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PubMed1999

Primary epithelioid leiomyosarcoma of bone. Case report and literature review.

We describe an epithelioid leiomyosarcoma of bone located in the right knee of a 51-year-old woman. Plain radiograph and CT scan revealed a poorly defined lytic and destructive mass in the upper metaepiphyseal right tibia which involved surrounding soft tissues. The lesion was composed of proliferating monotonous round cells with a high mitotic activity with scanty intersecting spindle cell fascicles. Immunohistochemistry of both areas demonstrated a strong positivity for actin (HHF-35 and alpha-SMA) and vimentin, and negative reactions for desmin, keratin (AE1 AE3), epithelial membrane antigen, S-100 protein, factor VIII-related antigen, CD 31 and CD 34. Ultrastructural study confirmed a diagnosis of leiomyosarcoma. This is the first detailed description of the microscopic and radiological features of primary epithelioid leiomyosarcoma of bone.

Bone Neoplasms↗

PubMed1999

Pancreas-sparing duodenectomy for a huge leiomyosarcoma in the third portion of the duodenum.

A duodenal leiomyosarcoma which was resected by pancreas-sparing duodenectomy is reported. The tumor arose in the third portion of the duodenum and grew in an extraluminal direction. The tumor was huge (13 cm x 9 cm x 8 cm) but did not involve the pancreas, and there were no findings of periduodenal lymph node metastases. Because the possibility of metastasis to the lymph nodes around the root of the superior mesenteric artery (which are removed only in a pancreatoduodenectomy) was judged to be low, pancreas-sparing duodenectomy was performed, with dissection of the pancreaticoduodenal lymph nodes. The proximal duodenum was transected between the second and third portions of the duodenum, and the distal end was cut in the jejunum at the portion of the first jejunal artery. Reconstruction was performed by end-to-side anastomosis between the duodenum and jejunum, using an end-to-end anastomosis instrument. Since the incidence of lymph node metastasis of leiomyosarcoma is low, resection of the head of the pancreas for extensive lymph node dissection does not always seem necessary. Pancreas-sparing duodenectomy can be a good option for a leiomyosarcoma in the third and fourth portions of the duodenum which does not invade the pancreas and is not accompanied by any apparent periduodenal lymph node metastases.

Angiography↗

PubMed2004

Primary adrenal leiomyosarcoma with inferior vena cava thrombosis.

We report a primary adrenal leiomyosarcoma in a 59-year-old man. Computed tomography demonstrated a poorly enhanced mass, measuring 10 cm, between the left kidney and the normal left adrenal gland, with tumor thrombus in the inferior vena cava (IVC). The patient underwent left radical nephroadrenalectomy with IVC thrombectomy. The histological diagnosis was adrenal leiomyosarcoma. Adrenal leiomyosarcomas are extremely rare. Only seven cases have been reported previously in the English-language literature.

Adrenal Gland Neoplasms↗

PubMed2005

Pancreatic and gastric metastases of leiomyosarcoma arising in the left leg.

Pancreatic or gastric metastases from other primary malignancies are rare, especially from leiomyosarcoma. We report a case of leiomyosarcoma in the left lower leg with metastases to the pancreas and stomach. A 61-year-old man had liver cirrhosis caused by hepatitis C virus infection and was followed up by his primary physician. Two years before presentation at our hospital, he had undergone surgical resection of leiomyosarcoma in the left lower leg and systemic chemotherapy for multiple metastatic tumors in the lung. On admission, endoscopic examination and computed tomography were performed for a routine checkup to exclude esophageal varices and liver tumor. Although the patient had no specific symptoms, multiple gastric and pancreatic metastases were identified by endoscopy and computed tomography, respectively. In general, metastases to the pancreas and stomach are rare. We discuss the clinical and diagnostic findings of pancreatic and gastric metastases by reviewing previously reported cases.

Humans↗

PubMed2002

A long-term survivor of leiomyosarcoma around the right side of the base of the skull: effective radiotherapy combined with intra-arterial chemotherapy.

We report a rare case of a leiomyosarcoma that developed around the right side of the base of the skull in a 51-year-old woman. The patient consulted our hospital complaining of pain in the right side of her neck and upper right arm in August 1994. A leiomyosarcoma, originating around the right side of the neck and base of the skull was diagnosed. Initially, surgery was planned, but invasion into the spinal canal was discovered. Curative resection of the leiomyosarcoma around the right side of the base of the skull was not possible. Therefore, external beam radiotherapy (EBRT) combined with intra-arterial chemotherapy and hyperthermia was employed. After the treatment, the tumor decreased in size to 45% of its initial volume, and, simultaneously, her symptoms completely disappeared. The patient initially remained clinically free of the disease, but showed reaggravations at the primary tumor site 3 years and 3 months, and 4 years and 11 months, after the first treatment. The reaggravations were treated with EBRT combined with intra-arterial chemotherapy. As a result, she survived for 5 years and 7 months after the first treatment.

