Surgical Management of a Giant Inflammatory Hepatocellular Adenoma in a Young Female.

Hepatocellular adenomas are rare and benign primary neoplasms of phenotypically mature hepatocytes. Our understanding of this pathology has greatly improved due to advances in molecular and anatomic knowledge. This article provides an in-depth review of hepatic adenomas (HCA) while presenting the case of a 20-year-old patient with a giant inflammatory hepatocellular adenoma with an atypical presentation, in whom surgical intervention was performed via right hepatectomy. In the post-surgical course, the patient had an in-hospital stay of three days with no complications. During outpatient monitoring via laboratory tests and imaging at eight months, the patient did not present any trace of recurrence.

Clinicopathological Characteristics and the First Mutational Analysis of Gastrointestinal Stromal Tumors From Mexico: A Single Institution Experience.

Background Gastrointestinal stromal tumors (GISTs) arise from Cajal's interstitial cell precursors and display a variety of genetic mutations, primarily in the KIT and PDGFRA genes. These mutations are linked to tumor location, prognosis, and response to treatment. This study delves into the mutational patterns of GISTs in a Mexican population and their impact on overall survival (OS) and disease-free survival (DFS). Methodology This retrospective study examined 42 GIST cases diagnosed at the Oncology Hospital of the National Medical Center XXI Century between January 2018 and December 2020. Clinical, histological, and immunohistochemical data were gathered, and mutational analysis of KIT and PDGFRA genes was conducted using second-generation sequencing. Results The study group consisted of 52.4% females and 47.6% males, with an average age of 62.6 years. The most common tumor site was the stomach (59.5%), followed by the small intestine (26.2%). KIT mutations were detected in 71.4% of cases, predominantly involving exon 11. PDGFRA mutations were observed in 7.1% of cases. Recurrence was noted in 9.5% of patients, all with high-risk tumors. No significant link was identified between specific mutations and OS or DFS. Conclusions This investigation sheds light on the genetic landscape of GISTs in the Mexican population. While no significant association was established between particular mutations and survival outcomes, the study emphasizes the importance of molecular profiling in treatment decision-making. Further studies with larger sample sizes and longer follow-up periods are necessary to validate these results and explore their clinical relevance.

A Giant Malignant Solitary Fibrous Tumor of the Pancreas: A Case Report and Review of the Literature.

We present a case report of a giant solitary fibrous tumor (SFT) with a review of the literature and discuss its biological features and diagnosis. A 43-year-old man presented to our emergency department with abdominal pain and distension with an evolution of two days. Contrast-enhanced computed tomography (CT) showed a large, well-circumscribed semisolid mass (12 cm x 10 cm x 12 cm) localized in the pancreatic head. The histological diagnosis obtained by endoscopic ultrasound-guided trans-duodenal tumor biopsy with fine-needle aspiration showed proliferating short spindle-shaped cells, suggesting a mesenchymal neoplasia of low grade. We proceeded to a Whipple surgical technique. The histopathological study of the resected tumor confirmed proliferating spindle-shaped cells in the tissue, and one mitotic figure was observed in 10 high-power fields (HPFs). Immunostaining was positive for CD34 and STAT-6. The histological diagnosis was a malignant pancreatic SFT. In the six months posterior to the surgical procedure, the patient has been free of recurrent disease. Preoperative diagnosis is difficult and requires comprehensive evidence including clinical, immunohistochemistry, and histological features. Since there are currently no recognized best practices, we advise total surgical excision and careful clinical monitoring.

[Extremity soft tissue sarcoma: does surgical margin impact survival?] / Sarcomas de tejidos blandos en las extremidades: ¿el margen quirúrgico impacta la sobrevida?

Objective: To define the impact of surgical margins on local recurrence (LR), distant recurrence (DR) and overall survival (OS) in patients with soft tissue sarcomas of the extremities (eSTS). Method: Patients treated for a primary eSTS from 2006 to 2010 were analyzed. Rates of local recurrence, distant recurrence, and overall survival were estimated using the Kaplan-Meier method. The association of possible prognostic factors such as local recurrence, metastasis, and survival was performed using the Cox proportional hazards model. Results: 128 patients were analyzed. The surgical margins were positive (R1 resection) in 22.7% and negative in 77.3%. The LR was 27%, the DR was 13% (70% of the population was free of disease at 5 years) and OS at 5 years was 84%. The prognostic factors for OS at 5 years were clinical stage, type and histological grade. The surgical margin had no impact on OS. Conclusions: Although an adequate oncological resection cannot be underestimated, this should be considered in the decision of the optimal treatment of eSTS when amputation or significant functional impairment of the limb is required to obtain negative surgical margins.

