Nested stromal-epithelial tumour of the liver : An unusual liver entity

Nested stromal-epithelial tumours (NSETs) of the liver have been reported to be extremely unusual primary hepatic neoplasms. To date, few cases have been described in the literature. NSETs have been defined as non-hepatocytic and non-biliary tumours of the liver consisting of nests of epithelial and spindled cells, myofibroblastic stroma and variable intralesional calcification and ossification. Here, we report a case of a young female who underwent liver resection for a large hepatic lesion that proved to be a calcifying NSET on pathological examination. Details about the clinical and histopathological features of the tumour are reported

Ratio-based staging systems are better than the 7th and 8th editions of the TNM in stratifying the prognosis of gastric cancer patients: A multicenter retrospective study.

BACKGROUND: The current and the previous editions of the tumor-node-metastasis (TNM) system for gastric cancer (GC; TNM8 and TNM7) have a high risk of stage-migration bias when the node count after gastrectomy is suboptimal. Hence, they are possibly not the optimal staging systems for GC patients. This study aims to compare the TNM with two systems less affected by the stage-migration bias, namely, the lymph nodes ratio (LNR) and the log odds of positive lymph nodes (LODDS), to assess which one is the best in stratifying the prognosis of GC patients. METHODS: The sample study included 1221 GC patients. Two 7-cluster staging systems based on the combination of pT categories and LNR and LODDS categories (TLNR and TLODDS) were compared with the two last editions of TNM, using the Akaike information criteria, the Bayesian information criteria, and the receiver operating characteristic (ROC) curve graphs. Further validation on an independent sample of 251 patients was carried out. RESULTS: The univariable and multivariable analyses and the ROC curves detected an advantage of the TLNR and TLODDS systems over the TNM. The TLNR and TLODDS showed the best accuracy both in the subgroup of patients with ≥16 nodes examined. The results were confirmed in the validation analysis. CONCLUSIONS: TLNR and TLODDS staging systems should be considered a valid implementation of the TNM for the prognostic stratification of GC patients. If these results are confirmed in further studies, the future implementation of the TNM should consider the introduction of the LNR or the LODDS along with the number of metastatic nodes

The sacral chordoma margin

[Objective]: Aim of the manuscript is to discuss how to improve margins in sacral chordoma. [Background]: Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery. [Methods]: A multidisciplinary meeting of the “Chordoma Global Consensus Group” was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies. This review aims to report on the outcome of the consensus meeting and to provide a summary of the most recent evidence in this field. Possible new ways forward, including on-going international clinical studies, are discussed. [Results]: En-bloc tumor-sacrum resection is the cornerstone of treatment of primary sacral chordoma, aiming to achieve negative microscopic margins. Radical definitive particle therapy seems to offer a similar outcome compared to surgery, although confirmation in comparative trials is lacking; besides there is still a certain degree of technical variability across institutions, corresponding to different fields of treatment and different tumor coverage. To address some of these questions, a prospective, randomized international study comparing surgery versus definitive high-dose RT is ongoing. Available data do not support the routine use of any medical therapy as (neo)adjuvant/cytoreductive treatment. [Conclusion]: Given the significant influence of margins status on local control in patients with primary localized sacral chordoma, the clear definition of adequate margins and a standard local approach across institutions for both surgery and particle RT is vital for improving the management of these patients

Preoperative doxorubicin plus ifosfamide in primary soft-tissue sarcomas of the extremities

A total of 51 patients with large, primary, high-grade soft-tissue sarcomas of the extremities were treated at our institute with two preoperative and three postoperative cycles of doxorubicin plus ifosfamide/mesna. Preoperative doxorubicin was given intra-arterially for lesions of the lower extremities. Of 47 evaluable patients, 22 (47%) showed clinical responses and 21 (45%), pathologic responses. Clinical and pathologic responses, however, were not necessarily coincident in the same patient. Conservative limb-sparing surgery was performed in 41 cases (87%), and in 4 cases amputation was avoided as a result of the local improvement induced by chemotherapy. Local recurrence occurred in 12 cases (25%), 3 of which required further amputation. Although the follow-up period was only 28 months, the 4-year actuarial survival was 91% and the 4-year metastasis-free value was 69%. An evident difference existed between patients who showed a documented clinical response to chemotherapy and those who did not, the 4-year metastasis-free value being 85% and 59% (P = 0.1), respectively. Adjuvant preoperative chemotherapy may allow for limb-salvage surgical procedures in selected cases

Chordoma: natural history and treatment results in 33 cases

Thirty-three chordomas were observed at the Istituto Nazionale Tumori of Milan from 1933 to 1983: 27 sacrococcygeal, 3 spheno-occipital, and 3 vertebral. The male: female ratio was 2.7, and the median age was 63 yr for patients with sacrococcygeal and 35.2 yr for those with nonsacral chordomas. After pathologic reassessment, distinct cytologic patterns were found: physaliphorous, syncytial, and mixed subtypes, with variable degrees of cytologic atypia. However, no evident difference in survival was documented in relation to these cytohistologic features. Four cases had a prior traumatic fracture, and the pathogenetic role of trauma is stressed. Eight cases were operated with adequate surgery and only three recurred, whereas of 11 inadequate operations, 10 developed local relapse. However, follow-up for recent adequate operations is short. Radiation therapy seemed to be effective with adjuvant or palliative aims. No chemotherapeutic regimen achieved any result; one case had a short complete remission after cis-dichlorodiammineplatinum + vinblastine + bleomycin (PVB). This analysis confirms the possibility of achieving radicality with high resection of the sacrum for lesions confined below the second sacral vertebra. Nonsacral chordomas were all unresectable. The best treatment for unresectable lesions seems to be palliative surgery plus radiotherapy

Kaposi's sarcoma: malignant tumor or proliferative disorder?

In order to provide information on the response to treatments and clinico-pathological pattern, the clinical course of 41 patients with classic Kaposi's sarcoma (KS) was reconsidered. Twenty-six cases presented a single nodular lesion, and 15 multiple, pluricentric lesions. Surgery was the first treatment for patients with single lesions, 14 of 26 (54%) patients had recurrences: the disease-free interval ranged from a few months to 11 years. Five cases had disseminated disease, three of these were preceded by local recurrence. Cases with multiple lesions were treated by a combination of surgery, chemo- and radiation therapy (RT). In three cases spontaneous regression of disease was observed and two of these are presently disease-free. After chemotherapy and RT, many patients had complete remission of disseminated disease for up to 40 months. The drugs of first choice were vinblastine and bleomycin. Over all, only one patient died of KS, 10 years after diagnosis, nevertheless the cure rate was very poor and the final overall disease-free rate was around 30%. Proper treatment for nodular or disseminated lesions provides a fair disease-free period. Final considerations: mortality of disease is exceptional despite the 80% risk of recurrence or dissemination. Data from our series do not provide proof that adequate control of the primary single lesion could screen against recurrence: the interval between treatment of the first lesion and recurrence is sometimes exceptionally long, up to more than 10 years, and for that it is not easy to state when disease is really cured. These considerations and other analogies between KS and lymphoproliferative disorders in immunodepressed people strongly suggests the possibility of a non-malignant or even non-tumoral pattern to this disease, with implications for therapeutic strategies