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Leiomyosarcoma of the testis is a very rare tumor in children. This report describes a massive leiomyosarcoma of the left testis in an infant that was treated successfully by excision and chemotherapy. During follow-up of 1 year, there has been no recurrence or metastases. No previous report of this tumor in an infant was found in the English-language literature.
OBJECTIVE: Bcl-2 protein is an apoptosis-inhibiting gene product that prevents the normal course of apoptotic cell death in a variety of cells. Additionally, bcl-2 can promote cell replication by reducing the requirement for growth factors. This protein seems, therefore, to play an important role in the growth of tumors. Our aim was to investigate the different expression of bcl-2 in uterine leiomyomas, smooth muscle tumors of uncertain malignant potential (STUMP), and leiomyosarcomas (LMS). Furthermore, the correlation between bcl-2 expression and various clinicopathologic parameters in leiomyosarcomas was assessed to evaluate its prognostic value. METHODS: This study included 26 cases of leiomyoma, 22 cases of STUMP, and 21 cases of LMS of the uterus. Bcl-2 expression was investigated by immunohistochemistry from paraffin-embedded tissue. The immunohistochemical findings were compared and correlated with different clinicopathologic parameters. Clinical information, including follow-up data, was obtained from the database of the Department of Gynecology and Obstetrics. RESULTS: Bcl-2 was present in 12 of 21 LMS, eight of 22 STUMP, and 20 of 25 leiomyomas. Significant differences regarding the frequency of bcl-2 expression and the staining intensity were observed between LMS and leiomyoma as well as between STUMP and leiomyoma (P <.05) but not between LMS and STUMP (P >.05). Regarding the outcome of uterine LMS, patients with bcl-2 positive tumors showed less vascular space involvement and longer overall survival (P <.05). CONCLUSION: Bcl-2 was expressed more frequently and more strongly in leiomyomas compared with LMS and STUMP. Regarding the outcome of uterine LMS, patients with bcl-2-positive tumors showed less vascular space involvement and longer overall survival. The stronger bcl-2 expression in benign leiomyomas and the better clinical outcome of bcl-2-positive LMS indicate that this protein seems to act as a good prognostic factor. Further studies including larger numbers of patients are necessary to establish bcl-2 as a routine marker for improved prognosis in malignant uterine smooth muscle tumors.
A case of leiomyosarcoma arising in the spermatic cord is described. A 83-year-old man required medical care for an irreducible inguinal hernia. The patient underwent herniorraphy and transinguinal radical orchiectomy. Macroscopically, the spermatic cord was enlarged by a gray-tan and ill-defined neoplasm measuring 4 x 4 x 3 cm. Histologically, this proliferation was composed of atypical spindle cells with blunted end nuclei and eosinophilic cytoplasm. Immunohistochemistry and ultrastructure confirmed the smooth muscle nature of the neoplastic cells. The diagnosis of leiomyosarcoma of the spermatic cord was made. To improve the assignment of this rare lesion to its specific anatomic location, we analyzed the immunohistochemical and ultrastructural characteristics of the smooth muscle tumoral cells and in particular those of the intracellular filament aggregates.
