[Dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features: a clinicopathological analysis of five cases].

Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of dedifferentiated liposarcoma (DDLPS) with inflammatory myofibroblastic tumor (IMT)-like features. Methods: Five cases of DDLPS with IMT-like features were collected from the First Affiliated Hospital of Nanjing Medical University, the Affiliated Hospital of Nanjing University of Traditional Chinese Medicine and the First People's Hospital of Qinzhou between 2013 and 2018. EnVision method and fluorescence in situ hybridization (FISH) were used to detect the immunophenotype of the tumor cells and the profile of MDM2 gene amplification respectively. Results: All five cases were male and the median age was 61 (range 53 to 65) years. The clinical symptoms were mainly related to the space-occupying lesions. The tumors were located in duodenal mesentery (two cases), intestinal wall (one case), retroperitoneum (one case), and spermatic cord (one case). Grossly, the tumors were not well encapsulated, ranging from 3 to 13 cm (median 6.7 cm) in diameter, with tan to gray and firm cut surface. Histologically, the dedifferentiated component closely resembled inflammatory myofibroblastic tumor (IMT), with spindle/polygonal/stellate-shaped cells arranged in storiform, sheet-like, or random pattern, with varying degrees of chronic inflammation and fibrosis. All three major patterns seen in IMT (myxoid, cellular and hypocellular fibrous) were observed, the hypocellular fibrous pattern was the most common. Well-differentiated liposarcomatous component was found in the peripheral areas of all the tumors. One case had high grade dedifferentiated component. Four cases were strongly positive for MDM2 and p16. Two cases were positive for SMA, and one case was focally positive for desmin and one for CD34. None of the cases stained for ALK-1. FISH demonstrated MDM2 gene amplification in all five cases. Clinical follow-ups were available in all five cases and the interval ranged from 3 to 66 months (median 23 months). Two patients developed recurrences and one patient had metastasis. The remaining two patients were alive with no evidence of tumor recurrence at 3 and 14 months after surgery respectively. Conclusions: DDLPS with IMT-like features is a more aggressive neoplasm than its histological mimic (IMT), and should not be misdiagnosed as other intermediate or low-grade malignant tumors, such as IMT, sclerosing liposarcoma, inflammatory liposarcoma, aggressive fibromatosis, solitary fibrous tumors, low-grade myofibroblastic sarcoma, and low-grade fibrosarcoma.

Feasibility and Efficacy of Microwave Ablation Combined with Iodine-125 Seed Implantation in Local Control of Recurrent Retroperitoneal Liposarcomas: Initial Clinical Experience.

INTRODUCTION: The objective of the present study was to evaluate the feasibility, safety, and short-term efficacy of microwave ablation (MWA) combined with iodine-125 (125I) seed implantation in recurrent retroperitoneal liposarcomas (rRPLs). MATERIALS AND METHODS: From September 2012 to March 2015, 11 patients were enrolled in this prospective study. Eleven tumors (median, 9 cm; range, 5.5-12.5 cm) were treated with computerized tomography-guided MWA for 11 sessions and 125I seed implantation for 18 sessions. 125I seed implantation was performed 4 weeks after MWA. RESULTS: There were no procedure-related deaths. Post-MWA pain (grade ≥2) was the most common complication (6 of 11 patients, 54.5%), and fever (grade ≥2) was observed in two patients. Reversible nerve injury, defined as transient limb paresthesia or leg weakness, was observed in one patient. There were fewer complications associated with the 125I seed implantation procedure compared with the MWA procedure. All 11 patients who underwent the MWA procedure achieved a partial response (PR), according to the modified Response Evaluation Criteria in Solid Tumors, 1 month post-ablation; after 125I seed implantation was performed, a complete response was observed in three, five, and six target tumors in 3, 6, and 12 months, respectively. CONCLUSION: In selected patients with rRPLs, MWA combined with 125I seed implantation is feasible and safe with favorable local control efficacy. IMPLICATIONS FOR PRACTICE: This study evaluated the feasibility, safety, and short-term efficacy of microwave ablation (MWA) combined with iodine-125 (125I) seed implantation in recurrent retroperitoneal liposarcomas (rRPLs). Results suggest that a single session of MWA may be not sufficient in large-volume rRPLs and that as a supplement treatment, 125I seed implantation is safe and easy accessible. MWA combined with 125I seed has excellent local control effectiveness, and long-term efficacy and survival benefit still need to be more comprehensively evaluated.

