Primary leiomyosarcoma of the thyroid gland

Primary leiomyosarcoma of the thyroid gland is extremely rare, and to the best of our knowledge only five well-documented cases have been reported in the world literature. We herein report a 58-year-old female patient with primary leiomyosarcoma of the thyroid who was successfully treated by total thyroidectomy with a modified neck dissection. Immunohistochemically, the tumor cells showed positive reactivity to α-smooth muscle actin and vimentin. Radical surgery was thus considered to be essential in the treatment of this rare but rather aggressive malignancy.     

Renal angiomyolipoma with sarcomatous transformation and pulmonary metastases

We report the case of a 49-year-old woman with a large renal angiomyolipoma that invaded the liver. In some areas, the tumor had the appearance of a typical angiomyolipoma; however, it also had foci where the spindle cells of the lesion showed marked cytologic atypia and mitotic activity, giving it the appearance of a high-grade sarcoma. Immunohistochemical studies demonstrated expression of vimentin, desmin, and muscle-specific actin by the sarcoma cells; these findings were consistent with leiomyosarcoma. A second, small typical angiomyolipoma was also present in the kidney. In addition, the liver exhibited focal nodular hyperplasia. Three weeks after resection of the primary renal tumor, pulmonary metastases were diagnosed by fine-needle aspiration biopsy. This is the first report of a case of angiomyolipoma with sarcomatous transformation and biopsy-proven metastatic disease.     

Duodenal metastatic leiomyosarcoma of the uterus. A case report

The digestive metastases of uterine leiomyosarcoma are rare. We report a case of a duodenal tumor detected in a 50 year-old woman, 3 years after she underwent a total hysterectomy for uterine leiomyosarcoma. The stenosing duodenal mass was thought to be a mesenchymal tumor. A pancreaticoduodenectomy was performed. The resected lesion was morphologically similar to the uterine leiomyosarcoma. In fact, the histopathological study confirmed a submucosal tumor with typical features of the uterine leiomyosarcoma. Immunohistochemistry was performed and it showed negative CD117 and CD 34. Markers displayed positivity for actin smooth muscle and desmin. Considering the patient history and the immunohistochemical observations, we diagnosed a duodenal metastasis of uterine leiomysarcoma. Through this exceptional observation we want to emphasize the epidemiological and the pathological features of the metastatic uterine leiomyosarcoma. We will also point out the progress of tumoral cells and the histopathological distinctive criteria with a primitive digestive mesenchymal tumor.     

Leiomyosarcoma of the kidney with tumor thrombus to the inferior vena cava

A 76-year-old female presented with edema in the bilateral lower legs and computed tomography (CT) subsequently revealed a tumor under the right side of the kidney that extended to the inferior vena cava. Under suspicion of a right renal sarcoma or malignant teratoma, we performed a right nephrectomy and tumor thrombectomy. Histopathological features of the resected specimen were compatible with a leiomyosarcoma originating from either extrarenal blood vessels or muscle fibers of the renal pelvis. Immunohistological findings showed that the tumor cells were spindle-shaped, arranged in bundles, and stained positive for α-smooth muscle actin and desmin. The patient was free from recurrence 10 months after surgery. The prognosis of patients with a renal leiomyosarcoma with venous thrombus has been considered to be poor. Herein, we provide details of our case and conclude that radical tumor resection is necessary for a long-term cure.     

Adenomyoepithelioma of the breast diagnosed by a mammotome biopsy: Report of a case

Adenomyoepithelioma is an uncommon primary breast tumor. It is conspicuous for two elements of the tumor, namely, ductal and myoepithelial components. Recently, a Mammotome biopsy, or stereotactic vacuum-assisted biopsy has become popular and various benign or borderline lesions are obtained. We report an adenomyoepithelioma of the breast in a 56-year-old woman. She was pointed out to have a cluster of some microcalcifications on mammography and a 9-mm hypoechoic mass lesion was detected by ultrasound. A Mammotome biopsy revealed a well-defined lesion. Histologically, the tumor demonstrated a thick and bi-cellular growth pattern consisting of ducts and myoepithelium. Immunohistochemically, epithelial cells were positive for cytokeratin AE1/AE3 and cytokeratin, epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA), negative for alpha-smooth muscle actin (alpha-SMA). In addition, myoepithelial cells were positive for alpha-SMA and CEA, which were scatterly positive for cytokeratin AE1/AE3, and negative for EMA. In examinations of non-palpable lesions found on mammography and ultrasound, a Mammotome biopsy is useful for making diagnosis, however, and adenomyoepithelioma is rarely found. In diagnosing such a rare disease from the limited information obtained from a needle biopsy, an immunohistochemical study was thus found to be useful for making a differential diagnosis.     

