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PubMed1993

Primary leiomyosarcoma of the skin. A histological and immunohistochemical analysis.

We report a rare case of a cutaneous leiomyosarcoma on the extensor surface of the proximal part of the left arm of an 84-year-old man. The tumour exhibited a number of histological and immunohistochemical features which are characteristic of a leiomyosarcoma. Leiomyosarcomas are frequently misdiagnosed on clinical grounds. Therefore, clinical features, differential diagnosis, histological and immunohistochemical criteria, therapy and prognosis of this rare malignant tumour are summarized on the basis of the present case and previously published reports.

Aged↗

PubMed1998

A case of giant leiomyosarcoma of the rectum.

A 63 year-old-man was admitted to our hospital with the chief complaint of dyschezia. Digital examination revealed a large solid mass on the posterior wall of the rectum. Endoscopically, the tumor was covered by an intact mucosal layer. Under the diagnosis of rectal leiomyosarcoma, abdominoperineal resection of the rectum was performed. The tumor was 10 x 9 x 8 cm in size, and its cross section showed a gray-white tumor with central necrosis. Microscopically, the large tumor of the rectum was mainly located in the proper muscle layer and adventitia, and showed cellular proliferation of spindle-shaped and mild pleomorphic stromal cells, arranged in interlacing fashion, and focal necrosis. The histologic findings support the diagnosis of leiomyosarcoma. Leiomyosarcoma of the rectum is a relatively uncommon disease. We report our case with reference to the literature.

Humans↗

PubMedDate not supplied

Leiomyosarcoma versus myofibrosarcoma: observations and terminology.

Two spindle cell sarcomas, which showed similarities in light microscopic histology and immunostaining and were diagnosed as leiomyosarcomas by these criteria, were compared ultrastructurally to show the value of electron microscopy in subtyping these neoplasms. Both were subcutaneous, case 1 occurring in the nasolabial fold and case 2 in the upper calf. Both consisted of fascicles of spindle cells, and both stained positively for vimentin and alpha-smooth muscle actin; only case 2 stained additionally for desmin. Case 1 showed strong and case 2 weak or negative staining for fibronectin. By electron microscopy, case 1 contained prominent rough endoplasmic reticulum, peripheral fine filaments with focal densities, and fibronexus junctions. By contrast, case 2 was characterized by an external lamina and well-developed bundles of fine filaments with focal densities. Case 2 was considered a typical leiomyosarcoma, and case 1 was interpreted as showing myofibroblastic differentiation. The nomenclature for these myofibroblastic tumors is discussed, and myofibrosarcoma and leiomyosarcoma, myofibroblastic variant are suggested as suitable terms.

Adult↗

PubMed1995

The prognostic significance of stage, tumor size, cellular atypia and DNA ploidy in uterine leiomyosarcoma.

To analyze the significance of DNA ploidy in uterine leiomyosarcoma, the traditional clinical and histopathological prognostic variables and DNA ploidy were studied in 70 patients with histologically verified uterine leiomyosarcoma. Evaluable flow cytometric DNA histograms from paraffin-embedded tissue from the tumor were obtained in 58 patients. In univariate analysis tumor diameter, FIGO stage and presence of residual disease after primary surgery were highly significant (p < 0.001) and also DNA ploidy (p = 0.043), age (p = 0.017), and menopause status (p = 0.028) obtained significance. Cellular atypia was almost significant (p = 0.056), while mitotic count, malignancy grade and vessel invasion were not. In Cox's multivariate analysis, FIGO-stage was found to be the most important prognostic factor (p < 0.001), followed by cellular atypia (p = 0.007) and tumor diameter (p = 0.016). DNA ploidy did not obtain significance when categorized as diploid/non-diploid. Patients with tumors with multiple aneuploid cell populations had a very poor prognosis. When categorized as multiple aneuploidy versus all other ploidy groups, DNA ploidy obtained marginal significance in multivariate analysis (p = 0.054). Tumor diameter, stage and cellular atypia are important prognostic parameters in uterine leiomyosarcomas.

Adult↗

PubMed1995

[A report of leiomyosarcoma of the esophagus].

