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Leiomyosarcoma of the breast: report of a case with fine needle aspiration cytologic, histologic and immunohistochemical features.

BACKGROUND: Leiomyosarcoma of the breast is a rare neoplasm. We present a case of primary leiomyosarcoma of the breast in a middle-aged female in whom fine needle aspiration cytologic features suggested sarcoma. CASE: A 55-year-old female presented with a rapidly growing breast lump of 1 month's duration. On examination, an ulcerating, 12 x 10 cm tumor was seen involving the lower medial and lateral quadrants of the right breast. Fine needle aspiration cytology showed variably sized, dissociated and loosely clustered polygonal, plump and spindle cells with pale blue cytoplasm and vesicular nuclei that were round, oval or irregular. Occasional giant forms and nucleolated and mitotic cells were present. A single cluster of benign ductal cells was seen. The tumor cells did not express immunocytologic reactivity to estrogen receptor protein. A cytologic diagnosis of sarcoma was given with differential diagnoses of metaplastic carcinoma and malignant phyllodes tumor. Histologic study established the diagnosis of leiomyosarcoma. Leiomyosarcoma of the breast shows fine needle aspiration cytologic features of sarcoma, but specific tumor typing may not be possible, especially when the cytologic material is inadequate for ancillary staining required to distinguish leiomyosarcoma from metaplastic carcinoma and malignant phyllodes tumor.

Biopsy, Fine-Needle↗

Fine needle aspiration biopsy of hepatic leiomyosarcoma. An unusual epithelioid variant posing a potential diagnostic pitfall in a hepatocellular carcinoma-prevalent population.

OBJECTIVE: To study the cytomorphologic features of hepatic smooth muscle tumors on fine needle aspiration biopsy (FNAB) material in a hepatocellular carcinoma (HCC)-prevalent population, in which a potential diagnostic pitfall is an unusual variant of metastatic epithelioid leiomyosarcoma seen on aspiration cytology. STUDY DESIGN: FNABs of five cases of metastatic hepatic leiomyosarcomas were studied cytohistologically and immunocytochemically. In addition, ultrastructural studies were performed in one unusual case. Resected hepatic specimens and all other relevant histopathologic specimens were reviewed. RESULTS: Classically, aspirates of leiomyosarcoma produced syncytial masses of spindle cells characterized by overlapping, cigar-shaped nuclei and truncated ends. An unusual variant of metastatic epitheliod leiomyosarcoma was studied, with cytohistologic, immunohistochemical and ultrastructural correlation. CONCLUSION: Hepatic spindle cell lesions consist mainly of metastatic leiomyosarcomas. The diagnosis of malignancy and metastatic status of hepatic smooth muscle tumors is facilitated by the presence of an extrahepatic primary tumor. In a hepatitis B virus-endemic population, HCC, sarcomatoid type, has to be considered in the differential diagnosis of hepatic spindle cell lesions and the classic type, in epithelioid variants of smooth muscle tumors.

Adult↗

Right atrial extension of primary venous leiomyosarcoma: pulmonary embolism and Budd-Chiari syndrome at presentation-a case report.

Venous leiomyosarcomas are rare and arise predominantly in the inferior vena cava (IVC). The clinical findings are nonspecific and may precede the diagnosis by several years. IVC leiomyosarcoma is predominantly seen in women. Intracavitary extension of vascular tumors tends to result from embolization or propagation along great veins, and this is a serious risk factor for pulmonary embolism and sudden death when it reaches the right heart. Modern imaging modalities using computed tomography, magnetic resonance imaging, individually or in combination with cavography, ultrasound, and echocardiography, allow an early and accurate preoperative diagnosis, resulting in a higher rate of surgical resection and improved survival. The authors present a 72-year-old woman who presented with pulmonary embolism and Budd-Chiari syndrome. Pathological examination revealed a leiomyosarcoma. The tumor, involving the IVC, was diagnosed with imaging techniques that showed intracardiac extension of a primary venous leiomyosarcoma. The literature discussing leiomyosarcoma of the IVC is briefly reviewed.

Aged↗

So-called "inflammatory leiomyosarcoma'': a series of 3 cases providing additional insights into a rare entity.

