Surgical management of urethral obstruction secondary to perineal liposarcoma in a dog: a case report.

BACKGROUND: Swelling of the perineal region in male dogs is most commonly caused by a perineal hernia. Clinical signs associated with perineal hernia are constipation, tenesmus or stranguria. This case report documents a rare cause of perineal swelling created by the growth of a malignant tumour leading to urethral obstruction and subsequent stranguria. CASE PRESENTATION: An 11-year-old neutered male German Shepherd was presented for swelling in the perineal region and stranguria for three days. Complete blood count and serum biochemistry were unremarkable. Ultrasound revealed a heterogeneous mass in the perineal region. Retrograde urethrography showed a severe narrowing of the urethra caudal to the pelvis. A fine-needle aspirate of the mass was highly suspicious for liposarcoma. Staging was performed by computed tomography (CT) of the thorax and abdomen. Total penile amputation in combination with pubic-ischial pelvic osteotomy, transposition of the remaining urethra through the inguinal canal, V-Y-plasty cranial to the prepuce and preputial urethrostomy were performed to remove the tumour. Histopathology confirmed a well-differentiated liposarcoma with complete histological margins. Six months after the surgery the dog was doing well and there were no signs indicating local tumour recurrence. CONCLUSIONS: Wide surgical excision is generally recommended for soft tissue sarcomas, however this is sometimes not feasible for large tumours. In the case reported here, tumour resection was achieved by a combination of several surgical techniques with a good clinical outcome.

Acquired isolated factor VII deficiency in a patient with myxoid pleomorphic liposarcoma, case report.

INTRODUCTION: Acquired factor VII (FVII) deficiency is a rare condition with various causes, including acquired inhibitors to FVII, liver disease, and malignancies. Myxoid pleomorphic liposarcoma is a rare and aggressive form of soft tissue sarcoma that can cause a range of clinical manifestations, including bleeding and clotting disorders. PATIENT CONCERNS AND DIAGNOSIS: We present a case report of a 21-year-old man with severe acquired FVII deficiency due to mediastinal myxoid pleomorphic liposarcoma. The patient presented with elevated International normalized ratio (INR) and a severe reduction in FVII coagulant activity, unresponsive to conventional therapy. While an acquired inhibitor to FVII was initially suspected, negative results from laboratory testing, including protein G sepharose adsorption and a Bethesda assay using Immunoglobulin G purified from patient plasma, made the diagnosis of an acquired inhibitor to FVII uncertain. INTERVENTIONS AND OUTCOME: The patient underwent surgical resection of the tumor, supported by recombinant FVII infusion, leading to the normalization of coagulation parameters. However, a relapse of the disease was detected 6 months later when he was noted to have a decline in FVII levels. CONCLUSION: This case highlights the importance of considering rare causes of bleeding and clotting disorders, particularly in unresponsive or atypical presentations. It also underscores the need for close monitoring and follow-up in patients with acquired FVII deficiency, even after successful treatment.

Sarcopenia is associated with worse surgical complications but not relapse-free survival and overall survival in patients with retroperitoneal liposarcoma.

OBJECTIVE: This investigation aimed to explore the relationship between sarcopenia and severe postoperative complications, relapse-free survival (RFS), and overall survival (OS) in patients with retroperitoneal liposarcoma (RLPS). MATERIAL AND METHODS: This retrospective study included 72 RLPS patients (47 men, 25 women; mean age, 57.49 years, SD 10.92) who had abdominal CT exams. Clinical information was recorded, including RLPS characteristics (histologic subtypes, grade, size), laboratory assessment (ALB, PALB, A/G, Hb, SCr), relapse-free survival, overall survival, and postoperative complications. The relationships between those variables and RFS and OS were analyzed using Cox proportional hazard models. RESULTS: There were 8 severe postoperative complications (Clavien-Dindo grade > 2). The chi-square test showed sarcopenia was associated with severe postoperative complications (P = 0.011). In multivariate analysis, sarcopenia was not associated with relapse-free survival (P = 0.574) and overall survival (P = 0.578). CONCLUSIONS: Sarcopenia predicts worse surgical complications but does not affect relapse-free survival and overall survival.

Glutamic acid decarboxylase (GAD) antibody-positive paraneoplastic stiff person syndrome associated with mediastinal liposarcoma.

