BACKGROUND: Leiomyosarcoma (LMS) is a rare neoplasm that arises from tissues of embryonic mesodermal origin. Primary tissues of origin can include smooth muscle cells of the abdominopelvic viscera, blood vessels, or arrector pili muscles. LMS is known to metastasize to the lungs, with few reported cases of spread to the central nervous system.
METHODS: A 66-year-old male with cutaneous LMS of the left forearm with metastases to the lungs and kidney that had been treated with chemoradiation presented with worsening headaches. Magnetic resonance imaging revealed a sellar lesion. An endocrine workup was unremarkable. Imaging over 6 months revealed rapid interval growth. Positron emission tomography demonstrated moderate uptake. Given the rapid growth, the patient was offered an endoscopic endonasal approach for resection. Pathology confirmed LMS.
CONCLUSIONS: To the authors\' knowledge, this is the first documented case of LMS metastasis to the sella. Pituitary carcinoma or metastases to the sellar region should be in the differential among patients with sellar region tumors with a rapid growth rate, bony erosion, or findings of lesions in the upper cervical lymph nodes or soft tissue. Tumors that show significant interval growth should raise suspicion for nonadenomatous lesions, and surgical intervention should be considered even in the absence of endocrinological dysfunction or cranial neuropathies. https://thejns.org/doi/10.3171/CASE2435.

Primary hepatic leiomyosarcoma (PHL) is a rare malignant tumor, which originates from smooth muscles. The imaging features are nonspecific and the diagnosis is often delayed until the tumor reaches a large size, which leads often to a dismal prognosis. We report a case of a 46-year-old male patient who was complaining about abdominal pain for 2 months. The imaging revealed the presence of a large mass in the liver with adrenal and liver metastasis. Diagnosis of PHL was confirmed by histopathological and immunohistochemical examinations. In this case report, we review the epidemiological, clinical, and paraclinical aspects of the disease, as well as the treatment modalities.

Renal leiomyosarcoma metastasis to the pancreas is exceptionally rare. Here, we present a case of metastatic recurrence in the pancreas seven years after renal leiomyosarcoma resection. A 73-year-old female with a history of renal leiomyosarcoma surgery seven years prior presented with a well-defined 40 × 30 mm pancreatic tail tumor detected by a computed tomography (CT) scan. The tumor exhibited hypo-enhancement in the arterial phase and a progressive enhancement pattern toward the equilibrium phase, similar to pancreatic cancer. Endoscopic ultrasound-guided fine-needle biopsy (EUS-FNB) revealed bundles of spindle cells that matched those in the previously resected renal sample. Immunohistochemistry showed positive staining for desmin, confirming the diagnosis of pancreatic metastasis from renal leiomyosarcoma. The patient underwent a distal pancreatectomy to remove the metastatic lesion. The extended interval of seven years before the detection of metastasis underscores the challenges in monitoring and diagnosing metastatic patterns of renal leiomyosarcoma. EUS-FNB can assist in distinguishing metastatic pancreatic leiomyosarcoma from primary pancreatic cancer, thus influencing treatment decisions.

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BACKGROUND: The addition of trabectedin to doxorubicin, followed by trabectedin maintenance, may have superior efficacy to doxorubicin alone as first-line treatment in patients with advanced leiomyosarcoma.
METHODS: We conducted a phase 3 trial involving patients with metastatic or unresectable leiomyosarcoma who had not received chemotherapy previously. Patients were randomly assigned to receive either single-agent doxorubicin (six cycles) or doxorubicin plus trabectedin (six cycles), with continued trabectedin as maintenance therapy in patients in the doxorubicin-trabectedin group who did not have disease progression. Surgery to resect residual disease was allowed in each group after six cycles of therapy. Analyses of progression-free survival (primary end point) and overall survival (secondary end point) were adjusted for two stratification factors: tumor origin site (uterine vs. soft tissue) and disease stage (locally advanced vs. metastatic). The primary end-point results were reported previously.
RESULTS: A total of 150 patients underwent randomization. At a median follow-up of 55 months (interquartile range, 49 to 63), a total of 107 patients had died (47 in the doxorubicin-trabectedin group and 60 in the doxorubicin group). The median overall survival was longer in the doxorubicin-trabectedin group (33 months; 95% confidence interval [CI], 26 to 48) than in the doxorubicin group (24 months; 95% CI, 19 to 31); the adjusted hazard ratio for death was 0.65 (95% CI, 0.44 to 0.95). In a finding consistent with earlier reports, progression-free survival was longer in the doxorubicin-trabectedin group (12 months; 95% CI, 10 to 16) than in the doxorubicin group (6 months; 95% CI, 4 to 7); the adjusted hazard ratio for progression or death was 0.37 (95% CI, 0.26 to 0.53). The incidence of adverse events and the percentage of patients with dose reductions were higher with doxorubicin plus trabectedin than with doxorubicin alone.
CONCLUSIONS: Combination therapy with doxorubicin and trabectedin induction, followed by trabectedin maintenance, was associated with improved overall survival and progression-free survival, as compared with doxorubicin alone, among patients with metastatic or surgically unresectable uterine or soft-tissue leiomyosarcoma. (Funded by PharmaMar and others; LMS04 ClinicalTrials.gov number, NCT02997358.).