Radiation induced intracranial leiomyosarcoma: Its histopathological features

Summary This is a case of intracranial sarcoma which was recognized 23 years after irradiation therapy for pituitary adenoma. Four operations were performed because of recurrences with a short interval between each operation. Immunohistochemically, the tumour cells were stained for smooth muscle actin, human muscle actin and vimentin. It was verified as a leiomyosarcoma. This report is the first case of intracranial leiomyosarcoma associated with radiation therapy in pituitary adenoma.     

Paratesticular leiomyosarcoma in an elderly man

Soft tissue tumors, both benign and malignant, of the testes are relatively uncommon. Primary leiomyosarcoma of the testis is rare, and its clinical behavior is difficult to predict. We report a case of paratesticular leiomyosarcoma in a 71-year-old man. Routine histologic examination and immunohistochemical staining were performed. The tumor was composed of interlacing fascicles of smooth muscle cells with marked nuclear pleomorphism and three to four mitoses per high power field, with few atypical mitoses. On the basis of the microscopic examination and immunohistochemical studies, the tumor was identified as a paratesticular leiomyosarcoma. The published studies on this entity are reviewed.     

Immunoprofile of a carcinosarcoma of the submandibular gland.

Carcinosarcomas are a very rare group of true malignant tumors of the salivary gland. As the name indicates, the tumor is composed of an epithelial and a mesenchymal component, both malignant. We report a case of carcinosarcoma of the submandibular gland in an 86-year-old woman. The epithelial component showed a squamous carcinoma phenotype, whereas the mesenchymal component was morphologically similar to a fibrosarcoma. The epithelial component was strongly positive for CK13, CK14, and AE1/AE, and groups of positive cells were seen for CK19 and vimentin. The whole mesenchymal component was positive for vimentin, negative for cytokeratins, and focal cells were positive for smooth- muscle actin. Both components were strongly positive for P53 and Cyclin D1, and focally positive for MDM2. Rare multinucleated giant cells showed expression of CD68, and focal dendritical cells on carcinomatous nests were positive for S-100. The CK7, CK8, Factor XIIIa, c-erbB-2, P16, CDK-4, Rb1, and E2F-1 were not detected in these 2 groups of malignant cell populations.     

Combined right hepatic and retrohepatic caval resection with reconstruction using a polytetrafluoroethylene graft for primary leiomyosarcoma of the liver: Report of case

We present herein the case of a 53-year-old woman who underwent successful surgical treatment for a leiomyosarcoma of the liver that originated from the posterior hepatic segment and involved the retrohepatic inferior vena cava (IVC). A computed tomographic scan and magnetic resonance imaging demonstrated a large tumor, with rich vascularity, in the liver. The IVC was found to be occluded on these scans, which was confirmed by venacavography. The patient underwent a combined right hepatic and caval resection with reconstruction using an expanded polytetra-fluoroethylene graft. The tumor consisted of spindle-shaped cells with cigar-shaped nuclei. It also had a moderate degree of cellularity and ten mitotic figures per ten high-power fields. Immunohistologically, desmin and alpha-smooth muscle actin were stained positive in the tumor cells, implying that the tumor was derived from smooth muscle cells. The patient is alive and well 15 months after her operation.     

Perforation of intestinal leiomyosarcoma: A case report

Introduction and ImportanceThe majority of gastrointestinal sarcoma is gastrointestinal stromal tumors and intestinal leiomyosarcoma is rare. Small intestinal mesenchymal tumors are often large at diagnosis, and they commonly present with bleeding or intussusception. We report a perforation associated with intestinal leiomyosarcoma.Case PresentationA 66-year-old man presented with severe epigastric pain. A physical examination showed tachycardia and a diffusely tender and rigid abdomen. Computed tomography showed a massive tumor and free air. A laparotomy was performed to treat lower digestive perforation. Massive tumor, which invaded surrounding intestine, was 20 cm in size at the ileum. The involved intestine was perforated. We confirmed that feeding artery was superior mesenteric artery and performed partial intestinal resection. His clinical course was uneventful and discharged 10 days postoperatively. The pathological findings showed spindle shaped and the tumor invaded the mucosa at the perforated site. Immunohistochemical spectrum resulted c-kit negative, S-100 negative, Desmin positive, alpha smooth muscle actin(αSMA) positive and Ki-67 30–40 %. The pathological findings were leiomyosarcoma.DiscussionGastrointestinal sarcoma is sometimes found by bleeding. In our patient, leiomyosarcoma invaded surrounding intestine, it made the intestine wall frail and caused perforation. The intestinal perforation which was involved by leiomyosarcoma has been rarely reported to the best of our knowledge since WHO refined leiomyosarcoma.ConclusionsAlthough intestinal leiomyosarcoma is rare, we should know that it can involve surrounding intestines and make them perforated.     

