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.

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.

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.

Experience with eribulin in pretreated patients with advanced liposarcoma: a case series.

Systemic treatments for advanced soft tissue sarcoma are limited. Eribulin showed activity in metastatic soft tissue sarcoma, particularly liposarcomas. Data from six patients with advanced liposarcoma that received eribulin as third- or fourth-line therapy are presented herein. Eribulin treatment was well tolerated; no grade 3-4 toxicity or therapy delay was observed. Two patients had a partial response; four had a prolonged stable disease. The first case, with pre-existing chronic renal dysfunction, achieved a 6-month stable disease with dose-reduced eribulin. The second case became resectable after eribulin treatment, with a 6-month complete surgical remission. The third case obtained a 7-month stable disease with eribulin and stereotactic body radiotherapy. The last three cases were ≥65 years old, and two of them had objective response with eribulin.

Long-term eribulin treatment in patients with a single kidney and retroperitoneal liposarcoma.

Retroperitoneal sarcomas are extremely rare malignant tumors. The most common type of sarcomas arising in the retroperitoneum are liposarcomas, occurring mostly in the sixth and seventh decades of life. The only potentially curative approach to liposarcomas is the complete surgical resection of the tumor with negative microscopic margins. However, retroperitoneal liposarcomas exhibit a propensity for local recurrence and distant metastasis despite the negative surgical margins, thus requiring additional therapy. Eribulin demonstrated a benefit in terms of overall survival in patients with advanced or metastatic liposarcoma. We report two cases of patients, both submitted to concomitant right nephrectomy, who experienced a long-lasting control of recurrent retroperitoneal liposarcoma before being submitted to eribulin-based therapeutic regimens (23 and 24 treatment cycles completed, respectively).

Spermatic cord liposarcomas incidentally found during hernia surgery: is histology of any lipoma mandatory? A review of the literature.

Purpose: Liposarcomas found incidentally during open or laparoscopic inguinal hernia surgery are extremely rare. It is unclear, whether any adipose tissue being removed during inguinal hernia surgery must be sent for histology due to the potential risk of liposarcoma of the spermatic cord. This study aims to evaluate the frequency of liposarcomas incidentally found in the inguinal canal during hernia surgery and tries to derive evidence-based recommendations regarding the optimal management of any fatty tissue found in the inguinal canal.Methods: A literature review of the PubMed/Medline electronic databases between January 1980 and January 2019 was performed using the search terms 'inguinal hernia' and 'liposarcoma'. There was only one study available on this topic. Therefore, an additional literature review was performed analyzing all reports on patients with incidentally detected liposarcomas of the spermatic cord in the inguinal canal during hernia surgery.Results: There was only one retrospective study evaluating the frequency of inguinal liposarcoma found at hernia operations with a frequency of less than 0.1%. There were 18 cases of spermatic cord liposarcomas that were truly found incidentally during operation for an unsuspected symptomatic or incarcerated inguinal hernia. These included 16 case reports with a total of 18 patients and 19 liposarcomas. All patients were male with a median age of 62.5 years (range: 24-86 years) years. Median size of liposarcoma was 10.5 cm (range: 3-30 cm). In seven patients, the inguinal liposarcoma was an extension of a retroperitoneal sarcoma. Treatment consisted of radical orchidectomy during the primary operation in 12 patients. Three out of the seven patients with retroperitoneal extension of the tumor underwent a secondary operation with complete resection of the tumor.Conclusions: Currently, there is no evidence-based recommendation available regarding the management of lipomas detected during open or laparoscopic inguinal hernia surgery. Due to the extremely low risk of the presence of a liposarcoma, routine histologic examination cannot be recommended unless the diameter exceeds 10 cm.

[Primary Orbital Liposarcoma]. / Primäres Liposarkom der Orbita.

