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Primary leiomyosarcoma of the seminal vesicle.

A case of leiomyosarcoma of the seminal vesicle detected as a prerectal mass on routine per-rectal examination is described in a 37-year-old man. Computed tomography scan confirmed it to be arising from the right seminal vesicle. Per-rectal trucut biopsy showed malignant cells. Radical cystoprostatectomy with bilateral pelvic lymphadenectomy with anterior resection of rectum and urinary diversion with ileal conduit was performed. Microscopic examination of the resected specimen showed moderately differentiated leiomyosarcoma from the seminal vesicle. The patient received adjuvant chemotherapy and sandwiched radiotherapy. He is well and free of tumor 20 months after surgery.

Adult↗

A rare case of leiomyosarcoma of the penis with a reappraisal of the literature.

Primary leiomyosarcomas arising in the penis are rare, with only 29 reported cases to date. We review the published records on this entity and describe an additional case of penile leiomyosarcoma occurring in a 53-year-old patient who underwent postectomy for a firm nodule in the prepuce. Four years later he experienced local recurrence which was successfully treated with partial penectomy.

Humans↗

Leiomyosarcoma of the duodenum: a report of three cases.

Three cases of leiomyosarcoma of the duodenum are reported in this paper. The first patient demonstrated the development of a leiomyosarcoma from a leiomyoma, and also posed diagnostic problems as a Pólya gastrectomy had previously been performed. The second patient presented with acute pancreatitis--a hitherto unreported complication of this condition. The third patient showed that even though the primary tumour was unresectable, the patient remained well for more than a year after diagnosis.

Acute Disease↗

Perforated leiomyosarcoma of Meckel's diverticulum.

A case is reported of perforated leiomyosarcoma of Meckel's diverticulum. The patient presented with localized peritonitis which responded to antibiotic therapy, and diagnosis was not made until elective resection of a tubulovillous adenoma of the rectum which was found in the course of investigation. Although rare, leiomyosarcoma is the commonest malignant tumour of Meckel's diverticulum.

Aged↗

Leiomyosarcoma of the parotid gland.

An unusual case of leiomyosarcoma of the parotid gland is reported. The tumour recurred thrice and metastasized to one of the adjacent lymph nodes. Histologically, a leiomyosarcoma in the salivary gland closely resembles a tumour of myoepithelial cells origin but lacks the dual cell morphology seen in the latter.

Adolescent↗

Gastric leiomyosarcomas: a general surgical experience.

Leiomyosarcomas of the stomach are rare malignancies. In Singapore, they account for only 0.7% of all gastric tumours. Nine cases of gastric leiomyosarcomas were treated in this department over a 20 year period from 1968 to 1987. Pre-operative diagnosis was difficult. All our cases' required laparotomy for histological diagnosis. Of the nine cases, there were eight gastrectomies (two repeated). None of the resected cases showed lymph node spread. There was only one long-term survival (8 years).

Aged↗

Leiomyosarcoma of the rectum and anal canal.

Leiomyosarcoma of the rectum and anal canal is rare and the optimal treatment is not clear. Eight patients with isolated anorectal leiomyosarcoma treated surgically were reviewed. The age ranged from 44 to 76 years (median 63 years) and the follow up ranged from 6 months to 4.5 years (median 2 years). All patients were symptomatic at presentation. All tumours involved the muscularis propria of the low and/or mid-rectum with three tumours also involving the anal sphincters. The tumour size ranged from 1.2 to 10 cm (median 4 cm). Mucosal involvement occurred in only three patients and there was no lymph node involvement. All showed microscopic infiltration at the advancing border, despite macroscopic circumscription. Only one patient was thought to have a tumour sufficiently small (3 cm) and localized on clinical and intrarectal ultrasound examinations (UST2N0) to be suitable for wide local excision. That patient remained tumour-free after 2 years. The remaining patients (88%) were treated by abdomino-perineal resection. The disease free interval in this latter group ranged from 3 months to 4.5 years. All recurrences were detected within 15 months of surgery and the mean interval from detection of recurrence to death was 9 months. Using a histological grading system that included tumour differentiation, mitotic count and amount of necrosis, high grade sarcomas were associated with a worse prognosis. Other factors associated with a poor outcome included large tumour size (> 6-7 cm) and prior incomplete local excision.

Adult↗

Leiomyosarcomas have a poorer prognosis than mixed mesodermal tumours when adjusting for known prognostic factors: the result of a retrospective study of 423 cases of uterine sarcoma.