A case of epithelial membrane antigen-positive leiomyosarcoma of the urinary bladder

A 41-year-old woman was admitted with gross hematuria and pain on urination. Cystoscopy showed a huge and lobulated submucosal non-papillary bladder tumor. Pelvic computed tomography demonstrated a heterogeneous and enhanced lobulated mass, 8 cm in diameter, with extravesical invasion but there appeared to be no metastatic lesions. Transurethral biopsy revealed leiomyosarcoma pathologically. Total cystectomy and construction of an ileal conduit were performed. The tumor was histologically diagnosed as leiomyosarcoma. Immunohistochemical studies revealed the tumor to be positive for epithelial membrane antigen (EMA) and muscle actin but negative for desmin, and S-100. We reviewed 102 cases of vesical leiomyosarcoma reported in Japan. Among these 102 cases, there were no EMA-positive cases. Immunohistochemical and electromicroscopic evaluation should be performed to evaluate this disease.     

A case of primary leiomyosarcoma of the chest wall successfully resected under the video-assisted thoracoscopic approach.

We report a case of a 62-year-old woman with primary leiomyosarcoma of the chest wall which was successfully resected under the video-assisted thoracoscopic approach. The disease was found during the treatment for a malignant melanoma of the left heel. On the preoperative CT images, the lesion was suspected to be a metastasis of the malignant melanoma. The thoracoscopic surgery revealed that the tumor originated from the parietal pleura, and it was resected with a 5-mm margin of normal pleura. Histopathologically, the tumor was diagnosed as low-grade leiomyosarcoma. Since no residual tumor cells were proven in the resected margins histologically, further resection was not performed. At present, she is alive and well with no sign of recurrence of leiomyosarcoma two years and one month after operation. Thoracoscopic surgery is worth trying for accurate diagnosis of and effective treatment for a chest lesion without apparent invasion of the chest wall on the preoperative CT images.     

Giant leiomyosarcoma of the rectum with lymph node metastasis: A case report and review of the literature

IntroductionLeiomyosarcoma of the gastrointestinal tract is very rare, with a reported frequency of less than 0.1% of all malignancies of the colorectum. It is important to diagnose leiomyosarcoma definitively by immunohistochemical profiling of smooth muscle actin, desmin, and CD34. True leiomyosarcoma of the colorectum diagnosed by immunnohistochemical profiling is extremely rare that only 13 reports have been published in reviews of resected gastrointestinal mesenchymal tumors after 1998. In addition, lymph node involvement is rare in patients with leiomyosarcoma. Herein we report an aggressive case of LMS in a rectosigmoid lesion with lymph node metastasis.Case presentationA 76-year-old woman visited our hospital complaining of intermittent anal bleeding that had lasted 5 months. Image studies aiming at examining the cause of her anal bleeding revealed a tumor located between the right ovary, uterus, and the rectosigmoid. Histopathology of biopsied materials from the colonoscopy suggested a malignant tumor of mesenchymal origin. Surgical resection was performed with curative intent. The tumor was diagnosed as leiomyosarcoma by pathological examination. Moreover, one of the 31 regional lymph nodes retrieved was metastasized by leiomyosarcoma. Eight months later, follow-up CT scans revealed multiple recurrent lesions in the liver and peritoneum. Despite systematic chemotherapy, she deceased 12 months after the surgery.ConclusionIt is crucial to diagnose leioyosarcoma precisely based on immunohistochemistry, and thereby distinguish it from GIST. Although lymph node metastasis is rare, lymphadenectomy appears to be important for high-risk LMSs to perform R0 resection. Further investigation on leiomyosarcoma cases so far is required to establish standard treatment strategies.     

One-stage surgery of low-grade malignant intravenous uterine leiomyosarcomatosis with right atrium extension

Uterine leiomyosarcoma is a rare and aggressive malignant tumor arising from the smooth muscle cells of the myometrium, which rarely metastasizes to the heart. We report the case of a 49-year-old Chinese woman who underwent a successful one-stage surgical intervention after an initial diagnosis of intravenous uterine leiomyomatosis. However, her postoperative biopsy showed the presence of low-grade malignant uterine leiomyosarcoma with intravenous and right atrium extension. It is our conclusion that surgical resection is the mainstay treatment for this type of condition.     

