, the surgical exploration of the neck. A strict clinic overseeing of the patient, which allowed her to carry out the pregnancy happily, was undertaken. Any thyroid carcinoma repercussion on pregnancy was not noted. A left inferior parathyroid adenoma was removed five months after the woman's delivery. The child psychosomatic development, at one year of age, was absolutely normal. A review of the literature indicates that when a pregnancy is complicated by hyperparathyroidism its prognosis is improved by parathyroidectomy, if possible during the second trimester. In patients with asymptomatic hypercalcemia and/or in late pregnancy surgical treatment may be postponed until after delivery. It is also demonstrated that thyroid carcinoma is not aggravated by pregnancy and that the latter can develop without any worry for mother and fetus.     

Diffuse cavernous hemangioma of the uterus in a pregnant woman: report of a rare case and review of literature

Introduction  

Diffuse cavernous hemangioma of the uterus in pregnant woman is an extremely rare condition. A total of eight cases have been described in the literature till date. The antenatal diagnosis as well as management requires considerable skill. Although it is a benign condition but it can have serious consequences for the mother as well as the baby.     

Embryonal rhabdomyosarcoma (botryoid type) of the cervix: A case report and review

A case report of embryonal rhabdomyosarcoma originating from the cervix in a 3-year-old girl is presented and a brief review of the literature on this topic given. The histopathology of the tumor is discussed and the effectiveness of a less aggressive surgical approach and chemotherapy is demonstrated in this particular case.     

Pleomorphic liposarcoma of the uterus: case report and literature review.

A 62-year-old woman with a history of breast carcinoma being treated with tamoxifen presented with a rapidly enlarging pelvic mass. Imaging studies suggested a uterine leiomyoma with possible sarcomatous transformation. Laparotomy revealed a 15-cm, oval, well-circumscribed mass emanating from the posterior cervix and left uterosacral ligament. The tumor had a variegated fleshy, tan, myxoid, and necrotic sectioned surface. Microscopic examination revealed a variety of patterns and cell types characteristic of liposarcoma that included myxoid/round cell, storiform/pleomorphic, epithelioid, and spindle cell areas. Lipogenic areas exhibited a "crow's feet" vasculature and characteristic lipoblasts. The tumor cells were highly pleomorphic with numerous mitotic figures, some of them atypical. The tumor cells were immunoreactive for vimentin, estrogen receptors, and S-100. The tumor recurred 9 months postoperatively. Although a variety of uterine tumors have been associated with tamoxifen treatment, this appears to be the first example of tamoxifen-associated uterine liposarcoma.     

Severe complication to phytomenadione after intramuscular injection in woman in labor. Case report and review of literature

A 28-year-old woman in labor developed a severe anaphylactoid reaction, necessitating acute cesarean section, with subsequent neonatal death, after receiving 10 mg of phytomenadione (Konakion) by the intramuscular route. Allergologic investigations revealed no type I reaction against the drug, and the symptoms were considered to be caused by drug-induced intolerance. Prophylactic administration of phytomenadione to the infant rather than to the parturient is recommended.