[Non-secreting paraganglioma retroperitoneal]. / Paragangliome non sécrétant rétropéritonéal: à propos d'une observation.

Non-functional retroperitoneal paragangliomas are rare tumors. They are often asymptomatic and can reach very large sizes. We report the case of a 49-year old woman with retroperitoneal tumor detected during CT scan examination performed to find the cause of non-specific abdominal pain. Malignant forms, more frequent than benign forms, show locoregional invasion and are characterized by delayed-onset metastases. The treatment of these tumors is based on the most complete degree of surgical resection, since prognosis depends on it. There is no consensus on the usefulness of complementary therapies which may nevertheless constitute a supportive treatment.

Effect of intraoperative radiotherapy in the treatment of retroperitoneal sarcoma.

BACKGROUND: Current National Comprehensive Cancer Network guidelines for the treatment of retroperitoneal sarcomas (RPS) endorse surgical resection, but the role of radiotherapy (RT) is less clear. We investigate the utilization and benefits of intraoperative RT (IORT) in the treatment of RPS. METHODS: We queried the Surveillance, Epidemiology and End Results (SEER) database (1988-2013) for the utilization of IORT and perioperative external beam RT (EBRT) in patients who underwent surgical resection of RPS. Groups were defined as any IORT (aIORT), IORT alone (IORT-), IORT with EBRT (IORT+) and preoperative and/or postoperative EBRT without IORT (EBRT). Demographics, tumor characteristics, extent of disease, and survival were compared between groups. RESULTS: We identified 908 patients with RPS who underwent surgical resection with perioperative RT. Demographics of age, sex, and race were similar between groups. There was no difference in baseline tumor characteristics of mean size, tumor grade, or histological subtype between groups. A higher percentage of patients receiving aIORT had tumors >20 cm in size, and extension beyond local tissues. Liposarcoma and leiomyosarcoma were the most common subtypes overall and in each subgroup. Patients with liposarcoma undergoing IORT and EBRT (IORT+) demonstrated a survival benefit over both IORT alone (IORT-) and EBRT alone. CONCLUSION: IORT was used infrequently for RPS but generated equivalent outcomes compared to EBRT, despite being utilized more often for larger tumors and those with peri-tumoral soft-tissue invasion. Patients with the most common subtype (liposarcoma) may benefit from combination IORT with adjuvant EBRT versus other regimens.

Refractory diarrhea: A paraneoplastic syndrome of neuroblastoma.

Neuroblastoma (NB) is the most common extracranial solid tumor in children. Diarrheal NB is quite rare and is not easy to diagnose in the early stage. Six cases of diarrheal NB in our hospital treated from 1996 to 2006 were retrospectively analyzed, including characteristics such as electrolyte imbalance, pathologic features, vasoactive intestinal peptide (VIP) immunohistochemical staining results, treatment, and prognosis. All patients were boys with 3-8 loose or watery stools each day and routine fecal tests were normal. Abdominal tumors were identified by B-ultrasound. Drugs were ineffective. Three patients underwent surgery, and the remaining three patients received surgery and chemotherapy. Diarrhea stopped after treatment in five patients. Two patients died due to intractable hypokalemia. The tumor was located in the adrenal gland in four patients, in the upper retroperitoneum in one patient, and in the presacral area in one patient. Pathologic findings were NB and ganglioneuroblastoma. Five patients were at clinical stage I-II, and one was at stage III. Four patients survived (followed-up for 6 mo to 4 years). Immunohistochemical staining for VIP was positive. Refractory diarrhea is a paraneoplastic syndrome of NB and is rare. Patients aged 1-3 years who present with chronic intractable diarrhea should be followed closely. Intractable diarrhea, hypokalemia, and dysplasia are the initial clinical manifestations. Increased VIP is characteristic of this disease. Potassium supplementation plays a vital role in the treatment procedure, especially preoperatively. The prognosis of diarrheal NB is good following appropriate treatment.

Beyond stage I germ cell tumors: current status regarding treatment and long-term toxicities.

