Role of surgery in treatment of advanced differentiated thyroid carcinomas.

Well-differentiated thyroid carcinomas are characterized by a long natural history. The evolution of the reconstructive techniques and the improvement of the peri-operative anaestesiologist management of the patient have contributed, over the last few years, to a progressive widening of demolitive surgery. The aims of enlarged surgical treatment in differentiated advanced thyroid carcinomas are to guarantee respiratory and alimentary functions as well as symptomatic benefits, to obtain local control of the disease and the recovery of adjuvant therapeutic options, such as metabolic and conventional radiation.

Thyroid-stimulating hormone suppression for protection against hypothyroidism due to craniospinal irradiation for childhood medulloblastoma/primitive neuroectodermal tumor.

Purpose: Hypothyroidism is one of the earliest endocrine effects of craniospinal irradiation (CSI). The effects of radiation also depend on circulating thyroid-stimulating hormone (TSH), which acts as an indicator of thyrocyte function and is the most sensitive marker of thyroid damage. Hence, our study was launched in 1998 to evaluate the protective effect of TSH suppression during CSI for medulloblastoma/primitive neuroectodermal tumor.

Efficacy of trabectedin (ecteinascidin-743) in advanced pretreated myxoid liposarcomas: a retrospective study.

Background: Previous studies have suggested that trabectedin (ecteinascidin-743) could have antitumour activity in soft-tissue sarcoma. We aimed to study the usefulness of trabectedin in the treatment of patients with myxoid liposarcomas, a subtype of liposarcoma that is associated with specific chromosomal translocations t(12;16)(q13;p11) or t(12;22)(q13;q12) that result in the formation of DDIT3-FUS or DDIT3-EWSR1 fusion proteins.

Methods: 51 patients with advanced pretreated myxoid liposarcoma who started treatment with trabectedin between April 4, 2001, and Sept 18, 2006 at five institutions in a compassionate-use programme were analysed retrospectively.

Myxoid/round cell and pleomorphic liposarcomas: prognostic factors and survival in a series of patients treated at a single institution.

Background: The objective of this study was to investigate prognostic factors and clinical outcome of myxoid/round cell and pleomorphic liposarcoma.

Methods: Three hundred twenty-nine patients with localized myxoid/round cell or pleomorphic liposarcoma who underwent surgery at the Istituto Nazionale per lo Studio e la Cura dei Tumori (Milan, Italy) over 25 years were reviewed. The rates of local recurrence, distant metastases, and survival were studied.

Intramedullary astrocytoma with granular cell differentiation.

Granular cell astrocytomas are uncommon tumors of the central nervous system (CNS) of which no cases have been documented in the spinal cord. This variant of glioma should not be confused with benign granular cell tumor which, although rare, has been well characterized in the spinal cord. We describe here the clinical, pathological, and radiological features of such an astrocytoma arising within the spinal cord at the dorsal level.

Postoperative radiotherapy for synovial sarcoma of the head and neck during pregnancy: clinical and technical management and fetal dose estimates.

Aims And Background: In vivo and phantom dosimetry is reported to estimate the fetal dose and evaluate the effectiveness of a special shielding device to reduce fetal exposure in a woman undergoing postoperative radiation therapy for synovial oral cavity sarcoma at the 30th week of pregnancy.

Methods: In vivo measurements were performed by placing thermoluminescent dosimeters on 3 points for fetal dose estimation: uterine fundus, umbilicus and pubis. A Rando anthropomorphic phantom was used to simulate radiotherapy.

Colorectal carcinoma in children and adolescents: the experience of the Istituto Nazionale Tumori of Milan, Italy.

Background: Colorectal carcinoma (CRC) is one of the most common tumors in adults, but extremely rare in young age. This study retrospectively reports on a group of 27 patients <30 years of age, and particularly on 7 cases <18 years old, treated at the Istituto Nazionale Tumori, Milan, Italy, between 1985 and 2005.

