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PubMed1992

Computed tomography appearance of bone metastases of leiomyosarcoma.

Bone lesions in leiomyosarcoma are uncommon. When encountered, they are usually a late manifestation of disease seen in the setting of widespread systemic metastases. We present five cases of metastatic leiomyosarcoma, including one in which a bone lesion predated detection of a primary gastric leiomyosarcoma and one in which bone metastases signaled disease recurrence. Computed tomography in 14 of 16 osseous leiomyosarcomas demonstrated a lytic, non-expansile pattern.

Adult↗

PubMed1987

Primary leiomyosarcoma of bone. A clinical, radiographic, pathologic-anatomic, and prognostic study of 16 cases.

Sixteen cases of primary leiomyosarcoma of bone are described. The patients, 11 males and 5 females, ranged in age from 9 to 74 years. The annual incidence of this tumor in Sweden was calculated to be 0.09 cases per million. This figure was obtained by reviewing a Swedish series of spindle cell sarcomas of bone of which one quarter (11/44) were diagnosed by us as primary leiomyosarcoma. The diagnosis was based on light- and electron-microscopic examinations using the same criteria as for leiomyosarcoma of soft tissues. Thirteen tumors were located in a long bone of an extremity (nine close to the knee joint) and three in the central skeleton. Radiographically, all the tumors presented as a purely osteolytic lesion, and three patients had sustained a pathologic fracture. In four of six cases angiography suggested malignancy by revealing hypervascularity, irregular tortuous vessels, and diffuse contrast opacification. Contrast-enhanced computed tomography, performed in two cases, showed hypervascular areas within the tumors. Scintigraphy showed a marked increase in radionuclide uptake in all five cases studied. The clinical behavior indicates that primary leiomyosarcoma of bone is highly malignant. Eight patients had died of the tumor and, of the eight patients who were alive at follow-up, two had metastases, and one had been operated on three times for a cutaneous metastasis, which had recurred locally twice. The remaining five patients had been continuously free of disease for 6.5 to 12.3 years.

Adolescent↗

PubMed1993

Intussusception and leiomyosarcoma of the gastrointestinal tract in a pediatric patient. Case report and review of the literature.

Intestinal leiomyosarcoma is a rare tumor in infants and children; only 46 cases have been reported in the English literature. Presenting signs and symptoms include abdominal pain and gastrointestinal obstruction and bleeding. We describe a neonate with the unique presentation of ileocecal intussusception accompanying an ileal leiomyosarcoma. In contrast to adult patients, where intussusception is associated with smooth muscle tumors in 30% of cases, leiomyosarcoma and subsequent intussusception is rare in infancy and childhood. The overall prognosis for long-term survival is similar for both pediatric and adult patients with leiomyosarcoma of the intestinal tract, with a five-year survival in reported cases of 53% and 40%, respectively.

Humans↗

PubMedDate not supplied

Primary leiomyosarcoma of bone: clinicopathologic, immunohistochemical, and molecular biologic aspects.

BACKGROUND: Primary leiomyosarcoma of bone is a very rare malignant tumor with uncertain pathogenicity. METHODS: The authors studied five cases of surgically treated primary leiomyosarcoma of bone. Clinical histories and radiographic findings were recorded. Regular clinical and radiographic controls were obtained postoperatively. In all cases, immunohistochemical studies were used to confirm the diagnosis. Molecular biologic examinations, using the polymerase chain reaction technique with microsatellite DNA markers from regions of tumor-relevant genes, were performed to determine the stability of the genome or to detect some typical genomic changes. RESULTS: The study included three women and two men, with an average age of 42 years. The tumor was located in the pelvis in two patients, in the femur in two patients, and in the proximal tibia in one patient. All tumors were classified as high-grade tumors (four stage IIB, one stage IIA). Radiographically, all tumors appear as purely osteolytic lesions, with a geographic or moth-eaten appearance and without any sclerotic margin. Three patients underwent limb salvage surgery followed by endoprosthetic replacement. The other two patients required amputation. The mean follow-up was 19 months (range, 8-29 months). Three patients died of disease, with a mean postoperative survival period of 18 months (range, 6-27 months). Four patients developed diffuse pulmonary metastases after an average of 10.5 months. One of those patients responded well to chemotherapy. In all cases, immunohistochemistry showed strong reactivity of the tumor cells for (alpha-SMA and vimentin. Molecular biologic investigations revealed a high rate of genomic instabilities in all of the stage IIB tumors. CONCLUSION: Clinical follow-up suggests that primary osseous leiomyosarcoma has an aggressive biologic behavior. The immunohistochemical studies are useful tools and suggest that osseous leiomyosarcoma arise from the vascular smooth muscle cells within the bone. The molecular biologic findings of a high rate of genomic instability confirm the hypothesis that this rare entity is of an aggressive nature.

