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PubMed2005

Metastasis of uterine leiomyosarcoma to the pancreas.

BACKGROUND: Metastasis of uterine leiomyosarcoma to the pancreas is extremely rare. CASE: A 46-year-old woman presented with hypermenorrhea and dysmenorrhea and underwent surgery. The histologic and immunohistochemical diagnosis was uterine leiomyosarcoma stage I with no metastasis to the ovaries or the pelvic lymph nodes. The mitotic count was very high. Thereafter, recurrences in the lung and subsequently in the pancreas were detected. The lesions in the lung and pancreas were resected and diagnosed as metastases of uterine leiomyosarcoma based on histology and immunohistochemistry. CONCLUSION: We report an extraordinarily rare case of uterine leiomyosarcoma with metastasis to the pancreas following initial metastasis to the lung, both of which were diagnosed using histology and immunohistochemistry of the specimens obtained at surgery.

Actins↗

PubMed1996

Intradermal and subcutaneous leiomyosarcoma: a clinicopathological and immunohistochemical study of 41 cases.

Superficial leiomyosarcomas are rare tumours. The lesions confined to the dermis, contrary to those involving the subcutis, have been reported to carry a favourable prognosis. A retrospective study of 41 consecutive cases of surgically treated intradermal and subcutaneous leiomyosarcomas was undertaken in order to determine the prognostic factors that may influence the survival of these patients. Seven tumours were predominantly intradermal and 34 involved the subcutaneous tissue. Fifty-four percent of the tumours were located in the lower extremities. All cases stained positively for smooth muscle antigen and 66% for desmin. The tumours were classified with regard to tumour grade I (low grade, 3%), II (intermediate, 12%), IIIA (high grade, 46%) and IIIB (high grade, 39%). In all patients, follow-up information was available. Mean follow-up time was 5 years. The patients with intradermal tumours were all alive without signs of recurrence, whereas 14 of those with leiomyosarcomas involving the subcutis have died with pulmonary metastases. Our study confirms that "pure" intradermal leiomyosarcomas independent of tumour grade behave in a benign fashion, probably due to small tumour size. Tumour size > or = 5 cm, deep localization with fascia involvement, and high malignancy grade (IIIB) were found to deteriorate survival based on a univariate analysis. However, in a multivariate analysis only tumour size was found to be an independent prognostic factor.

Adult↗

PubMed2006

Cutaneous leiomyosarcoma with myxoid alteration arising in a setting of multiple cutaneous smooth muscle neoplasms.

BACKGROUND: Cutaneous leiomyomas and leiomyosarcomas are rare tumors that originate from the arrector muscle of hair follicles or the smooth muscle of blood vessels. CASE REPORT: A 74-year-old male presented with a single, erythematous nodule on the left upper arm. This lesion developed within the excision scar of a piloleiomyoma that had been excised 3 years ago. Additionally, physical examination revealed a tender nodule on the right ear. RESULTS: Histological examination showed a cutaneous leiomyosarcoma with myxoid alteration on the upper arm and an angioleiomyoma on the right ear. CONCLUSION: Myxoid leiomyosarcomas have exclusively been reported in the uterus and gastrointestinal tract, but not in the skin. Here, we describe a case of cutaneous leiomyosarcoma with myxoid alteration that developed in the excision scar of a piloleiomyoma and was accompanied by a cutaneous angioleiomyoma in a different location.

Aged↗

PubMed1992

Leiomyosarcoma of the uterus: ultrasonography and serum lactate dehydrogenase level.

Between January 1, 1979, and September 30, 1990, a total of 1,886 patients in the National Defense Medical College Hospital, a self-referred population, had a hysterectomy because of signs and symptoms presumably resulting from uterine myomas. After hysterectomy with presumed benign disease, a histologic diagnosis of leiomyosarcoma was made in 7 patients (0.37%). Preoperative diagnosis of leiomyosarcoma was not made in any of the 7 patients. However, serum lactate dehydrogenase levels were abnormally elevated in 3 of them, and degenerative changes were found within the tumor by ultrasonography in 5 of them. Furthermore, increased lactate dehydrogenase levels and degenerative changes within the tumor were found in 3 of the patients whose tumors had 10 or more mitoses per 10 high-power fields. The prognosis for the leiomyosarcomas with increased mitotic rates is very poor. Therefore, a degenerative change within the uterine mass and an increased lactate dehydrogenase level, when present, should suggest the diagnosis of leiomyosarcoma.

