Adrenal lipomatous tumours: a 30 year clinicopathological experience at a single institution

AIMS: Fatty tumours of the adrenal gland are uncommon and their features have received little attention in the literature. The aim of this study is to analyse the features of adrenal lipomatous tumours. METHODS: The histological features of primary adrenal tumours reported over a 30 year period (1970 to 1999) in Queen Mary Hospital, Hong Kong were reviewed and the clinicopathological features of adrenal lipomatous tumours were analysed. RESULTS: Adrenal lipomatous tumours were noted in 20 patients (12 men, eight women), and they accounted for 4.8% of the primary adrenal tumours reported. The adrenal fatty tumours comprised 11 myelolipomas, three lipomas, three teratomas, two angiomyolipomas, and one liposarcoma. Calcification or bone was noted in one third (seven of 20) of the adrenal tumours. In some fatty tumours (myelolipoma and angiomyolipoma), the fatty component may be inconspicuous. This is the first report in the English literature of angiomyolipoma and liposarcoma of the adrenal gland. CONCLUSIONS: Different types of fatty tumours were noted in the adrenal gland. A high index of suspicion should be maintained with an aim of surgical treatment for selected patients with large and symptomatic adrenal lipomatous lesions. Histological confirmation is needed for diagnosis.published_or_final_versio

Murine double minute 2 (MDM2) expression by immunohistochemistry in lipomatous tumours and a clinical cohort

INTRODUCTION: Differentiating atypical lipomatous tumours (ALT) and well-differentiated liposarcomas (WDLS), also known as grade 1 liposarcomas (G1LS), from benign lipomas is a common diagnostic problem for pathologists. ALT/WDLS are characterised by amplification of the murine double minute 2 (MDM2) gene and our aim was to investigate if immunohistochemistry for MDM2 can be used to diagnose ALT/WDLS. We also reported treatment and calculated survival rates for patients with grade 1 liposarcomas (G1LS) at Helsinki University Central Hospital (HUCH). METHODS: 25 lipomas, 17 ALT and 21 G1LS were reviewed and initiated for MDM2 immunohistochemistry. For the clinical cohort we included 53 patients with a final diagnosis of G1LS during 1995-2007. RESULTS: The sensitivity of MDM2 immunohistochemistry for ALT and G1LS was 35% and 86%, respectively. The specificity in distinguishing ALT and G1LS from lipomas was 100%. The 5-year local-recurrence free survival (LRFS) for G1LS was 80%, and the 10-year LRFS was 68%. Local recurrence occurred in 16/53 patients. Retroperitoneal and intra-abdominal tumours had a 4.224 times greater risk of local recurrence compared to tumours in the extremities or trunk wall. Only one patient died due to G1LS. DISCUSSION: MDM2 immunohistochemistry is a good supportive method when diagnosing lipomatous tumours. It was highly specific in differentiating ALT and G1LS from lipomas and also relatively sensitive in recognising ALT and G1LS. We reported that G1LS are recurring tumours with tumour site strongly affecting the risk of local recurrence. Despite the high risk of local recurrence, the mortality of G1LS is low

Trabectedin arrests a doxorubicin-resistant PDGFRA-activated liposarcoma patient-derived orthotopic xenograft (PDOX) nude mouse model.

BACKGROUND:Pleomorphic liposarcoma (PLPS) is a rare, heterogeneous and an aggressive variant of liposarcoma. Therefore, individualized therapy is urgently needed. Our recent reports suggest that trabectedin (TRAB) is effective against several patient-derived orthotopic xenograft (PDOX) mouse models. Here, we compared the efficacy of first-line therapy, doxorubicin (DOX), and TRAB in a platelet-derived growth factor receptor-α (PDGFRA)-amplified PLPS. METHODS:We used a fresh sample of PLPS tumor derived from a 68-year-old male patient diagnosed with a recurrent PLPS. Subcutaneous implantation of tumor tissue was performed in a nude mouse. After three weeks of implantation, tumor tissues were isolated and cut into small pieces. To match the patient a PDGFRA-amplified PLPS PDOX was created in the biceps femoris of nude mice. Mice were randomized into three groups: Group 1 (G1), control (untreated); Group 2 (G2), DOX-treated; Group 3 (G3), TRAB-treated. Measurement was done twice a week for tumor width, length, and mouse body weight. RESULTS:The PLPS PDOX showed resistance towards DOX. However, TRAB could arrest the PLPS (p < 0.05 compared to control; p < 0.05 compared to DOX) without any significant changes in body-weight. CONCLUSIONS:The data presented here suggest that for the individual patient the PLPS PDOX model could specifically distinguish both effective and ineffective drugs. This is especially crucial for PLPS because effective first-line therapy is harder to establish if it is not individualized