A 30-year-old male patient presented with a painful globe subluxation of the left eye since two weeks. Painless left proptosis had been noted at least 2 years earlier. An MRI performed one year after onset of symptoms showed a retrobulbar mass, which on biopsy was diagnosed as spindle cell lipoma. Surgical debulking via a transconjunctival approach successfully reduced proptosis; repeated histology with extensive immunohistochemical analysis now led to the diagnosis of a well-differentiated orbital liposarcoma. The patient underwent orbital exenteration and received adjuvant radiotherapy. Genetic testing showed a heterozygote mutation of the ATM-gene on chromosome 11.

The impact of lipomatous tumors on type 2 diabetes: are adipose-derived tumors metabolically active?

BACKGROUND: The metabolic and immunologic properties of adipose tissue are linked to the pathogenesis of type 2 diabetes mellitus. Lipomatous tumors, such as liposarcomas, are rare but can reach significant size. We hypothesized that some lipomatous tumors are metabolically active and can alter systemic glucose homeostasis. METHODS: We performed a retrospective study of patients who underwent resection of a lipomatous tumor at a tertiary cancer referral center (2004-2015). We divided patients into nondiabetics, well-controlled diabetics (hemoglobin A1c [HbA1c] < 7), and poorly controlled diabetics (HbA1c ≥ 7). We compared patient demographics, tumor characteristics, and measures of glycemic control among these groups before and after tumor resection. RESULTS: We reviewed 217 operations for lipomatous tumors. No differences were observed in tumor characteristics in patients with and without diabetes. However, tumor characteristics differed significantly between the well-controlled and poorly controlled diabetics groups. Patients with poorly controlled diabetes had larger tumors that were more likely to be malignant, retroperitoneal, and well-differentiated. Tumor resection had no detectable impact on diabetes, as assessed by HbA1c, and requirement for diabetic medications. CONCLUSIONS: Poorly controlled diabetes was linked to the presence of large, malignant, and retroperitoneal lipomatous tumors. However, in limited follow-up, no detectable impact of tumor resection was apparent on glycemic control. These data suggest that most lipomatous tumors do not exert a clinically relevant impact on glucose homeostasis.

Multiply recurrent retroperitoneal liposarcoma.

Retroperitoneal liposarcomas (RPLPS) are rare tumors that represent at least 50% of all retroperitoneal sarcomas. Surgical resection remains the standard of care. Unfortunately, many RPLPS patients will develop a local recurrence and subsequently die in the absence of distant metastasis. This review outlines the factors that predict local recurrence and influence the management of first and subsequent multiply recurrent RPLPS.

Simultaneous pulmonary and inferior vena cava tumor thromboembolism secondary to retroperitoneal pleomorphic liposarcoma.

Retroperitoneal pleomorphic liposarcoma (RPLS) is the least common but the most aggressive subtype of liposarcoma. We herein report a case of tumor embolism (TE) to the inferior vena cava (IVC) and pulmonary arteries in a 54 years old woman with RPLS. The case suggests that fusion fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) can accurately detect occult TE. It also illustrates the usefulness of computed tomography (CT) and high 18F-FDG uptake by PET/CT imaging for diagnosing such findings as filling defect in the veins and beaded pulmonary arteries as TE. To our knowledge, this is the first reported case of simultaneous pulmonary and inferior vena cava thromboembolism secondary to RPLS. And occult TE from RPLS has not been reported previously by 18F-FDG PET/CT.

Association of Li-Fraumeni Syndrome With Small Cell Carcinoma of the Ovary, Hypercalcemic Type and Concurrent Pleomorphic Liposarcoma of the Cervix.

Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT), is a rare, highly lethal malignancy predominantly affecting young adult females. We report a patient with widely metastatic SCCOHT and concurrent uterine cervical pleomorphic liposarcoma. Clinical targeted next-generation sequencing was performed on both neoplasms and demonstrated hemizygous stop-gain TP53 mutations (p.R196*), and wild-type SMARCA4 in both tumors. Microarray analyses of both tumors revealed similar but not identical widespread loss of heterozygosity over most chromosomes associated with loss of chromosomal copy number in the SCCOHT and pleomorphic liposarcoma tumors, amplification of FGFR1 in both tumors, and amplification of MYC in the SCCOHT. Immunohistochemistry demonstrated that SMARCA4 and SMARCB1 were retained in both tumors, and that SMARCA2 expression was retained but TP53 expression was lost in the SCCOHT. Germline testing using Sanger sequencing showed heterozygous TP53 mutation, confirming the diagnosis of Li-Fraumeni syndrome. These findings are novel and for the first time associate SCCOHT with Li-Fraumeni syndrome.

Primary liposarcoma of the sigmoid presenting as colonic intussusception - A case report.

Liposarcomas are malignant soft tissue neoplasms usually located in the retroperitoneum, head, neck and extremities. Although secondary invasion of the gastrointestinal tract by retroperitoneal sarcomas is frequent, primary involvement is uncommon. We report a young patient with Crohn's disease in remission presenting with intussusception of the colon due to a primary liposarcoma of the sigmoid. Emergency resection confirmed the diagnosis. The patient remains in remission after five years. This represents the youngest diagnosis of liposarcoma to date and the first occurring in the sigmoid. We also highlight the concomitant diagnosis of Crohn´s disease.

Recurrence of paratesticular liposarcoma: a case report and review of the literature.

Paratesticular liposarcomas are rare tumors that typically affect adult. Diagnosis is very difficult and inadequate surgical excision leads to a high rate of recurrence.We report a case of local recurrence of paratesticular liposarcoma diagnosed six months following surgery.Since there is low response to adjuvant treatments, extensive surgery remains the only curative approach, as shown by the case described here and the following review of the literature.

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.

Late onset Li-Fraumeni Syndrome with bilateral breast cancer and other malignancies: case report and review of the literature.

BACKGROUND: Li-Fraumeni-Syndrome (LFS) is an autosomal-dominant, inherited tumour predisposition syndrome associated with heterozygous germline mutations in the TP53 gene. Patients with LFS are at a high risk to develop early-onset breast cancer and multiple malignancies, among which sarcomas are the most common. A high incidence of childhood tumours and close to 100% penetrance has been described. Knowledge of the genetic status of the TP53 gene in these patients is critical not only due to the increased risk of malignancies, but also because of the therapeutic implications, since a higher rate of radiation-induced secondary tumours in these patients has been observed. CASE REPORT: We report a patient with LFS harbouring heterozygous, pathogenic TP53 germline mutation, who was affected by four synchronous malignancies at the age of 40: a myxofibrosarcoma of the right upper arm, bilateral breast cancer and a periadrenal liposarcoma. Radiological treatments and a surveillance program were adjusted according to recommendations for LFS patients. CONCLUSION: Management of tumour treatment of patients with LFS is different to the general population because of their risk for secondary cancers in the radiation field. Screening procedures should take a possibly elevated risk for radiation induced cancer into account.

[A case of retroperitoneal liposarcoma with inflammatory pseudotumor].

A 54-year-old man presented with pollakiuria and dysuria. Ultrasonography, computed tomography (CT), and magnetic resonance imaging (MRI) showed a retroperitoneal tumor 10 cm in diameter. Pathological examination of a transrectal biopsy specimen obtained from the patient revealed the presence of an inflammatory myofibroblastic tumor (IMT). The patient underwent surgical resection of the tumor. Histopathologically, the tumor was composed of 3 parts : the largest part was compatible with an inflammatory pseudotumor: the second part, well-differentiated liposarcoma: and the third part, a myxoid liposarcoma. He has been followed up for 14 months ; but there was no evidence of local recurrence of the tumor. We believe that the inflammatory pseudotumor complicated in repairing the inflammation arising from the liposarcoma.