Adult↗

PubMed1981

Comparative study on the nuclear DNA content of leiomyoma, leiomyoblastoma and leiomyosarcoma of the stomach and small intestine.

The nuclear DNA content of tumor cells in 5 leiomyomas, 4 leiomyoblastomas and 2 leiomyosarcomas of the stomach and 1 leiomyosarcoma of the small intestine was examined. From the viewpoint of nuclear DNA content, leiomyoblastoma was between leiomyoma and leiomyosarcoma. A definite difference in nuclear DNA content exists among leiomyoma, leiomyoblastoma and leiomyosarcoma, and percentage of nuclei with a DNA content of 3 times or 4 times over the basic chromosome content represented good indices for distinguishing between benign and malignant conditions. A diagnosis of leiomyocarcinoma may be more accurate when over 80% of the nuclei have DNA content values over 3 times of basic nuclear DNA content and when over 50% exhibit more than 4 times of basic content.

Adult↗

PubMed1998

Giant leiomyosarcoma of the remnant stomach: report of a case.

We describe herein the case of a 73-year-old woman who developed a giant leiomyosarcoma in the remnant stomach 4 years after undergoing a distal gastrectomy for gastric carcinoma. Abdominal ultrasonography and computed tomography revealed a huge tumor, 22 cm in maximum diameter, in the left hypochondrial region. Selective abdominal angiography showed a hypervascular tumor fed by the branches of the splenic artery and left inferior phrenic artery. The tumor arose from the posterior wall of the remnant stomach, and demonstrated marked extragastric growth and direct invasion of the pancreas, transverse colon, and diaphragm on the left side. Total resection of the remnant stomach with en bloc resection of these adjacent organs was subsequently carried out. We reviewed the Japanese literature on this extremely rare tumor and evaluated its clinical profile. In comparison with leiomyosarcoma of the unresected stomach, that of the remnant stomach more frequently showed endogastric-type growth and was accompanied by ulceration of the gastric mucosa. The prognosis of patients with leiomyosarcoma of the remnant stomach appears to be greatly affected by the presence of liver metastases and the tumor diameter, similar to that of patients with leiomyosarcoma of the unresected stomach.

Aged↗

PubMed2004

Subperiosteal leiomyosarcoma of the tibia.

Primary leiomyosarcoma of bone is a rare malignant tumor of smooth muscle. We report a case of low-grade subperiosteal primary bone leiomyosarcoma in the tibial diaphysis, which radiologically appeared to be osteoid osteoma. A 35-year-old man presented with a several-year history of a palpable hard nodule in the distal left leg, which had enlarged and become painful over the previous 2 years. Radiographs showed solid periosteal reaction with a well-defined lytic lesion in the posteromedial cortical border of the left tibial diaphysis. Computed tomography demonstrated a small, well-defined lytic lesion, not calcified, in a subperiosteal location, surrounded by solid periosteal bone formation. The lesion was excised en bloc and the histological diagnosis of a low-grade leiomyosarcoma was made. To the best of our knowledge, the surface location of primary bone leiomyosarcoma has not been previously described in the literature.

Adult↗

PubMedDate not supplied

Hepatic metastases from leiomyosarcoma: MR features with histopathologic correlation.

BACKGROUND: To determine the magnetic resonance (MR) imaging features of hepatic metastases from leiomyosarcoma and determine the pathologic basis for the MR appearance of these neoplasms. METHODS: MR examinations of eight patients with a total of 16 hepatic metastases from leiomyosarcoma were retrospectively reviewed. Hepatic metastases originated from a primary gastrointestinal (n = 6), uterine (n = 1), or retroperitoneal (n = 1) leiomyosarcoma. Correlation between MR features and pathologic findings was performed in 11 metastases from which histologic material was obtained. RESULTS: Among the 10 metastases seen on T1-weighted MR images, nine were homogeneous and one was heterogeneous. The latter contained areas of necrosis and hemorrhage on pathologic examination. Among the 16 metastases seen on T2-weighted MR images, 12 were well delineated and completely homogeneous, with a marked hyperintense signal ("hemangiomalike" pattern). These neoplasms were composed of smooth fibrous tissue without macroscopically visible areas of necrosis on pathologic examination. The remaining four metastases were heterogeneous and contained varying degrees of necrosis and hemorrhage or gelatinous tissue. CONCLUSION: A hemangiomalike pattern is the most common feature on T2-weighted MR images. We found that homogeneous hyperintensity of hepatic metastases from leiomyosarcoma on T2-weighted MR image does not reflect cystic changes.

Adult↗

PubMed1998

[Leiomyosarcoma of the spermatic cord with paraneoplastic beta-hCG production].