OBJECTIVE: To determine the survival data for the various tumour types of uterine sarcoma and determine the influence of various prognostic factors on survival. DESIGN: Retrospective analysis of all uterine sarcoma cases registered in the 15 year period 1967-1981. SETTING: West Midlands Regional Cancer Registry, serving a catchment area of 2.6 million women. SUBJECTS: 423 women registered as having a uterine sarcoma; 367 of these were associated with the two main histological types, leiomyosarcomas (LMS) and mixed mesodermal tumours (MMT). MAIN OUTCOME MEASURES: Duration of survival was taken as the primary endpoint. RESULTS: The overall 5-year survival for uterine sarcomas in this series was 31%, with the major prognostic indicator being tumour stage. Survival for mixed mesodermal tumours is similar to other sarcomas despite a tendency towards less differentiation, wider dissemination and a greater age of the patient at diagnosis. Multivariate analysis shows that for cases with similar stage, age and grade, mixed mesodermal tumours have a better prognosis than leiomyosarcomas. CONCLUSIONS: These results demonstrate the danger of considering each variable in isolation when the relation between variables can lead to spurious significance or lack of significance because of the imbalances in the numbers between groups of prognostic importance. This study underlines the need for an adequate inspection of the intra-abdominal contents at the time of hysterectomy for uterine fibroids.

Adult↗

Uterine leiomyosarcoma--a Singapore experience.

Uterine leiomyosarcoma is an aggressive tumour. In our retrospective series of 27 patients, there were 25 with nonmyxoid high-grade leiomyosarcoma of the uterus. The stage distribution was Stage 1, 16; Stage 3, 5 and Stage 4, 4. In the patients with Stage 1 disease, 3 of the 8 patients who received adjuvant chemotherapy subsequently developed recurrent disease. In contrast, 6 of the 8 patients who did not receive adjuvant chemotherapy subsequently developed recurrent disease; 2 of the patients in the latter group also received adjuvant radiotherapy. Six of the 9 women with recurrences were distant 'failures' alone, 2 were both distant and pelvic 'failures' and 1 was pelvic 'failure' alone. All the patients with advanced-stage disease eventually succumbed to the disease despite the therapies given. This study is small and retrospective but it suggests that there might be a role for adjuvant chemotherapy in the management of the early stage of this disease.

Chemotherapy, Adjuvant↗

Management of uterine leiomyosarcoma in Australia.

Uterine leiomyosarcoma is an uncommon malignancy for which the management varies widely between individual gynaecologists and gynaecological oncology units. We have performed a retrospective review of patients treated at both the Royal Women's Hospital in Melbourne (1970-1997) and King George V Hospital in Sydney (1987-1993). In addition we have performed a survey of Certified Gynaecological Oncologists (CGO's) to assess the current management of uterine leiomyosarcomas in Australia. The results show varied management practices exist in Australia, many of which are not supported by evidence in the current literature. Oophorectomy in the premenopausal patient appears unnecessary unless the ovaries are macroscopically involved. The role of pelvic lymphadenectomy is debatable. This practice was recommended by many CGO's, yet these nodes are rarely positive unless obvious extrauterine disease is present. Adjuvant chemotherapy appears not to have a role at present unless in a trial setting. Adjuvant radiotherapy does appear to have a potential palliative role as it prevents locoregional relapse, although survival is not prolonged. Until suitable phase 3 trials are available, gynaecological oncology units should be meticulous in prospectively recording the clinical course of their patients and critically analyzing their current management strategies.

Adolescent↗

Leiomyosarcoma of the skin. Treatment of 34 cases.

BACKGROUND: Leiomyosarcoma is a rare soft tissue sarcoma that is one of the least common malignant lesions found in the skin. OBJECTIVE: We discuss the clinical, histopathologic, and prognostic factors related to superficial leiomyosarcoma from cases reported in the literature as well as from the Mayo Clinic experience from 1964 through 1994 with 34 patients. CONCLUSIONS: The vast majority of tumors were treated by excisional surgery with wide margins, but conceptually, Mohs' micrographic surgery should prove useful for the treatment of this tumor as it has with other skin cancers displaying contiguous growth.

Actins↗

Superficial leiomyosarcoma treated with Mohs micrographic surgery.