Treatment of lipomas and diffuse lipomatosis with NDYAG 1064 NM laser and their impact on the quality of life

Lipomas, the most common type of benign tumours, are generally developed from adipose tissue and present an incidence of 2.1 per 1000 inhabitants. In addition to lipomas, at least three other maladies of the adipose tissue lead patients to consult a doctor, especially for aesthetic purposes: multiple familial lipomatosis, diffuse congenital lipomatosis, and adiposa doloris. Unlike lipomas, these maladies are characterized by numerous lipomas, encapsulated or not, of different sizes, symmetrical or not, which may appear in the neck, limbs, or the trunk, sometimes being painful. The life quality of these patients is affected, not only from an aesthetic point of view but also from medical considerations, like pain. Chemical lipolysis has proven unsatisfactory for patients due to several reasons: the prolonged therapy, high cost, the partial dissolution of the lipomatosis, and high recurrence at one year. Surgical treatment remains the only viable option; sometimes when numerous lipomatous tumors required large and numerous incisions, treatment was refused by patients. The result after laser liposuction is excellent, the recovery time is short, without much pain for the patient, with minimal ecchymoses and edemas, without any recurrence in time, and with an excellent degree of patient satisfaction

Surgical treatment of retrosternal extraosseous Ewing Sarcoma in a 6-years old female: a clamshell approach with hemysternectomy and application of a non-crosslinked extracellular matrix

Background Ewing Sarcoma (ES) and Neuroblastoma (NB) belong to a family of tumours of primitive neuroectodermal origin (PNET) that occurs in both bone and soft tissue. Notwithstanding ES and NB are two distinct malignant tumours, sometimes there could be a link between them. Case report We describe a case of an extraosseous ES localized in the retrosternal region and the upper lobe of the right lung, which had been previously treated for NB in a 6 years old female. We treated this case with a clamshell approach which allows, in a one-step surgery, a complete excision of the mass reconstructing the hemysternectomy with a non-crosslinked matrix. Conclusion the clamshell approach is therefore useful to achieve the retrosternal space and the lung with a single surgical access. According to our experience, we consider appropriate to use a non-crosslinked matrix for sternal reconstruction

Multi-platform profiling of over 2000 sarcomas: Identification of biomarkers and novel therapeutic targets

Background: Drug development in sarcoma has been hampered by the rarity and heterogeneity of the disease and lack of predictive biomarkers to therapies. We assessed protein expression and gene alterations in a large number of bone and soft tissue sarcomas in order to categorize the molecular alterations, identify predictive biomarkers and discover new therapeutic targets. Methods: Data from sarcoma specimens profiled for protein expression, gene amplification/translocation and DNA sequencing was reviewed. Results: 2539 sarcoma specimens of 22 subtypes were included. TOPO2A was the most overexpressed protein at 52.8%. There was overexpression or loss of other sarcoma relevant proteins such as SPARC, PTEN and MGMT. Approximately 50% of the sarcomas expressed PD-L1 by IHC and presented with PD-1+ TILs, notably the LMS, chondrosarcomas, liposarcomas and UPS. Gene amplification/rearrangement of ALK, cMYC, HER2, PIK3CA, TOPO2A and cMET was relatively uncommon. EGFR gene amplification occurred at a rate of 16.9%. DNA sequencing of 47 genes identified mutations in 47% of the samples. The most commonly mutated genes were TP53 (26.3%) and BRCA2 (17.6%). Overexpression of TOPO2A was associated with TP53 mutation (P = 0.0001). Conclusion: This data provides the landscape of alterations in sarcoma. Future clinical trials are needed to validate these targets

Retroperitoneal liposarcoma : current insights in diagnosis and treatment

Retroperitoneal liposarcoma (RLS) are rare, biologically heterogeneous tumors that present considerable challenges due to their size and deep location. As a consequence, the majority of patients with high grade RLS will develop locally recurrent disease following surgery, and this constitutes the cause of death in most patients. Here, we review current insights and controversies regarding histology, molecular biology, extent of surgery, (neo)adjuvant treatment, and systemic treatment including novel targeted agents in RLS

Intraoperative Extracorporeal Irradiation for the Treatment of the Meningioma-Infiltrated Calvarium.

