Asunto(s)
Sarcoma de Células Dendríticas Interdigitantes/diagnóstico por imagen , Anciano , Sarcoma de Células Dendríticas Interdigitantes/patología , Células Dendríticas/patología , Humanos , Imagen por Resonancia Magnética , Masculino , Columna Vertebral/diagnóstico por imagen , Columna Vertebral/patologíaAsunto(s)
Endometriosis/patología , Músculo Liso/patología , Neoplasias Ováricas/patología , Tumor de Músculo Liso/patología , Adulto , Endometriosis/diagnóstico por imagen , Femenino , Humanos , Metaplasia/diagnóstico por imagen , Metaplasia/patología , Músculo Liso/diagnóstico por imagen , Neoplasias Ováricas/diagnóstico por imagen , Ovario/diagnóstico por imagen , Ovario/patología , Tumor de Músculo Liso/diagnóstico por imagen , Tomografía Computarizada por Rayos XRESUMEN
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a generally slow-progressing rare disorder of unknown etiology. The direct cause of death in cases of IPPFE is rarely investigated. We experienced an autopsy case of a Japanese man with IPPFE and found aspiration pneumonia to be the major trigger of death. The individual had left vocal cord paralysis at admission, which may have contributed to aspiration pneumonia, and which probably was affected by the fibrous adhesion of the left apex of the chest wall resulting from IPPFE. The prevention of aspiration pneumonia is important for maintaining the respiratory function, especially in IPPFE patients with repeated pneumothorax.
Asunto(s)
Pulmón/patología , Tejido Parenquimatoso/patología , Parálisis de los Pliegues Vocales/complicaciones , Parálisis de los Pliegues Vocales/patología , Anciano , Autopsia , Progresión de la Enfermedad , Fibrosis , Humanos , Masculino , Neumonía por Aspiración/patología , Tomografía Computarizada por Rayos X/métodos , Parálisis de los Pliegues Vocales/diagnósticoRESUMEN
We herein report the case of a 2-year-old girl with neurofibromatosis type 1 (NF1), who presented with a 12-cm mass in the right retroperitoneum and underwent tumor resection. Histologically, the tumor was composed of two distinct components: one was teratoma, showing mature morphology; and the other was embryonal rhabdomyosarcoma. An interphase fluorescence in situ hybridization (FISH) analysis of the rhabdomyosarcoma component revealed the absence of isochromosome 12p. Although it is well known that rhabdomyosarcoma occurs in infantile NF1, and that rhabdomyosarcoma can arise from teratoma as a somatic-type malignancy, to the best of our knowledge, this is the first case of an infantile NF1 patient, who developed rhabdomyosarcoma within a retroperitoneal teratoma. The absence of chromosome 12p alteration suggests a possibility that the rhabdomyosarcoma occurred due to the NF1 background, not as a somatic-type malignancy of germ cell tumor.
Asunto(s)
Neoplasias Primarias Múltiples/patología , Neurofibromatosis 1/patología , Neoplasias Retroperitoneales/patología , Rabdomiosarcoma Embrionario/patología , Teratoma/patología , Preescolar , Femenino , Humanos , Neoplasias Primarias Múltiples/diagnóstico , Neurofibromatosis 1/diagnóstico , Neoplasias Retroperitoneales/diagnóstico , Rabdomiosarcoma Embrionario/diagnóstico , Teratoma/diagnósticoRESUMEN
BACKGROUND: Nephroblastoma (NB) is a malignant embryonal neoplasm derived from nephrogenic blastemal cells. NB usually forms a solid mass, but in extremely rare cases, it may show cystic changes. CASE PRESENTATION: A six-month-old girl with persistent high fevers was found to have pyuria and bacteriuria. Ultrasonography revealed multilocular cysts in the right kidney. Right nephrectomy was performed with cyst wall rupture during surgery. An intraoperative rapid diagnosis, based on peritoneal fluid cytology, confirmed three components of blastemal, stromal, and epithelial cells. The blastemal cells were dyshesive, with scant to no cytoplasm and were the predominant cell type. The spindle-shaped stromal cells were arranged in fascicles. The epithelial cells demonstrated tubular structures. Macroscopically, the resected cystic tumor measured 80 mm in maximum diameter with a prominently thin cyst wall, but solid areas were also apparent. Histologically, the tumor was diagnosed as cystic NB (blastemal-predominant) displaying a triphasic pattern. Hyperchromatic nuclei and apoptotic bodies were found. The clinical stage classification of Japan Wilms Tumor Study group was 3. The patient was treated with chemotherapy and radiotherapy. Tumor recurrence and metastasis have not been observed in the 8 months since surgery. CONCLUSION: This is an extremely rare case of infantile cystic NB. We diagnosed the NB cells that appeared in the peritoneal fluid by intraoperative rapid cytology. Cytological examination proved to be a very useful technique for determining the clinical stage of NB. Additionally, we propose that massive tumor degeneration and necrosis be considered as a pathogenic mechanism of cyst formation in NB.
