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[Leiomyosarcoma of Meckel's diverticulum in cystic form].

BACKGROUND: Leiomyosarcoma is an uncommon small bowel tumor. About 60 cases of a Meckel's diverticulum localization have been reported. We present a very rare form of leiomyosarcoma arising in a Meckel's diverticulum, mimicking a very large abdominal cyst. CASE REPORT: Abdominal ultrasound evidenced a voluminous cystic mass in the abdomen of a 37-year-old man consulting for abdominal discomfort. Computed tomography visualized a tissular component with contrast uptake. Laparotomy was performed with resection of a 4 liter cyst on the Meckel's diverticulum. Histology reported leiomyosarcoma. DISCUSSION: Leiomyosarcoma of the small bowel mainly develops in an extraluminal localization. Necrotic remodeling explains the cyst formation. Computed tomography with contrast injection can be most helpful for diagnosis. Surgery provides the only possible cure in still localized forms. Other treatments have failed to demonstrate any benefit. Prognosis is mainly related to the histological grading according to the mitotic index.

Adult↗

Preoperative cytological diagnosis of gastric leiomyosarcoma. A report of three cases.

Gastric leiomyosarcoma has rarely been diagnosed prior to operation. Three cases are presented in which a cytological diagnosis compatible with leiomyosarcoma could be made preoperatively by exfoliative cytology using a brushing technique under direct vision. Data which favor the diagnosis of leiomyosarcoma are: (1) spindle-shaped cells with malignant looking nuclei; (2) cells with extremely long, fiber-like cytoplasms and hyperchromatic nuclei; (3) large, anaplastic malignant cells. Smears from 3 patients showed a mixture of these different cell types. This cytological presentation is probably unique and diagnostic for leiomyosarcoma.

Cell Nucleus↗

Myxoid leiomyosarcoma of the left atrium: a rare malignancy of the heart and its comparison with atrial myxoma.

Primary tumours of the heart are rare. The majority of these tumours are benign, with myxomas located in the left atrium being the most common form. Almost all malignant tumours are sarcomas and occur preferentially in the right side of the heart. An exception to this rule is leiomyosarcoma, a rare form of primary cardiac sarcoma that occurs predominantly in the left atrium, as does cardiac myxoma. The case of a 53-year-old woman who presented with symptoms of mitral valve stenosis and pulmonary hypertension is reported. Cardiac catheterization, angiography and echocardiography revealed a left atrial mass that was interpreted as atrial myxoma. At the time of operation, the myxoid appearance of the tumour mass further supported this assumption. The tumour, including a wide rim of atrial septum, was removed with cautery. Histopathological examination unexpectedly showed that the tumour was not an atrial myxoma but rather a myxoid variant of a primary leiomyosarcoma. Immunohistochemistry and electron microscopy confirmed the diagnosis. Local radiotherapy was considered but deemed contraindicated in view of the longstanding pulmonary hypertension. Two months after excision, a repeat echocardiogram indicated recurrence of tumour in the left atrium, and the patient died a few days later. The preferential left atrial location and the frequently myxoid appearance of primary leiomyosarcomas of the heart make it particularly difficult to differentiate them preoperatively from atrial myxomas. The authors recommend resection of all atrial myxoid tumours with a wide (at least 1 cm) margin, combined with intraoperative frozen section diagnosis, because complete surgical resection appears to correlate with prolonged survival in the few reported cases of atrial leiomyosarcomas. In cases of incomplete initial resection or local recurrence in the absence of metastatic disease, heart transplantation may be a valid option in appropriately selected patients.

Cardiac Catheterization↗

Leiomyosarcoma after uterine artery embolization. A case report.

BACKGROUND: Hysterectomy is the most common treatment for relieving symptoms attributable to uterine leiomyomas; however, alternatives to hysterectomy are becoming increasingly available. Uterine artery embolization is being used more frequently in this clinical setting. CASE: A leiomyosarcoma was diagnosed incidentally in a 51-year-old, nulliparous woman who underwent uterine artery embolization for symptomatic leiomyomata and subsequent total abdominal hysterectomy/bilateral salpingo-oophorectomy due to unsatisfactory results of the embolization procedure. CONCLUSION: While the occurrence of preoperatively undiagnosed uterine leiomyosarcomas among patients undergoing hysterectomies has been reported, there are no reports of unsuspected leiomyosarcomas after uterine artery embolization. Criteria for selection of patients for the procedure should consider the possibility of leiomyosarcoma. With the increasing popularity of uterine artery embolization, cases like this are likely to be encountered in the future.

Arteries↗

Pulmonary leiomyosarcoma.

