Primary Neoplasia Research Articles

Coordinated medical care for children with neurofibromatosis type 1 and related RASopathies in Poland.

Coordinated medical care offered in Poland for patients suffering from neurofibromatosis type 1 and related RASopathies combines complex multispecialty consultation with permanent supervision and the patient’s oriented longitudinal care. Neurofibromatosis type 1 is one of the most common single gene disorders in the global population, observed in 1 out of 2500–3000 live births. It is a primary neoplasia disease with 100% penetration of the gene mutation but remarkable age-dependent onset of different disease signs and symptoms, outstanding clinical heterogeneity between patients even in one family and lack of genotype-phenotype correlation, a high rate of spontaneous mutation exceeding 50%, and multiple comorbidities among which increased risk of malignancy is the most important. Medical practice proved that not only patient-oriented complex but also coordinated care provided in centers of competence is indispensable for patients and the families and provides a sense of medical security to them in conjunction with public health costs rationalization.

MON-432 Symptomatic Pituitary Metastases: A Report of 16 Cases

Introduction: Pituitary Metastases (MTS) are infrequently seen in clinical practice. The incidence ranges from 0.14 to 28%. Breast and lung cancer are the primary sites that most frequently metastasize to sellar region, between the sixth and seventh decades of life. Most cases are diagnosed in patients with advanced malignant disease, however, in 20-30%, symptoms of pituitary involvement can precede the diagnosis of the primary tumor. Objectives: To evaluate symptoms at presentation, hormonal, radiological and histological findings, management and outcome of a series of patients with pituitary MTS. Patients and methods: medical records of 16 patients from eight Endocrine Centers were reviewed. Ten patients had histological confirmation of the pituitary MTS, 6 were not operated, being the diagnostic criteria the presence of sellar mass associated with diabetes insipidus (DI) and / or sudden-onset of neuro-ophthalmological symptoms in patients with confirmed primary neoplasia. Results: The median age was 54 years (range 35-70), 9 women (56.2%). The sites of the primary tumor were: 7 lung (44%), 5 breast (32%), 1 follicular thyroid carcinoma (6%), 1 Hodgkin lymphoma (6%), 1 poorly differentiated carcinoma (6%), and 1 clear cell renal carcinoma (6%). The median time between the diagnosis of the primary neoplasm and the occurrence of the pituitary MTS was 12 months (range: 3-120). In 9 patients (56.2%), the diagnosis of the primary neoplasm was made after the finding of the symptomatic sellar mass. DI was found in 14 patients (87.5%), adenohypophyseal deficit in 12 (75%), visual disorders in 10 (62.5 %), headache in 6 (37.5%) and cranial nerve deficits in 6 (37.5%). In 68.7% (11 patients), other MTS were detected. Fifteen patients were evaluated by MRI and one by CT: 13 (81.3%) harbored supra / parasellar masses, and the remaining 3 had lesions limited to the pituitary gland, with stalk thickening and lack of spontaneous neurohypophysis hyperintensity in 2 of them. In all cases diffuse gadolinium uptake was present. Fourteen patients died (87.5%), with a median survival time of 6,5 months (range: 1-36); the remaining 2 are still alive with a follow-up period of 4 and 12 months respectively. Conclusions: In this series of 16 patients with pituitary MTS, the most frequent primary neoplasms were lung and breast. Median age was lower than in published series. DI was the most common condition at presentation, followed by hypopituitarism and visual disorders. The short survival was related to the extent of the disease at the time of diagnosis. In more than half of the cases the diagnosis of primary neoplasia was made through the symptomatic pituitary mass. In the presence of a pituitary lesion with diffuse gadolinium uptake, associated with DI and / or acute visual deficit, pituitary MTS should be suspected even in patients without a history of oncological disease.

Metastases of Merkel cell carcinoma in terms of diagnosing carcinomas with unknown primary localization.

Merkel cell carcinoma (MCC) is a rare malignant primary skin neoplasia with epithelial and neuroectodermal differentiation. The problem of MCC, which determines the relevance of its research, is often a high frequency of local recurrence, regional lymph node metastasis and further hematogenous and/or distant lymphogenous dissemination. Materials and methods. A retrospective analysis of the biopsy material of 6 patients with isolated MCC metastasis aged from 29 to 82 years (average 63.11 ± 16.24; median 62.5) was carried out. For the control group, 9 cases of postoperative material of primary skin tumors with MCC of patients aged from 27 to 76 years (average 52.17 ± 12.72; median 53.5) were selected to determine the differential diagnostic criteria. The results of the study. Carcinomas without primary localization may be interpreted as MCC if they demonstrate phenotype Cytokeratin, Pan AE1/AE3 (paranuclearly +) / CK20 (paranuclearly +) / Vimentin (-) / CD45 (-) / S100 (-) / Chromogranin A (+) / Synaptophysin (+), with morphometric parameters of the area and perimeter, which is 2 times the size of ordinary lymphocytes, (all p>0.05). Findings. Metastatic CMC without primary localization, in comparison with locally spreading, often exhibit low-differentiated small-cell and transitional forms, which require additional immunohistochemical study to exclude carcinomas of other origin.

Linfoma Anaplásico de Grandes Células primário do SNC, ALK-negativo: Relato de Caso

O Linfoma Anaplásico de Grandes Células (LAGC) é um subtipo raro de Linfoma não-Hodgkin (LNH). Citogeneticamente, tem forte expressão do marcador de ativação CD30 e, em cerca de 65-80% dos LAGCs, há expressão da quinase do linfoma anaplásico (ALK). Classificam-se em LAGC ALK-positivos quando expressam a quinase, relacionando-se a uma melhor resposta à terapia e uma melhor sobrevida global, ou em LAGC ALK-negativos em caso de não expressão. A incidência de LAGC no SNC é muito rara, com menos de 1% dos casos correspondentes a primários do SNC. Apresenta-se, no seguinte relato, um caso bastante raro de LAGC primário do SNC, ALK-negativo, em paciente jovem do sexo feminino, características epidemiológicas opostas às descritas na literatura para esse tipo de apresentação. O diagnóstico diferencial de primeira hipótese era de neoplasia glial primária do SNC de alto grau, entretanto, a imuno-histoquímica definiu como LAGC ALK-negativo, conforme o padrão de expressão citogenético (CD30 e CD3 positivos, alto índice de proliferação celular e negatividade para ALK-1). O tratamento de escolha foi quimioterapia em quatro ciclos com metotrexato e citarabina, em associação com radioterapia.

Nanoparticles for the Treatment of Metastatic Non Small Cell Lung Cancer with 225Ac

Non small cell lung cancer (NSCLC) is the most common form of primary lung neoplasia with nearly 40% of patients having metastasis at the time of diagnosis, resulting in a 5year survival rate ranging from 13-36% in patients with nodal metastasis, and decreasing to as little as 2% for those with distant metastasis. Furthermore, pulmonary metastatic disease is the most common form of secondary lung tumors, being identified in 30-55% of all cancer patients. Targeted α-radiotherapy (TAT) agents have great potential for treating micro-metastatic disease, given their short, densely ionizing track length and high relative biologic effect (RBE). The use of in vivo alpha generators allows for multiple alpha decays from a single radioactive nucleus, but retaining the radioactive daughters at the target site throughout the decay process is a challenge. We present preclinical data describing a multilayered nanoparticle-antibody conjugate that can deliver multiple α radiations from the in vivo α-generator 225Ac at biologically relevant receptor sites while also containing the radioactive daughters at those sites. The layered nanoparticles (NP) consist of an 225Ac-doped (La0.5Gd0.5)PO4 core coated with four layers of GdPO4 and an outer layer of Au. These multi-shell particles combine the radiation resistance of crystalline lanthanide (Ln) phosphate to contain atoms of the therapeutic radionuclide and its radioactive daughters, the magnetic properties of gadolinium for facile separation during synthesis, and the chemistry of gold for attachment of targeting agents to the nanoparticle surface. In a proximity delivery model of cancer, 225Ac-NPs conjugated to mAb 201b resulted in a 73% decrease in the number of EMT6 colonies in the lung five days after treatment [1]. On biodistribution studies and SPECT/CT imaging, over 85% of the injected dose was delivered to the target tissue, and approximately 90% of the fourth daughter, 213Bi, was retained in the target 24 hours after injection. Competition assays demonstrated specific binding of the conjugated radiopharmaceutical to the target. Current studies are evaluating the application of these 225Ac nanoparticles to A549 NSCLC grown in a mouse orthotopic lung cancer model, while a spontaneous cancer model in canine patients is also being explored. [1]M.F. McLaughlin, J.D. Robertson, P.H. Pevsner, J.S. Wall, S. Mirzadeh, and J. Kennel, “LnPO4 Nanoparticles Doped with Ac-225 and Sequestered Daughters for Targeted Alpha Therapy,” Cancer Biotherapy and Radiopharmaceuticals 29(1), 34-41 (2014).

AB043. P-11. Autoantibody screening in sera of patients with cholangiocarcinoma by serological proteomic approach

Background: Cholangiocarcinoma (CCA) is the second most common primary hepatic neoplasia. Antibodies are normally produced in response to a foreign protein or substance within the body. Autoantibodies can be used clinically for cancer detection which can be identified by proteomic analysis of tumor associated antigens (TAAs) that are potentially involved in malignant transformation. The purpose of the present study was to detect the autoantibodies in sera of patients with cholangiocarcinoma by serological proteomic approach for early cancer diagnosis.

Racjonalne przesłanki do wyłączenia nerwiakowłókniakowatości z rodziny RASopatii i fakomatoz – podstawy projektu zarządzania chorobą w ramach koordynowanej opieki medycznej

Term neurofibromatoses (NF) comprises three distinct medical entities of different clinical course with overlapping symptomatology and different molecular pathology. NF-1 and its allelic and mosaic forms is one of the most frequent monogenic disorders and together with Legius syndrome belongs to RASopathies. Remaining two, NF-2 and schwannomatosis (NF-3), are ultra-rare diseases and do not belong to RASopathies. Symptomatology, diagnostic and therapeutic requirements as well as complications of NF course are so different from those observed in other RASopathies and phacomatoses, that neurofibromatoses should constitute a separate classification group with distinct program of care. The crucial argument behind this thesis is that NFs are primary neoplasia syndromes, as benign tumors arise in all patients lifelong and a risk of malignancy significantly exceed populational risk in NF patients. Primary diagnostic problems, especially in small children with multiple cafè-au-lait spots required differentiation among almost 80 clinical entities, divers tumors and potential malignancies, varied multiorgan oncological and non-oncological complications of disease course, warrant not only complex multi-specialty consultations and comprehensive supervision, but the coordinated medical care in general. Proofs confirming title’s thesis are the subject of this article.

Abdominal CT evaluation of the liver and spleen for staging mast cell tumors in dogs yields nonspecific results

Canine mast cell tumor staging is commonly performed using abdominal ultrasonography and fine-needle aspiration cytology of masses, lymph nodes, and hepatic and splenic parenchyma. Computed tomography is used for abdominal, thoracic, or whole body imaging in staging mast cell tumors in the authors' institution enabling evaluation of multiple body areas in one examination. The aim of this study was to compare the CT examinations acquired for staging of mast cell disease to their subsequent liver and spleen cytology findings. Medical records of dogs with primary mast cell tumors that underwent abdominal CT and concurrent liver and spleen aspirates were reviewed. The CT examinations were evaluated for attenuation, size, and margination of the liver and spleen. The relationship between CT findings and cytology results was analyzed. Forty-nine dogs matched the inclusion criteria: five of forty-nine dogs with cutaneous mast cell tumors were positive for metastasis from liver and/or spleen aspirates. Of the five dogs with cytological evidence of liver or spleen metastasis, four had normal CT liver attenuation and size, one dog had concurrent primary hepatocellular neoplasia, four dogs had abnormal splenic parenchyma (two nodular and two diffuse heterogeneity), and one dog had a normal attenuation of the spleen. In four dogs, the spleen was subjectively enlarged. Computed tomographic evaluation of the liver showed no consistent pattern associated with mast cell metastasis and did not predict cytology results. Multifocal splenic hypoattenuating lesions more commonly coincided with mast cell metastasis. Sampling of the liver and spleen remains to be considered in the absence of abnormal CT findings for full staging.

18FDG PET/CT & arterial inflammation: predicting cardiovascular events in lung cancer.

18F-FDG PET/CT predicts cardiovascular disease. To analyze the predictive value of cardiovascular events from inflammation and arterial calcification in patients who underwent an 18F-FDG PET/CT for lung cancer. A retrospective study of 274 patients with primary lung neoplasia. We determined: (i) TBR (target-to-background ratio), and (ii) the calcium score, at eight common arterial segments. We took as arteriosclerosis, a TBR ≥1.6 and ≥15 Calcium Score sum. We registered cardiovascular risk factors, comorbidities, histology, stage, treatment, status at the last clinical review, cause of death and cardiovascular event during the follow-up. The territory presenting the greatest uptake of 18F-FDG, was the thoracic aorta with an average of 1.77 (± 0.27 TBR) in the aortic arch, while the greatest degree of calcification was obtained in the abdominal aorta (52% with a Calcium Score ≥ 3). 24% of the patients presented a sum Calcium Score ≥15, and 17% a TBR ≥1.6. Patients with high TBR, (17%), had not a higher frequency of cardiovascular comorbidities beforehand, nor did they in the follow-up. However, those with a sum Calcium Score ≥15 (24%), were older, had more cardiovascular risk factors and ischemic events during follow-up. The calcium score, but not the TBR, predicted the emergence of a cardiovascular event (HR 4.9 IC95% 2.1-9.1, P < 0.05). In our cohort, a high Calcium Score was an independent predictor for developing cardiovascular events.

Datasets for the reporting of neoplasia of the testis: recommendations from the International Collaboration on Cancer Reporting.

We here describe the development of an evidence-based cancer dataset by an International Collaboration on Cancer Reporting expert panel for the reporting of primary testicular neoplasia, and present the 'required' and 'recommended' elements to be included in the pathology report, as well as a commentary. This dataset encompasses the updated 2016 World Health Organisation classification of urological tumours, the results of an International Society of Urological Pathology consultation, and also staging with our preferred method: the American Joint Committee on Cancer version 8. Implementation of this dataset will facilitate consistent and accurate datacollection between different cohorts, facilitate research, and hopefully result in improved patient management.

A rare case of intracardiac fibrosarcoma with myxoid features inducing venous occlusion in a dog \u2013 a case report

BackgroundIn both humans and animals, cardiac fibrosarcoma is rare among primary cardiac malignant neoplasia. The overall prevalence of cardiac neoplasia in dogs is low, reported to be between 0.17% and 0.19% of hospital admissions. The aim of this report is to describe the clinical and pathological findings of a dog presenting signs of right sided congestive heart failure due to an intracardiac and venous obstructing mass, diagnosed by histopathology as cardiac fibrosarcoma with myxoid features.Case presentationA 7 years old male mix breed Husky weighing 23 kg was presented to our Veterinary Teaching Hospital the owner reporting weight loss, inappetence and exercise intolerance and on presentation exhibited breathlessness and an enlarged abdomen. A 5 minutes six leads electrocardiogram and cardiac ultrasonography were performed using standard, established techniques. Complete blood count, serum liver enzyme activities and renal parameters were assessed. Shortly after the cardiologic examination, the dog died and necropsy examination of the cardiovascular system revealed an elongated and branched mass attached dorsally to the endocardial insertion of the septal tricuspid valve leaflet. This mass extended retrogradely into the lumen of the cervical veins, obstructing the venous flow. Histological diagnosis of the mass was cardiac fibrosarcoma with myxoid features. Multiple metastases were found inside the lungs only.ConclusionThis is the first report describing a right cardiac fibrosarcoma with myxoid features and venous obstruction in a dog. Cardiac fibrosarcoma is a rare finding, however should be considered when an intracardiac mass is diagnosed.

Synchronous rectal and breast cancer in a 40-year-old woman

Multiple primary malignancies have an increasing incidence in the general population due to better diagnostic tools and the increased life expectancy. However, synchronous lesions are still rare and have a rate which varies between 0,17 and 0,69%. Second primary tumours usually develop after some time from the first cancer diagnosis. Although there is an arsenal of therapeutical options – the order and priority of the therapeutic choices are debatable and need to be tailored to every patient. The present paper illustrates the case of a 40 years old woman who presented to the emergency department with diffuse abdominal pain, nausea and bloating. The patient had done a fine needle biopsy of a suspicious lump in her right breast one week before the presentation and had no other relevant medical history. The CT scan revealed intraperitoneal free liquid with a paracolic mass at the rectosigmoid junction. The surgical team decided to perform an exploratory laparoscopy. At exploration, the mass was intensely adherent to the uterus and fixed to the pelvis. Conversion to laparotomy and extemporaneous exam of the mass were undertaken, which revealed adenocarcinoma. En-bloc rectosigmoidian resection with hysterectomy and bilateral adnexectomy. The histopathology report staged the tumour as pT4N2M0 adenocarcinoma. Breast biopsy pathology report revealed no special type (NST) carcinoma, with luminal B breast cancer on immunohistochemistry. Clinical staging of the breast was cT1N0. After discussion of the case in the multidisciplinary team, it was decided for Madden mastectomy with axillary lymphadenectomy. Breast reconstruction with retropectoral expander was done in the same operating procedure. Post-mastectomy pathology report revealed pT1N0 and no metastases were present at standard imaging. The immunohistochemical profile of the resected breast tumour proved to be Luminal A. Adjuvant therapy consisted in chemoradiation for the rectum. The breast neoplasia was treated with tamoxifen as adjuvant therapy. Synchronous primary neoplasia exists and even if they have a low incidence once identified their treatment requires particular treatment for each case. A multidisciplinary approach is essential.

Clear Cell Renal Cell Carcinoma Metastatic to the Gynecologic Tract: A Clinicopathologic Analysis of 17 Cases.

Clear cell renal cell carcinomas (CCRCC) rarely metastasizes to the gynecologic tract. In this study, we analyzed a multi-institutional data set to provide insights into the clinical, morphologic, and immunophenotypic features of this phenomenon. Seventeen metastatic CCRCC involving the gynecologic tract [ovary/fallopian tube (n=9), vulva (n=2), uterine corpus (n=3), cervix (n=2), uterine serosa (n=1)] were analyzed. Mean patient age was 62 yr (range: 45-79 yr). Most cases (15/17) presented as a recurrence 6 to 72 mo postnephrectomy, 1 case was concurrently diagnosed, and 1 case (a cervical metastasis) was diagnosed prenephrectomy. In 10 cases, metastases to other locations were identified within 6 wk of the gynecologic tract lesion. The adnexa were the most common site of metastases and the mean tumor size of adnexal metastases was 3.7 cm; in only 2 of 9 cases were metastases bilateral and only 1 had external surface nodules. The morphologic and immunohistochemical features of metastatic CCRCC were compared with those of 102 müllerian clear cell carcinomas (müllerian CCC: 49 endometrial, 53 ovarian). Although CCRCC and müllerian CCC displayed extensive morphologic overlap, a higher mitotic index and a higher frequency of an alveolar pattern were seen in CCRCC, whereas diffuse hobnail cells, hyaline globules, tubulocystic pattern, or any papillary pattern were more frequently seen in müllerian CCC. CA-IX, CD10, and renal cell carcinoma antigen were more frequently expressed in CCRCC than müllerian CCC, whereas Napsin-A, CK7, and p504S showed the reverse. PAX8 and HNF1β did not significantly distinguish between the 2 groups. In summary, gynecologic tract metastases most often occur as a relapse of a previously resected CCRCC, and these relapses may occur many years postnephrectomy. Gynecologic tract metastases are often accompanied by concurrent metastases to other organs. The gross pathology of metastatic CCRCC in the ovary may potentially overlap with primary neoplasia. However, the expected morphology and immunophenotype of CCRCC are maintained in most gynecologic tract metastases. As such, although metastatic CCRCC and müllerian CCC may display significant overlap in pathologic features, several morphologic and immunophenotypic features are useful in their distinction.

Programmed cell death protein-1 (PD-1)/programmed death-ligand-1 (PD-L1) axis in hepatocellular carcinoma: prognostic and therapeutic perspectives.

Hepatocellular carcinoma (HCC) is the most common primary neoplasia of the liver. There have been tremendous efforts in the development of therapeutic strategies in the last decades. As opposed to other cancer entities immunotherapy has just recently gained popularity in HCC. Among various immunotherapy approaches, programmed cell death protein-1 (PD-1), and its ligand programmed death receptor ligand-1 (PD-L1) axis became one of the most promising pathway of the decade. The scientific interest in PD-1/PD-L1 axis is definitely justified due to: ability to detect PD-L1 expression in patients that underwent resection for HCC with prognostic values; the role of serum PD-L1 as a tool to identify early recurrences and to monitor treatment outcome; PD-1/PDL1 is a highly targetable pathway, with possible predictive markers, and with high clinical applicability that might help us in selecting a subgroup of HCC patients who are most likely to benefit from PD-1/PD-L1 inhibitors. In this review we will first discuss the prognostic role of PD-1/PD-L1 as a bio-marker in various clinical scenarios. Afterwards we will critically analyse the recently published trials with PD-1/PD-L1 inhibitors in HCC either alone or in combination with other treatment modalities. The higher focus will be on clinical rather than preclinical studies. Nevertheless, the strengths and limits of PD-1/PD-L1 axis in both prognosis and therapy of HCC will be highlighted.

EUS Guidance in Diagnosing a Rare Case of Lung Adenocarcinoma Metastatic to Pancreas

: Lung cancer metastasizes via blood or the lymphatics. Organs that are commonly involved include liver, bone, adrenal glands, brain and lungs. Carcinoma metastatic to the pancreas is rare; the most commonly reported sources being renal cell carcinoma, followed by colorectal, melanoma, breast, lung carcinoma and sarcoma.Herein we report a case of metastatic adenocarcinoma of the lung to the pancreas diagnosed by endoscopic ultrasound (EUS) guided fine needle biopsy (FNB). Case: A 56-year-old male with a 30-pack-year history of tobacco smoking was seen for five months of neck pain and headache. Cervical imaging by computed tomography and magnetic resonance imaging showed C7 lytic lesion. Positron emission tomography (figure 1) showed hypermetabolic right hilar and right upper lung lobe mass concerning for malignancy. Additionally, hypermetabolic masses of the right adrenal and the pancreatic body were detected, concerning for metastatic lung cancer. Bronchoscopy with biopsy confirmed lung adenocarcinoma. The pancreatic finding was unclear in whether it represented another primary carcinoma or a metastatic mass. Endoscopic ultrasound with FNB was performed to further evaluate the pancreatic lesion (figure 2). A subtle 14 mm hypoechoic lesion with irregular borders in the body of the pancreas was sampled with 25-gauge fine needle biopsy. The tissue stained positive for thyroid transcription factor 1 (TTF1) and Napsin A immunohistochemistries (figure 3), consistent with metastatic adenocarcinoma of lung origin. Patient underwent palliative radiation to cervical spine and enrolled in carboplatin/paclitaxel/ascorbate study for Non-Small Cell Lung Cancer (NSCLC) phase II. Discussion: Pancreatic masses found on imaging usually represent primary neoplasia of the pancreas. Metastasis from other primaries such as lung adenocarcinoma is highly unusual. While uncommon, these metastases have a distinct pathologic morphology and immunohistochemically detectable expression. For patients with pancreatic lesions with unknown primaries, EUS-FNB is the most effective means of obtaining tissue diagnosis, with immunochemistries playing an important role in identifying the primary. As in our case, it has shown its utility especially when CT scans have been negative or inconclusive. Hence, a pancreatic lesion, in a patient with lung adenocarcinoma, especially if inconclusive on imaging, should raise concern for metastasis and be followed up by an EUS -FNB.1875_A Figure 1. PET scan showing uptake in the right lung and body of pancreas1875_B Figure 2. Endoscopic ultrasound showing pancreatic mass1875_C Figure 3. Napsin A stain

Caso clínico: Manifestación Extrapulmonar de Neoplasia Indiferenciada.

Introducción: El síndrome de neoplasia primaria de origen desconocido, se refiere a la presencia de lesiones metastásicas corroboradas histológicamente cuyo origen primario no puede ser identificado durante la evaluación pre-tratamiento. En el presente caso clínico se presenta una manifestación extrapulmonar de neoplasia no diferenciada por su rareza y descripción del estudio diagnóstico.&#x0D; Caso Clínico: Se trata de un hombre de 49 años, con una masa axilar derecha de 1 año de evolución, de 30 x 20 cm, que incluye el hemitórax, asociado a dolor 7/10, con valoración de la escala Eastern Cooperative Oncology Group (PS ECOG) de 3 en forma inicial. Fue tratado por sepsis por infección de lecho tumoral. La tomografía reportó la lesión tumoral extensa en región axilar derecha con áreas de necrosis, burbujas aéreas, una lesión nodular subcentimétrica en segmento apical de lóbulo superior del pulmón derecho y derrame pleural. La biopsia en cuña reportó una Neoplasia maligna indiferenciada CD–56 +, CD–79 A +, VIMENTINA +, CAM 5.2 +, CK – 20 +.Se concluyó como manifestación extrapulmonar metastásica de neoplasia maligna indiferenciada de origen pulmonar posiblemente de origen neuroendócrino.&#x0D; Evolución: Se estableció un tratamiento neoadyuvante con cisplatino + etopósido, por 3 meses. Escala PS ECOG 1, recuperación funcional de la extremidad, reducción tumoral del 50 % de lesión tumoral, en espera de tratamiento quirúrgico.&#x0D; Conclusión: En este reporte se presenta un caso de síndrome de metástasis de primario oculto el cual responde con reducción tumoral al tratamiento Neoadyuvante. Aunque son de mal pronóstico en la mayoría de los pacientes, en este reporte se presenta una respuesta adecuada al tratamiento.

Role of microRNAs in the main molecular pathways of hepatocellular carcinoma.

Hepatocellular carcinoma (HCC) is the most common primary liver malignant neoplasia. HCC is characterized by a poor prognosis. The need to find new molecular markers for its diagnosis and prognosis has led to a progressive increase in the number of scientific studies on this topic. MicroRNAs (miRNAs) are small non-coding RNA that play a role in almost all main cellular pathways. miRNAs are involved in the regulation of expression of the major tumor-related genes in carcinogenesis, acting as oncogenes or tumor suppressor genes. The aim of this review was to identify papers published in 2017 investigating the role of miRNAs in HCC tumorigenesis. miRNAs were classified according to their role in the main molecular pathways involved in HCC tumorigenesis: (1) mTOR; (2) Wnt; (3) JAK/STAT; (4) apoptosis; and (5) MAPK. The role of miRNAs in prognosis/response prediction was taken into consideration. Bearing in mind that the analysis of miRNAs in serum and other body fluids would be crucial for clinical management, the role of circulating miRNAs in HCC patients was also investigated. The most represented miRNA-regulated pathway in HCC is mTOR, but apoptosis, Wnt, JAK/STAT or MAPK pathways are also influenced by miRNA expression levels. These miRNAs could thus be used in clinical practice as diagnostic, prognostic or therapeutic targets for HCC treatment.

Excisão cirúrgica de um osteocondroma de raiz nervosa da cauda equina de cão

Background: Spinal neoplasms are classified into extradural, intradural/extramedullary or intramedullary. Intradural/extramedullary tumors include meningiomas and nerve sheath tumors, which arise from meninges or peripheral nerves around the spinal cord. Clinical signs are related to dysfunction of the involved nerve and include pain, nerve root signature and atrophy. Osteochondromas are benign tumors located within the bone, on its surface or in extra-osseous regions, when they are classified as soft tissue osteochondoma. The aim of this study is to describe a case of an osteochondroma in a nervous root of the cauda equina in a dog, whose surgical resection allowed the resolution of the clinical signs.Case: A 12-year-old, male, Labrador Retriever dog, was presented with a 40-day history of progressive, painful, pelvic limb paresis, with no improvement when treated with analgesics and acupuncture. Neurological abnormalities included paraparesis, sometimes worse in the left pelvic limb, that was carried flexed at the level of the stifle, hindlimb atrophy, decreased interdigital reflexes and pain in the lumbar spinal region, mainly over L6 vertebra. Results of blood count and serum biochemical analysis were unremarkable. Computed tomography (CT) of the lumbosacral area was performed and the evaluation of images in transverse and reconstructed dorsal and sagittal planes allowed the visualization of a hyperattenuating and calcified round structure with 8 mm x 6 mm, in the left side of vertebral canal, at the level of caudal epiphysis of L6. Lumbosacral (L7-S1) CT abnormalities, as subchondral sclerosis, mild disc margin bulging, spondylosis deformans and foraminal proliferation were also observed but were considered clinically insignificant. Then, a dorsal L6 laminectomy was performed and after opening of the vertebral canal it was observed the presence of an ovoid mass, in the left side of the medullary cone, measuring approximately 0.9 cm in length, originating from a sacral nerve root. This structure was excised and submitted to histopathological evaluation. The dog improved after surgery without any complications, and neurological functions were gradually recovered. The histopathology of the mass was confirmed as an osteochondroma.Discussion: The history and neurologic examination findings as well the improvement after surgery were compatible with the lesion visualized by TC in L6, rather than the degenerative changes in L7-S1. Regarding the position of the mass, the main differential would be nerve sheath tumor, one of the most common types of primary neoplasia of the peripheral nervous system, however in a CT study in dogs with brachial plexus and nerve roots neoplasms, calcification of the tumor was not observed. Most of benign cartilaginous tumors of soft tissue are chondromas/osteochondromas, and this lesion should not be confused with multiple osteochondroma or multiple cartilaginous exostoses, which have been reported as multifocal, proliferative lesions involving the surface of ribs, vertebrae and pelvis of young dogs. If this lesion is unique, it is called osteochondroma. There are two reports of chondromas into the first lumbar vertebrae of dogs, with adherence to the floor of the vertebral canal and one report of an osteochondroma originated from the left pedicle of T11 in a cat. Radiculopathy and myelopathy caused by osteochondromas inside the spine are rare in humans and description of this tumor arising from nerve roots were not found in the veterinary literature. Probably the origin of the mass was metaplasia of the meninges surrounding the nervous root. In conclusion, neurological examination, CT and surgical exploration allowed the diagnosis and removal of a soft tissue osteochondroma, a rare benign tumor, with good patient recovery.

Analysis of laparoscopic laser liver resection in standardized porcine model.

Hepatocellular carcinoma is a highly prevalent and lethal primary neoplasia of the liver and metastases of other malignancies affect most frequently the liver. Minimally invasive surgical approach for liver resections is advancing. Dissection of liver parenchyma by laparoscopic technique remains challenging and new technologies are in need. Therefore, we asked whether it is feasible to dissect liver tissue comparably in terms of speed and hemostasis with a non-contact 1.9-µm cw-laser device and whether there are differences in the postoperative healing process compared to a gold standard device (ultrasound aspirator) in an experimental model. Laparoscopic laser and ultrasound aspirator standardized partial liver resections were performed in seven pigs. Resection time, hemostasis time, and blood loss were evaluated. After at least 10days, representative specimen of the resection areas was collected via re-laparoscopy and biopsy and side effects like hematoma, abscess, or bilioma were noted. Histologically, coagulation necrosis margin, granulation tissue zone, tissue fibrosis, and giant cell count were analyzed. Laparoscopic laser liver resection was three times faster compared to the laparoscopic ultrasound aspirator. Blood loss was equal in both groups. No side effects like hematoma or bilioma occurred. Histologically, specimen showed the same expansion of coagulation necrosis zone and granulation tissue. Fibrotic scar could be determined in three cases in both groups, respectively. However, giant cell count was significant higher in the laser resection group. The 1.9-µm cw-laser device enables a safe and fast liver resection in an experimental pig model compared to a gold standard (ultrasound aspirator) laparoscopic liver resection method. Wound healing is not interfered by laser liver resection.

Robotic-assisted inguinal lymphadenectomy: a systematic review.

Lymphadenectomy represents the standard treatment for various types of cancer. The introduction of robotics in lymph node dissection may have an important impact on post-lymphadenectomy complications. A systematic literature review was performed. In our review, robotic inguinal lymphadenectomy was performed on 51 patients. Penile squamous cell carcinoma was the most common histological type of the primary neoplasia. No intra-operative complications were reported. One case of conversion to open was reported. The mean duration of hospitalization was 2 days. The duration of drainage ranged from 7 to 72 days. The most common postoperative complications were lymphocele (13.7%), lymphedema (7.8%), cellulitis (7.8%), seroma (3.9%), abscess (3.9%), wound breakdown/wound infection (3.9%), sepsis (1.9%), prolonged lymphorrhea (1 out of 51 patients, 1.9%) and skin necrosis (1 out of 51 patients, 1.9%). Until now there has not been sufficient evidence regarding the role of robotics in groin lymph node dissection, though this approach appears to be safe and oncologically effective, with morbidity rates relatively lower compared to open surgery.