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A resected case of giant leiomyosarcoma of the pancreas.

Leiomyosarcoma of the pancreas is very rare. We report a case of giant leiomyosarcoma of the pancreas treated by distal pancreatectomy. A 53-year-old female was admitted with an large abdominal mass. Computed tomography, magnetic resonance imaging, and ultrasonography revealed a huge tumor adjacent to the pancreas. However, we could not identify the primary organ with these imagings. Angiographic findings strongly suggested that the tumor originated from the pancreas, as main feeding arteries arose from the great pancreatic artery. Fourteen days after trans-catheteric arterial embolization, we performed a distal pancreatectomy with splenectomy; the patient's postoperative course was uneventful. Histologically, we confirmed the diagnosis as a leiomyosarcoma originating from the pancreas.

Female↗

Primary leiomyosarcoma arising in the lesser sac: report of a case.

We describe herein a very rare case of leiomyosarcoma arising in the lesser sac. A 58-year-old man with a psychiatric disorder was admitted to our department for the investigation of epigastralgia and nausea which he had been suffering since the previous month. A laparotomy revealed that the abdominal mass, found on physical examination, was a primary lesser omental tumor, histological examinations of which confirmed a diagnosis of leiomyosarcoma. The tumor cells showed a DNA diploid pattern. Leiomyosarcoma of the lesser sac is extremely unusual and it is important that it be distinguished from an extraluminal tumor of the abdomen.

DNA, Neoplasm↗

Leiomyosarcoma of the larynx.

Leiomyosarcomas account for approximately 7% of all soft-tissue sarcomas and occur most frequently in the gastrointestinal tract and uterus. Leiomyosarcomas of the larynx are extremely rare with only about 27 cases reported in the available literature. We present a leiomyosarcoma of the left vocal cord that was treated by transoral CO2 laser surgery. To date, the patient has been free of disease for more than 25 months. Since these tumors may be difficult to diagnose, immunohistochemical studies may be necessary for making an unambiguous diagnosis. Primary treatment should be surgical resection, with small tumors suitable for partial laryngectomy or transoral laser surgery.

Humans↗

Primary leiomyosarcoma of the jejunal mesentery: report of a case.

The occurrence of a primary leiomyosarcoma of the mesentery is rare. A 61-year-old man was admitted to the hospital complaining of an abdominal mass. The findings of both abdominal ultrasonography and a computed tomography (CT) scan revealed an irregular and heterogeneous mass located in the mesentery. A laparotomy was performed and a 7.0 x 6.5 cm tumor was thus found within the jejunal mesentery. The tumor was successfully resected by a combined resection of 40 cm of the jejunum and end-to-end anastomosis of the jejunum. Pathological examination of the resected specimen revealed leiomyosarcoma. The patient had an uneventful postoperative course, but multiple liver metastases were discovered 1 year and 5 months after the initial operation. A second operation was performed, but the patient died due to hepatic failure and unexpected bleeding from the cut surface of the remnant liver. Preoperative imaging examinations, including abdominal ultrasonography and CT scan, were thus found to be useful tools for both identifying and diagnosing the origin and extension of a mesenteric mass. However, even using such diagnostic techniques an accurate diagnosis of intraabdominal leiomyosarcoma remains difficult.

Humans↗

Leiomyosarcoma of the inferior vena cava: angiographic and computed tomography findings. Report of two cases and review of imaging criteria.

Leiomyosarcoma, a highly malignant tumor of the inferior vena cava, is rare. Only 55 cases have been reported in the world literature, and of these only 18 were evaluated with a special vascular procedure, either arteriography or inferior vena cavography. In two cases of leiomyosarcoma of the inferior vena cava, we performed arteriography and inferior vena cavography. In one, computed tomographic studies were also carried out. Cavography showed a lobulated filling defect in one case and complete caval occlusion with collateral circulation in the other. In the one case in which it was performed, computed tomography clearly demonstrated the tumor's size and its relationship to surrounding organs. Arteriographic studies, however, allowed only an indistinct delineation of the extent of tumor growth in one case. Venography followed by computed tomography should permit adequate assessment of most leiomyosarcomas of the inferior vena cava, with arteriography reserved for tumors involving the upper cava in which hepatic involvement must be evaluated.

Adult↗

Synchronous leiomyosarcoma and ameboma of the rectum: report of a case.

A case of leiomyosarcoma of the rectum associated with rectal amebiasis is presented. This is the first case to be reported. Initially, amebiasis obscured the histologic diagnosis of leiomyosarcoma, but incomplete regression following a course of treatment with metronidazole aroused the suspicion of malignancy, which was confirmed on repeat biopsy. A brief review of the features of leiomyosarcoma was also undertaken.

Aged↗

Renal vein leiomyosarcoma: a case report and literature review.