Leiomyosarcomas of the spermatic cord are very rare tumours, which, in contrast to the rhabdomyosarcoms of childhood, occur almost exclusively in adults. We report the case of a 51-year-old male patient with a metastasizing, pleomorphic leiomyosarcoma of the spermatic cord. The tumour showed paraneoplastic secretion of beta-chain human chorionic gonadotropin (beta-hCG), as was demonstrated by the elevated levels of beta-hCG in serum samples and by immunohistochemistry. Histologically, the tumour was a high-grade leiomyosarcoma which showed an aggressive course with pulmonary metastases appearing 4 months after primary surgery. A concomitant rise in the serum levels of beta-hCG was also noted at this time. A wide spectrum of tumours with a choriocarcinomatous component or paraneoplastic production of beta-hCG has been described, the vast majority being carcinomas. Only two leiomyosarcomas producing beta-hCG have so far been reported. The paraneoplastic production of beta-hCG should prevent confusion with germ-cell tumours, especially teratomas.

Adult↗

PubMed2002

Abdominal leiomyosarcomas: radiologic appearances at various locations.

Leiomyosarcomas are soft tissue tumors that account for approximately 15% of all soft tissue sarcomas. Leiomyosarcomas may be located at almost any part of the abdomen but especially are more common in the retroperitoneum, followed by gastrointestinal tract and genital system. They develop mainly in adult life and are very rare in children. In this article, imaging findings of leiomyosarcomas in various abdominal locations are presented. Radiologic studies are capable of providing useful information on the localization, size, changes in the internal structure of the tumor, its extension and invasion. Leiomyosarcoma should be considered in the differential diagnosis in case of detection of a large, circumscribed, and heterogenous abdominal mass. Histopathologically, diagnosis of malignancy depends particularly on mitotic counts, size, rate of necrosis, and infiltrating margins.

Abdominal Neoplasms↗

PubMed2002

Breast metastasis from uterine leiomyosarcoma: a case report.

Breast metastasis from extra-mammary malignancy is rare, constituting 2% of breast tumors. Uterine leiomyosarcoma is a rare soft tissue neoplasm comprising 1% of all uterine malignancies. This report presents the first case of uterine leiomyosarcoma metastasis of the breast. A 44-year-old woman, G4P3AA1, presented with a one-week history of painless bilateral breast lumps. Twelve years previously she had undergone a right salpingo-oophorectomy and hysterectomy for uterine leiomyosarcoma. An excisional biopsy of the breast nodule was performed, and the histopathological study revealed the nodule was composed of spindle cells, typical of breast metastasis with uterine leiomyosarcoma found in the immunohistochemical study. The patient was healthy and symptom free during the 12 month post-operative follow-up period.

Adult↗

PubMed1998

Anorectal leiomyosarcomas.

In adults, rectal leiomyosarcoma is uncommon, and anal leiomyosarcoma is even more rare. We report one patient with anal leiomyosarcoma and one patient with a biphasic clinical history, with three occurrences of perineal leiomyosarcomas after surgically resected rectal leiomyoma. We discuss the characteristics of these rare entities based upon a review of the literature.

Aged↗

PubMed2002

Leiomyosarcoma of the greater omentum with liver metastasis: report of a case.

We report herein a rare case of leiomyosarcoma arising from the greater omentum with concomitant liver metastases. A 63-year-old Japanese woman was admitted to our hospital with an elastic-hard mass in the abdomen, but no other symptoms. The findings of several examinations led us to suspect an omental leiomyosarcoma. A laparotomy revealed an elastic hard and foliaceous mass (12.5 x 9.0 x 8.0 cm) arising from the omentum, and six masses in the liver. The omental tumor was extirpated and chemotherapy was administered under the guidance of angiography. The final diagnosis was leiomyosarcoma of the omentum. About 9 months after the operation, multiple liver metastases were found and repeated chemotherapy was given, but without effect. A partial hepatectomy was done, accompanied by microwave coagulation therapy (MCT). She is currently well and asymptomatic, with no signs of recurrence, 1 year after the partial hepatectomy. Thus, surgical resection and MCT were effective against liver metastases of a leiomyosarcoma in this patient.

Female↗

PubMed2002

Primary leiomyosarcoma of the breast: report of a case.

We describe herein a case of primary leiomyosarcoma of the breast in a 59-year-old woman. Preoperative clinical examination and cytology findings indicated a benign breast tumor, but intraoperative histopathological examination of the tumor revealed malignant spindle cells, and a modified radical mastectomy was performed. Immunohistochemical analysis subsequently confirmed a diagnosis of leiomyosarcoma. Primary leiomyosarcoma of the breast is extremely rare and difficult to diagnose before surgery because of the need for mitotic figures and immunohistochemical staining to confirm the diagnosis. When pre- and intraoperative examinations indicate the possibility of a leiomyosarcoma, an extended excision with sufficient margins should be performed to remove all of the affected tissue. A review of the literature on this unusual tumor of the breast is presented.

