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[Primary leiomyosarcoma of the pancreas].

Primary leiomyosarcoma of the pancreas is a very rare malignant tumor, with only 12 cases reported. A case of primary pancreatic leiomyosarcoma in a 55 year-old female with diabetes mellitus is described. US and CT demonstrated tumor masses in the head and tail of the pancreas. By angiography, abundant tumor vessels corresponding to the pancreatic tumor masses were revealed in the pancreas. The patient underwent surgical resection of the tumor in the tail of the pancreas, and then, microscopically it was diagnosed as leiomyosarcoma. The tumor in the head of the pancreas was not resected on account of the presence of diabetes mellitus and the possibility of poor prognosis. Macroscopically, it seemed to be the same as the tumor in the pancreatic tail. By operative findings, there was invasion from the pancreatic head to the duodenum and there was nothing to justify suspicion of the extra-ductal growing type of primary leiomyosarcoma of the duodenum. There were no metastases noted at the time.

Diagnosis, Differential↗

[Carney's triad. Apropos of a case of recurrent gastric leiomyosarcoma and bilateral pulmonary chondroma].

Carney's triad is a rare syndrome characterized by gastric leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. Twenty-seven cases are reported in literature: the most commonly observed neoplasm is gastric leiomyosarcoma (26/27) followed by lung tumor (22/27), and gastric paraganglioma (15/27). Three patients bearing a gastric leiomyosarcoma developed liver metastases, and 6 had a local recurrence after some years. The occurrence of paraganglioma makes the prognosis worse: 4 deaths from local infiltration, metastases or severe hypertension. The triad only occurred in 9/27 cases, mostly young female patients. A common embryologic origin is difficult to assess. The case of a young female patient with recurrent gastric leiomyosarcoma (12 years later) and bilateral pulmonary chondroma is described. It is important that patients with any of the three tumors be periodically examined.

Child↗

[Leiomyosarcoma in the musculature of large vessel wall].

Leiomyosarcoma of the large arteries and veins is rare. In this paper, one case of primary leiomyosarcoma of the axillary vein is reported. The patient, a 66 year old man, was admitted because of a mass in the medial side of right upper arm for three years and increasingly growing. The tentative diagnosis was neurologic fibroma. A local resection was performed and the final diagnosis of leiomyosarcoma of the right axillary vein was established by pathology. So far, there are 86 cases reported in the foreign literature but only one in the Chinese literature. The neoplasm most frequently occurs in the large veins (68 cases) and approximately one half in the inferior vena cava (33/68). 18 neoplasms are originated from the large arteries and of them, 10 from the pulmonary arteries. Primary leiomyosarcoma in large veins is about four times as common as that in arteries. The primary sites in order of incidence: (1) inferior vena cava; (2) pulmonary artery; (3) other large veins and (4) other large arteries.

Aged↗

[A case report of hepatic metastasis from leiomyosarcoma of the rectum with right hepatic lobectomy].

A case of hepatic metastasis from leiomyosarcoma of the rectum submitted to right hepatic lobectomy is presented. Surgery for hepatic metastasis from intestinal leiomyosarcoma is rarely possible. The patient was a 67-year-old woman who was diagnosed as leiomyosarcoma of the rectum and underwent abdominoperineal excision of the rectum in fifteen months. Hepatic metastasis was detected by US, CT and angiography. Right hepatic lobectomy was performed, and the resected hepatic lesions were histologically identified as metastasis from leiomyosarcoma of the rectum. The patient is still alive 10 months after the second operation with no lesions of the liver.

Aged↗

Uterine leiomyosarcoma with myxoid stroma.

Myxoid leiomyosarcomas present a diagnostic problem. They are said to have low mitotic counts and are considered to be exceptions to the general rule that uterine smooth-muscle tumors with a mitotic rate of less than five per ten high-power fields rarely recur. We present two cases of myxoid leiomyosarcoma, both of which show high mitotic counts. In addition, one of the cases presented initially as a typical leiomyosarcoma, but contained abundant myxoid stroma in the metastases. The role of the myxoid stroma in influencing prognosis in leiomyosarcomas is, as yet, uncertain.

Adult↗

[Scrotal leiomyosarcoma].

We report a case of leiomyosarcoma arising in smooth muscle of scrotum (Dartros muscle). Morphological findings are similar to leiomyosarcomas in other sites. Because of deep location of smooth muscle in scrotal skin, scrotal leiomyosarcomas are deep located tumors lacking the indolent course described in pure dermal leiomyosarcoma.