Inflammatory leiomyosarcoma, a rare entity first described in 1995, has been characterized by smooth muscle differentiation, a near-haploid karyotype, and a surprisingly good prognosis. The morphology is similar to that of conventional leiomyosarcoma admixed with a chronic inflammatory infiltrate. Thus far, only 15 cases have been reported in the English language literature. We report the clinical and pathological features of 3 additional cases of inflammatory leiomyosarcoma. Two women (ages 64 and 25, respectively) and 1 man (age 32) presented with a thigh, ovary, and lung mass, respectively. Inflammatory symptoms, such as anorexia, fever, night sweats, abdominal pain, and diarrhea, coincided with the thigh and ovarian primaries. Immunohistochemical studies revealed diffuse positivity for desmin and poor expression for other smooth muscle and skeletal muscle markers (muscle-specific actin [0/3], alpha-smooth muscle actin 1/3 [focal], calponin [1/3], caldesmon [0/3], and myogenin [0/3]). CD68 was diffusely positive in both the histiocytes and spindle cell component in all cases. Ultrastructural evaluation of 1 case (lung primary) lacked definitive smooth muscle differentiation. Cytogenetic analysis in 1 of 2 cases that were karyotyped, identified a near-haploid karyotype, which has been reported in other cases of inflammatory leiomyosarcoma. The other case showed 2 clonal populations of cells with interstitial deletions of the short arm of chromosome 8 and the long arm of chromosome 9, respectively. The case without cytogenetic data was intimately associated with an ovarian mature teratoma. These data also suggest that inflammatory leiomyosarcoma may lack smooth muscle differentiation, characterized by diffuse immunoreactivity for desmin but lack of immunoreactivity for alpha-smooth muscle actin, calponin, and caldesmon. In addition, 2 of the 3 cases developed distant metastases to the lungs, which suggests that these lesions may have a worse prognosis than previously believed.

Adult↗

Leiomyosarcoma of the esophagus: radiographic findings in 10 patients.

OBJECTIVE: Leiomyosarcomas of the esophagus are rare malignant smooth-muscle tumors that have been described only anecdotally in the radiology literature. The objective of this study was to evaluate the clinical and radiographic findings of this unusual lesion. MATERIALS AND METHODS: A search of the radiology archives of the Armed Forces Institute of Pathology revealed 10 cases of esophageal leiomyosarcomas. Clinical and radiographic findings were reviewed retrospectively. RESULTS: All but one patient presented with dysphagia. The average duration of the dysphagia was 6.7 months, but five patients had dysphagia for 3 or fewer months. Frontal chest radiographs revealed a mediastinal mass in five patients. Barium studies revealed intramural lesions in six patients, intraluminal lesions in two, and infiltrative lesions in two. The intramural Lesions all had large exophytic components, and three contained ulceration or tracking. One of the intraluminal lesions appeared as a polypoid expansile mass and the other, as a smooth expansile sausage-shaped mass mimicking a fibrovascular polyp. CT revealed a mass involving the esophagus in five patients; three of these patients had heterogeneous lesions containing large exophytic components, central areas of low density, and extraluminal gas or contrast material within the tumor. In two patients, MR imaging revealed large masses that were isointense with skeletal muscle on T1-weighted images and hyperintense on T2-weighted images. CONCLUSION: Our experience suggests that esophageal leiomyosarcomas have radiographic findings similar to those of leiomyosarcomas elsewhere in the gastrointestinal tract. Esophageal leiomyosarcomas have a better prognosis than squamous cell carcinomas and are often amenable to surgical cure.

Adult↗

Epithelioid and spindle-celled leiomyosarcoma of the heart. Report of 2 cases and review of the literature.