BACKGROUND: In contrast to Epstein-Barr virus (EBV)-associated posttransplant lymphoproliferative disorders (PTLD), EBV-associated leiomyomatous tumors have thus far only rarely been described. CASE REPORT: Two years after heart transplantation with ATG induction, cyclosporine (CsA; trough levels of 250 ng/mL)-based triple drug immunosuppression), a 23-year-old patient developed a small round lesion within the left lateral liver segment. The patient underwent ultrasound-guided biopsy followed by liver resection. Histological and immunohistological examination showed a leiomyosarcoma. In situ hybridization using EBV-specific EB endoplasmic reticulum-RNA showed an intensive signal in almost all tumor cells. The tumor stained for EB nuclear antigen (EBNA)-2-protein. Immunosuppression was drastically reduced, namely, CsA levels <100 ng/dL, prednisolone 5 mg, azathioprine withdrawn, and antiviral chemotherapy initiated with 10 days of IV gancyclovir and acyclovir followed by oral famcyclovir. During the follow-up, anti-EBV-IgM, anti-early antigen antibodies, and anti-EBNA antibodies were continuously monitored excluding significant EBV replication. Eighteen months post-liver resection, and high-resolution computed tomography scan demonstrated two paravertebral tumors. These lesions and a small nodule at the left ankle were resected revealing identical leiomyosarcomata. Immunosuppression was further reduced (CsA levels 75 ng/dL) and famcyclovir maintenance therapy started. Nevertheless, 2 years later the patient again developed tumor recurrence (perirectal, liver, and right adrenal gland); the tumors were surgically removed. The therapy was switched to Rapamycin and famcyclovir was continued. Three years after the last surgical intervention, the patient is well and recurrence-free. CONCLUSION: Long-term survival in patients with posttransplant EBV-associated leiomyosarcoma can be achieved by combined surgical intervention, reduction of immunosuppression, switch to Sirolimus, and antiviral chemotherapy.
We report what we believe to be the first case of high-grade, radiation-induced, intratesticular leiomyosarcoma in a 30-year-old man who had had testicular relapse of acute lymphoblastic leukemia at age 12 years that was treated with standard testicular field radiation (2400 cGy) and chemotherapy. Radiation-induced tumors of this type are rare, have a median latency of 10 years, and are usually dose dependent (around 5000 cGy). Testicular leiomyosarcoma, especially high grade, remains to be fully characterized. After radical orchiectomy, patients should be followed up with serial germ cell tumor markers and imaging to monitor for metastatic spread. The use of retroperitoneal lymph node dissection and chemotherapy remains controversial but is probably not indicated.
Leiomyosarcomas of vascular origin is a rare and aggressive pathology, its presentation on the renal vein is infrequent, of which 29 cases have been reported world-wide. We describe a case of a 76 year-old woman, who presented with intermittent left flank pain, irradiated to the lumbar region of 6 months duration. A left radical nephrectomy with en-bloc resection of a para-aortic tumor was performed. Microscopic examination of the specimen reported a moderately differentiated leiomyosarcoma, originated in the wall of the renal vein. The patient received adjuvant chemotherapy and radiation therapy. The tumor recurred in the liver. After 24 months from the primary surgery, the patient is alive with evidence of multiple metastatic spread to the liver. She is now under palliative care.
BACKGROUND: Leiomyosarcoma (LMS) of the uterus is a rare neoplasm with an aggressive growth pattern. Although the majority of uterine LMS is diagnosed with disease confined to the uterus, the rate of recurrent disease is high. The most common sites of recurrent disease are lung, liver, and peritoneal cavity. The rate of lymph node involvement with disease confined to the uterus is less than 3%. Metastases to brain and skull are rare. CASE: A 39-year-old woman underwent a total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic and paraaortic lymph node biopsies, omentectomy, and appendectomy for Grade 2 FIGO Stage 1 uterine leiomyosarcoma. She remained disease-free for 2 years until she presented with metastases to the sphenoid sinus. She underwent incomplete resection of the recurrence and was treated postoperatively with adjuvant MAID chemotherapy with poor response. She then underwent a second resection at an outside institution. Five months later, she was treated with radiation therapy to the base of the skull with no response. She expired from the disease 13 months after the diagnosis of the recurrence. CONCLUSION: The prognosis for patients who recur is dismal. The treatment options are limited. Surgical management should be considered as uterine LMS has a low response to chemotherapy and surgical resection of LMS to sites such as lung and abdomen has been suggested to offer a benefit. Radiation therapy may provide palliative benefit in the setting of metastatic disease.
INTRODUCTION: Primary malignant lesions of the vagina represent less than 2% of all gynecologic malignancies. Primary vaginal sarcomas account for about 2% of all malignant vaginal lesions, with leiomyosarcoma being the most common vaginal sarcoma found in adult women. CASE: We report a case of primary vaginal leiomyosarcoma occurring in the field of a prior tension-free vaginal tape (TVT) procedure using a Bard Duraderm allograft. CONCLUSION: This report represents the first report, to our knowledge, of a vaginal sarcoma arising in the field of a Bard Duraderm TVT allograft. Although the product is no longer available for this use, the ongoing studies of the TVT procedure and outcomes should include this potential complication in their review.