Approximately 20% to 40% of patients with germ-cell tumors (GCT) will need advanced medical treatment because of relapse or initial metastatic disease. The survival and recommended treatment for men with metastatic disease varies according to histology, primary and metastatic sites, and the level of prechemotherapy tumor markers. For patients with a good prognosis, three cycles of bleomycin, etoposide, and cisplatin (BEP) or four cycles of etoposide, and cisplatin are recommended. For patients with intermediate- and poor prognosis, four cycles of bleomycin, etoposide, and cisplatin remains the preferred treatment option, although a switch to a more intensive regimen can be considered a new alternative. A major advance in salvage therapy for GCT in the last 5 years was the development of a new risk classification system. Initial salvage treatment includes both high-dose chemotherapy and standard-dose chemotherapy. There is clear consensus that patients with residual masses larger than 1 cm should undergo postchemotherapy retroperitoneal lymph node dissection (PC-RPLND); however, the role of PC-RPLND in patients with serologic and radiographic complete response to first-line chemotherapy is controversial. The rationale for PC-RPLND in patients with small residual masses is discussed, and only a small minority of advanced nonseminoma GCT (NSGCT) patients are suitable candidates for observation after first-line chemotherapy. Post-treatment long-term toxicity has emerged as an important issue for GCT survivors. Examples of late effects are secondary nongerm-cell cancers and cardiovascular disease, which represent the most severe and potentially life-threatening effects of cancer treatment. Follow-up of cancer survivors should include recommendations for maintaining a healthy lifestyle to reduce the risk of serious long-term and late effects of treatment.

[Operative therapy of abdominal and retroperitoneal sarcoma]. / Operative Therapie der abdominellen und retroperitonealen Sarkome.

Retroperitoneal soft tissue sarcomas are characterized by a high rate of local recurrence. Complete tumor resection is the only potentially curative therapeutic option. The concept of a systematic compartmental resection is to remove the tumor en bloc with a margin of uninvolved tissue and organs. This is frequently only achieved by multivisceral resection which often includes kidney, colon, pancreas and parts of the diaphragm or the psoas muscle. The adoption of such a policy of multivisceral organ resection improves the proportion of curative resections and, ultimately, results in lower local recurrence rates. The present article comprehensively describes the operative procedures, perioperative treatment and the oncological results of surgery for retroperitoneal sarcomas. The role of surgery in oncological treatment plans and the importance of specialized centres are outlined in detail.

Solitary ganglioneuromatosis of the descending colon, presenting as giant retroperitoneal tumour.

Ganglioneuroma (GN) is a benign neoplasia of the autonomous nervous system, colonic GN is uncommon in adults. There are three subgroups: polypoid GN, ganglioneuromatous polyposis and diffuse ganglioneuromatosis. Ganglioneuromatosis is highly-associated to neurofibromatosis type 1 (NF1) and multiple endocrine neoplasia type 2b (MEN2B). A 68-year-old female, with a discrete retarded emission of stools, was admitted for a large tumor in the left flank; CT scan, urography and barium enema demonstrated a large retroperitoneal mass, presumed as sarcoma. Open surgery discovered a 16 10 11 cm solid and encapsulated tumor, attached to the retroperitoneal descending colon, with no macroscopic mucosal involvement; the pathologic diagnosis of the resected specimen (en-bloc tumorectomy with limited colectomy) was intramural colonic ganglio-neuromatosis. Anamnesis, physical examination and complete endoscopic explorations showed no evidence of personal bearing or familial aggregation of genetic syndromes. In adults, association of transmural ganglioneuromatosis to NF1 or MEN2B is not mandatory; presentation often mimics obstructive carcinoma and positive diagnosis is provided by pathological examination of the resected specimen. In this peculiar case, the loose tissue of the retroperitoneal space favoured a slow development of intramural ganglioneuromatosis, presenting as a gigantic retroperitoneal mass with no radiological evidence of its colonic origin.

Primitive retroperitoneal tumors. Vascular involvement--a major prognostic factor.