BACKGROUND: Primary cardiac leiomyosarcomas are rare. Isolated reported cases and small series generally describe spindle-celled, high-grade tumors with poor short-term survival; however, the pathologic features of many of these tumors are incompletely documented. The authors report in detail the clinicopathologic features of 2 relatively low-grade epithelioid and spindle-celled primary cardiac leiomyosarcomas. METHODS: Cases 1 and 2 were studied using standard histochemical and immunohistochemical techniques, and case 1 was examined by electron microscopy. The literature was reviewed with regard to primary cardiac leiomyosarcomas. RESULTS: Both tumors showed epithelioid and spindle-celled areas. The tumor in case 1 was low grade, and the tumor in case 2 was predominately low grade with a high-grade focus. A review of 28 reported cases revealed a wide age range (mean, 43 years), equal male-to-female ratio, and a predilection for the left atrium (48%). Follow-up of reported cases with fewer than 5 mitoses per 10 high-power fields showed a mean survival of 22 months compared with a 9-month mean survival for all others. CONCLUSIONS: Short-term follow-up of reported cases of high-grade cardiac leiomyosarcoma suggests a poor prognosis. Long-term follow-up in our case 2, along with follow-up of reported cases that were histologically similar to our cases, suggests that cardiac leiomyosarcomas with low-grade features or mixed low- and high-grade features also have a poor overall long-term survival, with a high rate of local recurrence and systemic spread.

Adult↗

Leiomyosarcoma: a rare pedal finding.

Leiomyosarcoma of the skin and subcutaneous tissue is a rare soft tissue neoplasm comprised of spindle-shaped smooth muscle cells. The statistical incidence of superficial leiomyosarcomas varies from 2.3% to 5.3% of malignant soft tissue tumors and from 4.0% to 6.5% of soft tissue sarcomas. Its occurrence in the foot is even more unusual. In a literature review extending from 1936-present, the authors have been able to locate only 12 known cases of leiomyosarcoma involving the foot and ankle. A presentation of the incidence, demographics of distribution, clinical characteristics, histopathologic features, prognosis, and treatment of leiomyosarcoma will be presented along with a unique case history involving a recalcitrant heel ulcer complicated by leiomyosarcoma.

Aged↗

MR imaging of CNS leiomyosarcoma in AIDS patients.

Leiomyosarcomas of the central nervous system are extremely rare; however they are becoming more prevalent in immunocompromised patients. The authors present MRI (Magnetic Resonance Imaging) of six cases of pathological proved leiomyosarcomas of the central nervous system in patients infected with human immunodeficiency virus. MR images of 4 cases of intraspinal leiomyosarcoma showed lobulated masses expanding multilevel of neural foramina with extradural and intradural extension, giving dumbbell appearance which mimic neurofibroma. Two cases of intracranial leiomyosarcoma revealed a mass at the left cavernous sinus involving prepontine cistern in one case and two lesions in the other case showing masses with dural based appearance at the region of the planum sphenoidale and the posterior aspect of the falx cerebri which mimiced a meningioma. The leiomyosarcoma should be included in the differential diagnosis of extra-axial CNS lesions in HIV-infected patients.

Acquired Immunodeficiency Syndrome↗

[A case of primary hepatic leiomyosarcoma presenting with multiple subcutaneous scalp mass].

Leiomyosarcoma is an uncommon tumor which arises from various sites including uterus, stomach, retroperitoneum, superficial soft tissues, bladder, kidney, and lung. Primary hepatic leiomyosarcoma is a very rare tumor and fewer than 70 cases of primary hepatic leiomyosarcoma have been reported since the first publication in Japan. And there was only one case report of cutaneous metastasis from hepatic leiomyosarcoma. We recently experienced a case of primary hepatic leiomyosarcoma presenting as subcutaneous palpable mass. Herein we report this case with a review of literatures.

Head and Neck Neoplasms↗

[Determination of cell nuclear DNA content in leiomyoma and leiomyosarcoma of gastro-intestinal tract (G-I)].

Nuclear DNA content was measured by microspectrophotometry in 15 biopsy specimens from patients with G-I smooth muscle tumors (3 leiomyomas and 12 leiomyosarcomas subdivided into 3 groups with 4 cases to each). The mean DNA value increased steadily as follows: leiomyomas (14.39 +/- 0.62 Au); leiomyosarcoma Grade I (19.78 +/- 2.39 Au); Leiomyosarcoma Grade II (26.39 +/- 1.60 Au); leiomyosarcoma Grade III (30.66 +/- 2.39 Au). The difference of DNA value in the 4 groups had statistical significance (P less than 0.05-0.01). These results suggest that microspectrophotometric measurement of nuclear DNA content may serve as an objective quantitative method for diagnosis of G-I tract smooth muscle tumors and classification of leiomyosarcoma.