Genital Neoplasms, Male↗

[Renal leiomyosarcoma associated with penile carcinoma: a case report].

A case of renal leiomyosarcoma in a 55-year-old male is presented. The patient also had penile carcinoma. We believe this is the first report of renal leiomyosarcoma associated with penile carcinoma. Since 1957, thirty four cases of renal leiomyosarcoma have been reported in the Japanese literature. Some diagnostic problems of renal leiomyosarcoma are discussed, and the association of penile carcinoma with other malignant tumors is reviewed.

Carcinoma, Squamous Cell↗

Resection of leiomyosarcoma originating in internal iliac vein and extending into heart via inferior vena cava.

A patient's condition was initially diagnosed as constrictive pericarditis, but an echocardiogram revealed the presence of an intracardiac tumor. The tumor was a slow-growing leiomyosarcoma that originated in the left internal iliac vein, extended into the inferior vena cava, and reached the right ventricle. This tumor was considered to be a recurrent uterine leiomyosarcoma that had been resected 4 years before. After a second resection of this tumor, the patient was living a normal life 61/2 years postoperatively, in spite of recurrence in the pelvis and lung. We reviewed 12 cases of leiomyosarcoma in the world literature that reached the heart through the inferior vena cava. We added our case and evaluated the clinical features of all 13. This is the first successfully resected case of leiomyosarcoma of this nature so far as we could determine.

Adult↗

Primary leiomyosarcoma of the liver--a case report.

This is the 17th report on primary leiomyosarcoma of the liver in world literature. In 1980, a 69-year old white female patient was admitted with pain in the right upper abdomen and loss of weight of 12 kg. Increased blood sedimentation rate, elevated serum markers of the liver and ultrasound evolved a big tumor of the right liver without extrahepatic tumor nodules. Right hemihepatectomy was undertaken, the tumor was completely resected. After histologic examinations, leiomyosarcoma of the liver with 1 mitosis per 10 high power fields was diagnosed. 10 years later, the patient was readmitted with symptoms of pain, tiredness and night sweat. At this time, 3 intrahepatic and 1 retroperitoneal tumor nodules were detected. A sure-cut needle biopsy revealed the recurrent leiomyosarcoma with 4 mitoses per 10 high power fields. The survival period of 10+ years is the longest of all 17 patients with primary hepatic leiomyosarcoma reported so far. The low rate of mitoses per high power field and primary surgical resection are considered the reasons for the long survival period.

Aged↗

[Two cases of metastatic lung tumor from leiomyosarcoma of the uterus].

Three pulmonary resections were performed for two cases of metastatic lung tumor from uterine leiomyosarcoma. Case 1 was diagnosed as myoma uteri. Preoperative chest X-ray examination showed an abnormal shadow in the left upper lobe. Postoperative diagnosis was leiomyosarcoma. During radiotherapy for the pelvic lesion, the lung tumor was growing very rapidly and micrometastases were detected bilaterally without distant metastasis. Bilateral pulmonary resections were performed. Three months and 15 days later, second left pulmonary and thoracic wall resections were performed because of lung and pleural recurrence. Seven months after the first pulmonary operation, she died of systemic metastases. Case 2 was admitted to the hospital because of genital bleeding and diagnosed leiomyosarcoma of the uterus. The patient had a metastatic lung tumor in the left lower lobe preoperatively. Eighteen days after hysterectomy, left lower lobectomy was performed because progressive hemoptysis occurred for rapidly enlarged metastatic lesion. She died of intrapelvic bleeding three months after the operation without respiratory symptom. The prognosis of synchronously found out lung metastasis from uterine leiomyosarcoma is very poor. Neither chemotherapy nor radiotherapy is effective. Although it's difficult and controversial to decide the surgical indication, we need to operate on as soon as possible when there is no other distant metastasis. Even if the timing of operation is late, we can find the surgical significance of pulmonary resection for reduction of respiratory symptoms and probability of longer survival.

Female↗

[A resected case of leiomyosarcoma of the esophagus--the diagnosis was supported with DNA content analysis].