Objetivo: Definir el impacto de los márgenes quirúrgicos sobre la recurrencia local (RL), la recurrencia a distancia (RD) y la supervivencia global (SG) en pacientes con sarcomas de tejidos blandos de las extremidades (STBe). Método: Se analizaron pacientes tratados por un STBe primario desde 2006 hasta 2010. Las tasas de recurrencia local, recurrencia a distancia y sobrevida global se estimaron mediante el método de Kaplan-Meier. La asociación de posibles factores pronósticos como recidiva local, metástasis y supervivencia se realizó mediante el modelo de riesgos proporcionales de Cox. Resultados: Se analizaron 128 pacientes. Los márgenes quirúrgicos fueron positivos (resección R1) en el 22.7% y negativos en el 77.3%. La RL fue del 27% y la RD fue del 13% (el 70% de la población está libre de enfermedad a 5 años) y la SG a 5 años fue del 84%. Los factores pronósticos para la SG a 5 años fueron el estadio clínico, el tipo y el grado histológico. El margen quirúrgico no tuvo impacto en la SG. Conclusiones: Aunque no se puede subestimar una resección oncológica adecuada, esto se debe considerar en la decisión del tratamiento óptimo de los STBe cuando se requiere una amputación o un deterioro funcional significativo de la extremidad para obtener márgenes quirúrgicos negativos.

Desmoid tumors: diagnostic and therapeutic considerations.

A literature review on desmoid tumors was carried out, which are tumors that affect soft tissues with a locally aggressive behavior and are unable to metastasize. Sporadic cases are located on the extremities and chest wall; hereditary cases have an intra-abdominal predilection, and those associated with pregnancy occur on the abdominal wall. Imaging techniques assess disease extension. Trucut biopsy is the study of choice for diagnosis. Mutations in the CTNNB1 or APC genes cause an abnormal accumulation of b-catenin within the cell. In this review, an emphasis is made on therapeutic strategies' evolution and change, and current tools for decision making are analyzed, as well as clinical outcomes. Radiation therapy can play a therapeutic or adjuvant role. Advances in the understanding of the disease have allowed establishing better targeted treatments with lower morbidity; however, there are still unanswered questions regarding the choice of the ideal candidate for surveillance and/or early treatment. Data related to quality of life are also presented, as well as the uncertainty generated by this diagnosis for both doctor and patient.

Se realizó una revisión bibliográfica de los tumores desmoides, lo cuales afectan los tejidos blandos con un comportamiento localmente agresivo sin capacidad de producir metástasis. Los casos esporádicos se localizan en extremidades y pared torácica; los casos hereditarios tienen predilección intraabdominal y los asociados con el embarazo en la pared abdominal. Las técnicas de imagen evalúan la extensión de la enfermedad. La biopsia con aguja trucut es el estudio de elección para el diagnóstico. Las mutaciones en el gen CTNNB1 o en el gen de APC provocan acumulación anormal de betacatenina en la célula. En esta revisión se hace énfasis en la evolución y cambio de las estrategias terapéuticas y se analizan las actuales herramientas para la toma de decisiones, así como los resultados clínicos. La radioterapia puede tener un papel terapéutico o adyuvante. Los avances en la comprensión de la enfermedad han permitido establecer tratamientos mejor dirigidos y con menor morbilidad; sin embargo, aún existen interrogantes en cuanto a la elección del candidato ideal para la vigilancia o el tratamiento precoz. También se presentan datos relacionados con la calidad de vida y la incertidumbre que genera el diagnóstico en el médico y el paciente.

Tumores desmoides: consideraciones diagnóstico-terapéuticas / Desmoid tumors: diagnostic and therapeutic considerations

Resumen Se realizó una revisión bibliográfica de los tumores desmoides, lo cuales afectan los tejidos blandos con un comportamiento localmente agresivo sin capacidad de producir metástasis. Los casos esporádicos se localizan en extremidades y pared torácica; los casos hereditarios tienen predilección intraabdominal y los asociados con el embarazo en la pared abdominal. Las técnicas de imagen evalúan la extensión de la enfermedad. La biopsia con aguja trucut es el estudio de elección para el diagnóstico. Las mutaciones en el gen CTNNB1 o en el gen de APC provocan acumulación anormal de betacatenina en la célula. En esta revisión se hace énfasis en la evolución y cambio de las estrategias terapéuticas y se analizan las actuales herramientas para la toma de decisiones, así como los resultados clínicos. La radioterapia puede tener un papel terapéutico o adyuvante. Los avances en la comprensión de la enfermedad han permitido establecer tratamientos mejor dirigidos y con menor morbilidad; sin embargo, aún existen interrogantes en cuanto a la elección del candidato ideal para la vigilancia o el tratamiento precoz. También se presentan datos relacionados con la calidad de vida y la incertidumbre que genera el diagnóstico en el médico y el paciente.