Leiomyosarcoma of the esophagus is an uncommon disease of which only 97 cases including the present case have been reported in Japan. We report a case of the tumor which showed multiple hematogenous metastases after surgery. A 73-year-old male was admitted complaining of dysphagia and vomiting. Esophagography and endoscopy revealed a large protruding lesion in the lower esophagus. CT scanning revealed threefold-sized extramural mass. Boring biopsies failed to yield evidence of malignancy. However, we performed surgical treatment because of the uncommon size for a benign tumor. The excised tumor was 11 x 9 x 5 cm in size and was diagnosed histologically as leiomyosarcoma of the esophagus without any nodular involvement. Metastatic tumor in the right rib was found 14 months after the operation. Radiotherapy failed to decrease tumor size but eliminated pain. Bone metastases appeared successively and the patient died 3 years and 4 months after operation. Chemotherapy had no effect. Autopsy revealed metastases to the ribs, vertebrae, sternum, pelvic kidneys and diaphragm, but no local recurrence. There is a great need for the development of effective anti-cancer drugs for leiomyosarcomas, particularly in cases with extensive metastasis, such as presented here.

Adult↗

PubMed2004

[A patient with von Recklinghausen's disease associated with polymyositis, asymptomatic pheochromocytoma, and primary hepatic leiomyosarcoma].

A 72-year-old woman with von Recklinghausen's disease was referred to our hospital because of pain and muscle weakness in her thighs. She had elevated serum values of creatine kinase, aspartate aminotransferase, alanine aminotransferase, lactate dehydrogenase, and aldolase. Based on these results, a diagnosis of polymyositis was made. Treatment with prednisolone improved muscle strength, and laboratory values returned to normal. Computed tomography, magnetic resonance imaging of the abdomen, and 131I-metaiodobenzyl guanidine MIBG scintigraphy demonstrated a tumor 3 cm in diameter in the region of the left adrenal gland. Endocrinologic investigation disclosed elevation of serum and urine catecholamines. Since the blood pressure was normal, nonfunctioning pheochromocytoma was diagnosed clinically. The nonhypertensive course was attributed to reduced vascular response to noradrenaline. Serum lactate dehydrogenase. alkaline phosphatase. and asparate aminotransferase became elevated, and abdominal computed tomography showed a well-defined mass measuring 13 x 12 x 10 cm in the right lobe of the liver. The patient underwent right trisegmentectomy and left adrenalectomy. Histologically the adrenal tumor was a typical pheochromocytoma. The hepatic tumor was a leiomyosarcoma consisting of elongated spindle-shaped atypical cells arranged in intersecting bundles. Immunohistochemically, the cells of this tumor were reactive for alpha-smooth muscle actin and vimentin. The leiomyosarcoma recurred and metastasized to the liver. Eight months after onset of symptom, the patient developed hepatic coma and died. The mean age at presentation with pheochromocytoma in von Recklinghausen's disease patients age is 42 years. Our patient was considerably older. To the best of our knowledge this is the first report of a patient with von Recklinghausen's disease developing polymyositis. asymptomatic pheochromocytoma, and primary hepatic leiomyosarcoma and illustrates the need to remain aware of the possibility of cancer in von Recklinghausen's disease.

Adrenal Gland Neoplasms↗

PubMed2003

Pararenal leiomyosarcoma of the inferior vena cava.

A complete surgical resection is the only proven therapeutic modality that prolongs the survival in patients with leiomyosarcoma of the inferior vena cava (IVC). Reconstruction of the IVC is not always necessary but is often required to facilitate venous drainage of the kidney for the tumors at the pararenal area of the IVC. Controversy exists in postoperative adjuvant therapy. Recently, we experienced four cases of pararenal leiomyosarcoma of the IVC, of which treatment consisted of a complete resection of the tumor, ringed polytetrafluoroethylene (PTFE) graft interposition, and bilateral renal vein reconstructions in all patients. Postoperative radiation therapy was instituted in 3 of 4 patients. One patient who did not receive the postoperative radiation therapy was treated with adjuvant chemotherapy. The kidneys were preserved in all patients and no deep vein thrombosis (DVT) or venous insufficiency of the lower extremity veins developed. Distant metastasis to the lung was noted in one patient at 18 months after surgery, who was not received the postoperative radiation therapy but chemotherapy. In conclusion, a complete resection of the tumor, IVC reconstruction, and bilateral renal vein reconstruction followed by adjuvant radiation therapy is recommended for the treatment of pararenal leiomyosarcoma of the IVC.

