1/10. Primary cardiac sarcoma in pregnancy: a case report and review of the literature.Primary cardiac sarcoma (PCS) is a rare disease with a poor prognosis, because of diagnostic delay, therapeutic difficulties, and high metastatic potential. Surgery is the standard treatment. A case of PCS in pregnancy is reported, with a review of published surgical series of PCSs, focusing on the role of surgery and adjuvant therapy. Prompt surgery improved cardiac function and patients' outcome in comparison with untreated cases. The role of adjuvant treatment was analyzed only in a few series, mainly without distinction between postoperative chemotherapy and radiotherapy; adjuvant therapy improved survival in the larger series of resected PCSs. Only three other cases of PCS in pregnancy were reported. In the present case, resection was performed with no major complication for the mother and the infant. Even if the patient's survival was short, cardiac surgery allowed prolonging of pregnancy until an acceptable possibility of fetal survival was reached. Although resection is not curative in most cases, surgery remains the treatment of choice for PCS and has a definite palliative significance. The role of postoperative chemotherapy and radiotherapy is difficult to ascertain; however, adjuvant chemotherapy seems advisable in high-grade tumors.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/10. Combined resection and adjuvant therapy improves prognosis of sarcomas of the pulmonary trunk.BACKGROUND: diagnosis of pulmonary artery sarcoma is difficult and frequently delayed owing to the insidious, non-specific nature of the symptoms. We present our experience with surgery for this rare disease. methods: Between 1995 and 1998, four patients underwent surgery for pulmonary trunk sarcomas. They presented with similar symptoms and were initially treated for thromboembolic disease of the lung. Preoperative diagnosis was established using computer tomography and echocardiography. Postoperatively, two patients received radiotherapy and one combined radio-/chemotherapy. RESULTS: One patient underwent thrombendarteriectomy of the trunk with adjuvant radiotherapy and remains tumor-free after three years and one showed complete regression of metastases after chemotherapy and remains well after more than one year. The other two patients died within two months of surgery, one due to a tumor mass in the inferior caval vein and the other because of cerebral metastases. CONCLUSIONS: According to the literature, mean survival rate is 1.5 months without resection and 10 months with resection, with survival up to four years following combined resection and adjuvant therapy. Our findings are in agreement with this.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/10. Malignant triton tumor of the nasal cavity.BACKGROUND: Malignant triton tumor is usually an aggressive sarcoma consisting of a malignant schwannoma with rhabdomyoblastic differentiation. However, malignant triton tumor of the nasal cavity is very rare. methods: A case report of a 38-year-old woman with polypoid mass, which bled easily in the right nasal cavity, and nasal obstruction is presented with a review of the literature pertaining to this unusual case. RESULTS: The malignant triton tumor was treated with wide surgical excision followed by radiation therapy. Histopathological diagnosis of the malignant triton tumor was made on the surgical specimen. The patient's postoperative course was unremarkable, and follow-up at 5 years reveals no evidence of disease. CONCLUSIONS: A malignant triton tumor in the nasal cavity is a rare disease. head and neck surgeons should recognize the possibility of malignant triton tumor occurring in the nasal cavity.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/10. Locally advanced and metastatic breast cancer with cartilaginous and/or osseous metaplasia showing excellent response to chemotherapy.breast cancer with cartilaginous and/or osseous metaplasia is considered a rare disease, but several cases have been reported recently. We report a case of breast cancer with cartilaginous and/or osseous metaplasia that was StageIV,(T4bN0M1b (PUL)), on the basis of the Japanese General Rules for Clinical and Pathological Recording of breast Cancer, which responded well to chemotherapy. A 58-year-old women visited our hospital with a chief complaint of a palpable breast mass that had increased in size in March 2002. It was 20 x 15 x 14 cm and occupied the entire right breast. Chest computed tomography (CT) demonstrated multiple lung metastases. histology of the biopsy specimens revealed a spindle-shaped cell carcinoma. It was ER(-), PgR(-), and HER2/neu Score 0. CAF was given to the patient as preoperative chemotherapy. Five cycles of treatment yielded improvement at the primary site and improvement of the metastatic lung lesions, which was judged as a partial response. Subsequently, one cycle of weekly paclitaxel 80 mg/m2 and oral administration of 5'-DFUR 800 mg/day were given. In November 2002, the patient underwent a right simple mastectomy with whole-layer skin grafting from the abdomen. The final pathological diagnosis was a rare type of breast cancer with cartilaginous and/or osseous metaplasia. Preoperative chemotherapy had caused necrosis in most of the tumor