Objectives Complete removal of infiltrated bone is required to achieve a Simpson Grade 1 meningioma resection. Reconstruction of the resulting bone defect is typically achieved with a nonnative implant that can result in poor cosmesis, foreign body reaction, or infection. Extracorporeal irradiation and reimplantation of tumorous bone has been used for limb-sparing surgery with excellent results, but this treatment option is not routinely considered in meningioma surgery. We present a case of anterior fossa meningioma with tumorous overlying calvarium that was successfully managed with intraoperative extracorporeal irradiation and reimplantation. Design, Setting, and Participant A 37-year-old woman with persistent chronic headaches was found to have an anterior skull base meningioma with extension into the forehead frontal bone. Concurrently with mass resection, the bone flap was irradiated intraoperatively with 120 Gy. After resection of the tumor, the bone flap was replaced in its native position. Main Outcome Measures and Results Twenty-nine months postoperatively, the patient had an excellent cosmetic outcome with no radiographic evidence of tumor recurrence or significant bone flap resorption. Conclusion Intraoperative extracorporeal irradiation of tumorous calvaria during meningioma surgery is an effective, logistically feasible treatment option to achieve local tumor control and excellent cosmetic outcome

Commentary on "a case of paratesticular leiomyosarcoma successfully treated with orchiectomy and chemotherapy"

We have read with great interest the article written by Ko and colleagues on a particularly rare type of malignant mesenchymal tumor that is paratesticular leiomyosarcoma and we did appreciate the argumentation on the utility of adjuvant chemotherapy as treatment of stage III disease. As for our experience, we would like to shed light on a very rare and little-known aspect surrounding this neoplasm, which is the capability of dedifferentiation exerted in order to recur or metastasize

Primary intrathoracic liposarcoma: a clinicopathologic study and prognostic analysis of 23 cases

BACKGROUND: Primary intrathoracic liposarcoma is an extremely rare malignancy as well as a rare histologic subtype of intrathoracic sarcoma. Relatively few reports appear in the world literatures. We explored the clinicopathologic features and prognostic factors of this tumor in this study. METHODS: We retrospectively analyzed the clinicopathological data of 23 patients with primary intrathoracic liposarcoma who were treated in Shanghai chest Hospital affiliated to Jiao Tong University, from January 2003 to March 2013. These patients were classified into three groups according to the distinct tumor locations, including mediastinum, pleura and lung liposarcoma. Also, these patients could be divided into four types, including well-differentiated, myxoid, dedifferentiated and pleomorphic liposarcoma. The influences of age, sex, tumor size, tumor location, tumor histologic type and therapy on the prognosis of the patients were analyzed. RESULTS: There were no significant difference for survival among distinct liposarcoma locations. However, significant difference for survival among distinct liposarcoma types were observed. Poor disease-free survival (DFS) was observed in the myxoid, pleomorphic and dedifferentiated types as compared to well-differentiated type (P = 0.038). Inferior overall-survival (OS) was observed in dedifferentiated, pleomorphic and myxoid types relative to well-differentiated type (P = 0.027). The radical surgery was a favorable prognostic factor for OS, as demonstrated by the better OS of the radical surgery group as compared to that of the non-radical surgery group ( P = 0.029). Notably, there were no significant differences for DFS and OS in other clinical parameters including tumor size, gender and age. In addition, radiotherapy and/or chemotherapy could not improve the prognosis of the patients receiving non-radical surgery or suffering from relapse. CONCLUSIONS: The histological type and the radical surgery are the factors that influence the behavior and prognosis of liposarcoma. In general, radiotherapy and chemotherapy are believed to be ineffective therapeutic modalities for survival. So it is essential to completely resect the primary intrathoracic liposarcoma as radical cure of the disease