Stiff person syndrome (SPS) is a rare, debilitating neurological illness characterised by rigidity and spasms of the axial muscles, causing severe restrictions to mobility. SPS can be classic, partial or paraneoplastic. We report a case of a young woman who presented with seizures and painful spasms of the thoracolumbar muscles who was subsequently diagnosed with SPS. Serological work revealed glutamic acid decarboxylase (GAD) antibodies and imaging showed a large mediastinal mass. The patient underwent surgical resection of the mediastinal mass and final pathology revealed well-differentiated mediastinal liposarcoma. She received five sessions of plasma exchange and her neurological symptoms gradually improved after surgery. This case highlights a rare case of GAD antibody-positive paraneoplastic SPS associated with mediastinal liposarcoma.

A Liposarcoma Arising in a Pulmonary Hamartoma, Coexisting With Benign Metastasizing Leiomyoma.

Pulmonary hamartoma is a common benign tumor that rarely degenerates into malignancy. This report documents a unique case of pulmonary hamartoma with malignant transformation into well-differentiated liposarcoma, coexisting in proximity to pulmonary nodules representing benign metastasizing leiomyoma in a 60-year-old woman.

Successful Resection of a Primary Dedifferentiated Tracheal Liposarcoma Causing Tracheal Stenosis.

A 68-year-old woman was admitted with a persistent cough and dyspnea that had persisted for 4 months prior. Chest computed tomography revealed a tumor protruding from the membranous portion of the trachea. She underwent tumor resection via rigid and flexible bronchoscopy to relieve the symptoms and obtain a diagnosis. After the procedure, she was diagnosed with tracheal liposarcoma. Three months after the procedure, she underwent complete surgical tumor resection. Liposarcoma is a mesenchymal tumor that usually develops in the extremities and the retroperitoneum. Tracheal liposarcoma is extremely rare. To the best of our knowledge, this is only the second reported case.

Atrapamiento mesentérico como manifestación inicial de metástasis de carcinoma sarcomatoide pleomórfico de pulmón / Mesenteric involvement as the initial manifestation of pleomorphic sarcomatoid lung carcinoma metastasis

Mujer de 69 años no fumadora, que acude a urgencias por dolor abdominal, intolerancia oral y síndrome constitucional. Se solicita TAC identificando adenopatías axilares, mediastínicas y mesentéricas, derrame pleural bilateral, obstrucción ureteral derecha y lesión ósea blástica en D12 compatible con metástasis, sin objetivar tumor primario. La evolución clínica fue desfavorable, precisando nuevo TAC, observándose cambio de calibre en íleon terminal sin visualizar causa obstructiva. Se decide intervención quirúrgica, objetivándose dilatación intestinal, engrosamiento mesentérico, con obstrucción ileal adyacente a válvula íleocecal, secundaria a tumoración mesentérica retroperitoneal, realizándose hemicolectomía derecha. El diagnóstico anatomopatológico fue de metástasis de carcinoma pulmonar tipo sarcomatoide en su variante pleomórfica, hallazgo excepcional por lo infrecuente de la diseminación mesentérica de estos tumores. Había afectación de 15 ganglios mesentéricos, positividad en la citología del derrame pleural y de ganglios axilares. La paciente presentaba una diseminación peritoneal. Dado el mal pronóstico, se decidió quimioterapia paliativa (Carboplatino-Paclitaxel) (AU)

A 69 year-old women, non-smoker, who came to the emergency for abdominal pain, oral intolerance and constitutional syndrome. A CT scan identified axillary, mediastinal and mesenteric lymphadenopathy, bilateral pleural effusion, right ureteral obstruction and blast bone metastasis lesion in D12, without observing a primary tumor. The clinical course was unfavorable, requiring new CT scan, where a change in caliber in terminal ileum was observed without an obstructive cause. Surgical intervention was proposed, intestinal dilation and mesenteric thickening were found, with ileal obstruction adjacent to the ileocecal valve, secondary to a retroperitoneal mesenteric tumor, a right hemicolectomy was performed. The pathological diagnosis was pleomorphic sarcomatoid lung carcinoma metastasis. This finding is exceptional due to the infrequency of the mesenteric dissemination of these tumors. There were involvement of 15 mesenteric lymph nodes, positive cytology of the pleural effusion and axillary lymph nodes. Due to the bad prognosis, palliative chemotherapy (Carboplatin-Paclitaxel) was proposed (AU)

Recurrent transient loss of airway secondary to regurgitated pedunculated hypopharyngeal liposarcoma.