Asunto(s)
Líquido Ascítico/patología , Neoplasias Renales/patología , Neoplasias Quísticas, Mucinosas y Serosas/patología , Tumor de Wilms/patología , Biopsia , Quimioterapia Adyuvante , Femenino , Humanos , Lactante , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/cirugía , Neoplasias Quísticas, Mucinosas y Serosas/diagnóstico por imagen , Neoplasias Quísticas, Mucinosas y Serosas/cirugía , Nefrectomía , Tomografía Computarizada por Rayos X , Resultado del Tratamiento , Ultrasonografía , Tumor de Wilms/diagnóstico por imagen , Tumor de Wilms/cirugíaRESUMEN
BACKGROUND: The occurrence of malignant lymphoma after delivery is an extremely rare event. Although several cases of Hodgkin lymphoma and B cell lymphoma and a few cases of peripheral T cell lymphoma (PTCL) after delivery have been reported, there are no report of autopsy cases of PTCL in the puerperal period. CASE PRESENTATION: A 32-year-old Japanese woman with a past medical history of atopic dermatitis and bronchial asthma presented with generalized eruptions four days after the delivery of her first child; generalized skin induration and lymphadenopathy subsequently emerged. A skin biopsy specimen showed the diffuse proliferation of atypical lymphoid cells that were immunohistochemically-positive for CD4 but negative for CD8. She was diagnosed as PTCL, not otherwise specified (PTCL, NOS). She died one year and three months after the onset of symptoms. At autopsy, the systemic infiltration of lymphoma cells into the whole body was observed. Unexpectedly, these lymphoma cells were immuno-reactive with CD8 but not with CD4. CONCLUSION: The occurrence and development of PTCL after delivery with the shift from CD4 positivity to CD8 positivity may be associated with not only the selection of resistant subclone as a result of chemotherapy but also the changes of immune status before and after delivery.
Asunto(s)
Linfocitos T CD4-Positivos/patología , Linfocitos T CD8-positivos/patología , Linfoma de Células T Periférico/patología , Periodo Posparto , Adulto , Autopsia , Linfocitos T CD4-Positivos/inmunología , Linfocitos T CD8-positivos/inmunología , Resultado Fatal , Femenino , Humanos , Linfoma de Células T Periférico/inmunología , EmbarazoRESUMEN
Multicentric Castleman disease (MCD) is a systemic inflammatory disease potentially caused by an increase in the serum interleukin-6 (IL-6) level. Idiopathic MCD (iMCD) is histopathologically classified into three types: plasmacytic (PC), mixed, and hypervascular (hyperV) types. Recently, a unique clinical phenotype with a poor prognosis overlap with iMCD, thrombocytopenia, anasarca, fever, renal failure or reticulin fibrosis, and organomegaly (TAFRO syndrome), has been reported from Japan, but its detailed clinicopathological features remain unclear. In this study, we performed a clinicopathological analysis of 70 nodal cases of iMCD with and without TAFRO syndrome (nâ¯=â¯37 versus nâ¯=â¯33). Compared with iMCD without TAFRO, iMCD with TAFRO showed more atrophic lymphoid follicles (LF), greater distances between follicles, increased glomeruloid vascular proliferation within the germinal center, and increased follicular dendritic cells. In addition, the hyperV type in particular demonstrated severe atrophic LF and interfollicular vascular proliferation. Among the mixed-type cases, the serum IL-6 levels in iMCD with TAFRO were significantly higher than those in iMCD without TAFRO. Furthermore, compared to iMCD without TAFRO, the numbers of immunoglobulin G4 (IgG4)-positive and CD38-positive plasma cells were significantly decreased in iMCD with TAFRO.