Three cases of pulmonary leiomyosarcoma were presented. The characteristic clinical features were described with review of literature. In comparison with bronchogenic carcinoma, the leiomyosarcoma has some characteristics: 1) On chest X-ray, it usually appears as a sharply demarcated, even density round mass, growing rapidly within the lung, it unusually accompanies with hilar or mediastinal lymph node metastasis; 2) The preoperative cytological or pathological diagnosis is difficult either by sputum smear or by bronchoscopic biopsy or by fine needle percutaneous aspiration biopsy; 3) Pathological differential diagnosis of leiomyosarcoma of lung from anaplastic lung cancer is difficult. In conclusion, the primary pulmonary leiomyosarcoma is a rare malignant tumor, detecting the present illness seriously, paying attention to the chest X-ray films characterize, early surgical resection is the only way to get diagnosis and effective treatment method.

Adult↗

Gigantic jejunal leiomyosarcoma.

Jejunal leiomyosarcoma is a rare neoplasm. We report a case of gigantic leiomyosarcoma of the jejunum in a 30-year old man who presented with abdominal distension, pain and constipation. Computerized tomography scan revealed a giant tumor filling the entire abdominal cavity. Fine needle aspiration cytology was suspicious. At laparotomy, a huge jejunal leiomyosarcoma measuring 30 x 25 x 19 cm and weighing 13 kg was completely excised. To the best of our knowledge, this is the first case of a giant jejunal leiomyosarcoma.

Adult↗

[Leiomyosarcoma of the renal vein].

The authors report a new case of leiomyosarcoma of the right renal vein, marked by a rapidly fatal course within 9 months, despite the initial absence of metastasis. This exceptional vascular tumour (only about thirty cases have been published) must be distinguished from primary renal leiomyosarcoma and retroperitoneal leiomyosarcomas involving adjacent structures. This difficult diagnosis was suggested by preoperative CT and angiography. Leiomyosarcomas of the renal vein generally have an unfavourable outcome in the medium term. The prognosis is related to the localized nature of the tumour and the risk of local and distant recurrence. Limited tumour resection is rarely sufficient and radical nephrectomy is usually necessary, possibly associated with a vascular procedure on the inferior vena cava in the case of contiguous extension.

Adult↗

[Imaging evaluation and interventional therapy of hepatic metastases from small intestinal leiomyosarcoma].

OBJECTIVE: To evaluate the imaging appearances and the short-term curative effect of hepatic metastases from small intestine leiomyosarcoma treated by interventional methods. METHODS: Seven cases with 46 lesions of hepatic metastases from small intestinal leiomyosarcoma were analyzed retrospectively. The diameters of the metastases were from 2 cm to 18 cm. CT triphasic scan and hepatoarterial angiography were performed in all cases. The interventional therapy methods included transcatheter arterial chemoembolization (TACE) (all 7 cases), percutaneous left subclavian artery port-catheter system implantation (PCS) (1 case). Three cases were also treated with percutaneous tumor necrosis drainage (PTND) because of obvious liquefaction necrosis within the tumor. RESULTS: The lesions were substantial occupation and some were mixed density with cystic component. The cystic component increased in the larger tumor. The hepatic metastases were enhanced remarkably during the hepatic arterial-dominant phase scan and had abundant tumor vascular and tumor stain on hepatoarterial angiography. The size of tumor in 4 cases was reduced after TACE. The tumor in the patient who was treated with PCS had no changed. The size of the tumor in 3 cases was slightly enlarged. The tumors in the 3 patients treated with PTND were reduced remarkably. CONCLUSIONS: The predominant blood-supply of the hepatic metastases from small intestinal leiomyosarcoma is the hepatic artery abundant in blood vessels. TACE, PCS and PTND are effective methods to treat hepatic metastases from small intestinal leiomyosarcoma.

Angiography↗

[A case of primary pleural leiomyosarcoma].

A 70-year-old man was admitted to our hospital with complaints of chest pain and exertional dyspnea. Chest radiography and computed tomography (CT) revealed right pleural effusion and pleural thickening on admission. The pleural fluid was bloody. Microbiological and cytologic examinations of the fluid were negative. The chest CT revealed progress of pleural thickening after hospitalization. A thoracoscopic pleural biopsy was performed, and the histological finding of the excised specimen was leiomyosarcoma. Because no organ of origin of the leiomyosarcoma, other than the pleura, was detected, this case was diagnosed as a primary pleural leiomyosarcoma. It is thought that leiomyosarcoma originating from the pleura is rare.

Aged↗

Evaluating prognostic parameters in women with uterine leiomyosarcoma. A clinicopathologic study.