BACKGROUND: Epithelioid leiomyosarcomas arising from the uterine cervix are extremely rare neoplasms, with only three cases reported in the English literature. CASE: A 53-year-old Japanese woman was admitted to our university hospital due to massive vaginal bleeding from a cervical tumor. A total hysterectomy with bilateral salpingo-oophorectomy was performed. Histological findings, including immunohistochemical study using desmin, SMA, cytokeratin, S-100, HMB-45, vimentin, melan-A, and CD68, led to a diagnosis of epithelioid leiomyosarcoma of the uterine cervix. The patient underwent adjuvant chemotherapy and has been disease-free for over 20 months. CONCLUSION: Immunohistochemical studies may be needed to differentiate among the alternative diagnoses of malignant melanoma, metastatic carcinoma, and epithelioid sarcoma. Additional cases are needed to develop optimal management strategies and to predict prognosis.
INTRODUCTION: The leiomyomatous type of uterine sarcoma with osteoclast-type giant cell component is a rare variant of uterine tumors with poor prognosis. The histological diagnosis of these rare tumors can be problematic and only five such tumors have been published previously. CASE REPORT: A 54-year-old woman presented with fever and weight loss for 7 months and laboratory findings suggestive of inflammation. After extensive clinical investigation, a uterine tumor was found, which was considered to be an incidental finding and was thought to be unlikely to explain the symptoms. After hysterectomy, the patient had a surprising and quick recovery with the complete relief of systemic symptoms and normalization of laboratory changes. The tumor was a dedifferentiated leiomyosarcoma with osteoclast-like giant cells and contained extensive necrosis. The patient continues to do well and is tumor-free 1 year after the operation. DISCUSSION: To our knowledge, this is the first report of a patient being alive and disease-free 12 months after surgery with a dedifferentiated uterine leiomyosarcoma with osteoclast-like giant cells.
The pathogenesis of uterine leiomyosarcomas (LMS) is poorly understood. It is unknown if these tumors arise de-novo or from pre-existing leiomyomata (LM) or atypical leiomyomata. In this study, we evaluated morphologic heterogeneity within uterine LMS to identify possible precursor lesions. We reviewed 11 cases of total hysterectomy in which the final diagnosis was LMS. All slides from the grossly recognized tumor were evaluated for the degree of atypia and mitotic counts within the leiomyosarcomas. The slides with the lowest and highest mitotic count were stained with monoclonal antibody to p53, MIB-1 and ER/PR. The number of cells stained was subjectively assessed to nearest 5%, with 1% for rare positive cells. Morphologically benign tumor areas were identified in 5 of the 11 tumors. These areas showed <5 mitoses/10 HPF, with 1+ atypia in 4 cases and 1-2+ atypia in 1 case. No necrosis was seen by definition. Immunostains could be done in 4 of these 5 cases. These morphologically benign areas showed a p53 expression of 1% in each of the 4 cases, with low MIB-1 (5 to 15%) and high ER/PR expression (ER: 50-100%, PR: 10-100%). Morphologically malignant areas had 13 to 31 mitoses/10 HPF, 2+ to 3+ atypia, p53 expression of 70% to 100%, MIB-1 expression of 40% to 100%, ER expression of 1 to 100% and PR expression of 1 to 100%. The benign and malignant areas merged in all cases. Morphologic and immunohistochemical spectrum of changes from benign to malignant is seen in 50% of LMS. This raises the possibility of progression of some leiomyomata to LMS.
Leiomyosarcoma is a malignant tumour of smooth muscle cells. Usually is found in the gastrointestinal and female genital tracts, but is extremely rare in the larynx. Diagnosis is based in immunohistochemical studies. From the related literature is concluded that this tumour has a very aggressive behavior, with a very bad prognosis in spite of a radical surgery. A case of glottic Laryngeal Leiomyosarcoma (LL) treated with partial surgery is presented, There isn't any evidence of recurrent or metastases after a two years follow-up.