Leiomyosarcoma of the urinary bladder in a patient with bilateral retinoblastoma

Abstract:   We report a case of bladder leiomyosarcoma in a 27-year-old woman who had previously been treated with surgery and radiation for bilateral retinoblastoma. The patient was admitted to hospital with discomfort on micturition. Cystoscopy revealed a bladder tumor covered by normal urothelium. Transurethral resection of the bladder tumor was performed, and the histopathological diagnosis was leiomyosarcoma. Partial cystectomy was performed. The leiomyosarcoma of the bladder did not invade the muscle layer. However, bladder tumors recurred at new intravesical locations repeatedly. After transurethral resection of the bladder tumor had been performed twice, total cystectomy and creation of an ileal conduit were performed.     

Leiomyosarcoma of the external iliac vein

Leiomyosarcoma of the iliac vein is an uncommon tumor. We report a case of a 63-year-old Japanese woman with leiomyosarcoma of the right external iliac vein. The patient complained of right inguinal pain and swelling. Computed tomography demonstrated a mass surrounding the right external iliac artery and vein. Metastases in the lungs and liver were found. Complete resection of the tumor along with the involved vessels was performed. Polytetrafluoroethylene grafts were used to reconstruct the vessels. Pathological examination revealed leiomyosarcoma of the external iliac vein. Although the prognosis of leiomyosarcoma is poor, en bloc tumor resection is the treatment of choice.     

Myoepithelial Carcinoma: A Rare Neoplasm of the Breast

BACKGROUND: Malignant myoepitheliomas of the breast are extremely rare. There has been a limited number of published reports of myoepithelial carcinomas originating from the breast. CASE REPORT: We describe a malignant myoepithelioma of the breast in a 56-year-old woman. Histological examination showed polygonal epithelioid cells and spindle cells with moderate to marked nuclear atypia. Immunohistochemistry showed reactivity in the spindle cells for smooth muscle actin, cytokeratin (AE1/AE3), and p63, indicating a myoepithelial cell lineage of tumor cells. The patient underwent radical surgical excision of the lesion and axillary lymph node dissection. She demonstrated no evidence of recurrence over an 11-month follow-up. CONCLUSIONS: We suggest myoepithelial carcinomas of the breast be managed with appropriate surgical clearance. A multidisciplinary approach is usually required.     

Spinal manifestation of metastasizing leiomyosarcoma.

STUDY DESIGN: Case report. OBJECTIVE: To provide additional information on possible relations between uterine and spinal manifestations of leiomyosarcoma. SUMMARY OF BACKGROUND DATA: Spinal metastases and primary spinal manifestation of leiomyosarcoma and other malignant smooth muscle tumors are rarely observed. METHODS: Clinical and radiologic follow-up of a patient with a spinal tumor. RESULTS: A 46-year-old women had rapidly progressive paraplegia caused by an extramedullary lesion in the extradural space at T2-T3, with spinal cord compression. After surgical decompression, the resected tumor was histologically classified as a leiomyosarcoma. Diagnostic work-up failed to detect the primary tumor site. Previous medical history had been uneventful except for hysterectomy 3 years earlier for a leiomyoma. Extended necroses and focal metaplasia were already described in the hysterectomy specimen indicating malignant disease; however, it was not definitely diagnosed. Multiple leiomyosarcoma metastases developed 22 months later. CONCLUSIONS: In retrospect, the origin of the leiomyosarcoma that manifested in the thoracic spine of the patient must be reconsidered. Rather than a primary dural leiomyosarcoma, this tumor represented the first evidence of recurrence of a missed diagnosis of early-stage uterine leiomyosarcoma.     

Huge retroperitoneal leiomyosarcoma: a case report

This is a case report of retroperitoneal leiomyosarcoma in a 61-year-old woman. She presented with a chief complaint of back pain. Computed tomography showed a left huge retroperitoneal tumor. The tumor was removed with left nephrectomy and left hemi-colectomy. Histological examination demonstrated leiomyosarcoma 26 x 20 x 16 cm in diameter and, 3.84 kg in weight. She died of local recurrence causing ileus 2 months after the surgery. Fifty-four cases of retroperitoneal leiomyosarcoma including the present case in the Japanese literature are reviewed.