OBJECTIVE: To evaluate the prognostic impact of different clinicopathologic parameters in patients with uterine leiomyosarcoma. STUDY DESIGN: Twenty-one patients with histologically proven uterine leiomyosarcoma were included in the analysis. Leiomyosarcomas were defined as uterine smooth muscle tumors with > or = 5 mitoses per 10 high-power fields and nuclear atypia and/or necrosis. RESULTS: The median follow-up time was 47 months; 5-year overall survival was 41%. A univariate Cox model revealed that early tumor stage (P = .00001), age at diagnosis < 50 years (P = .02), absence of vascular space involvement (P = .04), low myometrial invasion (P = .006) and low histologic grade (P = .04) were associated with lengthened overall survival. Adjuvant radiotherapy and/or chemotherapy (P = .1) did not influence overall survival. CONCLUSION: Early tumor stage, age at diagnosis < 50 years, absence of vascular space invasion, low myometrial invasion and low histologic grade were parameters of a good prognosis in women with uterine leiomyosarcoma. Adjuvant chemotherapy and/or radiotherapy showed no benefit in these patients.

Adult↗

Immunohistochemical analysis of a primary ovarian leiomyosarcoma. Case report.

BACKGROUND: Primary ovarian leiomyosarcoma is an extremely rare malignant smooth muscle neoplasm. We report a case of a primary leiomyosarcoma of the ovary managed at our institution. CASE REPORT: Surgical exploration in a 71-year-old woman revealed a large left adnexal tumor and one separate metastatic implant in one part of the omentum, being classified as a primary ovarian leiomyosarcoma stage III C. The patient was treated with an adjuvant chemotherapy regimen consisting of cisplatin and ifosfamide. The patient remained without any evidence of disease until she died due to apoplexy 14 months after initial surgery. High cellularity, nuclear polymorphism as well as patchy necrosis and large areas of hemorrhage, characterized the tumor. Mitotic activity exceeded 5 mitoses per 10 high-power fields with lots of atypical mitoses. Immunohistochemically, the tumor showed a strong positive staining reaction for muscle actin, alpha-smooth muscle actin and neuron-specific enolase as well as a weak positive reaction for vimentin. Furthermore, immunohistochemistry revealed a positive staining for Ki-67, MMP-1 and MMP-2, and VEGF. CONCLUSION: Until recently, prognostic parameters in ovarian leiomyosarcoma have still not been identified. Thus, the prognostic value of different clinicopathological and immunohistochemical parameters, especially Ki-67, MMPs and VEGF, should be proven in additional cases with long-term follow-up.

Aged↗

Leiomyosarcoma in dogs: 44 cases (1983-1988).

During a 5-year period, leiomyosarcoma was diagnosed in 57 dogs. Forty-four dogs were included in the study on the basis of completeness of medical records. All dogs underwent exploratory laparotomy, and dogs were allotted to 4 groups according to primary site of tumor: spleen (16 dogs, median age 10.3 years), stomach/small intestine (13 dogs, median age 10.3 years), cecum (10 dogs, median age 11.8 years), and liver (5 dogs, median age 9 years). All dogs with leiomyosarcoma of the liver had visible metastasis and were euthanatized at surgery. In the other 3 groups, 79% of the dogs had no gross evidence of metastasis at surgery, and 64% survived greater than 2 weeks. Median survival in these 3 groups was 10 months (range, 1 month to 7 years); 48% died of metastasis, 32% died of unrelated causes, and 16% died of unknown causes. The prognosis in dogs with leiomyosarcoma of the spleen, stomach, small intestine, and especially the cecum is good to excellent if surgery is performed. In dogs with leiomyosarcoma of the liver, the prognosis is poor.

Animals↗

[Leiomyosarcoma of the breast].

A 62-year old patient was treated at our hospital for a palpable change in the upper part of the left breast. The clinical finding was preoperatively confirmed by radiologic, ultrasound and cytology studies, however, the potential biologic process (malignant/benign) was not thus verified. Considering undefined diagnostic nature of the process, biopsy, pathohistology and immunoanalysis were performed to indicate leiomyosarcoma mammae. The incision interspace at certain sites was less than two centimeters, indicating radical breast excision in toto (ablation with evacuation of the contents of the axilla). At the time of scheduled surgery, the patient sustained cardiologic discomfort, so tumorectomy was supplemented by locoregional radiotherapy. Leiomyosarcoma of the breast is a very rare primary malignant breast tumor, with only 14 cases reported in the literature. Considering the rare occurrence of leiomyosarcoma of the breast, in radical surgery we used attitudes like in other breast tumors and leiomyosarcoma of other localizations.

Breast Neoplasms↗

Primary venous leiomyosarcoma: a rare but lethal disease.