A case of leiomyosarcoma of the renal vein occurring in a 48-year-old woman is reported, and the literature is reviewed. Ultrasonography (US) revealed a rounded uniform echogenic mass in the renal hilus. Computed tomography (CT) showed a well-demarcated, uniform, soft tissue density mass. Both US and CT were useful to detect the renal mass in the present case, and may be useful for staging of renal vein leiomyosarcoma. However, these imaging procedures did not reveal diagnostic features of leiomyosarcoma.

Female↗

Leiomyosarcoma of the female breast.

Leiomyosarcomas of the breast are rare tumors. Less than 15 such cases have been reported in the literature so far. In this paper authors describe a case of leiomyosarcoma of a female breast presenting as a firm lobulated mass, mimicking a phylloid tumor radiographically. By fine needle aspiration biopsy, on the smears discohesive malignant looking cells were conclusive to a poorly differentiated invasive ductal carcinoma of the breast. The mastectomy specimen contained a lobulated mass, microscopically showing a partly epithelioid spindle cell tumor, immunoreactive for vimentin, desmin, smooth muscle actin antibodies, and negative for epithelial markers, hormone and growth factor receptors. Axillary lymph nodes were free of tumor. A primary leiomyosarcoma of the breast was diagnosed.

Actins↗

[Leiomyosarcoma of the esophagus. Clinical aspects, diagnosis and therapy based on an individual case].

Leiomyosarcomas of the esophagus are rare tumors of mesenchymal origin. Apropos of a case we present clinicopathological features, diagnostic procedures and management of this seldom tumor of the esophagus. Primary gastrointestinal sarcomas cause less than 0.5% of all esophageal malign tumors, and present in ca. 5% as esophageal leiomyosarcomas. The most frequent incidence ranges between the fourth and fifth decade of life. The tumors originate from the muscular layers of the esophageal wall and are localized predominantly in the middle and distal third of the esophagus. Dysphagia is the most important and leading symptom although it presents late in the course of the illness. Endosonography is at the time the most accurate method to establish the tumor size. Differentiation between leiomyoma and leiomyosarcoma is only possible by histopathological examination and may be difficult in certain cases. Histopathological grading of the tumors as low- and high-grade sarcomas in dependence of the number of mitosis affects predominantly the prognosis of these patients. Differential diagnosis includes spindle cell carcinoma and carcinosarcoma of the esophagus. The most effective therapy consists in the complete operative removal of the tumor, in these cases five years survival rates of 30 to 40% are achieved, strongly influenced by tumor differentiation and size.

Adult↗

[Leiomyosarcoma of the larynx].

Leiomyosarcoma is usually found in the female genital tract, retroperitoneum, the wall of the gastrointestinal tract and subcutaneous tissues. An appearance of this malignant tumor in the larynx is extremely rare and may be difficult to diagnose. Because of its rarity, little information exists on management and prognosis. We report on a 64-year old male with a supraglottic leiomyosarcoma who was treated with supraglottic laryngectomy and postoperative radiotherapy. After 19 months the patient was well and free of disease. The most commonly used diagnostic and therapeutic procedures are analysed in a review of the 46 published cases of laryngeal leiomyosarcoma.

Combined Modality Therapy↗

Skeletal muscle metastasis from uterine leiomyosarcoma.

A case of a 68-year-old woman who presented with a rapidly enlarging painful right thigh mass is presented. She had a known diagnosis of uterine leiomyosarcoma following a hysterectomy for dysfunctional uterine bleeding. She subsequently developed a single hepatic metastatic deposit that responded well to radiofrequency ablation. Whole-body MRI and MRA revealed a vascular mass in the sartorius muscle and a smaller adjacent mass in the gracilis muscle, proven to represent metastatic leiomyosarcoma of uterine origin. To our knowledge, metastatic uterine leiomyosarcoma to the skeletal muscle has not been described previously in the English medical literature.

Aged↗

Primary adrenal leiomyosarcoma.

Leiomyosarcomas of adrenal origin occur infrequently, always present as huge abdominal masses, and are associated with poor prognoses when other organs are invaded. Radiologic images of small adrenal leiomyosarcomas have not been published. This report presents magnetic resonance images of a 3-cm left adrenal leiomyosarcoma from a 49-year-old male. Ten months after adrenalectomy, the patient was alive without tumor recurrence.

Adrenal Gland Neoplasms↗

[Syntopical and synchronous leiomyosarcoma and papillary carcinoma of the female breast].