Primitive retroperitoneal tumors, although very rare, arouse an increased interest, because of the poor prognosis, unsatisfactory surgical and complementary therapy results. Up to now, the very low number of cases has impeded the acquisition of a unitary view of these tumors, a unanimously accepted algorithm of diagnostic and treatment being absent. Randomized trials regarding the effects of different therapies have not been possible. The main factor that can fundamentally increase the survival of these patients is radical resection, some authors even recommending compartmental surgery. We found no significant statistical difference between the survival rates of the patients with different types of non-radical interventions, that shoud be therefore, as much as possible, avoided. Our study evidences that vascular involvement is the main limiting factor in achieving radicality. The involvement of large retroperitoneal vessels makes often impossible a radical intervention, usually because of the lack of an adequate material and human endowment for ample vascular resections followed by laborious reconstructions. That is why, in our study, vascular involvement was associated with a decreased survival rate for operated patients. Therefore, we underline the necessity both of a solid material base and of establishing multidisciplinary surgical teams for adequate vascular interventions in oncologic general surgery.

Clinical considerations on the retroperitoneal liposarcomas.

AIM: It presents a clinical case of undifferentiated retroperitoneal liposarcoma with 5 years' recurrence from the first operation for the rarity of the occurrence, the problems related to surgery and complementary therapeutic approach. MATERIAL OF STUDY: Male patient aged 73 was operated for removal of retroperitoneal mass with involvement of the right kidney at the Second University of Naples in the 2003. In accordance with the interdisciplinary board, complementary therapy is not indicated and follow-up program. The successive controls were negative until at least 2007. Reoperation for recurrence in 2008 for the presence of massive bone formation occupying a large part in the right half of the abdomen at the sub-hepatic level. In both cases histological examination showed undifferentiated liposarcoma. DISCUSSION: It is of unknown etiology and only 25% occurs in well-differentiated cells are also more than 100 histological subtypes, 85% are malignant. The most affected is the male sex and from the beginning looks like malignancy. The trend of growth in general is slow, and in most cases tends to recur over time. The role of chemotherapy and radiation therapy is controversial. CONCLUSIONS: The peculiarity of our case is higher than the average survival despite advanced age and presence of recurrence. This confirms the importance of surgical treatment, thus offering the patient a chance of better long-term survival.

Peritoneal sarcomatosis: is there a subset of patients who may benefit from cytoreductive surgery and hyperthermic intraperitoneal chemotherapy?

BACKGROUND: Unlike novel molecular-targeted therapies for metastatic gastrointestinal stromal tumors (GIST), conventional treatments for peritoneal sarcomatosis (PS) are mostly ineffective. As with carcinomatosis of epithelial origin, a rationale base supports an aggressive locoregional treatment of PS, but the use of CRS and HIPEC in this setting is still controversial. We assessed the outcome of clinically and pathologically homogeneous subsets of patients with PS uniformly treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). METHODS: A prospective database of 37 patients who underwent CRS and close-abdomen HIPEC with cisplatin and doxorubicin or mitomycin-C was reviewed. PS originated from GIST (pre-imatinib era) in 8 patients, uterine leiomyosarcoma (ULS) in 11, retroperitoneal liposarcoma (RPLP) in 13, and other sarcoma in 5. RESULTS: CRS was macroscopically complete in 28 patients (75.7%). Operative mortality was 3.7% and morbidity 21.6%. After median follow-up of 104 (range, 1-131) months, peritoneal disease progression occurred in 16 patients, distant metastases in 5, and both in 13. For all patients, median overall survival was 26.2 months; 7 patients were alive at 46-130 months (ULS, n = 4; RPLP, n = 2; GIST, n = 1). RPLP had the best overall survival (median, 34 months) but 100% peritoneal relapse; GIST had dismal overall, local-regional-free and distant-free survival; ULS had the higher proportion of long survivors and best local-regional-free survival. CONCLUSIONS: Overall, results of CRS and HIPEC did not compare favorably to those of conventional therapy. In a subgroup analysis, the combined approach did not change GIST and RPLS natural history. The interesting results with ULS may warrant further investigations.

Neuroblastoma: treatment outcome after incomplete resection of primary tumors.

