ABSTRACT
BACKGROUND: Ampullary adenocarcinoma (AAC) is a rare neoplasm that accounts for only 0.2% of all gastrointestinal cancers. Its incidence rate is lower than 6 cases per million people. Different prognostic factors have been described for AAC and are associated with a wide range of survival rates. However, these studies have been exclusively conducted in patients originating from Asian, European, and North American countries. AIM: To evaluate the histopathologic predictors of overall survival (OS) in South American patients with AAC treated with curative pancreaticoduodenectomy (PD). METHODS: We analyzed retrospective data from 83 AAC patients who underwent curative (R0) PD at the National Cancer Institute of Peru between January 2010 and October 2020 to identify histopathologic predictors of OS. RESULTS: Sixty-nine percent of patients had developed intestinal-type AAC (69%), 23% had pancreatobiliary-type AAC, and 8% had other subtypes. Forty-one percent of patients were classified as Stage I, according to the AJCC 8th Edition. Recurrence occurred primarily in the liver (n = 8), peritoneum (n = 4), and lung (n = 4). Statistical analyses indicated that T3 tumour stage [hazard ratio (HR) of 6.4, 95% confidence interval (CI) of 2.5-16.3, P < 0.001], lymph node metastasis (HR: 4.5, 95%CI: 1.8-11.3, P = 0.001), and pancreatobiliary type (HR: 2.7, 95%CI: 1.2-6.2, P = 0.025) were independent predictors of OS. CONCLUSION: Extended tumour stage (T3), pancreatobiliary type, and positive lymph node metastasis represent independent predictors of a lower OS rate in South American AAC patients who underwent curative PD.
ABSTRACT
Liposarcomas are malignant mesenchymal tumours usually located in the retroperitoneum, rarely occurring as a single lesion in the kidney. We present a case of a 59-year-old male patient with a left renal mass detected by computed tomography scan. He underwent radical nephrectomy and the histopathological study reported a primary undifferentiated liposarcoma of the kidney without nodal involvement. After 15 months of surgery, he remained asymptomatic and without evidence of disease recurrence. The objective of this report is to present a case and literature review with current evidence of treatment options and prognostic factors for survival.
ABSTRACT
El schwannoma microquístico reticular (SMR) es una rara variante de schwannoma con predilección por el tracto gastrointestinal; es una neoplasia neural benigna sin potencial significativo de recurrencia. Microscópicamente exhibe un patrón de crecimiento microquístico y reticular de células fusiformes que se anastomosan y entrelazan alrededor de islas de estroma mixoide o colagenoso/hialinizado. La actividad mitótica es baja y no se observan atipia ni necrosis. Por inmunohistoquímica expresa positividad nuclear y citoplásmica para S-100, con positividad variable para proteína fibrilar glial ácida (PGAF). Los diagnósticos diferenciales incluyen el tumor del estroma gastrointestinal y el perineuroma, y en casos con morfología más epitelioide se deben considerar las neoplasias epiteliales. Hasta la fecha los reportes de schwannoma microquístico reticular son pocos, por ello presentamos 2 casos, el primero en el intestino delgado y el segundo en el mesoapéndice (AU)
Reticular microcystic schwannoma (RMS) is a rare variant of schwannoma found most frequently in the gastrointestinal tract; it is a benign neural neoplasm with a low rate of recurrence. Microscopically, it shows a striking microcystic and reticular lesional growth pattern with anastomosing and interlacing strands of spindle cells around islands of myxoid or collagenous/hyalinized stroma. Mitotic activity is low and both atypia and necrosis are absent. Immunohistochemically, there is a strong nuclear and cytoplasmic positivity for S-100 and a variably strong glial fibrillary acidic protein staining (GFAP). Differential diagnoses include gastrointestinal stromal tumour, perineurioma and, in cases with epithelioid morphology, epithelial neoplasms should be considered. There are few reported cases to date. We present 2 cases, one in small bowel and the other in mesoappendix (AU)
Subject(s)
Humans , Male , Middle Aged , Aged , Neurilemmoma/pathology , Intestinal Neoplasms/pathology , Appendiceal Neoplasms/pathology , Diagnosis, Differential , S100 Proteins/analysis , Immunohistochemistry/methodsABSTRACT
Undifferentiated (embryonal) liver sarcoma is a rare tumor about 2% of all malignant liver tumors with a poor prognosis and usually occurs in children, this review aims to assess cases of primary embryonal sarcoma of the liver presented at our institution the past 8 years and improve recognition of its variants and evaluate immunohistochemical characteristics that help differentiated it from other tumors. Six cases of undifferentiated liver sarcoma were histologically evaluated and investigated by immunohistochemistry with a panel of antibodies using the equipment âÅAutostainer Link 48â. Usually masses were on average more than 20 cm, with solid, cystic, mucinous areas. The microscopic features include cells of spindle cell appearance, oval, starry, epithelioid and multinucleated cells densely arranged in a myxoid matrix. Trapped bile ducts and hepatic cords often present in the periphery of tumors. Intracellular and extracellular PAS positive hyaline globules. Immunohistochemistry showed very divergent differentiation.