A 61 years old female was treated by total lumpectomy due to a palpable and radiologically demonstrable tumor measuring 22 cm in diameter in the left breast. Two different but directly neighboured malignant tumours were found: a poorly differentiated leiomyosarcoma and a well differentiated invasive papillary carcinoma of the breast. The latter had metastasized in 4 axillary lymph nodes. Three months after surgery the patient died. Post-mortem studies showed many metastases of the leiomyosarcoma in both lungs, the heart, the liver, the left kidney and both adrenals. Cause of death were relapsing pulmonary thrombembolisms. Case reports of leiomyosarcomas following radiotherapy of the breast cancer are published but coincidental occurrence of both metastasizing tumors are so far not known from the literature.

Breast Neoplasms↗

[Leiomyosarcoma in the thyroid gland--primary tumor or metastasis?].

In a 69-year-old female patient a moderately pleomorphic spindle-cell thyroid tumour measuring 5 cm in diameter was initially misinterpreted as primary anaplastic thyroid carcinoma. During clinical investigations to elucidate the cause of severe anaemia, 17 months later an ulcerated duodenal leiomyosarcoma was detected and removed by duodenopancreatectomy. Reevaluation of the thyroid nodule led to revision of the initial diagnosis to metastatic leiomyosarcoma. Six months later the patient died from cerebral stroke. Autopsy findings confirmed the diagnosis of primary leiomyosarcoma of the duodenum with initial manifestation as thyroid metastasis.

Aged↗

Uterine leiomyosarcoma metastatic to the brain stem.

A patient with a history of an uterine leiomyosarcoma presented with diplopia, gait disturbances, and hypesthesia of the right face. MRI of the head showed a lesion located in the pons and causing obstructive hydrocephalus. Open biopsy revealed a metastatic tumor with histological features of leiomyosarcoma. Despite whole-brain irradiation, the patient died due to respiratory arrest. This case illustrates that brain stem metastasis may occur as a rare complication in uterine leiomyosarcoma.

Brain Neoplasms↗

High levels of cellular retinol binding protein-1 expression in leiomyosarcoma: possible implications for diagnostic evaluation.

Retinoid bioavailability is regulated by the activity of specific cytoplasmic receptors. High levels of cellular retinol binding protein-1 (CRBP-1) have been documented during experimental arterial and wound-healing processes, but data concerning neoplastic smooth muscle tissues are scarce and/or controversial. This study reports that the expression of CRBP-1 is markedly higher in uterine and gastrointestinal leiomyosarcomas than in leiomyomas and normal myometrium; CRBP-1 was practically absent in normal gastrointestinal smooth muscle tissue. CRBP-1 positivity was particularly elevated in the epithelioid variant of leiomyosarcoma; it was associated with increased proliferative and apoptotic rates and inversely related to smooth muscle differentiation evaluated by alpha- and gamma-smooth muscle actin and desmin expression. Western blotting and reverse-transcription polymerase chain reaction confirmed the observations concerning CRBP-1 and actin isoform expression and revealed higher NF-kappa-Bp65 and RAR alpha and lower Bax protein levels in leiomyosarcoma than in the other conditions. These findings document that a high CRBP-1 expression is associated with smooth muscle malignancy and suggest that CRBP-1 expression represents a new useful marker for the classification of unusual variants of smooth muscle tumors.

Adult↗

Expression of drug resistance related proteins in sarcomas of the pulmonary artery and poorly differentiated leiomyosarcomas of other origin.

Sarcomas are known to develop resistance to current chemotherapeutic strategies, displaying a multidrug-resistant phenotype. Mechanisms involved in drug resistance include reduced cellular drug accumulation, drug detoxification as well as alterations in drug target specificity. In seven sarcomas of the pulmonary artery (SPA) and ten leiomyosarcomas of other origin, we studied the immunohistochemical expression of P-glycoprotein (P-gp), multidrug-resistance protein (MRP), lung resistance protein (LRP), metallothionein (MT) and topoisomerase IIalpha. Upregulation was found in tumour cells for P-gp but not for MRP in SPA and other leiomyosarcomas. Topoisomerase IIalpha was expressed at high levels in tissue of primary tumours as well as recurrent tumours. Both P-gp and topoisomerase IIalpha were present in numerous tumour-associated vessels. LRP was expressed at high levels in SPA but to a lesser extent in the other leiomyosarcomas. MT was expressed at low levels but was markedly present at the border of necrosis. The overall survival and the relapse-free survival did not correlate with the expression of these factors. There was no significant relationship between treated and non-treated patients with respect to the expression of the examined molecules. P-gp, but not MRP, may play a role in the development of drug resistance. P-gp, LRP and topoisomerase IIalpha contribute to drug resistance through expression in tumour-associated vessels. Unique high levels of topisomerase IIalpha reflect the high proliferation rate of these tumours. MT seems to serve as a detoxifying agent of metabolites at the border of necrosis. Our findings underline the fact that multiple factors contribute to chemoresistance and that examination of a spectrum of relevant molecules is probably necessary to plan the best therapy.

ATP Binding Cassette Transporter, Subfamily B↗