PURPOSE: For International Neuroblastoma Staging System (INSS) stages III or IV neuroblastoma (intermediate or high risk), complete excision of the primary tumor is not always feasible. Most current studies on the treatment outcome of these patients have reported on the complete excision status. The aim of this study is to review the treatment outcome after the incomplete resection. METHODS: The medical records of 37 patients that underwent incomplete resection between January 1986 and December 2005 were reviewed retrospectively. Incomplete resection was assessed by review of the operative notes and postoperative computerized tomography. Age, gender, tumor location, INSS stage, N-myc gene copy number, pre- and postoperative therapy, and treatment outcome were reviewed. The treatment outcome was evaluated according to the postoperative treatment protocol in the high-risk group. RESULTS: Intermediate-risk patients were treated with conventional chemotherapy, isotretinoin (ITT) and interleukin-2 (IL-2). High-risk patients were treated with peripheral blood stem cell transplantation (PBSCT), ITT, and IL-2 (N = 11). Before the introduction of PBSCT, the high-risk patients were also treated with the conventional chemotherapy (N = 19). Intermediate-risk patients (N = 5) currently have no evidence of disease (NED). For the high-risk patients (N = 32), 19 patients were treated with chemotherapy alone; 15 patients died of their disease while four patients currently have an NED status. Eight of 11 patients that underwent PBSCT are currently alive. CONCLUSIONS: For intermediate risk, conventional chemotherapy appears to be acceptable treatment. However, for high-risk patients, every effort should be made to control residual disease including the use of myeloablative chemotherapy, differentiating agents and immune-modulating agents.

Multifocality in retroperitoneal sarcoma: a prognostic factor critical to surgical decision-making.

OBJECTIVE: To evaluate the significance of multifocality on overall survival (OS) in patients with retroperitoneal sarcoma (RPS) and establish a data-derived, prognostically and therapeutically useful definition of sarcomatosis. SUMMARY BACKGROUND DATA: The incidence, clinical features, and prognostic significance of multifocality in RPS is unknown. No current standardized definition for sarcomatosis is available. METHODS: We conducted a retrospective analysis of 393 patients with primary or recurrent nonmetastatic RPS treated at a comprehensive cancer center between 1996 and 2006. Baseline and treatment variables were compared in patients with unifocal and multifocal disease. A multivariate model was used to evaluate the association of multifocality and OS and identify additional prognostic factors in patients with multifocal disease. RESULTS: The median follow-up time for all patients was 69 months; 79 patients (20%) presented with multifocal disease. The 5-year OS rate was less in the multifocal group compared with the unifocal group (31% vs. 60%, respectively; P < 0.0001). After multivariate analysis, multifocality remained an independent predictor of worse OS {hazard ratio (HR) 1.7 (95% confidence interval (CI), 1.2-2.5); P = 0.004}. Additionally, patients with more tumors had significantly worse prognosis (>7 tumors, HR 2.1 (95% CI, 1.1-3.9); P = 0.03), with a 5-year OS rate of 7%. CONCLUSIONS: Multifocal RPS is associated with worse OS in patients with either primary or recurrent RPS; Patients with >7 tumors had the worst prognosis. This criterion can be used to define sarcomatosis, thereby identifying patients whose survival will ultimately depend on effective systemic therapy.

A case of relapsing secondary bladder adenocarcinoma after right colonic cancer.

BACKGROUND: A 71-year-old woman was referred to a surgical oncology clinic after CT raised suspicion for a bladder neoplasm. She had previously undergone right hemicolectomy and received adjuvant chemotherapy for pT3N1MX cancer of the cecum. A retroperitoneal recurrence had been deemed unsuitable for surgical resection, and had instead been treated with chemoradiation therapy. Follow-up CT raised suspicion for a possible bladder neoplasm. INVESTIGATIONS: CT, physical examination, urinalysis, cystoscopy with biopsy, pathological analysis and immunohistochemical analysis. DIAGNOSIS: Adenocarcinoma of the cecum metastatic to the bladder. MANAGEMENT: The patient underwent open bladder resection with total excision of the neoplasm and was administered adjuvant chemotherapy consisting of irinotecan and cetuximab. Subsequent recurrences at the same site were treated with transurethral resection, while chemotherapy was still in progress. At 7 months' follow-up, the patient remained alive, with no evidence of further recurrence.

Giant retroperitoneal sarcomas.