Cell Nucleus↗

[A case of leiomyosarcoma of the rectum with widespread lymph node metastases].

A case of leiomyosarcoma of is reported. A 78-year-old man was admitted with a history of constipation and deformation of the stools. Barium contrast study and sigmoidoscopy showed a submucosal tumor of the rectum. On computed tomography and angiography, the tumor was suspected of being leiomyosarcoma. Abdominoperineal resection was performed. Microscopic examination revealed a leiomyosarcoma of the rectum and widespread lymph node metastases were found. Leiomyosarcoma of the rectum is an uncommon tumor. In our country, 89 cases have been reported. Six had lymph node metastases initially and another six subsequently. We have referred to the literature on leiomyosarcoma of the rectum.

Aged↗

[2 cases of the independent coexistence of early cancer and leiomyosarcoma in the same stomach].

In the past 10 years, malignant diseases have been revealed in 157,836 autopsy cases (in Japan). Among them, gastric cancer was found in 17.5% and leiomyosarcoma in 0.45%. In addition, the frequency of leiomyosarcoma of the stomach was only 0.08%. In the literature, 20 cases of the coexistence of cancer and leiomyosarcoma in the same stomach (were) reported in Japan prior to (data). Eight of these were early cancer. Subsequently, three cases, including two of our own, were added. In our two patients, early cancer and leiomyosarcoma coexisted independently in the same stomach. It is suggested that the stomach with leiomyosarcoma may have a tendency to be cancerous.

Adenocarcinoma↗

[Leiomyoma and leiomyosarcoma of the esophagus: report of 9 cases and review of the Japanese literature].

Seven cases of leiomyoma and two cases of leiomyosarcoma of the esophagus were presented. Review of the Japanese literature revealed a total of 260 cases of the former and only 35 cases of the latter, including our own cases. They occurred more frequently in males than females, by a ratio of 1.6:1 and 1.8:1, respectively. The patients with leiomyosarcoma distributed in higher age groups. The most frequent symptom was dysphagia in both groups of the cases, but in cases of leiomyosarcoma the incidence was much higher and the duration of the symptom was shorter. About 20% of the patients with leiomyoma were asymptomatic. Although the size of leiomyosarcoma tended to be larger, giant leiomyomas more than 10 cm in diameter were found in 18% of the cases. The lower third of the esophagus was the site of both tumors in over half of the cases, the middle third being the next frequent. The surgical removal by enucleation was the procedure of choice for leiomyoma. However, when either the tumor is densely adherent to the mucosa for considerable length or accidental extensive mucosal tear occurs, esophageal resection is recommended. Postoperative morbidity was minimal and results were generally excellent. On the contrary, esophageal resection including the tumor was indicated for leiomyosarcoma. The long-term results were poor particularly in cases of infiltrating-type lesions, though survival data could not be fully evaluated.

Adult↗

[Primary leiomyosarcoma of the liver (author's transl)].

The occurrence of leiomyosarcoma was demonstrated in a tissue cylinder obtained by liver biopsy in a 68-year-old woman with unclear hepatomegaly. The patient died 8 months after she had experienced first signs of illness. Autopsy revealed a primary leiomyosarcoma of the liver with metastases in the lungs, bilaterally in the pleura, in the kidneys, and in the periportal and spleno-pancreatic lymph nodes. The occurrence of a primary tumour in the urogenital system or in the gastrointestinal-tract could be excluded. The diagnosis of leiomyosarcoma was based on the microscopical demonstration of smooth muscle fibres with enlarged red-like and partially atypical cell nuclei and atypical mitoses. Furthermore, we observed extensive necroses and haemorrhagia in the tumour tissue. Preexisting tumours of the liver as, e.g., teratoma or hepatoblastoma were not found. No cirrhosis of the liver could be detected. Obviously, the leiomyosarcoma had its origin in the smooth muscle fibres of the liver vascular system.--According to the literature primary leiomyosarcomas in the liver are only rarely found.

Aged↗

[Two case reports of retroperitoneal leiomyosarcoma].