Abstract A literature review on desmoid tumors was carried out, which are tumors that affect soft tissues with a locally aggressive behavior and are unable to metastasize. Sporadic cases are located on the extremities and chest wall; hereditary cases have an intra-abdominal predilection, and those associated with pregnancy occur on the abdominal wall. Imaging techniques assess disease extension. Trucut biopsy is the study of choice for diagnosis. Mutations in the CTNNB1 or APC genes cause an abnormal accumulation of b-catenin within the cell. In this review, an emphasis is made on therapeutic strategies’ evolution and change, and current tools for decision making are analyzed, as well as clinical outcomes. Radiation therapy can play a therapeutic or adjuvant role. Advances in the understanding of the disease have allowed establishing better targeted treatments with lower morbidity; however, there are still unanswered questions regarding the choice of the ideal candidate for surveillance and/or early treatment. Data related to quality of life are also presented, as well as the uncertainty generated by this diagnosis for both doctor and patient.

Adrenal schwannoma in a female patient; A rare benign neoplasm: Case report and literature review.

INTRODUCTION: Schwannomas are rare, slow-growing, usually benign tumors that originate from myelin-producing Schwann cells. Adrenal schwannomas are an exceptionally rare subset of these tumors, with few cases reported in the literature. PRESENTATION OF CASE: We present the case of a 44-year old female patient being evaluated for chronic abdominal pain at the outpatient clinic. Clinical and laboratory workup was unremarkable. An abdominal CT scan was performed, revealing a left suprarenal solid mass (5 × 6 cm). Surgical resection of the adrenal gland was performed, given the patient's symptoms, the size of the tumor, and its malignant potential. The patient completed the postoperative period satisfactorily, and her symptoms improved. Histopathological findings were compatible with a benign adrenal schwannoma. DISCUSSION: Schwannomas generally appear in the head, neck and extremities, with the vestibulocochlear nerve being the most frequently involved site. Retroperitoneal schwannomas account for 1-5% of retroperitoneal masses and comprise only 1-3% of all schwannomas. Their incidence increases with age, from 4% in the general population, reaching 7% in patients over 70 years of age. CONCLUSION: Adrenal incidentalomas represent a diagnostic challenge. Because of the malignant potential of large (> 4 cm) adrenal masses and the lack of characteristic findings using conventional imaging techniques and laboratory diagnostic tools, surgical excision with histopathology and immunohistochemistry analysis are required for definitive diagnosis and optimal management.

Desmoid-Type Fibromatosis.

Desmoid tumors represent a rare entity of monoclonal origin characterized by locally aggressive behavior and inability to metastasize. Most cases present in a sporadic pattern and are characterized by a mutation in the CTNNB1 gene; while 5-15% show a hereditary pattern associated with APC gene mutation, both resulting in abnormal ß-catenin accumulation within the cell. The most common sites of presentation are the extremities and the thoracic wall, whereas FAP associated cases present intra-abdominally or in the abdominal wall. Histopathological diagnosis is mandatory, and evaluation is guided with imaging studies ranging from ultrasound, computed tomography or magnetic resonance. Current approaches advocate for an initial active surveillance period due to the stabilization and even regression capacity of desmoid tumors. For progressive, symptomatic, or disabling cases, systemic treatment, radiotherapy or surgery may be used. This is a narrative review of this uncommon disease; we present current knowledge about molecular pathogenesis, diagnosis and treatment.

Gallbladder osteoma in a 66-year-old female; Case report and review of literature.

INTRODUCTION: Gallbladder mesenchymal tumours are rare. The more common include fibroma, lipoma or haemangioma. A gallbladder osteoma is very rare indeed there is only one other case reported in medical literature). We report a new case. PRESENTATION OF CASE: A 66-year-old female presented to the emergency department complaining of colicky epigastric pain and generalised abdominal discomfort for 1 month. The pain was scored 5/10 but there were no associated symptoms of fever, nausea or vomiting. Vital signs were normal as were all laboratory parameters. An abdominal ultrasound revealed a thin walled gallbladder with a solitary 3 mm polyp. Motility studies confirmed gallbladder dyskinesia. Laboratory studies were ordered reporting normal findings. Abdominal ultrasound was ordered reporting an image suggesting a gallbladder polyp and gallbladder dyskinesia. Cholecystectomy was done without any incidents and the gallbladder was sent to pathology. Pathology reported mature bone tissue in the stromal gallbladder tissue. Due to the rareness of the presence of mature bone tissue in the gallbladder we decided to report the case for the medical community. DISCUSSION: Osteomas are mesenchymal cell tumors derived from the mesoderm; the gallbladder may be the primary site of numerous types of mesenchymal tumors, although these tumors are common the location is not. CONCLUSIONS: We report only the second case of gallbladder osteoma. These mesenchymal tumours are common but this location is not. This case report will serve to remind readers of both benign osteomas and unusual conditions causing gallbladder disease.