BACKGROUND: Leiomyosarcoma (LMS) is a rare cutaneous malignancy with an infiltrative growth pattern and high risk of local recurrence (30-45%) with conventional wide excision. Mohs micrographic surgery (MMS) may offer an improved cure. OBJECTIVE: To illustrate by case reports the spectrum of clinical presentation of this rare neoplasm and management with MMS. METHODS: The outcomes of three cases of LMS treated by Mohs micrographic are described and compared with other cases in the medical literature. RESULTS: Three cases of LMS were excised using MMS. Two cutaneous tumors located on the trunk and arm were easily removed and have not recurred. The third tumor on the leg of an immunosuppressed patient demonstrated deep soft-tissue invasion that developed subsequent cutaneous metastases but no recurrence adjacent to the primary site. The patient remains disease free at 36 months after amputation below the knee. A total of 15 cases of LMS treated by MMS are reported in the literature, including these cases, with an aggregate recurrence rate of 13%. CONCLUSIONS: The overall cure rate of leiomyosarcoma treated by MMS is 87%, which compares favorably to wide excision and offers the advantage of tissue sparing. Immunosuppression may promote aggressive tumor behavior. Deeply invasive tumors may result in cutaneous or distant metastases regardless of the method of excision.

Female↗

Leiomyosarcoma of the vagina: case report and literature review.

Leiomyosarcoma of the vagina is quite rare. Extensive search of the literature discloses 137 case reports published over the past 40 years. Collectively, these reports give only cursory guidelines for diagnosis, management, and prognosis. We add another case to the sparse literature on leiomyosarcoma of the vagina. Treatment consisted of surgical excision. Tumor was found to extend to the margin of the excised specimen. The malignancy quickly recurred with lung metastases and the patient died. The very poor prognosis associated with this lesion emphasizes the critical need for early diagnosis and expeditious therapy. In the interest of improving salvage by prompt recognition and intervention, it is important for health-care professionals to be knowledgeable about and alert to this condition.

Adult↗

Cutaneous leiomyosarcoma in a child.

Superficial leiomyosarcomas are infrequent tumors that are very rare in childhood. We report on a 12-year-old white boy with a cutaneous leiomyosarcoma of his left groin. Immunohistochemical study revealed positive immunostaining with antibodies to vimentin, desmin, and smooth muscle actin. The tumor was removed with wide surgical margins.

Child↗

Neuron-specific enolase-producing leiomyosarcoma of the mesentery.

A case of neuron-specific enolase (NSE)-producing leiomyosarcoma arising in the mesentery of a 62-year-old female is presented. Preoperatively, serum level of NSE was markedly elevated. A well-defined but unencapsulated tumor measuring 14 x 12 x 9 cm was histologically characterized by a fascicular or herring-bone arrangement of atypical spindle or oval tumor cells with vesicular cigar-shaped nuclei, inconspicuous nucleoli and abundant clear to brightly eosinophilic cytoplasm. Immunohistochemically, the tumor was positive for vimentin, alpha-smooth muscle actin, muscle actin, desmin, and gamma-NSE. Postoperatively, serum level of NSE decreased to within normal range. The NSE levels were, however, elevated again as metastatic lesions in the liver had enlarged. The patient died of the liver metastases 10 months after surgery. Serum NSE level can be a useful index of tumor extent and for monitoring treatment of some patients with leiomyosarcoma.

Fatal Outcome↗

Leiomyosarcoma of the dartos muscle.

Smooth muscle is seen in the skin in three locations, the arrectores pilorum muscles, the wall of vessels and the specialized muscles in genital skin (the dartos, vulvar, and mamillary muscles of the scrotum, labia majora, and the breast, respectively). We report a case of a leiomyosarcoma of the dartos muscle in a 57-year-old man. The histological features of this tumor are similar to the previously reported cases of leiomyosarcomas of the skin thought to originate in arrectores pilorum muscles and the wall of vessels.

Genital Neoplasms, Male↗

Intestinal leiomyosarcoma in a cat.

Intramural ileocaecocolic leiomyosarcoma is described in an elderly neutered male domestic shorthaired cat with poor appetite and weight loss. Histological examination of the resected lesion revealed a poorly differentiated soft tissue sarcoma and a diagnosis of leiomyosarcoma was confirmed by positive immunohistochemical staining for vimentin and a-smooth muscle actin. Postoperative survival time was 102 days before local recurrence justified euthanasia.

Animals↗

Diagnosis and treatment of primary intraoral leiomyosarcomas in four dogs.

This paper describes the clinical and pathological features, diagnosis and management of primary intraoral leiomyosarcomas in the oral cavity of four dogs; they involved the gingivae of the rostral maxilla and mandible, the mucosa of the upper lip and the soft palate. Their features are compared with the those of leiomyosarcomas commonly encountered in the gastrointestinal tract of dogs. Two of the dogs responded well to surgical treatment; one died after its airways were obstructed by a large palatal tumour; and the fourth was lost to follow-up after its owners declined treatment.

Animals↗