Adult↗

PubMedDate not supplied

Ultrasound-guided fine needle aspiration cytodiagnosis of leiomyosarcoma metastatic to the breast. A case report.

BACKGROUND: Leiomyosarcomas are characterized by slow growth and late metastasis and most often involve the lung, liver and subcutaneous tissue. Metastasis to the breast is rare, with only four cases published in the English-language literature to date. Because of its rarity and the prolonged time interval between the diagnosis of the primary tumor and involvement of distant sites, accurate diagnosis of these tumors can be challenging. Evaluation of the cytomorphologic features of the tumor, ancillary immunocytochemical staining and detailed medical history of the patient are essential to making a correct diagnosis. CASE: A case of leiomyosarcoma metastatic to the breast occurred in a 60-year-old woman whose primary uterine leiomyosarcoma had been resected 10 years before the diagnosis of the metastasis. Fine needle aspiration performed with ultrasound guidance yielded a moderately cellular specimen composed of minimally pleomorphic spindle cells with smooth, blunt-ended nuclei arranged in an interlacing pattern. Immunocytochemical staining for muscle-specific actin confirmed the smooth muscle origin of the neoplasm. CONCLUSION: The results of cytomorphologic analysis and immunocytochemical staining performed after fine needle aspiration and the patient's history of primary uterine leiomyosarcoma allowed us to correctly diagnose metastasis to the breast and avoided inappropriate management of the metastasis as a primary tumor.

Biopsy, Needle↗

PubMed1990

Leiomyosarcoma of the os calcis.

A primary leiomyosarcoma of the os calcis, occurring in a 68-year-old woman and treated by below-the-knee amputation, is reported. The diagnosis was confirmed by electron microscopy, and supported by immunochemistry. These techniques can be performed in a primary spindle cell osseous neoplasm to distinguish leiomyosarcoma from fibrosarcoma and malignant fibrous histiocytoma. A total of 28 cases of primary leiomyosarcoma of extragnathic bones have been reported in the literature with one occurring in the foot. This is the first reported case involving the os calcis and the first reported leiomyosarcoma of the foot in the English literature.

Aged↗

PubMed1978

Angiography of abdominal leiomyosarcoma.

The spectrum of angiographic findings of abdominal leiomyosarcoma in 33 cases is presented, and the value of angiography in the diagnosis and management of such patients is discussed. Angiographic features of abdominal leiomyosarcomas are different depending upon the site of origin. Small bowel lesions are hypervascular, and those in the stomach and colon moderately vascular. Well circumscribed masses with enlarged feeding arteries and draining veins form the predominant presentation of small bowel leiomyosarcoma. In the retroperitoneum these neoplasms are usually hypovascular to moderately vascular, and displacement of major vessels, particularly the inferior vena cava, is the most common angiographic finding. Bladder leiomyosarcoma can be either moderately vascular or hypervascular. Vascularity of metastases is usually similar to that of the primary lesion.

Abdominal Neoplasms↗

PubMed1990

Leiomyosarcoma of the rectum with sarcoid-like reaction--a case report.

A 57 year-old man was admitted to the hospital because of bloody stools. A barium enema and coloscopy disclosed a tumor of the rectum. The resected tumor was diagnosed as a leiomyosarcoma, histologically. Many epithelioid granulomas with Langhans type giant cells were found in the tumor, and there was no evidence suggestive of generalized sarcoidosis, tuberculosis or mycosis. Thus, the epithelioid granulomas seen in the leiomyosarcoma were interpreted as a sarcoid-like reaction. As far as could be determined, there has been no reported case of a sarcoid-like reaction associated with a leiomyosarcoma. The occurrence of the sarcoid-like reaction in the present case could be due to the reaction to the metabolites or a degenerative substance of the leiomyosarcoma, or to host resistance to the tumor itself.