Adult↗

PubMedDate not supplied

Transient shrinkage of a uterine leiomyosarcoma treated with GnRH agonist for a presumed uterine leiomyoma: comparison of magnetic resonance imaging finding before and during GnRH agonist treatment.

We present a case of a premenopausal woman treated with GnRH agonist for a presumed uterine leiomyoma. This tumor reduced at first, but subsequent surgical specimens revealed a leiomyosarcoma. Magnetic resonance imaging (MRI), one of the most useful modalities for distinguishing between uterine leiomyoma and leiomyosarcoma, was undertaken twice, before GnRH agonist administration and then after 6 months of GnRH agonist administration. Apparent differences were observed between these MRI findings. Tumor-size reduction with GnRH agonist treatment does not always mean that the possibility of a leiomyosarcoma should be ruled out.

Antineoplastic Agents, Hormonal↗

PubMed1999

A case of primary leiomyosarcoma of the lesser omentum.

Leiomyosarcoma is a rare tumor that originates in the smooth muscle, usually in the gastrointestinal tract, the retroperitoneum and the genitourinary tract. The omentum has been rarely reported as a primary site of occurrence with leiomyosarcoma. A 72 year-old woman, with palpable mass on the left upper quadrant of abdomen and weight loss, was admitted to St. Paul's Hospital. Abdominal CT scan showed a 12 x 8 x 8 cm-sized cystic mass in the left upper quadrant of the abdomen. Endoscopic ultrasonography showed a large cystic mass between the stomach and the liver, which was 1.6 cm length in wall thickness. Laparotomy and resection of the mass was performed. A 12 x 8 x 8 cm-sized mass, originated from the lesser omentum, was discovered near the lesser curvature of the stomach. Microscopic examination revealed spindle-shaped cells with 7-8 mitoses per high power field. She was diagnosed as primary leiomyosarcoma originated from the lesser omentum.

Aged↗

PubMed2002

A case of primary myxoid leiomyosarcoma of the liver.

Primary hepatic leiomyosarcoma is very rare and no primary myxoid leiomyosarcoma in the liver has been reported yet. Most cystic space-occupying lesions in the liver are benign in nature. But, rarely, malignancy could appear as a cystic lesion by ultrasonographic examination. A 64-year-old woman with a huge cystic mass detected by hepatic ultrasonography was diagnosed as primary hepatic myxoid leiomyosarcoma by immunohistochemical and ultrastructural studies after various image studies and fine needle aspiration biopsy of the liver mass.

Female↗

PubMed2006

[Penile leiomyosarcoma: case report and bibliographic review].

OBJECTIVE: Primary penile leiomyosarcoma is a rare entity. Since 1930 only 30 cases have been reported in the bibliography. We wanted to add a new case to the international literature, in addition to a review of all available publications on the topic from 1957. A 54-year-old patient presented with a lobulated 8x4x3 cm lesion in the balanopreputial groove over two years; he was treated initially with partial penectomy and subsequently with total penectomy We analyze the evolution, progression and adjuvant treatments of this rare pathology. METHODS/RESULTS: We report one case with its clinical presentation, diagnostic tests performed for staging, treatment and follow-up. CONCLUSIONS: The first case of penile leiomyosarcoma was described by Levi in 1930. In 1957 Ashley and Edwards reported the first case in the British literature and in 1963 Pack reported the first in the American literature. MacKenzie et al. were the first to recognize two types of leiomyosarcoma: superficial and deep. The first develop from smooth muscle cells from the superficial dermal layers of the glans penis or distal third of the penis, they are generally asymptomatic and less malignant, rarely invading deeper structures and without involvement of the urethra on physical exam. The best prognostic predictors are type of tumor at presentation (superficial vs. deep) and treatment choice, being total penectomy more effective for failures of local resections than for primary treatment of deep tumors.

Humans↗

PubMedDate not supplied

Canine gastric adenocarcinoma and leiomyosarcoma: a retrospective study of 21 cases (1986-1999) and literature review.