A case of leiomyosarcoma of the esophagus was reported. The tumor was located in the lower esophagus. The esophagus was very stenotic, but the mucosa was clear. We thought the tumor had not invaded the mucosa. Enucleation was performed on the tumor. The tumor was encapsulated, so at the time of operation, the microscopic diagnosis was leiomyoma. However, after the operation, the diagnosis was changed to the leiomyosarcoma, because there are many spindle-shaped atypical cells, and many mitoses. We also analyzed the DNA content of the tumor. The DNA index of this tumor was 0.9 and 1.48, therefore, this tumor was DNA aneuploidy. The result of DNA content analysis showed that the tumor is malignant. DNA content analysis is helpful for diagnosis of this kind of sarcoma, because microscopic examination can not readily distinguish benign leiomyoma from malignant leiomyosarcoma. This patient was still alive one year after the operation. Thus, we thought that enucleation of the tumor is a good operational procedure to the leiomyosarcoma of the esophagus, if the tumor is polypoid type and encapsulated.

Aneuploidy↗

[Bone metastasis of leiomyosarcoma. Apropos of a case].

INTRODUCTION: Bone leiomyosarcoma is a rare tumor, whether it may be primary or secondary. The authors report on the case of a woman, aged 67, admitted in January 1992 complaining of pain in the left hip and the upper end of the femur. CASE REPORT: In 1985 the patient underwent surgical excision of a soft tissue tumor in the right thigh, histologically diagnosed as a benign fibrous tumor. This lesion recurred locally four times and repeated excisions were performed throughout the years, always with a histological diagnosis of a benign lesion. On admission to hospital, the physical examination as well as laboratory data and plain roentgenograms were unremarkable. Both tomography and MRI showed a lesion in the upper end of the left femur. An isotopic bone scan showed marked increased uptake in the left hip extending to the femoral diaphysis. An open biopsy was performed for histology, immunohistochemistry and electron microscopy. A diagnosis of metastatic leiomyosarcoma was made. The retrospective histological examination of specimens of the soft tissue tumor excised in 1985 showed the same immunohistochemical features of the contralateral leiomyosarcoma. On this basis, one stage resection of the left hip and the upper end of the femur was performed and a Kotz modular prosthesis was inserted. Postoperative healing was achieved without any complications and the function of the operated limb was satisfactory. Three months after the operation pulmonary lesions were noted on chest radiographs and CT scan. The patient died two years after the first admission for widespread metastasis. DISCUSSION: In the reported case, the bony metastasis appeared to be the presenting finding of the soft tissue tumor of the contralateral thigh. This presentation is rare in previously published series. The misdiagnosis of the primary tumor had caused local recurrences, and an increased malignity occurred. According to the literature, a soft tissue leiomyosarcoma can be easily confused with other spindle cell lesions. Therefore an accurate histological and ultrastructural diagnosis is necessary for adequate surgical treatment.

Aged↗

Leiomyosarcoma of the left atrium: a case report.

Primary malignant cardiac tumors are uncommon, and cardiac leiomyosarcoma is extremely rare. We reported a case of left atrial (LA) leiomyosarcoma with unusual clinical manifestations. A 28-year-old female presented with unknown cause of fever, body weight loss and anemia for two months. Echocardiography and magnetic resonance image study disclosed a 5 x 3 x 3.6 cm3 lobulated mass in the LA with invasion to its posterior wall. Histologic and immuno-histochemical studies of the resected specimen revealed a picture of leiomyosarcoma. The patient improved after surgical resection and post-operative chemotherapy. The literature was reviewed with a discussion of the clinical manifestations, diagnosis and treatment strategy of this rare tumor. Diagnosis of LA leiomyosarcoma is frequently delayed to make a very poor prognosis. Postoperative chemotherapy should be considered because of highly possible incomplete resection. However, an optimal treatment regimen remains unknown.

Adult↗

[Primary bone leiomyosarcoma. Anatomo-clinical case with immunohistochemistry study and review of the literature].

We describe a primary leiomyosarcoma arising in the proximal part of the right tibia of a 38-year-old man. The diagnosis was confirmed by immunohistochemistry (positivity of tumor cells for alpha smooth muscle actin, HHF 35, desmin and vimentin). To the best of our knowledge, this is the 49th documented case of primary leiomyosarcoma of bone outside the facial skeleton. This exceptional tumor arises more commonly in adults (mean age: 53 years) and in the long bones of lower limbs, near the knee. The most frequent symptom is pain with or without swelling or fracture. Radiological findings invariably consist of a non specific osteolytic lesion. Although their histological appearance does not differ from that of extraosseous leiomyosarcomas, their diagnosis is difficult and often requires immunohistochemical and/or ultrastructural study. From a practical point of view, the diagnosis of primary leiomyosarcoma of bone also requires an intensive review of the case history and of previous pathology. This is necessary in order to eliminate an extraosseous primary site (mainly in uterus, gastrointestinal tract and soft tissues). As well as clinicopathological features, modes of treatment and results are also reviewed.