Breast Neoplasms↗

PubMed1991

Myxoid leiomyosarcoma of the ovary: analysis of three cases.

The first three cases of myxoid leiomyosarcoma occurring in the ovary are reported. Two cases in stage III were found in postmenopausal patients and a further case was found in stage I in a 32-year-old. All masses were large and gelatinous with cystic change, necrosis, and hemorrhage, but both uteri and ligaments and contralateral adnexa appeared normal. Microscopically, the tumors showed a predominantly reticular meshwork of elongated cells surrounded by abundant basophilic material. While electron microscopy proved inconclusive due to nondifferentiation, the use of monoclonal antibodies against smooth muscle actin demonstrated a smooth muscle type of differentiation. The differential diagnosis of this rare ovarian condition includes other myxoid ovarian lesions, such as ovarian edema, myxoma, endodermal sinus tumors, and the sarcomatous component of malignant mixed müllerian tumor and carcinosarcoma, as well as lymphovascular tumors. Since mitotic count due to decreased cellular density is unusually low in myxoid leiomyosarcoma, capsular rupture and clinical stage seem to be more reliable prognostic markers. The highly aggressive behavior of myxoid leiomyosarcoma parallels that of typical ovarian leiomyosarcoma. Two of the three patients in this series died of tumor at 13 and 24 months after diagnosis; the other patient is free of disease at 3 years after diagnosis.

Actins↗

PubMed1990

Complex karyotypic changes, including rearrangements of 12q13 and 14q24, in two leiomyosarcomas.

Cytogenetic investigation of short-term cultures from two leiomyosarcomas revealed complex karyotypic changes in both cases. The first tumor, a subcutaneous leiomyosarcoma of the knee, had the karyotype 70-80,XY, +X, +Y, +1, +1, +2, +2, +3, +3, +4, +4, +7, +7, +8, +8, +9, +10, +15, +15, +16, +16, +18, +19, +20, +21, +21, +22, +22,t(?;5)(5;21)(?;q35p11;q11), t(?;5)(5;21)(?;q35p11;q11), +del(11)(q22),der(13)t(12;13)(q13;q22),der(14)t(9;14)(p11;p11), +14p+, +t(20;?)(q13;?), +t(20;?)(q13;?), +2 mar. A polyploidized clone with 120-150 chromosomes was also observed. DNA flow cytometry revealed only one abnormal peak, corresponding to a DNA index of 1.76. The other tumor, a uterine leiomyosarcoma, had the karyotype 61-67, X, -X, +1, +3, +5, +6, +7, +8, +9, +12, +13, +15, +t(1;1)(p32;q32), +der(1)t(1;8)(p13;q11), +del(2)(p11), +del(2)(q22), +del(2)(q22), +del(3)(p13), +i(5p),t(8;14)(q24;q24), +der(8)t(8;14) (q24;q24), +del(10)(p12),der(11)t(11;15)(p15;q11),t(16;?)(p13;?),t(16;?)(q24;?), der dic(17) (17pter----cen----17q25::hsr::17q25----cen----17pte r), +t(19;?)(p13;?), +der dic(20)(20pter----cen----20q12::hsr::20q12----cen----+ ++20pter), +mar. The DNA index was 1.59. The finding in these leiomyosarcomas of rearrangements of the same regions of chromosomes 12 and 14 that are involved in the tumor-specific t(12;14)(q14-15;q23-24) of uterine leiomyoma indicates that the same genes in 12q and 14q might be important in the pathogenesis of benign and malignant smooth muscle tumors.

Aged↗

PubMed1996

Gastric leiomyosarcoma in a child.

Gastric leiomyosarcoma is a rare tumor in pediatric patients. The histological differentiation between leiomyoma and leiomyosarcoma is difficult. Computed tomography may allow the presumptive diagnosis of leiomyosarcoma to be made preoperatively and the surgical treatment can be planned in advance. We report a case of gastric leiomyosarcoma in a 2-year old boy. Ultrasonography and computed tomography demonstrated an inhomogeneous mass with areas of tumor necrosis and calcifications in the left upper quadrant.

Calcinosis↗

PubMed2004

Subglottic leiomyosarcoma of the larynx; a case report.

Leiomyosarcoma of the larynx is very rare. We herein report an especially rare case of leiomyosarcoma originating in the subglottic mucosa. The majority of cases reported in the literature indicate that laryngeal leiomyosarcomas originate in the supraglottic or glottic area. Our patient underwent simple total laryngectomy and is free of disease 5 years after the operation. The literature is reviewed regarding the choice of treatment and the distribution of the reported sites of origin of laryngeal leiomyosarcoma.

Disease-Free Survival↗