Subject(s)
Liver Neoplasms/diagnosis , Sarcoma/diagnosis , Adult , Biomarkers, Tumor/metabolism , Child , Female , Humans , Liver/metabolism , Liver/pathology , Liver Neoplasms/metabolism , Liver Neoplasms/pathology , Male , Middle Aged , Peru , Sarcoma/metabolism , Sarcoma/pathologyABSTRACT
El sarcoma indiferenciado (embrionario) hepático es un tumor poco frecuente, alrededor del 2% de todos los tumores malignos del hígado, con un pobre pronóstico y usualmente se presenta en niños, la presente revisión tiene como objetivo evaluarlos casos de sarcoma embrionario primario de hígado presentados en nuestra institución los 8 últimos años y mejorar el reconocimiento de sus variantes y evaluar sus características inmunohistoquímicas que ayuden a diferenciarlo de otros tumores. Seis casos de sarcoma indiferenciado hepático fueron evaluados histológicamente e investigados por inmunohistoquímica con un panel de anticuerpos utilizando el equipo ôAutostainer Link 48õ. Por lo general eran grandes masas en promedio mayor de 20 cm, con áreas sólidas, quísticas y gelatinosas. Las características microscópicas incluyen células de aspecto fusocelular, oval, estrellada, epitelioide o células multinucleadas densamente dispuestos en una matriz mixoide. Conductos biliares atrapados y cordones hepáticos a menudo presentes en la periferia de los tumores. Glóbulos hialinos intracelulares y extracelulares PAS positivos. La inmunohistoquímica mostró diferenciación muy divergente...
Undifferentiated (embryonal) liver sarcoma is a rare tumor about 2% of all malignant liver tumors with a poor prognosis and usually occurs in children, this review aims to assess cases of primary embryonal sarcoma of the liver presented at our institution the past 8 years and improve recognition of its variants and evaluate immunohistochemical characteristics that help differentiated it from other tumors. Six cases of undifferentiated liver sarcoma were histologically evaluated and investigated by immunohistochemistry with a panel of antibodies using the equipment ôAutostainer Link 48õ. Usually masses were on average more than 20 cm, with solid, cystic, mucinous areas. The microscopic features include cells of spindle cell appearance, oval, starry, epithelioid and multinucleated cells densely arranged in a myxoid matrix. Trapped bile ducts and hepatic cords often present in the periphery of tumors. Intracellular and extracellular PAS positive hyaline globules. Immunohistochemistry showed very divergent differentiation...