This retrospective study describes the clinical course, treatment, and outcome of 21 dogs with gastric adenocarcinomas (n=19) and leiomyosarcomas (n=2). Medical records from 1986 to 1999 were reviewed for signalment, weight, diagnosis, tumor location, clinical signs, radiographic imaging procedures, surgical procedures, chemotherapy, duration of follow-up monitoring, outcome, cause of death, metastatic rate, metastatic sites, and method of detection of metastasis. Fourteen of 19 (74%) dogs with gastric adenocarcinomas had metastasis. Metastatic sites included gastric lymph nodes, omentum, liver, duodenum, pancreas, spleen, esophagus, adrenal glands, and lungs. Both cases of a gastric leiomyosarcoma had metastatic disease involving the liver (n=2) and duodenum (n=1). Surgery, consisting of either a Billroth I or a gastrojejunostomy, provided immediate relief of the gastric outflow obstruction and clinical improvement in the early postoperative period. The beneficial effects of chemotherapy alone or adjuvant chemotherapy are still unknown. Recurrence of clinical signs 3 days to 10 months after surgery caused all owners to elect euthanasia. The long-term prognosis for most cases of gastric adenocarcinomas and leiomyosarcomas is poor because of the presence of advanced disease. Surgical resection, however, does alleviate gastric outflow obstruction in the immediate postoperative period.

Adenocarcinoma↗

PubMed2000

Epithelioid leiomyosarcoma with osteoclast-like giant cells in the rectum.

We report a rare case of rectal epithelioid leiomyosarcoma with osteoclast-like giant cells. A 71-year-old Japanese man was admitted to a hospital with melena. Results of a colonoscopy test revealed a polypoid tumor in the rectum, and a biopsy specimen from the lesion showed a sarcoma; the patient underwent rectosigmoidectomy. At gross inspection, the tumor measured 8 x 7 x 4 cm and was polypoid with ulcerations. Necrotic and hemorrhagic foci were scattered. Microscopically, the tumor consisted of 2 cell types: malignant tumor cells with epithelioid features and benign-appearing osteoclast-like giant cells. The tumor cells were polygonal and epithelioid in shape and had eosinophilic or clear cytoplasms, with scattered giant tumor cells. Immunohistochemical examination revealed that the tumor cells were positive for vimentin, muscle actin, alpha-smooth muscle actin, and desmin, whereas the osteoclast-like giant cells were positive for CD68, leukocyte common antigen, and lysozymes. We diagnosed this case as epithelioid leiomyosarcoma with osteoclast-like giant cells. To the best of our knowledge, this is the first case of rectal epithelioid leiomyosarcoma with osteoclast-like giant cells.

Aged↗

PubMed2001

Primary leiomyosarcoma of the pancreas: a case report and review of literature.

Primary leiomyosarcoma of the pancreas is a rare tumor for which only 21 reports appear in the world literature. We describe an additional case of pancreatic leiomyosarcoma in a 76-year-old man, who complained of persistent high fever. Histologic examination revealed a pleomorphic spindle cell tumor. Reactivity for muscle-specific actin, alpha-smooth muscle actin, and basement membrane components, along with negative staining for epithelial and neural markers, were consistent with a smooth muscle sarcoma. The patient died of disease 1 year after complete surgical excision. This report highlights the need to use a complete antibody panel in order to accurately immunophenotype pleomorphic malignant tumors of the pancreas. A review of the cases compiled in the literature indicates that pancreatic leiomyosarcoma, like its counterpart arising in deep soft tissues, is an aggressive neoplasm characterized by short survival and a high rate of metastases.

Actins↗

PubMed2002

Epithelioid leiomyosarcoma of the external deep soft tissue.

Epithelioid leiomyosarcoma in the external deep soft tissue is extremely rare. Most epithelioid leiomyosarcomas occur in the uterus. We present a case of epithelioid leiomyosarcoma occurring in the muscle of the thigh of a 78-year-old man. Histologically, the tumor predominantly consisted of round or polygonal cells arranged in sheets with a focal spindle cell component. Immunohistochemical analysis revealed that the tumor cells expressed vimentin, alpha-smooth muscle actin, and alpha-sarcomeric actin. The tumor was negative for desmin, S100 protein, glial fibrillary acidic protein, pan-keratin, epithelial membrane antigen, CAM 5.2, HMB-45, leukocyte common antigen, factor VIII-associated antigen, and CD34. Electron microscopically, some tumor cells contained abundant actin-type filaments in their cytoplasm.

Actin Cytoskeleton↗

PubMed2003

Pleomorphic leiomyosarcoma of the adrenal gland.