cells, and the efficacy was judged as Grade 2. From the third week postoperatively, weekly paclitaxel (80 mg/m2) was given. Six months after the operation, the multiple lung metastases were completely eliminated and new metastasis to liver or bone or local recurrence have not been observed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/10. Multimodal treatment using surgery, radiotherapy, and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus.Perivascular epithelioid tumor (PEComa), a recently defined tumor, is a very rare disease affecting various organs, most often the uterus. This tumor displays a variety of histologic and clinical features and at this point is regarded as a tumor with uncertain malignant potential. A 9-year-old girl with abdominal pain and vaginal spotting was diagnosed with PEComa of the uterus with metastasis. She received chemotherapy comprising vincristine, ifosfamide, and doxorubicin, as well as radiotherapy after surgery. After this multimodal treatment, there was no evidence of recurrence or further metastasis. She remains disease-free 1.5 years after her initial diagnosis. PEComa of the uterus displaying malignant characteristics may have a more favorable response to more aggressive therapy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/10. langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report.langerhans cell sarcoma (LCS) is a neoplastic proliferation of langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomographic (CT) scan revealed multiple tiny nodules in both lungs. The thoracoscopic lung biopsy revealed LCH. The patient quit smoking, but he received no other specific treatment. One year later, the follow up chest CT scan showed a 4 cm-sized mass in the left lower lobe of the lung. A lobectomy was then performed. Microscopic examination of the mass revealed an infiltrative proliferation of large cells that had malignant cytologic features. Immunohistochemical stains showed a strong reactivity for S-100 and CD68, and a focal reactivity for CD1a. We think this is the first case of LCS arising from LCH.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/10. Synchronous prostate stromal sarcoma and gastrointestinal stromal tumor of rectum: case report and review of the literature.We report a case of prostate stromal sarcoma with initial presentation of obstructive voiding symptoms. The synchronous rectal gastrointestinal stromal tumor was also finally discovered. The patient underwent pelvic exenteration as the choice of treatment. Synchronous prostate stromal sarcoma and rectal gastrointestinal stromal tumor is an extremely rare disease in the literature view.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/10. Unusual survival after pulmonary artery sarcoma.A 46-year-old man with a primary sarcoma of the pulmonary artery is presented. The tumor was responsive to radiotherapy, and the patient is the longest reported survivor of this rare disease. The tumor spread distally in the pulmonary vasculature, with probable embolization to the central nervous system, and he died 27 months after the initial diagnosis, with sudden massive hemoptysis. pulmonary artery sarcomas are a rare cause of obstruction of the right ventricular outflow. The course of our patient suggests that early recognition and therapy may provide some of these patients with effective palliation and prolonged survival.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/10. A case of spindle cell sarcomatous change of hepatic ducts manifesting as obstructive jaundice.Spindle cell carcinoma is a rare tumor commonly occurring in the upper aerodigestive tract. We report a 62-year-old male with spindle cell sarcomatous change located at the hepatic hilum, resulting in obstructive jaundice. The patient died after an extended resective operation. The rare disease and its histogenesis is discussed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/10. Lymphangiomyomatosis of the uterus associated with tuberous sclerosis and malignant neoplasia of the female genital tract: a report of two cases.Lymphangiomyomatosis (LAM) is a rare disease that does not generally affect the female genital tract. We report two cases of uterine involvement by LAM in young women with tuberous sclerosis and renal angiomyolipomas. In both, the uterine lesions were grossly inapparent and were discovered during microscopic examination of hysterectomy specimens removed during surgical treatment for a primary ovarian adenocarcinoma with peritoneal and lymph node metastases in one case and a retroperitoneal lymphangiomyoma in the other. In one case, an area of uterine LAM with atypical features was interpreted as focal sarcomatous transformation. This patient also had pelvic and paraaortic lymph node involvement by typical lymphangiomyomas, a small uterine angiomyoma, and an occult primary endometrial adenocarcinoma. Immunostains for HMB-45 were strongly positive in the uterine LAM in both cases, the retroperitoneal and lymph node lymphangiomyomatous lesions, the uterine angiomyoma, and a resected renal angiomyolipoma. Although LAM is a rare uterine lesion, it must be distinguished from a variety of uterine smooth-muscle tumors.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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