Two cases of retroperitoneal leiomyosarcoma are presented. The first case was in a 67-year-old female, whose chief complaint of right upper abdominal mass and dull pain. The tumor, 13 x 12 x 8 cm in size, developing in the retroperitoneum was removed with the right kidney and vena cava. The pathological diagnosis was reported as leiomyosarcoma. The second case was in a 62-year-old male, whose complaint was left abdominal swelling, also with general fatigue. A large tumor mass, invading over all of the left flank organs, was palpable by physical examinations, from which the mass was far beyond surgical approach. Needle biopsy revealed the pathological finding of leiomyosarcoma. One month later, the patient died of cachexia. Review of the literatures for the retroperitoneal leiomyosarcoma, revealed only a few cases; 1.7%, of all leiomyosarcoma to date.

Aged↗

Gastric leiomyosarcoma: a clinicopathological review.

OBJECTIVE: To report our experience of eight patients with primary gastric leiomyosarcoma. DESIGN: Retrospective study. SETTING: Teaching hospital, Taiwan. SUBJECTS: 8 patients who presented with primary gastric leiomyosarcoma between 1986 and 1990. MAIN OUTCOME MEASURES: Endoscopic, radiological, and histopathological features, DNA ploidy, and outcome. RESULTS: Abdominal pain and gastrointestinal bleeding were the most common presenting symptoms. In 5 of the 7 patients who had endoscopy preoperatively a diagnosis of submucosal tumour was made, but a definite diagnosis of leiomyosarcoma was made in only 3. A provisional diagnosis of leiomyosarcoma was made in 3 of the 4 patients who had computed tomography. All patients underwent some form of gastric resection ranging from palliative resection to total gastrectomy. Patients in whom the tumour had invaded adjacent organs had a poor prognosis. Histopathological grading of tumours correlated well with DNA ploidy. CONCLUSION: Both histopathological grading and DNA ploidy could be helpful objective prognostic criteria in patients with primary gastric leiomyosarcoma.

Aged↗

Leiomyosarcoma of colon: report of two cases.

Leiomyosarcoma of the colon is a rare tumor, with fewer than 45 cases being reported by 1980. Wide resection, 10 cm margin, and adjacent mesentery will decrease chances of locoregional relapse. We recently encountered two leiomyosarcomas in the colon in the past two years. One is a 63-year-old female who was seen with rectal bleeding, pain, and was found to have a large intraluminal leiomyosarcoma in the descending colon. Biopsy revealed leiomyosarcoma, grade III. She underwent a wide subtotal colectomy with ileosigmoid anastomosis, with metastases to three of the nodes in the mesentery, and remains well. The second is a 69-year-old female with general weakness who had previously undergone a hysterectomy and cholecystectomy. On examination she was found to have an abdominal mass. This proved to be a large leiomyosarcoma arising in the proximal portion of the transverse colon. There were no metastatic areas to the liver, chest, or any other areas. She has undergone extended right colectomy and remains well. Because of the rarity of this type of cancer, there is no established protocol concerning its treatment.

Aged↗

Quantitative measurements of desmin and vimentin immunostains and cell density in leiomyomas and leiomyosarcomas.

The distinction between benign and malignant smooth muscle tumours relying on histological features such as the mitotic index and pleomorphism remains a generally acknowledged difficulty in modern pathology. A cell image processor was therefore used to quantitatively assess the desmin and vimentin immunostain in 39 smooth muscle tumours which included 26 benign (leiomyomas) and 13 malignant (leiomyosarcomas) cases. The 13 leiomyosarcomas were primary (non-recurrent and non-metastatic). Ploidy level and cell density were also assessed on each of these 39 tumours by means of the computer-assisted microscopic analysis of 5-microns thick Feulgen-stained histological sections. The results show that while neither the ploidy level determination nor the quantitative assessment of the vimentin immunostain made it possible to distinguish between leiomyomas and leiomyosarcomas, cell density determination and the quantitative assessment of the desmin immunostain enabled such a distinction to be made. Indeed, the leiomyomas exhibited a much higher level of desmin positivity than the leiomyosarcomas, as did diploid tumours as compared to the aneuploid (benign or malignant) ones. Furthermore, the leiomyoma group exhibited a significantly lower mean cell density value than the leiomyosarcoma group. The present study further confirms the lack of relationship between ploidy level and cytological malignancy in smooth muscle tumours.

Adolescent↗