Diagnosis, Differential↗

PubMed2007

Leiomyosarcoma of kidney: a case report with long term result after radiotherapy and chemotherapy.

Leiomyosarcoma of the kidney is a rare entity with poor prognosis. Among renal sarcomas, it is the most common histologic subtype amounting to 50-60% of all cases. The tumors usually arise from either the renal capsule or smooth muscle tissue in the renal pelvic wall. We describe a case of renal leiomyosarcoma that presented with history of left flank pain. Physical examination was normal. Abdominal ultrasound showed a solid lesion of 38 x 36 mm arising from the left kidney. Intravenous urography showed cut off of both major and minor calyces of the left kidney from its pelvis with a normal right kidney. Exploratory laparotomy revealed a well-encapsulated mass confined to the renal parenchyma for which radical nephrectomy was performed. Histopathology showed a Grade I leiomyosarcoma arising from the renal capsule. Adjuvant chemotherapy and sandwich radiotherapy was given and he is disease free after six and half years of follow up. The rarity of this disease, its severity and poor prognosis, uncommon form of presentation, resulting delay in diagnosis and difficulty in management is highlighted and reported.

Combined Modality Therapy↗

PubMed1991

Primary cardiac leiomyosarcomas.

Two patients with primary cardiac leiomyosarcoma, one of the rarest malignant tumors of the heart, are described. The first patient had a tumor of the pulmonary trunk and was admitted with symptoms suggestive of pulmonary thromboembolism. The second had a tumor of the left atrium and had signs and symptoms of mitral valve obstruction and regurgitation. The 2 patients were operated on and later underwent adjuvant chemotherapy. Both died more than 22 months after operation, having required multiple hospital admissions for treatment of complications related to the pathology or the treatment of the primary cardiac leiomyosarcoma. Nonetheless, aggressive surgical excision followed by chemotherapy appears to have improved survival in these patients with primary cardiac leiomyosarcoma, as they are among the longest survivors reported.

Adult↗

PubMed1993

Leiomyosarcoma metastatic to the oral region. Report of three cases.

Leiomyosarcoma, a malignant lesion of smooth muscle origin, is rare in the oral region. Metastatic leiomyosarcoma may originate from several potential primary sites, and the lung is the most common target tissue for metastatic deposits. This article describes three cases of leiomyosarcoma that were metastatic to the oral cavity and discusses the clinical and histopathologic differential diagnosis.

Aged↗

PubMed1986

Spermatic cord sarcoma. Leiomyosarcoma and retroperitoneal lymph node dissection.

Two cases of leiomyosarcoma of the spermatic cord are described. The first case is a seventy-eight-year-old white man with a twenty-year history of leiomyosarcoma of the cord. Twice irradiated, he has had some twenty local recurrences. These have been to the contralateral cord, scrotum, suprapubic region, both inguinal regions, and the penis. The second case is a seventy-three-year-old white man who had radical orchiectomy for leiomyosarcoma of the cord one year ago. The patient remains free of recurrence without other therapy. Other adnexal sarcomas are described, the literature is reviewed, the usefulness of retroperitoneal lymph node dissection discussed, and a plan for therapy and follow-up proposed.

Aged↗

PubMed1985

Leiomyosarcoma of the uterus: clinicopathologic study of 21 cases.

A detailed clinicopathologic study of 21 patients with uterine leiomyosarcoma was undertaken. The diagnosis of leiomyosarcoma was made for those uterine smooth muscle tumors showing cellular atypia and 5-9 mitoses per 10 high-powered fields (M/10 HPF) and those with 10 or more M/10 HPF. Using these strict pathologic criteria to define leiomyosarcoma, menopausal status, margin type (pushing vs infiltrating), tumor size, grade, location, and the presence of vascular invasion and/or hemorrhage were not associated with prognosis. The 5-year survival rate was 25%. Survival rates were not improved by the use of adjuvant chemotherapy in those patients rendered free of gross disease by initial surgery.

Adult↗

PubMed1988

Cytogenetic findings in leiomyosarcoma of the small bowel.

A cytogenetic study of a leiomyosarcoma of the small bowel revealed numerical and structural chromosome changes. Several of these changes, in particular the loss of one chromosome #14, #15, #18, and #22, and del(1)(p12p13) have been reported previously in an unrelated leiomyosarcoma of the small bowel. We suggest that these or part of these findings could be characteristic of leiomyosarcoma of the small bowel.

Chromosome Aberrations↗

PubMed1989

Three possible cytogenetic subgroups of leiomyosarcoma.