We describe a rare case of hypopharyngeal liposarcoma with an atypical presentation. The patient presented with a 3-month history of intermittent, transient acute airway obstruction. In between episodes, he was asymptomatic. A pedunculated tumour originating in the postcricoid region was seen to be suspended into the oesophagus and intermittently regurgitated into the larynx to cause airway obstruction. The lesion was endoscopically removed and examined histologically to confirm the diagnosis. On-going management of rare lesions such as this should be through multidisciplinary team meetings at a tertiary sarcoma centre.

Health-related quality of life and pain with selinexor in patients with advanced dedifferentiated liposarcoma.

Objective: Compare health-related quality of life (HRQoL) of selinexor versus placebo in patients with dedifferentiated liposarcoma. Materials & methods: HRQoL was assessed at baseline and day 1 of each cycle using the European Organization for Research and Treatment of Cancer 30-item core quality of life questionnaire. Results were reported from baseline to day 169 (where exposure to treatment was maximized while maintaining adequate sample size). Results: Pain scores worsened for placebo versus selinexor across all postbaseline visits, although differences in HRQoL at some visits were not significant. Other domains did not exhibit significant differences between arms; however, scores in both arms deteriorated over time. Conclusion: Patients treated with selinexor reported lower rates and slower worsening of pain compared with patients who received placebo.

Lay abstract The goal of this study was to compare the health-related quality of life (HRQoL) of patients with advanced unresectable dedifferentiated liposarcoma treated with selinexor compared with those treated with placebo. HRQoL was measured prior to treatment initiation and at the first day of each cycle of their treatment using the European Organization for Research and Treatment of Cancer 30-item core quality of life questionnaire. Pain scores worsened for placebo compared with selinexor across all visits after treatment, but differences at some visits were not significant. Other domains did not exhibit significant differences between arms; however, scores in both arms worsened over time reflecting the progressive disease burden in this patient population. As pain is one of the most devastating symptoms associated with advanced and progressing cancers, the significant reduction in pain in the selinexor arm, according to patient perception, represent a relevant added value of this drug in dedifferentiated liposarcoma.

Changes of the hepatic subcapsular blood flow in a case of high-grade retroperitoneal liposarcoma: what to expect?

Changes of the hepatic subcapsular blood flow with the early appearance of hypervascularity near the falciform ligament are rare radiologic findings. They present most frequently in cases of superior vena cava (SVC) obstruction and are related to the recruitment of the cavo-mammary-phrenic-hepatic-capsule-portal and the cavo-superficial-umbilical-portal pathways. We present the case of a 52-year-old female patient with an highly aggressive retroperitoneal liposarcoma with SVC obstruction caused by external compression due to a mediastinal metastatic mass. The patient exhibited no symptoms of SVC obstruction due to the collateral cavo-portal pathways.

Concurrent minimal change disease and retroperitoneal liposarcoma successfully treated by tumor resection and steroid therapy.

A 54-year-old Japanese woman developed simultaneous abdominal distension and bilateral leg edema. Her medical history and results of periodic medical check-up were unremarkable. Blood tests revealed severe hypoproteinemia and acute kidney injury, and urinalysis revealed 4+ proteinuria and 2+ hematuria. Abdominal computed tomography revealed a large intra-abdominal mass with fat tissue density. She underwent emergency tumor excision, splenectomy, and distal pancreatectomy. However, hypoproteinemia and acute kidney injury worsened. Therefore, she was transferred to the nephrology division for confirmation of diagnosis and for treatment of acute kidney injury and nephrotic syndrome. We conducted percutaneous kidney biopsy and diagnosed minimal change disease (MCD). Intravenous prednisolone was started, and heavy proteinuria and systemic edema were gradually alleviated. She achieved complete remission 2 months later, and oral prednisolone was tapered. Histopathological diagnosis of abdominal tumor was dedifferentiated liposarcoma of retroperitoneal origin. Immunohistochemical staining revealed strong expression of vascular endothelial growth factor in the tumor cells in the dedifferentiated component. Currently, her clinical course is stable without recurrence of liposarcoma and nephrotic syndrome. MCD develops in patients with Hodgkin's lymphoma, solid organ cancers, hematological malignancies, and thymoma, whereas concurrent MCD and liposarcoma are rare. Remission of nephrotic syndrome and normalized kidney function induced by steroid therapy are important for better management of patients with malignancy.

Successful intraoperative management in patients with abdominal compartment syndrome induced by giant liposarcomas: Two case reports.