Primary leiomyosarcomas arising in the adrenal gland are exceedingly rare, with only 3 cases reported in the literature. We present the clinical, morphologic, and immunohistochemical features of a pleomorphic leiomyosarcoma, a variant of leiomyosarcoma that has not been described in the adrenal gland. A 63-year-old man presented with a 1-year history of enlarging right upper quadrant mass and pulmonary nodule. A diagnosis of metastatic pulmonary carcinoma to the adrenal gland was rendered on a needle biopsy specimen. Preoperative chemotherapy reduced only the pulmonary mass but not the adrenal mass, which continued to enlarge. Documented by computed tomography and confirmed at surgery, the tumor had completely replaced the right adrenal gland, invading into both the posterior aspect of the right liver and the superior pole of the right kidney. Histologic sections showed a diffuse proliferation of pleomorphic, large, and polygonal neoplastic cells with prominent nucleoli. Many bizarre mitotic figures were present. The neoplastic cells were strongly positive for desmin, calponin, and vimentin. Approximately 80% of the neoplastic cells were positive for the proliferation marker Ki-67. They were negative for smooth muscle actin, muscle-specific actin, myoglobin, myogenin, CD117, cytokeratins, carcinoembryonic antigen, epithelial membrane antigen, chromogranin, CD34, CD31, S100 protein, and HMB-45.

Adrenal Gland Neoplasms↗

PubMed2004

Leiomyosarcoma of the urinary bladder in a teenage male.

Malignant neoplasms of the bladder are typically transitional cell carcinoma with squamous cell carcinoma and adenocarcinoma comprising nearly all of the remaining cases. Leiomyosarcoma is a rarely seen neoplasm of the bladder with a generally poor prognosis. It is important to distinguish leiomyosarcomas from rhabdomyosarcoma with the aid of immunohistochemical markers. A therapeutic dilemma exists with regard to the need for urethrectomy. We present the third reported case of leiomyosarcoma of the bladder in a teenage male patient.

Adult↗

PubMed1998

[Diagnostic imaging of gastrointestinal leiomyosarcoma. Experience with 12 cases].

PURPOSE: Gastrointestinal (GI) leiomyosarcoma is an uncommon malignant cancer arising in the smooth muscle of the alimentary tract. It is known for its widely variable patterns and aspecific symptoms and signs preventing correct clinical assessment in the majority of cases. We will illustrate the key role of diagnostic imaging in the detection and staging of this lesion, describing the most suggestive imaging findings for the correct diagnosis. MATERIAL AND METHODS: January, 1990, to June, 1998, we examined 12 patients with GI leiomyosarcoma; they were 10 men and 2 women whose age ranged 42 to 85 years (mean: 63.7 years). Four lesions were found in the stomach, 3 in the jejunum and ileum, and 2 in the rectum. Due to the difficult clinical assessment of this type of lesion and to the development of emergency conditions, we could plan no diagnostic protocol in advance; thus, the most suitable diagnostic imaging approach was decided on the spot for studying the supposedly involved GI portions. Double contrast studies, US, CT and endoscopy were performed and each patient underwent at least two examinations. RESULTS: Barium contrast studies were performed in 9 patients: the lesion was detected in 7 cases and tumor site and extent were defined in 5, while the double contrast study of the colon allowed to exclude large bowel involvement in 2 ileal tumors. In all 9 cases US and US-guided endoscopy permitted better assessment of extra-luminal spread and involvement of adjacent organs. CT, which is essential to staging, provided useful information suggesting the lesion nature: a round, inhomogeneous mass in continuity with the intestinal wall, with irregular margins, peripheral enhancement after i.v. injection of contrast material and a central necrotic area. Histology confirmed CT diagnosis in 7/9 cases while an aspecific diagnosis of large retroperitoneal and abdominal lesion was made in 2 cases. CT did not allow to define the origin of 2 large exophytic lesions in the stomach and jejunum and missed peritoneal metastases in 3 cases. CONCLUSIONS: Although the aspecific and quite variable clinical patterns make it extremely difficult to plan a correct diagnostic protocol, in our experience all diagnostic imaging techniques played a fundamental role in identifying and staging alimentary tract leiomyosarcoma. Particularly, CT showed high sensitivity and specificity in characterizing and staging this lesion but exhibited rather poor sensitivity in recognizing peritoneal spread.

Adult↗