Humans↗

PubMed1986

Leiomyosarcomas in childhood: a clinical and pathologic study of 10 cases.

Ten children (5 males, 5 females) with leiomyosarcomas were treated at the Children's Hospital, Boston, during the 20-year period from 1963 through 1983. The patients ranged in age from 2 1/2 to 15 years, the average age at diagnosis being 10 1/2 years. The tumors originated in the following sites: stomach (4 cases), jejunum, cecum, endocervix, retroperitoneum, urinary bladder, and base of tongue (1 case each). In each case the primary tumor was surgically resectable, with the average diameter being 6.7 cm (range 2-11 cm). The tumor proved fatal for 3 of 6 children with regional or distant metastases. Three others were alive with metastatic leiomyosarcoma 6-18 years following diagnosis. A fourth death was attributed to a massive chondrosarcoma of the lumbosacral area, which developed 7 years after hysterectomy, chemotherapy, and radiation therapy for an endocervical leiomyosarcoma. While leiomyosarcomas are extremely rare in the pediatric age group (less than 2% of all soft tissue sarcomas), diagnostic features and biologic behavior appear similar to those in adults. Tumor size and mitotic counts seem to be the most important features in assessing malignant potential, but absolute minimum criteria for malignancy are not well defined.

Adolescent↗

PubMedDate not supplied

Pleomorphic leiomyosarcoma of the soft parts: a reassessment by histology and immunohistochemistry of pleomorphic soft tissue sarcomas.

Histologically it is often difficult to differentiate malignant fibrous histiocytoma (MFH) from leiomyosarcoma. Sixty-three cases formerly diagnosed as pleomorphic soft tissue sarcomas were examined by immunohistochemistry. We identified three different types according to its positivity for the myogenic markers: A) positive cells with a fascicular pattern, 13 cases (26%); B) a small number of scattered positive cells, 29 cases (57%); C) negative for markers, 9 cases (17%). We reassessed 51 MFHs as follows: A type as pleomorphic leiomyosarcoma, B+C types as MFH. Pleomorphic leiomyosarcomas were also positive for collagen type IV around the compactly arranged individual tumor cells. An imunohistochemical analysis and detection of fascicular structures are thus considered to be necessary in order to distinguish between leiomyosarcoma and MFH.

Adult↗

PubMed2004

Malignant gastrointestinal leiomyosarcoma and gastrointestinal stromal tumor with prominent osteoclast-like giant cells.

CONTEXT: One case of leiomyosarcoma and one case of gastrointestinal stromal tumor with prominent osteoclast-like giant cells have so far been reported in the digestive tract. OBJECTIVE: To ascertain the clinicopathologic features and biologic behavior of these tumors, we report 3 additional cases of leiomyosarcoma of the gastrointestinal tract and one malignant gastrointestinal stromal tumor. DESIGN: Histologic and immunohistochemical examinations were performed. Clinical and follow-up data were recorded, and the literature was reviewed. RESULTS: The age of the patients ranged from 50 to 68 years (mean, 62 years). One of the lesions arose in the stomach, one in the ileum, and 2 in the colon. Three tumors showed a strong positivity for muscle actin and desmin and were diagnosed as leiomyosarcomas, 2 of them showing spindle cells and 1 of them showing epithelioid cells. The fourth tumor reacted strongly positive for c-Kit (CD117) and vimentin, and it was diagnosed as an epithelioid malignant gastrointestinal stromal tumor. All tumors were characterized by numerous osteoclast-like giant cells that were unevenly distributed and that, using immunohistochemistry, reacted strongly with CD68. CONCLUSIONS: Malignant stromal tumors with osteoclast-like giant cells of the gastrointestinal tract are rare entities, are more commonly of a myogenic origin such as leiomyosarcoma, and seem to have an aggressive behavior.

Actins↗

PubMed1999

Gastric leiomyosarcoma presenting as a sentinel hemorrhage.