Cytogenetic analysis was performed on short-term cultured tumor cells from ten patients diagnosed as having leiomyosarcoma. Of these, five tumors from five unrelated patients had clonal chromosome abnormalities. The combined data from this report and previous studies on leiomyosarcomas indicate that at least three subtypes may be identified chromosomally within leiomyosarcomas. One subtype is characterized by a hypodiploid chromosome number ranging from 41 to 43 and a common chromosome pattern of monosomies of chromosomes specific to this subgroup, including partial monosomy of the short arm of chromosome 1 (1p13----pter), monosomy 18, and consistent monosomy 22. In contrast to the previous subtype, for the pathogenesis of which the "tumor suppressor gene" hypothesis may be suggested, another subtype was characterized by a pseudodiploid chromosome number associated with simple reciprocal translocations; so far, these translocations are unique to individual tumors, the pathogenesis of which may involve a translocation-mediated gene deregulation pathway. A third subtype contained tumors with heterogeneous karyotypic findings.

Adult↗

PubMed1987

Leiomyosarcoma of the maxilla.

A case of leiomyosarcoma in the maxilla is presented together with a review of the literature. The most commonly encountered symptom at the early stage of oral leiomyosarcoma is a slowly enlarging, nonulcerated and painless mass. This tumor shows high incidence of local recurrence or metastasis, and poor prognosis. The histological confirmation of leiomyosarcoma seems difficult in the routine examination using biopsy specimen. Special staining methods and immunohistochemical analysis are useful for diagnosis of this tumor. The tumor slightly responded to chemotherapy consisting of adriamycin and cyclophosphamide.

Adult↗

PubMed1996

Leiomyosarcoma of the small intestine presenting as a pelvic mass.

We present a challenging case of differential diagnosis of leiomyosarcoma of the small intestine in a patient presented with a pelvic mass. This 43-year-old Japanese woman complained of hypermenorrhea and was diagnosed as myoma uteri. She underwent partial resection of the ileum with a primary end-to-end anastomosis, omentectomy, and appendectomy, as well as a simple hysterectomy and bilateral salpingo-oophorectomy. CT and MRI indicated an intestinal tumor at the gaseous site. The histological diagnosis was leiomyosarcoma of the small intestine and leiomyoma of the uterus. Although such leiomyosarcomas are rare, they can appear as pelvic masses and must be differentiated from gynecologic disease. Preoperative CT and MRI of the abdomen were useful in obtaining the diagnosis.

Adult↗

PubMed1994

Leiomyosarcoma of the base of the tongue treated with radiotherapy: a case report.

Soft tissue sarcomas of the oral cavity are uncommon malignancies; those of smooth-muscle origin are extremely rare. The world literature reveals about 80 leiomyomas and 26 cases of leiomyosarcoma. Only 4 of the cases with leiomyosarcoma occurred in the tongue. This report of leiomyosarcoma of the base and dorsum of the tongue adds 1 case which was primarily treated with irradiation and achieved an excellent response. A 1.5-year follow-up after radiation-therapy is included and a review of the literature is presented.

Aged↗

PubMed2005

Leiomyosarcoma of the head and neck: report of two cases and review of the literature.

Leiomyosarcoma is a malignant smooth-muscle tumor that is exceedingly rare in the head and neck region. Two cases of this uncommon tumor localized in the larynx and the parapharyngeal region are presented in this report, which might be the second leiomyosarcoma in the parapharyngeal region reported so far to our knowledge. We also discuss the diagnosis and treatment of leiomyosarcoma in this aspect.

Head and Neck Neoplasms↗

PubMed2005

No evidence for epigenetic inactivation of fumarate hydratase in leiomyomas and leiomyosarcomas.

Germline mutations in Fumarate Hydratase (FH) cause the development of leiomyomas and leiomyosarcomas in the syndromes Multiple Cutaneous and Uterine Leiomyomata (MCUL1) and Hereditary Leiomyomatosis and Renal Cell Cancer (HLRCC). There is little evidence, however, that FH mutation plays a role in the development of sporadic leiomyomas or leiomyosarcomas. Such observations do not, however, exclude a role for FH in tumour development outside the context of MCUL1/HLRCC, as it is possible that FH expression could be silenced by epigenetic mechanisms. To explore this possibility we have developed a highly specific antibody to FH and analysed a series of forty-five fresh-frozen uterine leiomyomas and nine leiomyosarcomas for FH expression.

Adult↗

PubMed2006

Primary leiomyosarcoma of the great saphenous vein: case report.

OBJECTIVE: To describe a case of primary leiomyosarcoma of the great saphenous vein. PATIENT RECORD: A 59-year-old Chinese lady presented with two painful lumps in the right thigh in the line of the great saphenous vein. At surgery, in September 2004, two tumors in the right great saphenous vein above the knee were excised with the intervening normal vein. Histopathological studies confirmed both masses as leiomyosarcoma. Radiotherapy was given postoperatively. The patient has been followed-up for 300 days after surgery with no evidence of local or distant metastasis. CONCLUSION: Leiomyosarcoma of the great saphenous vein can exist at more than one site. Tumor resection and radiotherapy was associated with good patient prognosis.

Female↗