PURPOSE: To report a case of spermatic cord leiomyosarcoma metastatic to the orbit. DESIGN: Case report. METHODS: A 78-year-old man presented with progressive redness, proptosis, and decreasing vision of the left eye of 3 weeks' duration. Computed tomography revealed an intraconal mass (1.5 x 2 cm) inferior to and displacing the left optic nerve. RESULTS: A left lateral orbitotomy was performed, and a well-demarcated mass was removed from the surrounding tissue. A follow-up computed tomography of the chest and abdomen was consistent with metastatic disease to the lungs and liver. CONCLUSIONS: Spermatic cord leiomyosarcoma is a rare cause of metastatic lesions to the orbit.
We are using an approach that is based on the cre/loxP recombination process and involves a binary system of Cre-producing and Cre-responding transgenic mice to achieve ubiquitous or tissue-specific expression of oncoproteins. To develop mouse models of tumorigenesis, Cre-producers are mated with responder animals carrying a dormant oncogene targeted into the 3' untranslated region of the locus encoding cytoplasmic beta-actin (actin cassette). Production of oncoprotein from a bicistronic message is accomplished in bitransgenic progeny by Cre-mediated excision of a segment flanked by loxP sites that is located upstream from the oncogenic sequence. Widespread Cre-dependent activation and expression of an actin-cassette transgene encoding the T antigens of the SV40 early region (SVER) commencing in embryos was compatible with normal development and did not impair viability. However, at approximately 3 months of age, all female animals developed massive uterine leiomyosarcomas, whereas practically all males exhibited enormously enlarged seminal vesicles because of pronounced hyperplasia of the smooth muscle layers. In addition, because of smooth muscle hyperproliferation, marked dilation of the gallbladder was observed in mice of both sexes. To begin exploring aberrant signaling events in the SVER-triggered tumorigenic pathways, we analyzed the expression profile of leiomyosarcomas by DNA microarray analysis.
A case of Peutz-Jeghers syndrome with long history and multiple resections is presented. Despite the large number of intestinal polyps in these patients, malignant transformations are rare. In this case, an epithelioid leiomyosarcoma developed within a single polyp. At the time of resection this tumor had already metastasized to the liver. The histology and ultrastructure of this tumor is presented. Reviews of the clinical syndrome and leiomyosarcoma are given, with discussion of the possible interaction of these two processes as presented in this patient.
A 12-year-old female sarus crane (Grus antigone) developed a recurrent proliferative lesion in the subcutaneous tissue of the tarsometatarsus, which failed to respond to medical and surgical therapy. The crane was killed and microscopic examination of the tissues taken at necropsy revealed a myxomatous, poorly-differentiated sarcoma with metastasis to the liver and kidney. Immunohistochemical staining for muscle actin, smooth muscle myosin and vimentin were positive, indicating that the primary and metastatic tumours were leiomyosarcomas. Location of the metastatic lesions in only the portal venous system of the liver and veins of the kidney indicated that the route of metastasis was the portal circulation via the ischiatic vein, caudal mesenteric vein and both the renal-portal shunt and hepatic portal vein. This is the first report of metastatic subcutaneous leiomyosarcoma in an avian species.
Leiomyosarcomas of the vasculature are exceedingly rare tumors in childhood. The case of a 2-year-old girl who underwent three local excisions with two courses of adjuvant chemotherapy over 3 years for a leiomyosarcoma of a saphenous vein tributary is described. Follow-up over the following 9 years has shown no evidence of further recurrence or metastases suggesting that cure has been achieved. Treatment options are discussed and pertinent literature is reviewed.
A rare case of a newborn infant with leiomyosarcoma of the transverse colon is reported. The condition was associated with meconium peritonitis due to a perforation proximal to a portion of the transverse colon that was completely surrounded by the tumor. The 12 previously published cases of leiomyosarcoma of the colorectum in childhood are reviewed, and the pathogenesis of meconium peritonitis is discussed.