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Because renal leiomyosarcoma is extremely rare, the experience in the management of this tumor type is necessarily scant. Our surgical and chemotherapeutic approach in a 17-year-old male patient with renal leiomyosarcoma achieved partial remission of tumor recurrence and a survival of 31 months following diagnosis.
A case of primary cardiac leiomyosarcoma diagnosed at Iaşi Cardiology Center is presented. The study was made on the surgical biopsy specimen, and the diagnosis by routine morphological techniques. The gross examination revealed a large intracavitary left atrial tumor attached by a small sessile base to the left posterior atrial wall, having a smooth, white-gray surface, and a dense consistency. On the cut surface, the tumor had a whirled white appearance, with focal brown areas. The microscopic examination revealed the presence of a spindle cell tumor, forming fascicles orientated at right angles. The study revealed the morphological aspect characteristic to leiomyosarcoma.
A 70-year-old woman with leiomyosarcoma, followed up for 5 years as tuberculoma, is described. The tumor, in the left lower lobe, had enlarged from 1.5 x 1.5 cm to 3.4 x 3.4 cm in 5 years, and doubling time was calculated to be 540 days. Another tumor (1.8 x 1.8 cm) was also recognized in the right lower lobe at the later time. Since not only a benign tumor but metastatic disease was suspected, thoracotomy was performed, and the intraoperative pathologic diagnosis was leiomyosarcoma. However, only partial lung resection was performed because of the low growth rate of the tumor, the patient's advanced age, limited lymphatic metastasis and favorable prognosis with only partial resection. DNA analysis of the tumor based on flow cytometry showed a diploid pattern, and this was thought to account for the long doubling time of the tumor. The postoperative course was satisfactory without any signs of recurrence at the operation site.
A 46-year-old female patient suffered from uterine malignant leiomyosarcoma. Ten months after abdominal subtotal hysterectomy, multiple lung, bone and spine metastases developed. She received 3 cycles of infusional ADI chemotherapy (adriamycin 60 mg/m2, 96-hour i.v. infusion, dacarbazine 500 mg/m2, 96-hour i.v. infusion and ifosfamide 5,000 mg/m2, 72-hour i.v. infusion, repeated every 3 weeks), but the metastatic tumors progressed. An empirical ADI-TAM chemotherapy which incorporated high-dose tamoxifen (TAM) (150 mg/m2/day, in 4 divided doses) into the original ADI regimen was applied from the fourth cycle. High-dose tamoxifen consisted of 240 mg/day for 4 days during chemotherapy and 40 mg/day between cycles. Good partial response with nearly total tumor reduction was achieved after 3 cycles of ADI-TAM therapy. The excellent tumor response by simple addition of high-dose tamoxifen into a previously inactive ADI regimen advocates further investigation for the development of chemotherapy in metastatic uterine leiomyosarcoma.
Leiomyosarcoma is a malignant smooth-muscle tumor that has a predilection for the gastrointestinal tract and the female genital tract. It is locally fast-spreading and highly aggressive, and the prognosis is poor. We report two cases of leiomyosarcoma of the maxilla in patients who sought treatment for maxillary swelling, nasal obstruction, and epistaxis; one patient also had orbital involvement and cervical lymph node metastasis. Both patients underwent radical surgery followed by chemo- and/or radiotherapy. The patient with orbital involvement died shortly thereafter, but the other was disease-free at 18 months.
Twenty eight leiomyomas and one leiomyosarcoma were cytogenetically analysed and also examined for ras and myc oncoprotein expression. Chromosome alterations were found in seven leiomyoma cases. In four of them 12q14-15 was involved. P21 product of H-ras and P62 product of c-myc genes were detected in paraffin embedded parallel tissue sections. A high expression of H-ras was apparent in most tumors. C-myc expression was weak or negative in most leiomyomas with normal karyotype while on the contrary in three out of seven cases with abnormal cytogenetic findings the gene product stained moderately positive. Considerable chromosome abnormalities and oncogene overexpression were also identified in the leiomyosarcoma.
Bladder leiomyosarcoma is an uncommon tumour. Early diagnosis and aggressive surgical treatment are necessary to ensure patient survival. The authors report a case of bladder leiomyosarcoma treated by radical cystoprostatectomy and enterocystoplasty with a favourable outcome at 32 months. The author analyse the clinical and histological features and the treatment of this rare tumour.
The Authors report a rare case of retroperitoneal leiomyosarcoma in a 80 years woman with respiratory symptoms and right abdominal pain. A large neoplasm occupied the right abdomen, looking asymmetric. Upper abdominal CT showed a retroperitoneal neoplasm close to right kidney, liver, aponeurosis of right oblique muscles, producing a left-side dislocation of the intraabdominal organs. A surgical "en bloc" resection of the neoplasm was performed; neoplasm was plurilobed and capsulated. The histologic examination confirmed the diagnosis of leiomyosarcoma. The best treatment of these neoplasms is surgery, that in the last years has drawn advantage from more sensible and specific diagnostic procedures, which show a more radical surgical option. Though metastases are occasional, local recurrences can be taken into consideration and, after a careful tumoral re-staging, they can be resected once more. Therefore, a careful follow-up is necessary on the basis of neoplastic grading, extension and involvement of the closer structures.