Adult↗

Pulmonary leiomyosarcoma--report of three cases.

Three cases of pulmonary leiomyosarcoma were presented. The characteristic clinical features were described with review of literature. In comparison with bronchogenic carcinoma, the leiomyosarcoma has some characteristics: 1) On chest X-ray, it usually appears as a sharply demarcated, even density round mass, growing rapidly within the lung, it rarely accompanies with hilar or mediastinal lymph node metastasis. 2) The preoperative cytological or pathological diagnosis is difficult either by sputum smear or by bronchoscopic biopsy or by fine needle percutaneous aspiration biopsy. 3) Pathological differential diagnosis of leiomyosarcoma of lung from anaplastic lung cancer is difficult. In conclusion, the primary pulmonary leiomyosarcoma is a rare malignant tumor, detecting the present illness seriously, paying attention to the chest X-ray films characterize, early surgical resection is the only way to get diagnosis and effective treatment method.

Adult↗

Detection of K-ras mutations in resected primary leiomyosarcoma.

Mutation of the K-ras oncogene occurs frequently in human malignancy. However, there are few reports concerning K-ras mutations in soft-tissue sarcoma, including leiomyosarcoma. We therefore designed a study to determine the prevalence of mutations in the first exon of K-ras in leiomyosarcoma and to evaluate its prognostic potential. Fifty-one leiomyosarcomas were reviewed, and their diagnoses were confirmed on pathological review. Tissue blocks were retrieved, and new sections were prepared for confirmation of diagnosis. Additional tissue sections were used for DNA isolation. PCR and denaturing gradient gel electrophoresis (DGGE) were used to detect K-ras mutations in the first exon of genomic DNA isolated from the specimens. Seven (14%) K-ras mutations were detected using DGGE. Subsequent sequencing of the K-ras gene from each of the mutated tumors confirmed the DGGE results in each case. The median survival for patients whose tumors did not contain mutations of K-ras was 42 months (n = 42) versus 25 months (n = 7) for those with mutations (P = 0.06). However, patients with stages I and II tumors had a median survival of 82 months (n = 28) compared to 28 months for those with stages III and IV disease (n = 20, P = 0.02). The results suggest that K-ras codon 12 mutations are uncommon in leiomyosarcoma; however, when such mutations are found, there is a trend toward worse survival. Furthermore, the data confirm that stage is a significant prognostic indicator.

Adult↗

[Leiomyosarcoma of the inferior vena cava and magnetic resonance imaging].

Venous leiomyosarcomas are rare and predominantly arise in the inferior vena cava (IVC). The clinical findings, often not very suggestive and nonspecific, sometimes precede the diagnosis by several years. According to the literature, leiomyosarcoma of the IVC generally occurs in middle-aged women. Modern imaging techniques, especially magnetic resonance imaging (MRI) can now establish the diagnosis of leiomyosarcoma of the IVC with a high probability and allow assessment of operability. The authors report a case of leiomyosarcoma of the IVC in a 24-year-old male patient, confirmed by intravenous biopsy. They present the MR features of this malignant tumour, rarely reported in the literature, and emphasize the value of this examination in the operability staging. The 3D imaging provided by MRI allows intra and extraluminal staging and involvement of adjacent organs. It also allows optimal evaluation of the effects on flow, circulatory slowing or thrombosis and, due to its high contrast resolution, it is more sensitive to distinguish clot from tumour nodule.

Adult↗

[Extracellular matrix components in leiomyosarcoma].

This report describes the glycosaminoglycans, collagen and elastin--composition of leiomyosarcoma. Studies were performed on leiomyosarcoma removed during surgery. The material was taken from 7 patients. Rearrangement of extracellular matrix in leiomyosarcoma has been found. There was an increase of total collagen content in comparison to control uterus. In both tissues type I collagen was found to be the predominant one. Both tissues, normal and neoplastic contain all known glycosaminoglycans types. Among them heparan sulphate was found to be the most abundant. A slight decrease in the content of this glycosaminoglycan was observed in leiomyosarcoma. A possible role of these alterations in tumour biology is discussed.

Adult↗