Retroperitoneal sarcomas are rare malignant tumors, which are developing from mesenchymal stem cells residing in muscle, fat, and connective tissues. Underlying the rarity of this kind of tumors in general population, the aim of this paper is to present three cases of retroperitoneal sarcomas operated in Surgical Department of Military Hospital of Craiova (a retroperitoneal liposarcoma, a dedifferentiated liposarcoma and a malignant fibrous histiocytoma). From clinical point of view, we note the poverty of symptoms and non-specificity of these and a great tolerability of retroperitoneal space that offers the possibility for a great development of the tumor. CT-scan and MRI are the best investigations for diagnosis but surgical exploration is the best way for a good evaluation of these tumors. From histological point of view, we try to present new features about these kinds of tumors in order to classify them. Results of surgery correlated with complementary therapies were good without per-operative mortality or postoperative morbidity but we noted a recidive of one tumor (with different histological pattern) 21 months after the surgical intervention. The rarity of retroperitoneal sarcomas, combined with the vast array of histologic subtypes, has complicated our understanding of these tumors and impeded the development of effective therapies.

Burkitt's lymphoma in the boy: infiltration in the stomach, colon and the retroperitoneum--ileocecal invagination.

A 4-year-old boy was hospitalised because showing signs of weakness, slight pain in the abdomen and while urinating. The symptoms occurred 7 days before hospitalisation. The boy did not vomit, nor did he have the urge to vomit, the defecation was regular showing no traces of blood. The physical visit a soft and painless tumefaction was confirmed ileocecally. The echography tests and the computed tomography suggested invagination, not excluding the second substrate. Barium enema showed irreductible invagination. The operative test showed that it was about the ileocolic invagination with extreme thickening of the cecum, the ascedental colon, the intestine and the retroperitoneum walls. A resection of the small intestine and a ileocolic anastomosis was performed. The pathohistological test shows the primar abdominal Burkitt's lymphoma. In spite of the subsequent therapy the boy dies three weeks after the first symptoms' manifestation. We, herewith, suggest at the importance of the echography analysis when diagnosing the Burkitt's tumor and give advantage to this analysis against the computerized tomography. We also point at the huge level of malignancy of the Burkitt's tumor in this boy.

Schwannoma antiguo: variante rara de schwannoma / Old schwannomaa. a rare type of schwannoma

OBJETIVO: A propósito de un caso deschwannoma retroperitoneal con características histológicaspoco frecuentes, se evalúa su significación clínica eindicaciones de manejo terapeutico.MÉTODOS: Presentación de un caso de schwannomaretroperitoneal benigno, como hallazgo incidental, alpracticar una Resonancia Magnética, en una paciente de70 años cuya clínica era de una lumbociática de seismeses de evolución. Revisión bibliográfica.RESULTADOS: Tras la exéresis quirúrgica se llegó al diagnósticode schwannoma retroperitoneal antiguo. No seadministra ningún tratamiento complementario. No se haevidenciado recidiva durante seguimiento. Los cambioshistológicos que se ven en los casos de schawannomaantiguo se han interpretado como degenerativos, indicandouna larga evolución, que ha motivado su denominaciónde “antiguos”. Algunos de los casos fueron diagnosticadoscomo malignos.CONCLUSIONES: El schwannoma antiguo es una variantepoco habitual del schwannoma común benigno.Microscópicamente muestra áreas de tejido hipocelular ynúcleos pleomórficos sugestivos de malignidad. Su manejoterapéutico es similar al de otros procesos benginosretroperitoneales, mediante exéresis quirúrgica y seguimiento

OBJECTIVES: We report one case ofretroperitoneal schwannoma with unfrequent histologicalcharacteristics evaluating its clinical significance andtherapeutic options.METHODS: We report the case of a benign retroperitonealschwannoma as an incidental MRI finding in a 70-yearoldfemale patient with symptoms of lumbosciatic painfor 6 months. We perform a bibliographic review.RESULTS: After surgical excision the diagnosis wasretroperitoneal old schwannoma. No additionaltreatment was given. There is no evidence of recurrenceon follow-up. The histological changes apparent in thecases of old schwannoma have been interpreted asdegenerative, indicating a long evolution, which hasmotivated its denomination as old. Some cases havebeen diagnosed as malignant.CONCLUSIONS: The old schwannoma is a rarevariety of the benign common schwannoma.Microscopically its shows areas of hypocellular tissueand pleomorphic nuclei suggestive of malignancy.Therapeutic management is similar to other benignretroperitoneal processes, surgical excision and followup