Pathology and genetics of tumors of soft tissue and bone

Cystic echinococcosis (CE) is a worldwide zoonosis caused by larval stages of the cestode Echinococcus granulosus. Surgery, chemotherapy, and interventional procedures are the therapeutic options. Surgery can cure the patient if the parasite is removed entirely. However, the technical procedures are inconsistent and comprise partial liver resection or opening of the parasitic cyst and removal of the parasite. Laparotomy is the most common approach. In selected cases laparoscopic methods are successful. Retrospective studies outweigh prospective ones by far. However, proper management gives favorable results. We critically review the literature and present a brief summary of current surgical strategy and focus on issues relevant for surgeons: diagnosis, indication for medical treatment, indication for surgical treatment, surgical procedures, scolicidal agents, morbidity, mortality, recurrence, perioperative medication, standards. All surgical procedures aim at the complete removal of the parasite. Liver resection and pericystectomy are procedures that resect the closed cysts with a fairly wide safety margin. A meta-analysis shows the best results regarding lethality (1.2%), morbidity (11.7%), and recurrence rates (2%) for resective operations. However, most surgeons consider these methods as too radical for a benign disease. Procedures that remove the parasite and keep the pericyst (=cystectomy) are easier to carry out than resective ones. The meta-analysis presented revealed a lethality of 2%, morbidity of 23%, and recurrence rate of 10.4% for these operations. Omentoplasty is the option of choice for the management of the remaining cyst cavity. Despite alternative procedures surgery is the treatment of choice. Supportive measures comprise the use of scolicidal agents and postoperative benzimidazole administration. However, a critical review of the literature disclosed a lack of scientific confirmation of established treatment modalities and procedures. The results of ultrasound imaging were classified and correlated to the developmental phases of CE. Cystectomy and omentoplasty for CE should be the standard surgical procedure because it is safe, simple, and effective and meets all criteria of surgical treatment for hydatid disease: entire elimination of the parasite, no intraoperative spillage especially by using a cone, and saving healthy tissue. Pericystectomy should be used for peripherally located liver cysts that are surrounded by parenchyma only partially. Ultrasonic classification of the parasitic lesion should be used as a guideline for therapeutic measures.

Echinococcus granulosus infection: the challenge of surgical treatment

Cystic echinococcosis (CE) is a well-known neglected parasitic disease. However, evidence supporting the four current treatment modalities is inadequate, and treatment options remain controversial. The aim of this work is to analyse the available data to answer clinical questions regarding medical treatment of CE. A thorough electronic search of the relevant literature without language restrictions was carried out using PubMed (Medline), Cochrane Central Register of Controlled Trials, BioMed, Database of Abstracts of Reviews of Effects, and Cochrane Plus databases up to February 1, 2017. All descriptive studies reporting an assessment of CE treatment and published in a peer-reviewed journal with available full-text were considered for a qualitative analysis. Randomized controlled trials were included in a quantitative meta-analysis. We used the standard methodological procedures established by the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. We included 33 studies related to the pharmacological treatment of CE in humans. Of these, 22 studies with levels of evidence 2 to 4 were qualitatively analysed, and 11 randomized controlled trials were quantitatively analysed by meta-analysis. Treatment outcomes are better when surgery or PAIR (Puncture, Aspiration, Injection of protoscolicidal agent and Reaspiration) is combined with benzimidazole drugs given pre- and/or post-operation. Albendazole chemotherapy was found to be the primary pharmacological treatment to consider in the medical management of CE. Nevertheless, combined treatment with albendazole plus praziquantel resulted in higher scolicidal and anti-cyst activity and was more likely to result in cure or improvement relative to albendazole alone.

/pdf/medical-treatment-of-cystic-echinococcosis-systematic-review-2y80razyoe.pdf

Medical treatment of cystic echinococcosis: systematic review and meta-analysis.

Background
The World Health Organization recently recognized a family of neoplasms showing at least partial morphological or immunohistochemical evidence of a putative perivascular epithelioid cell (PEC) differentiation. These tumors include angiomyolipoma (AML), clear cell "sugar" tumors of the lung (CCST), lymphangioleiomyomatosis (LAM), clear cell myomelanocytic tumors of the falciform ligament and distinctive clear cell tumors at various other anatomic sites.

/pdf/perivascular-epithelioid-cell-tumor-pecoma-of-the-uterine-2tizbetzup.pdf

Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis.

The uterus and retroperitoneum have emerged as the most frequently reported anatomic sites of origin of perivascular epithelioid cell tumors (PEComas), a poorly defined neoplasm that is characterized by varying amounts of spindle and epithelioid cells with clear to eosinophilic cytoplasm that display immunoreactivity for melanocytic markers, most frequently HMB-45. Published reports on 41 previously reported uterine PEComas are reviewed in this report. Of these 41 cases, 31 originating in the corpus and for which there was adequate follow-up information (or clinical malignancy) were categorized into 2 groups: (1) a malignant group that was comprised of cases associated with patient death of disease and/or clinical malignancy as evidenced by local and/or distant extension outside of the uterus (n=13, group 1) and (2) a "nonmalignant" group of cases in which neither of the above features were present (n=18, group 2). Groups 1 and 2 did not significantly differ regarding duration of follow-up (25 mo vs. 24.3 mo, respectively, P=0.9) or patient age (45.61 y vs. 43.46 y, respectively, P=0.7). Five of the group 1 patients experienced distant (extra-abdominal) metastases. The group 1 tumors were significantly larger than the group 2 tumors (averages 9.6 cm vs. 4.67 cm respectively, P=0.04); however, there were no size thresholds that, in of themselves, reliably classified 75% or more of the cases in both groups. Coagulative necrosis was highly associated with group 1, being present in 82% of cases as compared with only 11.8% of group 2 cases (P=0.0002). Eighty-eight percent of the group 2 cases had a mitotic rate of 1/10 HPF and/or coagulative necrosis are features that, if present, raise the definite potential for aggressive behavior.

Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases.

Background/Aims : It is important to single out the indications for surgery of hydatid disease of the liver at an early stage For this, a fully comprehensive diagnostic work up of the patient is required Surgery represents the most effective therapy and involves resection of the cysts and their outer capsule This must be as complete as possible Material and Methods : Between 1970 and June 1992, 95 patients received this combined treatment for hydatid disease although only those presenting from 1985 were followed up Results : No serious complications were noted apart from pyrexia due to secondary cholangitis or reactive pleuritis This responded quickly to antibiotics and there was no mortality No cases of relapse occurred although one case did show evidence of reappearance of the disease on serological testing alone, all other tests being negative Conclusion : The most effective treatment for echinococcus cystic disease of the liver is radical surgery Results are best when surgery is combined with medical therapy of benzoimidazole drugs given pre- and post-operatively From our experience, we are now confident that surgical treatment must always be preceded and then followed by treatment with benzoimidazole antihelminthics

Hepatic echinococcus disease: our experience over 22 years.

The authors describe the first ever reported case of a malignant pecoma of the uterus infiltrating the ovary, the tube and two bowel loops. This extremely rare tumour usually shows a benign behaviour and seems to arise from the perivascular epithelioid cells (PEC).

[A uterine pecoma: a case report].

We report the experiment of five cases of neurinoma observed in the division of Day Surgery of the Third Surgery Clinic, operating in the Hematology Institute of University of Studies "La Sapienza" in Rome. The problems of the diagnosis of this neoplasia has been faced, though it is commonly unknown or confused with other cervical especially lymphonodules tumefactions, from which it is almost impossible to distinguish before the operation. In the period of six years on 775 cases of cervical neoformations have been operated only 5 patients bearers of neuromi; two originated from vago cervicale (right and left), one from simpatico cervicale on the left, another from the left spinal accessory and the last from the secondary medial trunk of the left brachial plexus. All the patients have been operated in Day-Surgery anaesthesia, locoregional and with calming of spontaneous breath. One case, that originated from the secondary medial trunk of the left brachial plexus, has been operated with microsurgical technique. On all the cases has been done a subcapsular exeresis to preserve nervous continuity. No post operating complications have been recorded; all the patients discharged in the evening. The diagnosis about the nature is left to the final histological test on the operating piece. The surgical removal remains even today the elective treatment able to exclude probable relapses and potential malignant transformation of these tumours.

[Cervical neurinomas. (Considerations in five cases operated in Day-Surgery)].

A retrospective study on patients with differentiated thyroid carcinoma operated on at the 3rd Department of General Surgery of the University "La Sapienza" of Rome from 1970 to 1996 was performed. In 709 patients total thyroidectomy was performed as the minimal procedure acceptable, while 19 patients had subtotal thyroidectomy out of necessity. A functional ipsilateral or bilateral lymphnectomy of the neck was performed in 256 cases. This wider operation is indicated in the presence of metastatic lymph nodes and on principle in patients older than forty-five years in which at least another risk factor is present. Long term follow-up (12 years) was assured in 302 patients and the survival rate was 92% independently from the histotype (papillary or follicular). The survival rate of a group of 120 patients (80 with papillary and 40 with follicular carcinoma) was analyzed in relation to the risk factors. This group analysis demonstrated a very low mortality rate in patients with low risk index and an increased rate in patients with a high risk index.

Surgery of differentiated cancer of the thyroid

Nodular thyroid pathology weighs heavily for 4-5% of the general population, being the most common among the endocrinopathies. Because of this these diagnostic methods have gained more and more importance as FNA able to select already in the pre-operation phase the malignant nodules from the benign ones and to decide on the most suitable surgical intervention. In the period of time between January 1989 and June 1992 in the Institute of the III Surgical Clinic in Rome there have been performed 403 FNA. From the whole total we have extrapolated 221 patients. In this study we stressed how the FNA method has a sensibility of 80%, a specificity of 93% and afterwards an accurate diagnosis of 92%. Besides we have verified that sonography and radionuclide scanning have a truth worthiness respectively of 64% and 52%. The FNA in our opinion allows us to have in the pre-operation phase a much more accurate diagnosis as regards that obtained with only sonography and radionuclide scanning therefore the latter methods must be considered complementary to FNA.

[Preoperative diagnosis of thyroid nodule. Ultrasonography-guided fine-needle aspiration].

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