Primary venous leiomyosarcoma is rare, only 197 patients have been reported. To examine progress in diagnosis, treatment, and clinical outcome, we retrospectively reviewed data of 13 patients, 12 women and one man (mean age, 55 years; range, 19 to 75 years), who in the last 35 years underwent surgical treatment for primary venous leiomyosarcoma at our institution. The tumor arose from the inferior vena cava in eight, iliac vein in two, ovarian vein in one, and greater saphenous vein in two patients. Primary venous leiomyosarcoma was detected by physical examination in nine patients, symptoms were present in six. The 13 patients underwent 16 surgical procedures to resect primary (12), recurrent (2), or metastatic (2) tumors. Local excision alone was performed in seven, and local excision with reconstruction of the inferior vena cava or iliac vein was performed in six patients. The tumor was greater than 10 cm in eight patients. The perioperative mortality rate was 15% (2 of 13). Median survival of the 11 early survivors was 3.5 years (range, 6 months to 17 years). Currently five patients are alive (four of them free of known tumor), with a median survival of 3 years (6 months to 17 years) after surgery. Of the eight deceased patients, primary venous leiomyosarcoma recurred after resection in six. Tumor recurrence was not affected by tumor grade, size, or adjuvant treatment. Although early detection with modern imaging techniques could potentially be of benefit, wide local excision with selective venous reconstruction affords the only hope for prolonged survival.

Adult↗

Leiomyosarcoma of the small intestine associated with von Recklinghausen's disease: report of a case.

A 54-year-old woman with intestinal multiple smooth muscle tumors including leiomyosarcoma, epithelioid leiomyoma, and leiomyomas in association with von Recklinghausen's disease is reported. Thirteen years after the excision of an intestinal leiomyosarcoma, another leiomyosarcoma arose in a different area of the jejunum and was also completely resected. Although leiomyomas are occasionally recognized in patients with von Recklinghausen's disease and a gastrointestinal neoplasm, there have been no other reports of leiomyosarcomas. Careful observation of the tumor is necessary and, if a rapid increase in tumor size is recognized, malignant tumor may have arisen and early surgical treatment is required.

Aortography↗

An unusual abdominal tumor--leiomyosarcoma of the mesentery: a case report.

We report the case of an 82-year-old woman who presented with an ill-defined mass and tenderness in the right lower quadrant of the abdomen. On computerized tomography scan, she had an irregular large bosselated mass of the mesentery located medial to the cecum and associated with multiple loops of small intestine. Preoperative differential diagnoses included leiomyosarcoma, lymphoma and gastrointestinal stromal tumor. The mesenteric mass was resected and identified as a leiomyosarcoma. This patient's tumor is typical of a mesenteric leiomyosarcoma and recurred with hepatic metastases two years after its initial removal. Such tumors usually do not become symptomatic until late in the course of disease, often after distant metastases have occurred. Mesenteric leiomyosarcomas are rare and have a reported incidence of 1: 350,000.

Aged, 80 and over↗

Lower gastrointestinal bleeding due to small bowel metastasis from leiomyosarcoma in the tibia.

Small bowel metastasis from primary bone leiomyosarcoma is very rare. Here we report on a 50-year-old man who presented with general weakness, weight loss (six kg in two months) and intermittent tarry stools for two months. He had undergone an above-knee amputation for left tibia leiomyosarcoma seven years previously. No local recurrence and/or distant metastasis developed during a seven-year period of follow-up. Subsequent imaging study revealed a multilobulated mass in the ileum. He received segmental resection of the small bowel and a multilobulated mass was noted in the submucosal layer of the ileum with mucosa ulceration. His postoperative course was uneventful. Histopathological examination of the resected mass revealed small bowel metastatic leiomyosarcoma. No local recurrence or distant metastases were detected during a six-month follow-up period. To the best of our knowledge, this is the first report of small bowel metastasis from primary bone leiomyosarcoma presenting with lower gastrointestinal bleeding.

Bone Neoplasms↗

[Leiomyosarcoma of the Meckel's diverticulum and multiple diverticulosis of the ileum. Apropos of a case].

We report a case of a leiomyosarcoma arising in a Meckel's diverticulum in a man of 90 years, with multiple ileal diverticulosis. The clinical picture was similar to acute appendicitis and diagnosis was not made until resection of an abdominal mass with histological appearance of leiomyosarcoma. 59 cases of leiomyosarcoma of Meckel's diverticulum have previously been reported in the literature. No case until now has been reported in a patient of Caribbean origin neither in association with ileal diverticulosis. Although rare, leiomyosarcoma is the commonest sarcoma of Meckel's diverticulum, and with full resection of the tumor the prognosis is very good.

Aged↗