RATIONALE: Giant intra-abdominal liposarcomas weighing over 20 kg often increase the intra-abdominal pressure (IAP), which has severe effects on the cardiovascular and respiratory systems. Abdominal compartment syndrome is defined typically as the combination of a raised IAP of 20 mm Hg or higher and new onset of organ dysfunction or failure. The anesthetic management and perioperative management are very challenging. PATIENTS CONCERNS: We presented 2 patients with rare giant growing liposarcoma of the abdomen, weighing 21 kg and over 35 kg, respectively. Circulatory management was particularly difficult in the first case, while respiratory management and massive blood loss was very challenging in the second one. DIAGNOSIS: With a computed tomography scan and peritoneal-to-abdominal height ratio measurement, preoperatively the risk of developing intra-abdominal hypertension and abdominal compartment syndrome was recognized early in each patient. The inferior vena cava and right atrium of the first patient was compressed and malformed due to the uplifted diaphragm, while there was severe decreased lung volume and increased airway resistance, because of rare giant retroperitoneal liposarcomas in the second case. Histologic examination revealed dedifferentiated liposarcoma in both cases. INTERVENTIONS: Both of the patients underwent resection surgery with multiple monitoring; transesophageal echocardiography monitoring in the first case and pressure-controlled ventilation volume guaranteed mechanical ventilation mode in both cases. OUTCOMES: Intraoperatively and postoperatively no cardiopulmonary complications in both patients. The first patient was discharged without any complications on postoperative day 10, and the second patient underwent another surgery because of anastomotic leakage resulting from bowel resection. LESSONS: Multiple monitorings, in particular transesophageal echocardiography should be considered in patients with increased IAP due to a giant mass, while an appropriate lung protection ventilation strategy is crucial in these patients.

Primary pleural liposarcoma combined spindle cell lipoma of the lung.

Liposarcoma is a malignant adipose tissue tumor which mainly originates from the extremities and retroperitoneum. Primary pleural liposarcoma is very rare. Spindle cell lipoma is a rare benign adipose tissue tumor. A 66-year-old male was referred to our hospital for the evaluation of a mass-like opacity visible on chest X-ray. Computed tomography (CT) scan revealed a well-defined soft tissue mass with internal low attenuations and adjacent multiple nodules in the upper lobe of the left lung, and surgical excision was subsequently performed. Histopathological findings revealed adipose tissue with lipoblasts and spindle cells and immunohistochemical staining (IHC) revealed the tumor cells were strongly positive for CDK4 and MDM2. Histopathological examination of the small lung nodules showed spindle cell proliferation and adipose tissue without positivity for MDM2. Here, we report a rare case of primary pleural liposarcoma combined adjacent spindle cell lipoma of the lung.

Retroperitoneal sarcomas: from diagnosis to treatment. Case series and review of the literature.

BACKGROUND: Retroperitoneal sarcoma is a rare malignancy arising from mesenchymal cells, most commonly presented as an abdominal mass and is associated with poor prognosis. The most effective treatment modality for retroperitoneal sarcomas is complete surgical resection, including sometimes adjacent organs infiltrated by the tumor. Radiotherapy is frequently applied and has shown some benefit, while the role of chemotherapy and molecular-targeted agents is still not clear. Local recurrence is common for retroperitoneal sarcomas and still remains the main cause of death. The major factors associated with the overall survival are tumor grade, histological subtype, complete macroscopic excision and multifocality. AIM: To report our experience via the presentation of patients with retroperitoneal sarcomas managed in our department during the period 2014-18; and to review the current literature. PATIENTS AND METHODS: Eight patients appeared with chronic non-specific complaints including abdominal distension and changes in bowel or bladder habit, while one patient presented with acute abdominal pain due to mass rupture. All of the patients underwent surgical resection of the tumor. RESULTS: Among the patients, seven were operated for primary disease and one only for recurrent. The most common histologic type was liposarcoma (well-differentiated, dedifferentiated), found in five patients; followed by leiomyosarcoma found in two cases. Fibrous histiocytoma was found in only one case. The masses were removed with macroscopically clear margins (R0 and R1 resections) in four cases. In five patient cases adjuvant therapy was required. Three patients are still alive and free of disease. CONCLUSIONS: Retroperitoneal sarcomas present to be a therapeutic challenge based on their location, their extent at the time of diagnosis and the high risk of local recurrence or distant metastasis. Their management requires a multidisciplinary approach, with the surgical resection remaining the mainstay of curative treatment, combined with surveillance for early detection of recurrence or metastases.