Subject(s)
Humans , Male , Adult , Female , Child , Middle Aged , Diagnosis, Differential , Immunohistochemistry , Meta-Analysis as Topic , Liver NeoplasmsABSTRACT
El tumor fibrohistiocítico plexiforme (TFHP) es una rara neoplasia mesenquimal de malignidad intermedia, con alta tasa de recidiva local y potencial metastásico ganglionar o pulmonar. Suele afectar tejidos blandos superficiales de extremidades superiores de niños y adultos jóvenes, con predilección femenina. Presentamos el caso de un niño de 10 años con tumoración de crecimiento progresivo, dependiente del plano cutáneo en rodilla derecha con adenopatías inguinales ipsilaterales. Se le realiza resección amplia de la lesión con linfadenectomía inguinocrural. El estudio histopatológico reveló una neoplasia de patrón plexiforme con componente bifásico (células mononucleares tipo histiocitos asociado a células gigantes tipo osteoclasto, CD68 positivas y células fusiformes, actina de músculo liso positivas); favoreciendo el diagnóstico de esta entidad con metástasis ganglionar (AU)
Plexiform fibrohistiocytic tumour (TFHP) is a rare mesenchymal neoplasm of intermediate malignancy but with a high rate of local recurrence and potential for lymph node or pulmonary metastases. It usually affects superficial soft tissues of the upper extremities of children and young adults and is more frequent in females. We report a case of a 10 year old boy with progressive tumour growth, under the skin in the right knee with ipsilateral inguinal lymphadenopathy. He underwent wide resection of the lesion with inguinal lymphadenectomy. Histopathology revealed a plexiform pattern with a biphasic component (mononuclear histiocytic cells associated with CD68 positive osteoclast-like giant cells and smooth muscle actin-positive spindle cells); favoring the diagnosis of TFHP with lymph node metastasis (AU)
Subject(s)
Child , Humans , Male , Neurofibroma, Plexiform/surgery , Neurofibroma, Plexiform , Chondrosarcoma, Mesenchymal/complications , Knee/pathology , Knee , Knee Joint/pathology , Diagnosis, Differential , Soft Tissue Neoplasms/complications , Soft Tissue Neoplasms/pathology , Granuloma Inguinale/complications , Granuloma Inguinale/pathology , Inguinal Canal/pathology , Solitary Fibrous Tumors/complications , Solitary Fibrous Tumors/pathology , Magnetic Resonance Imaging , Magnetic Resonance Spectroscopy/methods , Immunohistochemistry/methodsABSTRACT
El Tumor Fibroso Solitario es una neoplasia mesenquimal muy infrecuente a nivel hepático. Reportamos un nuevo caso en una paciente de 52 años de edad. La imagen tomográfica revelaba una tumoración sólida bien delimitada, localizada en el lóbulo hepático derecho. Macroscópicamente era de bordes regulares, blanquecina, de carácter expansivo. La microscopia reveló una tumoración fusocelular, de vasculatura hemangiopericitoide, con extensas áreas de hialinización; positiva para marcadores de inmunohistoquímica para CD34, CD 99 y Bcl-2. Pese a su rareza, el tumor fibroso solitario debe de ser considerado entre los diagnósticos diferenciales de las tumoraciones fusocelulares a nivel hepático.
Solitary fibrous tumor is a very uncommon mesenchymal neoplasia in the liver. We report a new case in 52 year old patient. The tomographic image revealed a solid, well delimitated tumor, localized at the right hepatic lobe. Macroscopically, it had rounded borders, was expansive and white. Microscopically it had a spindle cell pattern with extensive areas of hyalinization and hemangiopericytic vessels; it was positive with inmunohistochemestry markers for CD34, CD99 and Bcl-2. Despite its rarity, solitary fibrous tumor should be considered among the differential diagnosis of the spindle cell tumors of the liver.
Subject(s)
Humans , Female , Middle Aged , Liver , Immunohistochemistry , Liver Neoplasms , Solitary Fibrous TumorsABSTRACT
Solitary fibrous tumor is a very uncommon mesenchymal neoplasia in the liver. We report a new case in 52 year old patient. The tomographic image revealed a solid, well delimitated tumor, localized at the right hepatic lobe. Macroscopically, it had rounded borders, was expansive and white. Microscopically it had a spindle cell pattern with extensive areas of hyalinization and hemangiopericytic vessels; it was positive with inmunohistochemestry markers for CD34, CD99 and Bcl-2. Despite its rarity, solitary fibrous tumor should be considered among the differential diagnosis of the spindle cell tumors of the liver.