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Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome is an autosomal-dominant disorder that results from a germline pathogenic variant in the fumarate hydratase (FH) gene on chromosome 1, characterised by renal cell carcinoma (RCC), cutaneous leiomyoma and uterine leiomyoma. Leiomyosarcomas are reported in less than 1% of those with HLRCC. We report a case of a man in his 30s who had a long-standing plaque excised from the left upper arm after undergoing a radical nephrectomy for a fumarate-deficient RCC, with histological exam revealing a grade 1 leiomyosarcoma. Genetic testing confirmed a heterozygous pathogenic variant in the FH gene. This is a rare case of leiomyosarcoma associated with HLRCC, and our patient remains under surveillance with interval abdominal imaging and skin examination. Leiomyosarcomas are difficult to distinguish clinically from their benign counterpart; therefore, histopathological examination is paramount with a low threshold for excision.

Leiomyosarcoma (LMS) is an extremely rare malignant pathology affecting smooth muscle cells, with the uterus being the predominant location of LMS. Its occurrence in the duodenum is rare, making it a diagnostic challenge for radiologists. Patients with duodenal LMS can present with very vague symptoms such as abdominal discomfort, loss of weight, or manifestations associated with internal gastrointestinal bleeding. In this case report, we have an 82-year-old female presenting with duodenal LMS, which is a very atypical location. An esophagogastroduodenoscopy and further workup revealed a duodenal mass, which was biopsied. The lump was identified as an LMS using immunohistochemistry and histopathology. Despite its rarity, it presents diagnostic and therapeutic challenges due to its nonspecific clinical manifestations and radiological findings. By exploring the existing literature and clinical insights, we aim to provide a comprehensive understanding of this rare condition, highlighting the need for interdisciplinary collaboration and tailored therapeutic strategies to diagnose and manage this disease entity effectively.

UNASSIGNED: Primary ureteral neoplasms are extremely rare in dogs, and ureteral involvement usually occurs owing to the invasion of renal and bladder tumors.
UNASSIGNED: This case report describes a 12-year-old intact male mixed-breed dog referred to a private clinic with a six-month history of abdominal distention. A physical examination revealed mild abdominal pain. Hematological tests detected normocytic-normochromic anemia (hematocrit 33.6% [reference interval-RI: 37%-55%], red blood cells 4.93 M/µl [RI: 5.5-8.5 M/µl], and hemoglobin 12.4 g/dl [RI: 12-18.0 g/dl]). The results from the leukogram, thrombogram, renal, and hepatic panels were within the reference intervals for dogs. Abdominal ultrasonography revealed a cavitary mass measuring approximately 12 cm in diameter as the largest tumor in the left abdominal region over the left hepatic lobe or mesenteric site. Chest radiography did not reveal any metastasis. Therefore, the patient underwent exploratory laparotomy, during which the left ureter was found to be affected by a 12-cm mass that adhered to the left kidney. A unilateral left ureteronephrectomy was performed, and histology and immunohistochemistry (IHC) confirmed well-differentiated primary ureteral leiomyosarcoma. The patient survived for 130 days but died of lung metastasis.
UNASSIGNED: Ureteral leiomyosarcoma should be investigated and included in the list of differential diagnoses for primary ureteral neoplasms. Regardless of the therapeutic modality, the prognosis of ureteral leiomyosarcoma may be unfavorable, as shown in this report.

UNASSIGNED: LMS of IVC needs a multidisciplinary approach. Surgical excision with free margin is the cornerstone of management. Upon case-by-case selection, adjuvant chemotherapy may play a role in better oncologic outcome.
UNASSIGNED: Leiomyosarcoma (LMS) of the inferior vena cava (IVC) is a rare form of mesenchymal origin malignancy with less than 400 cases reported to date. Surgery is the mainstay of management but it requires vast experience in vascular and visceral surgery to attain a free tumor margin. Subsequent adjuvant treatment with chemotherapy and radiation remains as an area of gray zone. We report the case of a 61-year-old man with an 8-month history of abdominal pain. Upon physical examination, an ill-defined mass over the right side of the lower abdomen and bilateral lower extremity edema were detected. Abdominal ultrasound with Doppler revealed a right-side retroperitoneal mass invading the IVC with extensive venous thrombosis for which anticoagulation was initiated. Computed Tomography of the abdomen revealed a huge heterogeneously enhancing mass involving the whole length of the infrarenal IVC obstructing the IVC lumen with collateral veins draining through the paralumbar veins and inferior epigastric veins bilaterally. With a top differential of primary IVC LMS, a midline longitudinal laparotomy was performed with an intraoperative finding of a tumor arising from the infra-renal IVC which was excised. Gore-Tex graft was used to reconstruct the IVC. There was an injury to the right common iliac artery and it was repaired by end-to-end anastomosis. Histopathology confirmed a high-grade LMS of the IVC and surgical margin status was unknown. He was given adjuvant Chemotherapy consisting of Doxorubicin and Dacarbazine. He has been on follow-up at the Oncology side with a good performance status.