Patients And Methods: Among the children/adolescents (age 9-18, median 12 years), 5/7 had unfavorable CRC histotypes (poorly differentiated or mucinous adenocarcinoma) and all but one had advanced disease at onset.

Bax mutation and overexpression inversely correlate with immature phenotype and prognosis of childhood germ cell tumors.

Primary childhood germ cell tumors (GCTs) represent a rare and heterogeneous group of tumors that varies in histologic differentiation, age of presentation and clinical outcome. In malignant neoplasms, apoptosis is a prognostic marker and a predictive factor of response to therapy. Therefore, the study of the expression and mutation of molecules involved in the regulation of apoptosis could be useful in order to both predict the clinical outcome and design self-tailored therapeutic approaches.

Kit protein (CD117) and proliferation index (Ki-67) evaluation in well and poorly differentiated neuroendocrine tumors.

Aims And Background: Kit protein expression seems to be associated with a poor outcome in cancer patients and may be an important target for new anticancer drugs. We examined by immunohistochemistry the presence of Kit protein in neuroendocrine tumors (NETs) to explore its relationship with histological grade and proliferation index.

Patients And Methods: Thirty-five tumor specimens from patients with 24 well differentiated and 11 poorly differentiated NETs were examined for the presence of Kit protein and the proliferation index marker Ki-67.

Extremity soft tissue sarcoma: adding to the prognostic meaning of local failure.

Background: We explored the prognostic meaning of local relapse and surgical margins in adult soft tissue sarcoma of the extremities.

Methods: Out of a series of 1017 patients with extremity soft tissue sarcoma treated over 20 years, we picked a group of 238 patients operated on at our institution for their first local relapse: 88 after their primary operation performed at the same center and 150 elsewhere. At operation for relapse, margins were microscopically negative in 77% and 75% of patients, respectively.

Response to melphalan in up-front investigational window therapy for patients with metastatic Ewing's family tumours.

The aim of the study was to determine the activity and toxicity of melphalan as a single agent given in up-front therapy for patients with newly-diagnosed Ewing's family tumours with bone/bone marrow metastases. Nineteen patients were enrolled from 2001 to 2004. The treatment consisted of up-front therapy with melphalan (two courses of 50 mg/m2, 3 weeks apart).

Treatment of sporadic nonmedullary thyroid carcinomas in pediatric age.

Nonmedullary thyroid carcinomas are rare malignancies in pediatric ages. The vast majority of them are papillary carcinomas with an overall survival of approximately 100%. Their outcome is independent of strong prognostic factors of adults, such as papillary carcinoma histological subtype, invasion into soft tissue of the neck, presence and site of distant metastases, relapse and type of surgery.

The murine Pou6f2 gene is temporally and spatially regulated during kidney embryogenesis and its human homolog is overexpressed in a subset of Wilms tumors.

We have previously suggested the transcription factor gene POU6F2 as a novel tumor suppressor involved in Wilms tumor (WT) predisposition. Since WT arises from pluripotent embryonic renal precursors, in this study we analyzed the expression of the murine homolog Pou6f2 during kidney embryogenesis and compared it to that of Wt1, the homolog of WT1, a known WT related gene involved in mesenchyme to epithelium conversion. Quantitative real-time reverse transcription-polymerase chain reaction (RT-PCR) performed for Pou6f2 on kidney specimens from embryos, pups, and adult mice, showed that the Pou6f2 mRNA was more abundant in the earliest analyzed phase of kidney organogenesis (E13) than in more advanced fetal stages and in adult animal.

Advanced extremity soft tissue sarcoma: prognostic effect of isolated limb perfusion in a series of 88 patients treated at a single institution.

Purpose: To explore the prognostic impact of isolated limb perfusion (ILP) in locally advanced extremity soft tissue sarcomas (ESTS).

Methods: From August 1982 to April 2005, 1,119 patients affected by ESTS (girdle excluded) were observed and treated at our institution. Eighty-eight (7.

