1/8. Angiomyomatous hamartoma and associated stromal lesions in the right inguinal lymph node: a case report.Angiomyomatous hamartoma is a rare disease with a predisposition for the inguinal lymph nodes. A 51-year-old male patient visited a local hospital because of a right inguinal mass, measuring 3 x 4 cm in size, which was resected. The resected specimen showed irregularly distributed thick-walled vessels in the hilum, extending into the medulla and focally into the cortex of the node, eventually becoming more dispersed and associated with smooth muscle cells splaying into sclerotic stroma. These findings are compatible with an angiomyomatous hamartoma. Another tumor-like mass appeared shortly after the resection at the same location, but was not an angiomyomatous hamartoma, rather it was composed of edematous stromal tissue with proliferating smooth muscle cells. The stromal component included thick-walled blood vessels and lymphatics. Although it could not be determined whether these associated changes in the surrounding stroma are a cause or an effect of angiomyomatous hamartoma, they indicate the clinical difficulty in determining an appropriate area of resection and may provide clues to the pathogenesis of angiomyomatous hamartoma.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/8. Lymphangiomyomatosis arising in the pelvic cavity: a case report.lymphangioleiomyomatosis (LAM) is a rare disease usually occurring in young women of child-bearing age. It is characterized by a distinctive proliferation of lymphatic smooth muscle cells, especially occurring in the pulmonary parenchyme. The majority of primary LAM occurs in the lung, but there are a few reports of extrapulmonary cases. We report a case of a 21-yr-old female who first complained of low abdominal pain and was referred from a local clinic with the impression of an ovarian cyst. Gynecologic ultrasonography revealed a large posterior pelvic mass with an irregular echogenicity measuring 9.7 x 4.2 cm in size. Pelviscopy showed a large, thin walled, partly cystic, pelvic mass. The mass was partly removed. Microscopically, the mass was characterized by a haphazard proliferation of smooth muscle cells arranged in fascicular, trabecular, and papillary patterns around a ramifying network of endothelium-lined spaces. The cells were plump or epithelioid with abundant eosinophilic cytoplasm and showed a positive reaction for both alpha-smooth muscle actin and HMB-45 antigen. Surgical and pathological findings were consistent with pelvic retroperitoneal LAM. Despite the numerous treatment attempts, the patient suffered from intractable chylous ascites and developed pulmonary LAM and died due to severe respiratory distress.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/8. Extraskeletal Ewing's sarcoma. Presentation of two cases and review of the literature.We present here two cases of extraskeletal Ewing's sarcoma, the first in a 50-year-old female and the second in a 25-year-old male. We discuss the clinical picture, histopathology and therapeutic management. The literature is also reviewed, with major emphasis on the treatment of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/8. Pelvic lipomatosis with cystitis cystica, cystitis glandularis and adenocarcinoma of the bladder: first reported case.Pelvic lipomatosis is a rare disease which may cause obstructive uropathy. It has been reported with cystitis cystica and cystitis glandularis. cystitis cystica and cystitis glandularis have been reported previously as progressing to adenocarcinoma of the bladder. The first reported case of pelvic lipomatosis, cystitis cystica, cystitis glandularis and adenocarcinoma of the bladder is presented and the literature reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/8. Leiomyomatosis peritonealis disseminata.A 49 year old woman presented with leiomyomatosis peritonealis disseminata (LPD) which is a benign and very rare disease entity. This patient is particularly interesting because she presented with many atypical features. She is the second Oriental case reported, and the follow-up period was long. The disease was symptomatic and progressive despite bilateral salpingo-oophorectomy, hysterectomy and repeated excision of all the recurrent nodules. She had documented recurrences of LPD in three episodes at 4 years apart. There was no obvious systemic hormonal abnormality including pregnancy or oestrinizing granulosa tumour, and she had never taken any contraceptive drugs. The importance of differentiation of this condition from generalized metastatic intra-abdominal malignancy is discussed as LPD has a very good prognosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/8. MRI of pelvic neurofibromatosis.Pelvic neurofibromatosis is a rare disease and rarely involves the prostate. A 19-year-old male presented with irritative and obstructive voiding symptoms. magnetic resonance imaging showed a large mass extending from the sacral promontory to the perirectal and perivesical spaces and to the proximal root of the penis. The mass also involved the prostate. The characteristics of the mass were highly suggestive for neurofibroma. prostate biopsy showed neurofibroma, and the immunohistochemistry stain for S-100 protein was positive.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/8. Massive low-attenuation mediastinal, retroperitoneal, and pelvic lymphadenopathy on CT from lymphangioleiomyomatosis. Case report.lymphangioleiomyomatosis (LAM) is a rare disease of women of child-bearing age characterized by the proliferation of smooth muscle throughout the lymph nodes and lymphatic channels of the retroperitoneum, mediastinum, and lungs. Most previous reports of LAM have concentrated on the pulmonary findings. Although mediastinal and retroperitoneal lymphadenopathy is known to be a prominent feature of the disease, it is rarely imaged. We present a case of a young woman with LAM who presented with massive pelvic, retroperitoneal, and mediastinal lymphadenopathy associated with a chylous pleural effusion on the left. On computed tomography (CT) the lymphadenopathy was heterogeneous in nature but was predominantly of low attenuation. We believe that when CT demonstrates low-attenuation lymphadenopathy in the mediastinum or retroperitoneum of a woman of child-bearing age, LAM should be considered as a likely diagnosis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/8. The use of an ultrasonic assisted lipectomy device for the treatment of obstructive pelvic lipomatosis.Pelvic lipomatosis is a rare disease of unknown etiology characterized by overgrowth of pelvic fat. We describe a 60-year-old man with pelvic and retroperitoneal lipomatosis causing severe urinary obstruction with massive hydroureteronephrosis. The diagnostic procedure and the management of the patient is described, emphasizing the value of the Ultrasonic Assisted lipectomy device in performing ureterolysis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |