Giant orbital leiomyoma in a pediatric patient: diagnostic and therapeutic challenge.

This manuscript describes an exceptional case of a long-standing orbital leiomyoma in a 14-year-old male. The tumor was unusually large, causing severe proptosis and significant involvement of the ocular muscles. The patient presented with amaurosis, complete ophthalmoplegia, spontaneous eye pain, and the inability to close the eyelids, leading to psychological distress. Due to the tumor's size and progression, a right orbital exenteration was performed to remove all orbital contents, including the tumor and the eyeball. The surgical procedure aimed to prevent tumor recurrence and improve the patient's quality of life. The histopathological analysis confirmed the diagnosis of orbital leiomyoma. This case presents a particular interest due to the degree of evolution it has reached. Complete tumor excision and long-term follow-up are necessary to prevent recurrence and ensure optimal patient outcomes. This report underscores global healthcare disparities and the complexity of managing rare orbital neoplasms in diverse country settings.

Genetic characterization of intramuscular myxomas.

Introduction: Intramuscular myxomas are benign tumors that are challenging to diagnose, especially on core needle biopsies. Acquired chromosomal aberrations and pathogenic variants in codon 201 or codon 227 in GNAS complex locus gene (GNAS) have been reported in these tumors. Here we present our genetic findings in a series of 22 intramuscular myxomas. Materials and methods: The tumors were investigated for the presence of acquired chromosomal aberrations using G-banding and karyotyping. Pathogenic variants in codon 201 or codon 227 of GNAS were assessed using direct cycle Sanger sequencing and Ion AmpliSeq Cancer Hotspot Panel v2 methodologies. Results: Eleven tumors carried chromosomal abnormalities. Six tumors had numerical, four had structural, and one had both numerical and structural chromosomal aberrations. Gains of chromosomes 7 and 8 were the most common abnormalities being found in five and four tumors respectively. Pathogenic variants in GNAS were detected in 19 myxomas (86%) with both methodologies. The detected pathogenic variants were p.R201H in nine cases (seven with abnormal and two with normal karyotypes), p.R201C in five cases, all with normal karyotypes, p.R201S in three cases (two with abnormal and one with normal karyotype), p.R201G in one case with a normal karyotype, and p.Q227E in one case with a normal karyotype. Conclusion: Firstly, our data indicate a possible association between chromosomal abnormalities and GNAS pathogenic variants in intramuscular myxomas. Secondly, the presence of the rare pathogenic variants R201S, p.R201G and p.Q227E in 26% (5 out of 19) of myxomas with GNAS pathogenic variants shows that methodologies designed to detect only the common "hotspot" of p.R201C and p.R201H will give false negative results. Finally, a comparison between Ion AmpliSeq Cancer Hotspot Panel v2 and direct cycle Sanger sequencing showed that direct cycle Sanger sequencing provides a quick, reliable, and relatively cheap method to detect GNAS pathogenic variants, matching even the most cutting-edge sequencing methods.

Rapidly growing intramuscular lipoma: a unique entity of benign lipomas in children.

This report presents a case of an intramuscular lipoma observed in the left back of a healthy female toddler. It was resected after 3 months of observation because of rapid enlargement, raising suspicion of malignancy. Histopathological examination confirmed a diagnosis of intramuscular lipoma without malignant and blastemal components. Intramuscular lipomas are benign neoplasms that mostly appear as a rapidly growing tumour. Several hypotheses regarding the pathogenesis of this characteristic growth pattern have been proposed, including atrophy of the surrounding muscle, reactive adipocytic neoformation and multiple contractive interactions between the lipoma and the surrounding muscle.

Inflammatory Rhabdomyoblastic Tumor: From a Nebulous Smooth Muscle Neoplasm to a Novel Skeletal Muscle Tumor Subtype.

Inflammatory rhabdomyoblastic tumor is a recently introduced name for neoplasms currently included in the World Health Organization classification of soft tissue tumors under the rubric inflammatory leiomyosarcoma. Inflammatory rhabdomyoblastic tumor is an excellent example of how surgical pathologists working in conjunction with tumor biologists can greatly improve tumor classification to the benefit of patients. Over the last 28 years, understanding of this entity has undergone a fascinating evolution. This review serves as a summary of the latest findings in inflammatory rhabdomyoblastic tumor research and a diagnostic manual for the practicing surgical pathologist.

Inflammatory Rhabdomyoblastic Tumor: Clinicopathologic and Molecular Analysis of 13 Cases.

Inflammatory rhabdomyoblastic tumors (IRMTs) are newly recognized skeletal muscle tumors with uncertain malignant potential. We investigated 13 IRMTs using clinicopathologic, genetic, and epigenetic methods. The cohort included 7 men and 6 women, aged 23 to 80 years (median, 50 years), of whom 2 had neurofibromatosis type 1. Most tumors occurred in the deep soft tissues of the lower limbs, head/neck, trunk wall, and retroperitoneum/pelvis. Two tumors involved the hypopharyngeal submucosa as polypoid masses. Eight tumors showed conventional histology of predominantly spindled cells with nuclear atypia, low mitotic activity, and massive inflammatory infiltrates. Three tumors showed atypical histology, including uniform epithelioid or plump cells and mitotically active histiocytes. The remaining 2 tumors demonstrated malignant progression to rhabdomyosarcoma; one had additional IRMT histology and the other was a pure sarcoma. All 11 IRMTs without malignant progression exhibited indolent behavior at a median follow-up of 43 months. One of the 2 patients with IRMTs with malignant progression died of lung metastases. All IRMTs were positive for desmin and PAX7, whereas myogenin and MyoD1 were expressed in a subset of cases. Targeted next-generation sequencing identified pathogenic mutations in NF1 (5/8) and TP53 (4/8). All TP53 mutations co-occurred with NF1 mutations. TP53 variant allele frequency was much lower than that of NF1 in 2 cases. These tumors showed geographic (subclonal) strong p53 immunoreactivity, suggesting the secondary emergence of a TP53-mutant clone. DNA methylation-based copy number analysis conducted in 11 tumors revealed characteristic flat patterns with relative gains, including chromosomes 5, 18, 20, 21, and/or 22 in most cases. Widespread loss of heterozygosity with retained biparental copies of these chromosomes was confirmed in 4 tumors analyzed via allele-specific profiling. Based on unsupervised DNA methylation analysis, none of the 11 tumors tested clustered with existing reference entities but formed a coherent group, although its specificity warrants further study.

AI-powered radiomics: revolutionizing detection of urologic malignancies.

PURPOSE OF REVIEW: This review aims to highlight the integration of artificial intelligence-powered radiomics in urologic oncology, focusing on the diagnostic and prognostic advancements in the realm of managing prostate, kidney, and bladder cancers. RECENT FINDINGS: As artificial intelligence continues to shape the medical imaging landscape, its integration into the field of urologic oncology has led to impressive results. For prostate cancer diagnostics, machine learning has shown promise in refining clinically-significant lesion detection, with some success in deciphering ambiguous lesions on multiparametric MRI. For kidney cancer, radiomics has emerged as a valuable tool for better distinguishing between benign and malignant renal masses and predicting tumor behavior from CT or MRI scans. Meanwhile, in the arena of bladder cancer, there is a burgeoning emphasis on prediction of muscle invasive cancer and forecasting disease trajectory. However, many studies showing promise in these areas face challenges due to limited sample sizes and the need for broader external validation. SUMMARY: Radiomics integrated with artificial intelligence offers a pioneering approach to urologic oncology, ushering in an era of enhanced diagnostic precision and reduced invasiveness, guiding patient-tailored treatment plans. Researchers must embrace broader, multicentered endeavors to harness the full potential of this field.

Cotyledonoid dissecting leiomyoma of the uterus: a case report and review of the literature.

BACKGROUND: Cotyledonoid dissecting leiomyoma, also named Sternberg tumor, is a rare variant of uterine leiomyoma. The tumor is benign, but the appearance and growth pattern are unusual and alarming. In this article, we report a case of cotyledonoid dissecting leiomyoma in a 55-year-old woman as well as review relevant literature. CASE PRESENTATION: We report a case of cotyledonoid dissecting leiomyoma in a 55-year-old Iranian woman who presented with vaginal bleeding 4 months after menopause. Ultrasound showed two heterogeneous hypoechoic masses on the uterine fundus. Total abdominal hysterectomy and bilateral salpingo-oophorectomy were performed for the patient. Macroscopically, a large heterogeneous intramural mass (140 mm × 120 mm × 120 mm) with a grape-like exophytic mass on the fundus was observed. Her health status was good after surgery, and the patient was discharged from the hospital after 2 days. In a 1-year follow-up period, no recurrence or any other related complications were found. CONCLUSION: It is important to recognize this rare variant of leiomyoma to prevent aggressive and inappropriate overdiagnosis and overtreatment. It is suggested to try to use frozen sections for better diagnosis and to preserve fertility in young women suffering from this lesion.

Solitary fibrous tumor resembling pulmonary fractionation disease: A case report.

RATIONALE: Preoperative differentiation between pulmonary fractionation and solitary fibrous tumors (SFTs) is challenging. Diaphragmatic primary tumors are relatively rare among SFTs, with limited reports of abnormal vascularity. PATIENT CONCERNS: A 28-year-old male patient was referred to our department for surgical resection of a tumor near the right diaphragm, Thoracoabdominal contrast-enhanced computed tomography (CT) scan revealed a 10 × 8 cm mass lesion at the base of the right lung. The inflow artery to the mass was an anomalous vessel in which the left gastric artery bifurcated from the abdominal aorta, and its origin was the common trunk and right inferior transverse artery. DIAGNOSIS: The tumor was diagnosed as right pulmonary fractionation disease based on the clinical findings. The postoperative pathological examination determined a diagnosis of SFT. INTERVENTIONS: The pulmonary vein was used to irrigate the mass. The patient was diagnosed with pulmonary fractionation and underwent surgical resection. Intraoperative findings revealed a stalked, web-like venous hyperplasia anterior to the diaphragm, contiguous with the lesion. An inflow artery was found at the same site. The patient was subsequently treated using a double ligation technique. The mass was partially contiguous with S10 in the right lower lung and stalked. An outflow vein was identified at the same site, and the mass was removed using an automatic suture machine. OUTCOMES: The patient received follow-up examinations that involved a chest CT scan every 6 months, and no tumor recurrence was reported during 1 year of postoperative follow-up. LESSONS: Differentiating between SFT and pulmonary fractionation disease may be challenging during preoperative diagnosis; therefore, aggressive surgical resection should be considered as SFTs may be malignant. Identification of abnormal vessels using contrast-enhanced CT scans may be effective in reducing surgical time and improving the safety of the surgical procedure.

Molecular classification of muscle-invasive bladder cancer based on a simplified immunohistochemical panel using GATA3, CK5/6 and p16.

The choice of therapy for muscle-invasive bladder cancer (MIBC) could be influenced by the tumor's molecular subtype. Currently, well-defined consensus subtypes are based on tumor microarray mRNA data. Clearly defined and easy-to-use surrogate molecular subtypes, based on immunohistochemistry (IHC) performed on whole slides, are needed to make subtyping cost-effective and useful in routine work and future research. To aid in the development of a simple immunohistochemical classifier, a retrospective single-center series of 92 cases of localized bladder cancer was identified. Routine IHC for GATA3, cytokeratins 5 and 6 (CK5/6), and p16 was performed on whole tissue blocks containing muscle-invasive disease. Electronic medical records were retrieved and searched for clinical variables, treatment, and survival data. The mean age was 69.6 years, and 73% were males. Conservative treatment was used in 55% of cases, while cystectomy with chemotherapy was used in 45%. GATA3 and CK5/6 expression divided cases into broad luminal and basal subtypes, respectively, while p16 expression was used to subclassify luminal cases into luminal papillary and luminal unstable types according to the consensus molecular classification. When subtyped in this way, GATA3 and CK5/6 negative cases showed worse overall survival. Molecular subtyping of MIBC on whole slides containing muscle-invasive tumor using only three commonly used, consensus-based antibodies, is a feasible and cost-effective method for detecting subtypes of invasive bladder cancer. Future work combining morphological analysis and IHC is needed to fully translate the consensus molecular classification into a comprehensive, cost-effective subtyping strategy.

Leiomyosarcoma, a rare smooth muscle cancer in the pancreas: An adjuvant radiotherapy treatment approach.

BACKGROUND: Leiomyosarcoma of visceral organs is uncommon, and pancreatic primary occurrence is even rarer. In terms of curative treatment, patients are generally managed with surgery alone, without significant data on the role or efficacy of adjuvant chemotherapy. CASE PRESENTATION: This manuscript presents a case of a 22-year-old female with advanced primary leiomyosarcoma of the pancreas, treated with radical surgery and adjuvant radiation therapy. CONCLUSION: With a low-survival rate, consideration of radiation therapy in some advanced and unresectable cases could be potentially beneficial.

Treatment of muscle-invasive urothelial cancer with nivolumab (CheckMate 274 study): a plain language summary.

WHAT IS THIS SUMMARY ABOUT?: This is a summary of a paper published in a medical journal that describes the results of a study called CheckMate 274. This study looked at a new treatment for muscle-invasive urothelial cancer, a type of cancer found in the urinary tract that has spread from the inner lining of the urinary tract or bladder and into the surrounding muscle wall where it can then spread to other parts of the body. The standard treatment for muscle-invasive urothelial cancer is surgery to remove affected parts of the urinary tract. However, cancer returns in more than half of people after this surgery. Adjuvant therapy is given to people after surgery with muscle-invasive urothelial cancer with a goal to reduce the risk of the cancer coming back; however, at the time this study started, there was no standard adjuvant treatment. WHAT HAPPENED IN THE STUDY?: In the CheckMate 274 study, researchers compared nivolumab with a placebo as an adjuvant treatment for people with muscle-invasive urothelial cancer. The aim of the study was to understand how well nivolumab worked to reduce the chance of the cancer returning after surgery. The study also looked at what side effects (unwanted or unexpected results or conditions that are possibly related to the use of a medication) people had with treatment. WHAT DO THE RESULTS MEAN?: The results showed that people who received nivolumab versus placebo: Survived longer before the cancer was detected again, including people who had programmed death ligand-1 (shortened to PD-L1) on their cancer cells. Survived longer before a secondary cancer outside of the urinary tract was detected. Experienced no differences in health-related quality of life (the impact of the treatment on a person's mental and physical health). Had similar side effects to the people who received nivolumab in other studies. Clinical Trial Registration: NCT02632409 (ClinicalTrials.gov).

Leiomyoma presenting as an anterior vaginal mass.

Leiomyoma, otherwise known as a fibroid, is commonly encountered in the uterus. Vaginal leiomyomas are extremely rare with a relative paucity of cases reported in the literature. Due to the rarity of the disease and complexity of the vaginal anatomy, definitive diagnosis and treatment are challenging. The diagnosis is often only made postoperatively after resection of the mass. They usually arise from the anterior vaginal wall and women may present with dyspareunia, lower abdominal pain, vaginal bleeding or dysuria. Imaging with a transvaginal ultrasound scan and MRI can confirm the vaginal origin of the mass. Surgical excision is the treatment of choice. The diagnosis is confirmed following histological assessment. The authors present the case of a woman in her late 40s presenting to the gynaecology department with an anterior vaginal mass. Further investigation with a non-contrast MRI was suggestive of a vaginal leiomyoma. She underwent surgical excision. Histopathological features were in keeping with the diagnosis of a hydropic leiomyoma. A high index of clinical suspicion is required to establish the diagnosis as it can be mistaken for a cystocele, Skene duct abscess or Bartholin gland cyst. Although it is a benign entity, local recurrence following incomplete resection and sarcomatous changes have been reported.

Risk factors associated with surgical site infection in patients with musculoskeletal cancer / Fatores de risco associados à infecção de sítio cirúrgico em portadores de câncer musculoesquelético / Factores de riesgo asociados a la infección del sitio quirúrgico en pacientes con cáncer musculoesquelético

Background and objectives: surgical site infections (SSI) continue to be a major concern in orthopedic oncology and pose as great a challenge as cancer recurrence, despite the preventive potential of surgery. SSI can be avoided if evidence-based measures are taken. The objective was to assess the frequency of infections in oncological orthopedic surgeries and associated risk factors and inflammatory markers in a reference hospital in the state of Pernambuco. Methods: the frequency of SSI, the identification of isolated microorganisms, the risk factors and the profile of Th1 and Th2 inflammatory markers (IL-2, IL-4, IL-6, IL-10, TNF and IFN-Ƴ) in patients with musculoskeletal cancer were analyzed. Results: SSI were found in 9.1% of patients undergoing orthopedic surgery. Bivariate analysis revealed that a surgical team comprising more than five members (p=0.041) and the need for intraoperative transfusion (p=0.012) were correlated with a higher risk of SSI. The measurement of ultrasensitive C-reactive protein levels to assess the inflammatory response after SSI showed results that were superior to the reference values for each sample, ranging from >5 to >200mg/dl by the immunoturbidimetric method. Of the IL-2, INFγ and TNF (Th1) and IL-4, IL-6, IL-10 (Th2) levels, only interleukin 6 showed high levels, between 6.68 and 58.76 pg/mL. Conclusion: the study found that surgical team with five or more members and blood transfusion were factors associated with the development of SSI in orthopedic surgery in patients with musculoskeletal cancer. Among the inflammatory markers, interleukin 6 (IL-6) showed the highest correlation with the outcome.(AU)

Justificativa e objetivos: as infecções do sítio cirúrgico (ISC) continuam sendo uma grande preocupação na oncologia ortopédica e representam um desafio tão grande quanto a recorrência do câncer, apesar do potencial preventivo da cirurgia. As ISC podem ser evitadas se forem tomadas medidas baseadas em evidências. O objetivo foi avaliar a frequência de infecções em cirurgias ortopédicas oncológicas e os fatores de risco e marcadores inflamatórios associados em um hospital de referência no estado de Pernambuco. Métodos: foram analisados a frequência de ISC, a identificação de microrganismos isolados, os fatores de risco e o perfil de marcadores inflamatórios Th1 e Th2 (IL-2, IL-4, IL-6, IL-10, TNF e IFN-Ƴ) em pacientes portadores de câncer musculoesquelético. Resultados: as ISC foram encontradas em 9,1% dos pacientes submetidos à cirurgia ortopédica. A análise bivariada revelou que uma equipe cirúrgica composta por mais de cinco membros (p=0,041) e a necessidade de transfusão intraoperatória (p=0,012) foram correlacionadas com maior risco de ISC. A dosagem dos níveis de proteína C reativa ultrassensível para avaliação da resposta inflamatória após ISC apresentou resultados superiores aos valores de referência para cada amostra, variando de >5 a >200mg/dl pelo método imunoturbidimétrico. Dos níveis de IL-2, INFγ e TNF (Th1) e IL-4, IL-6, IL-10 (Th2), apenas a interleucina 6 apresentou níveis elevados, entre 6,68 e 58,76 pg/mL. Conclusão: o estudo constatou que equipe cirúrgica com cinco ou mais membros e transfusão sanguínea foram fatores associados ao desenvolvimento de ISC em cirurgia ortopédica em pacientes com câncer musculoesquelético. Entre os marcadores inflamatórios, interleucina 6 (IL-6) apresentou maior correlação com o desfecho.(AU)

Justificación y objetivos: las infecciones del sitio quirúrgico (ISQ) siguen siendo una preocupación importante en la oncología ortopédica y representan un desafío tan grande como la recurrencia del cáncer, a pesar del potencial preventivo de la cirugía. Las ISQ se pueden prevenir si se toman medidas basadas en la evidencia. El objetivo fue evaluar la frecuencia de infecciones en cirugías ortopédicas oncológicas y los factores de riesgo y marcadores inflamatorios asociados en un hospital de referencia en el estado de Pernambuco. Métodos: se analizaron la frecuencia de ISQ, la identificación de microorganismos aislados, los factores de riesgo y el perfil de marcadores inflamatorios Th1 y Th2 (IL-2, IL-4, IL-6, IL-10, TNF e IFN-Ƴ) en pacientes con cáncer musculoesquelético. Resultados: se encontraron ISQ en el 9,1% de los pacientes sometidos a cirugía ortopédica. El análisis bivariado reveló que un equipo quirúrgico compuesto por más de cinco miembros (p=0,041) y la necesidad de transfusión intraoperatoria (p=0,012) se correlacionaron con un mayor riesgo de ISQ. La medición de los niveles de proteína C reactiva ultrasensible para evaluar la respuesta inflamatoria después de la ISQ presentó resultados superiores a los valores de referencia para cada muestra, variando de >5 a >200 mg/dl por el método inmunoturbidimétrico. De los niveles de IL-2, INFγ y TNF (Th1) e IL-4, IL-6, IL-10 (Th2), solo la interleucina 6 mostró niveles elevados, entre 6,68 y 58,76 pg/mL. Conclusión: el estudio encontró que el equipo quirúrgico con cinco o más miembros y la transfusión el estudio encontró que un equipo quirúrgico con cinco o más miembros y transfusión de sangre fueron factores asociados con el desarrollo de ISQ en cirugía ortopédica en pacientes con cáncer musculoesquelético. Entre los marcadores inflamatorios, la interleucina 6 (IL-6) mostró la mayor correlación con el resultado.(AU)

Malignant ectomesenchymoma in children: The European pediatric Soft tissue sarcoma Study Group experience.

Malignant ectomesenchymoma (MEM) is an extremely rare soft tissue tumor typical of young children, currently included in the category of skeletal muscle malignancies and characterized by a neuroblastic component. This study describes a series of 10 patients prospectively registered in the European paediatric Soft tissue sarcoma Study Group (EpSSG) database Of the 10 cases, seven had an initial local diagnosis of rhabdomyosarcoma. All patients received chemotherapy according to rhabdomyosarcoma strategy, four had radiotherapy. Overall, six patients were alive in first remission, two in second remission and one after second tumor. Only the patient with initially metastatic tumor died of disease.

Muscle mass loss in breast cancer patients of reproductive age (≤ 45 years) undergoing neoadjuvant chemotherapy.

PURPOSE: Loss of muscle mass is associated with negative clinical outcome in breast cancer (BC) patients. Therefore, the aim of the study is to evaluate if there is pectoralis muscle area (PMA) depletion, reflecting loss of muscle mass, in breast cancer patients of reproductive age (≤ 45 years) undergoing neoadjuvant chemotherapy (NAC) and to correlate PMA with clinical and histopathological data. MATERIAL AND METHODS: This monocentric study, approved by our institutional review board, enrolled a total of 52 consecutive patients (mean age 37 ± 4.96 years) with histologically proven primary breast cancer between January 2019 and September 2021, treated with NAC and in whom tumor response and PMA were assessed with breast MRI. Two radiologists calculated PMA before and after NAC independently and blindly on axial 3D FLASH pre-contrast T1-weighted images. Wilcoxon-Mann-Whitney U test compared median values and percentage changes of pectoralis muscle area at the beginning and at the end of NAC (158 ± 25.5 days). Multivariate regression analysis on ΔPMA (difference between PMA pre-NAC and PMA post-NAC) was done according to clinical and histopathological data. Inter-reader and intra-reader agreement was estimated with K statistics. RESULTS: Pre-NAC PMA mean value was larger than post-NAC PMA mean value (9.6 ± 2.6 cm2 vs. 8.7 ± 2.2 cm2, p < 0.001, delta value 1.41). According to the RECIST criteria, no significant differences between complete and partial response were found. Multivariate regression analysis did not show any significant relationships between ΔPMA and age, time between MRI examinations, estrogen and progesterone receptor status, human epidermal growth factor receptor 2 status, Ki-67 expression, lymph node involvement, RECIST criteria, histological type, and different regimes of NAC. Inter-reader (k = 0.74) and intra-reader agreement (0.67 and 0.73) in PMA assessment was good. CONCLUSIONS: PMA variation in BC young patients, directly estimated on breast MRI, could be a potential tool to monitor body composition during NAC with potential implications in improving outcome.

Tumor de células granulares com acometimento pericárdico / Granular cell tumor with pericardial involvement. Final paper

Objetivo: Apresentar um raro caso de tumor de células granulares, também conhecido como tumor de Abrikossoff com acometimento pericárdico. Relato do caso: Paciente de 69 anos, com história de tumor de células granulares maligno, com lesão lombar ressecada em 1998 e paraesternal ressecada em 2000 e agora descobriu uma massa pericárdica. Refere dor torácica leve, dispneia aos mínimos esforços, perda ponderal importante por hiporexia, eventualmente rosto avermelhado sem edema. História de hipertensão, asma brônquica e tabagismo, 11 maços/ano. Murmúrio vesicular reduzido na base pulmonar direita, sibilos na ausculta pulmonar, bulhas hipofonéticas, circulação colateral visível em tórax. Discussão: Na ecocardiografia visualiza-se lesão homogênea, isoecogênica, de contornos imprecisos envolvendo o folheto pericárdico visceral das paredes inferior, ínfero-lateral e ântero-lateral do ventrículo esquerdo associado a derrame pericárdico moderado sem sinais de elevação da pressão intrapericárdica. Na tomografia computadorizada evidencia massa pericárdica, heterogênea, com áreas hipodensas de permeio, de limites imprecisos, associada a derrame pericárdico. Há também derrame pleural a direita. O diagnóstico histopatológico da lesão foi de tumor de células granulares, com comportamento maligno devido as atipias celulares. O aspecto histológico era semelhante as amostras anteriores da lesão lombar e paraesternal, podendo corresponder a metástase. Na imunohistoquímica mostrou a expressão da proteína S-100. O tumor de células granulares é um tumor incomum de partes moles, sendo que apenas 2% são malignos, os locais mais comuns são a língua e o dorso, sendo o acometimento pericárdico raríssimo. Suspeita que tenha origem neural, devido a expressão da proteína S-100. Comumente se manifesta como uma lesão nodular, medindo entre 1 e 2 cm de diâmetro. O tratamento preconizado é a remoção cirúrgica total da lesão. Conclusão: O diagnóstico de tumor de células granulares com acometimento pericárdico é difícil, mas deve ser considerado especialmente nos casos em que o paciente já apresenta o tumor em outras partes. O diagnóstico definitivo é histopatológico e os exames de imagem servem para guiar a biópsia e avaliar a extensão da doença

Objective: Report a rare case of granular cell tumor, also known as Abrikossoff's tumor with pericardial involvement. Case report: A 69-year-old patient with a history of malignant granular cell tumor. She had a lumbar lesion resected in 1998 and a parasternal lesion in 2000 and now she has discovered a pericardial mass. She reports mild chest pain, dyspnea on minimal exertion, significant weight loss due to hyporexia, sometimes reddened face without edema. History of hypertension, asthma and smoking, 11 packyears. Reduced breath sounds in the right lung base, wheezing on pulmonar auscultation, hypophonetic heart sounds, visible colateral circulation in the chest. Discussion: The echocardiography showed a homogeneous, isoechoic lesion with imprecise contours involving the visceral pericardium of the inferior, inferolateral and anterolateral walls of the left ventricle associated with moderate pericardial effusion. Computed tomography showed a heterogeneous pericardial mass with hypodense areas, imprecise limits, associated with pericardial effusion. There is also a right pleural effusion. The histopathological result was granular cell tumor with malignant behavior due to cytological atypia. The histological aspect was similar to previous samples of lumbar and parasternal lesion, possibly corresponding to metastasis. In immunohistochemistry showed expression of S-100 protein. Granular cell tumor is an uncommon soft tissue tumor, only 2% are malignant. The most common sites are the tongue and the back, while pericardial involvement is very rare. It is suspected that the lesion has a neural origin due to the expression of the S-100 protein. It commonly presents as a nodular lesion measuring between 1 and 2 cm in diameter. The recommended treatment is the total surgical removal of the lesion. Conclusion: The diagnosis of granular cell tumor with pericardial involvement is difficult, but it should be considered especially when the patient already has the tumor in other sites. The definitive diagnosis is histopathological and imaging tests are used to guide the biopsy and assess the extent of the disease

Laparoscopic resection of a rare diaphragmatic haemangioma: a case report.

This case report describes the laparoscopic resection of a rare diaphragmatic haemangioma. A 45-year-old male patient was diagnosed incidentally with a left subphrenic mass by computed tomography. Laparoscopic left subphrenic mass excision was performed under general anaesthesia. A phrenic haemangioma was confirmed by postoperative pathology. Tumours originating in the diaphragm are rare, with only approximately 200 cases reported in the past century. The diaphragmatic tumour was determined to be primary because intraoperative imaging showed that the tumour was relatively isolated and had no obvious relationship with the surrounding tissues and organs.