A 43-year-old Asian woman who was initially seen because of hematemesis later had a gastric leiomyosarcoma diagnosed. Epigastric palpation, computed tomography, and magnetic resonance imaging assisted in determining the size, borders, and location of the tumor while a second esophagogastroduodenoscopy revealed friable gastric mucosa with erosions. Biopsy specimens taken for frozen section during surgical abdominal exploration revealed a malignant gastric stromal tumor. An en bloc excision of the mass then followed, with the final pathologic diagnosis a gastric leiomyosarcoma. Metastases were later found in the liver, peritoneum, and mesentery. Differentiation of gastric leiomyosarcoma from other stromal tumors is difficult and requires standardized nuclear and cellular evaluation of atypia, necrosis, mitosis, and tumor doubling time. The most common symptoms at initial presentation are abdominal pain and gastrointestinal bleeding. Abdominal computed tomography remains more specific in suggesting gastric stromal tumors than esophagogastroduodenoscopy and upper gastrointestinal barium series. Lymph node involvement in gastric leiomyosarcoma is rare and affords the first-line therapy of laparoscopic wedge gastrectomy with a good prognosis in tumors less than 6 cm in diameter. The prognostic factors include metastasis, size of the tumor, histologic grade, DNA ploidy of the tumor, and ulceration of overlying gastric mucosa.

Adult↗

PubMed1999

[Rectal malignant stromal tumor (leiomyosarcoma): echoendoscopic, computed tomographic aspects and anatomopathologic correlations].

We report the CT and endorectal us features of a malignant stromal tumor of the rectum (leiomyosarcoma). On CT scan, rectal leiomyosarcoma appeared as a large soft tissue mass, arising from the anterior rectal wall with exorectal extension. On endorectal US, the rectal leiomyosarcoma presented as a well-defined hypoechogenic and heterogeneous mass arising from the rectal muscularis propria. The uncommon location of leiomyosarcoma to the rectum may simulate other rectal tumors such as adenocarcinoma on CT. As suggested by this report, endorectal us demonstrated the muscular origin of the tumor.

Adenocarcinoma↗

PubMed1999

[Mediastinal leiomyosarcoma].

Leiomyosarcomas are extremely rare tumors which develop from smooth muscle, usually in the esophagus and large vessels (inferior vena cava, pulmonary artery, and superior vena cava). In very rare cases, leiomyosarcomas develop from small vessels in the soft tissue of the mediastinum. Clinical expression of mediastinal leiomyosarcomas (dysphagia, dysphonia) is related to their large size and the subsequent compression of mediastinal structures. At pathology examination, the gross aspect is one of a single cell tumor. Microscopically, the tumor may be highly undifferentiated making it necessary to use specific immune markers (actin and desmin) or ultra-structural analysis to establish the diagnosis. Treatment of localized tumors is based on surgical excision, either alone or in combination with radiotherapy of the mediastinum. Chemotherapy, generally dexorubicin, is indicated in case of metastatic dissemination, but outcome remains uncertain. As for all soft tissue sarcomas, the prognosis of mediastinal leiomyosarcoma depends on the size of the tumor, its histological structure and its resectability.

Antineoplastic Agents↗

PubMed1999

[Primary cutaneous leiomyosarcoma: 32 cases].

BACKGROUND: Superficial leiomyosarcomas are rare tumors, which may be confined to the dermis or extend to subcutaneous tissues. PATIENTS AND METHODS: We report the results of a retrospective study of 32 patients treated for leiomyosarcomas through a twenty-two year period (from 1975 to 1997). RESULTS: Mean age was 45 years, with 50 p. 100 of patients less than 35 years of age. Forty seven percent of the tumors were located on the lower limbs and mean diameter was 2.8 cm. Three clinical types have been isolated: nodule beneath normal epidermis (50 p. 100), purple nodule ulcerated or not (28 p. 100), swelling tumor (22 p. 100). Sixteen percent were intradermal, whereas sixty nine percent involved subcutaneous tissues. With regard to tumor grade, 37 p. 100 of tumors were grade I, 44 p. 100 of tumors were grade II, and 19 p. 100 were grade III. Immunohistochemical staining showed positive reactions for all tumors with anti-vimentin and anti-alpha smooth muscle actin. Main treatment was complete surgical excision. Follow-up informations were available for all patients and 75 p. 100 of them had a follow up period longer than a year. Five patients with leiomyosarcomas involving the subcutis developed local recurrences, and two of them died of the disease. DISCUSSION: Leiomyosarcomas can occur at any age without predominant sex-ratio. Main prognostic factors are tumor size, distal location, depth of tumor invasion and pathological grade. Immunohistological staining with anti-alpha smooth muscle actin is more sensitive and specific than with anti-desmin or anti-HHF 35. Main treatment is surgical excision with wide margins.