Detecting CD56+/NB84+/CD45- immunophenotype in the bone marrow of patients with metastatic neuroblastoma using flow cytometry.

Background: In order to identify neuroblastoma cells infiltrating the bone marrow, a triple-color flow-cytometric assay was developed combining CD56 and CD45 with the intracellular anti-NB84 specific antibody.

Materials And Methods: The bilateral aspirates obtained from 27 consecutive children over the age of one year with stage 4 neuroblastoma were evaluated.

Results: Neuroblastoma cells were detected in the bone marrow of 17/27 (63%) and 19/27 (70%) cases using cytomorphology and triple-color flow-cytometry, respectively.

Papillary carcinoma of the thyroid gland of childhood and adolescence: Morphologic subtypes, biologic behavior and prognosis: a clinicopathologic study of 42 sporadic cases treated at a single institution during a 30-year period.

Papillary thyroid carcinomas (PTCs) in pediatric age show an excellent outcome, independently of sex, stage at diagnosis, occurrence of relapse, and type of treatment. Our aim was to study the biologic behavior and the impact on survival of PTC subtypes in childhood. From 1968 to 2002, 42 sporadic pediatric PTCs were subclassified into PTC, not otherwise specified and PTC variants.

Malignant peripheral nerve sheath tumors: prognostic factors and survival in a series of patients treated at a single institution.

Background: The authors explored the prognostic factors and clinical outcomes of patients who had malignant peripheral nerve sheath tumors (MPNST) with and without neurofibromatosis type 1 (NF-1).

Methods: Two hundred five patients with localized MPNST who underwent surgery at the Istituto Nazionale per lo Studio e la Cura dei Tumori (Milan, Italy) over 25 years were reviewed. Forty-six patients had concomitant NF-1 syndrome, and 159 patients did not.

Salvage treatment for childhood ependymoma after surgery only: Pitfalls of omitting "at once" adjuvant treatment.

Purpose: To discuss the results obtained by giving adjuvant treatment for childhood ependymoma (EPD) at relapse after complete surgery only.

Methods And Materials: Between 1993 and 2002, 63 children older than 3 years old entered the first Italian Association for Pediatric Hematology and Oncology protocol for EPD (group A), and another 14 patients were referred after relapsing after more tumor excisions only (group B). Prognostic factors were homogeneously matched in the two groups.

GB virus C and HIV-1 RNA load in single virus and co-infected West African individuals.

Background: Investigations on the impact of GB virus C (GBV-C) co-infection on HIV disease progression relied essentially on clinical follow-up but not on virologic parameters.

Objectives: To detect and quantify GBV-C RNA in West African populations co-infected or not with HIV-1 and to correlate the RNA load of HIV-1 and GBV-C in co-replicating patients with different clinical conditions.

Methods: Three Ghanaian populations (blood donors, pregnant women and HIV-infected patients) were subdivided into six groups according to HIV-1 and clinical status and GBV-C and HIV-1 RNA load was tested by quantitative real time reverse transcriptase-polymerase chain reaction.

Salivary gland neoplasms in children: the experience of the Istituto Nazionale Tumori of Milan.

Background: Epithelial salivary gland tumors are very uncommon in pediatric age. We report a series of 52 cases treated at the Istituto Nazionale Tumori of Milan, Italy, over a 30-year period. These results are presented in conjunction with a literature review of salivary tumors with a view to providing an up-to-date overview of the clinical course, prognosis, and treatment options for this rare tumor.

Prognostic effect of re-excision in adult soft tissue sarcoma of the extremity.

Background: We explored the outcome of patients with primary adult soft tissue sarcoma (STS) of the extremities undergoing re-excision after previous unplanned surgery.

Methods: A total of 597 consecutive adult patients with primary extremity STS were treated with conservative surgery at our institution over a 20-year time span. A total of 318 patients were referred after unplanned excisions, and the remaining 279 underwent primary resection at our center.