OBJECTIVES: To present one case of paratesticular leiomyosarcoma, a rare tumor which has been reported in 5 cases only in our country. To review the management of this type of tumor. METHODS: A 73-year-old male presenting with a 4 cm mass adjacent to the right testicle underwent excision of the mass, spermatic cord and testicle through an inguinal approach. RESULTS: The tumor was classified as a grade 3 leiomyosarcoma, with positive immunohistochemical staining for desmin and smooth muscle actin. After this pathology report we did not consider adjuvant oncological treatment. Tumor relapse has not appeared so far. CONCLUSIONS: Radical surgery is the only feasible and successful treatment for paratesticular sarcomas, with the exception of rabdomyosarcoma, because they are radio and chemoresistant. The inguinal approach is the ideal and obligatory one, including excision of the spermatic cord and adjacent testicle. Long-term periodic follow-up visits are mandatory because relapses may occur even long time after diagnosis. In case of relapse, surgery is again the only tool we have to control this type of tumor. Currently radiotherapy is recommended in cases of local recurrence after surgery. 5-year survival rates are around 75%, and 10-year 50%.
Leiomyosarcomas are rare in the head and neck region. The treatment of choice is surgical resection. We present a case of leiomyosarcoma in a 50-year-old female patient, arising from the inferior turbinate. The tumor was resected by a transnasal microendoscopic approach and no evidence for tumor recurrence was found during an endoscopic and radiologic follow-up of two years. Cytogenetic analysis by means of comparative genomic hybridization revealed chromosomal gains at 4p13p15, 6p21p22, 7q22qter, 9q22qter and chromosomal losses at 10q22qter and 19p12p13.1. With augmented experience, the transnasal approach may be appropriate to resect circumscribed malignant tumors located in the nasal cavity.
Scrotal leiomyosarcoma is a rare tumour arising from the dartos layer: We describe a case of scrotal leiomyosarcoma in a 40-year-old man. The patient was treated by a wide surgical excision and no recurrence has been recognized 36 months later. A review of the literature is presented, summarizing the principal clinical and morphological characteristics of this rare tumor.
Primary ovarian leiomyosarcomas are extremely rare tumors that comprise less than 0.1% of all ovarian malignancies. We present a case of 62-year-old postmenopausal woman with a slightly enlarged right ovary and a Color Doppler sonography resistance index (RI) measuring 0.54. The patient, after being managed with surgery alone, is alive after 14 months without any evidence of disease. This is an unusual case in that primary ovarian leiomyosarcoma was diagnosed in the setting of a slightly enlarged irregular postmenopausal ovary with a concomitant intermediate RI value on color flow Doppler evaluation. A high index of suspicion may help prevent delay in the diagnosis of this rare neoplasm.
Until recently, leiomyosarcoma of the inferior vena cava was rarely diagnosed preoperatively. The authors describe a patient who was treated for 12 months on the basis of a presumptive diagnosis of thrombosis of the inferior vena cava. Abdominal ultrasonography and computed tomography demonstrated interval growth, and venous biopsy revealed a malignant spindle-cell neoplasm. Surgical excision confirmed the presence of a leiomyosarcoma, which had grown slowly for at least several months before entering a phase of accelerated growth.
Soft-tissue masses uncommonly completely occlude or engulf a major vessel. Complete occlusion of a major vein suggests that the mass originates in the wall of the vein. Such tumours are rare, the most frequently encountered type being leiomyosarcoma. The authors present a case of leiomyosarcoma of the femoral vein.
Gastric leiomyosarcoma was diagnosed in a 12-year-old Thoroughbred gelding. The horse was examined because of anorexia, weight loss, and intermittent fever of 1.5 months' duration. Antemortem diagnostic testing was extensive, but a definitive diagnosis could not be reached with noninvasive methods. Exploratory celiotomy confirmed an intra-abdominal mass, and the horse was euthanatized. Necropsy revealed a firm mass associated with the distal portion of the esophagus, cranial two thirds of the stomach, and visceral surface of the liver. The histopathologic diagnosis was leiomyosarcoma.
One case of pulmonary metastasis of leiomyosarcoma responsive to CTP therapy was reported. The patient was a 44-year-old woman who had undergone total simple hysterectomy for uterine myoma 6 years before. Five years after the operation she underwent ovariectomy for ovarian tumor. Based on the histopathological findings, she was diagnosed to have leiomyosarcoma. One year later the patient was referred to use because of pulmonary metastasis. After admission to our hospital CTP therapy was started. Disappearance of the metastatic lesion was demonstrated by the chest X-ray findings at the end of five courses of treatment.
Leiomyosarcomas arising from the wall of the blood vessels, are highly malignant tumours. Although 75% of the cases have been reported in the inferior vena cava, nevertheless scanty mention is made in the literature of an analogous tumor originating from smaller blood vessels. This paper deals with a 53-year-old man with a tumor in the right axilla. Surgery consisted in wide local excision. Histopathology revealed a mesenchymal tumor in an axillary blood vessel. Electron microscopy revealed tumor cell with all the characteristics of a leiomyosarcoma. The patient refused adjuvant therapy and died 10 months later with local recurrence and lung metastases.
Leiomyosarcoma of the rectum is a rare entity. Approximately 150 cases have been described in the literature. Differentiation from its benign counterpart, leiomyoma, and other connective-tissue tumors is often difficult, but it is important because each tumor has an entirely different prognosis. The case of a patient in whom an 11 x 5.5 cm leiomyosarcoma of the rectum was surgically excised by abdominoperineal resection is presented. Literature review shows disagreement over the therapeutic approach, most likely due to the lack of a large series of patients with this disease. At present, a selective treatment approach appears to be the most advocated. Lesions less than 2.5 cm in size and limited to the bowel wall can still be treated by wide local excision. More radical surgical resection is indicated for larger tumors and those extending outside the bowel wall.