Actins↗

PubMed1999

[Leiomyosarcoma of Meckel's diverticulum--a rare cause of acute hemorrhage in the lower part of the digestive system].

Meckel's diverticulum is a rare cause of haemorrhage into digestive tract in adults. A tumour arising in the diverticulum is also a rare cause of bleeding. Leiomyosarcomas represent 10-20% of malignant tumours in the small bowel and 1% of those located in the large bowel. These tumours have non-specific symptoms depending on their size, location and histology. Intestinal leiomyosarcomas may be the cause of surgical emergencies. We describe the case of an adult male with acute painless intestinal bleeding the cause of which was a leiomyosarcoma arising in Meckel's diverticulum. It was diagnosed during an acute operation. The main cause of the surgical emergency was an extremely rare complication of gastrointestinal leiomyosarcoma--intestinal bleeding.

Gastrointestinal Hemorrhage↗

PubMed1999

Hormone replacement therapy after uterine leiomyosarcoma treatment. Case reports.

Uterine sarcomas are extremely rare uterine malignancies; with a review of the literature we could not find any data dealing with exogenous oestrogens or combined hormone replacement therapy (HRT) after leiomyosarcoma treatment. We report two cases of patients with leiomyosarcoma of the uterine corpus. Both patients were without pelvic irradiation or exogenous oestrogen treatment before the diagnosis. Leiomyoma of the uterus was found during surgery in both cases. Both patients were receiving HRT with non-conjugated oestrogens, after an intensive non-hormonal treatment had failed. No recurrence was established after surgical treatment in the patient with 12 mitoses per 10 high power fields (HPF). The patient is still on HRT (61 months). The other patient with a leiomyosarcoma with very high mitotic activity (40 mitoses per 10 HPF) received cytostatic and irradiation therapy after surgery because of locally widespread disease. Ten months after the diagnosis and 3 months after beginning HRT, recurrence was observed. The patient thereupon stopped HRT. After two additional operations, the patient is alive and without evidence of disease. We presume that the present case reports observations might suggest that HRT did not appear to have a pronounced adverse effect on the leiomyosarcoma outcome in our patients. Nevertheless, until more collected data determine that HRT is safe, caution is needed.

Adult↗

PubMed1999

[Primary bone leiomyosarcoma. Anatomo-clinical, immunohistochemical, and ultrastructural study].

A variety of tissues in bone can be the origin of neoplastic primitive lesions. Consequently, it can be the site of various sarcomas of tissue type, incursing leiomyosarcoma. The leiomyosarcoma of the bone is considered as primitive after exclusion either a bony extension of a soft tissue tumor of vicinity or the presence of a leiomyosarcoma elsewhere. We report a case of a primary leiomyosarcoma arising on the left femur of a 40-year-old woman. The diagnosis was confirmed by immuno-histochemistry and electron microscopic study. A bloc resection followed by a prothesis was performed. The patient underwent a fractioned postoperative radiotherapy with a total dose of 75 Gy. The postoperative course one year later revealed local recurrence and pulmonary metastases. Despite the resection of pulmonary metastases and the amputation of left lower limb, the patient had developed local recurrence on the stump of amputated limb and was on the outside of all therapeutic resource. In this report, we present the clinicopathologic, immuno-histochemical and ultrastructural profiles of these rare primary bone tumors.

Adult↗