The Object of the Study: The author of the given paper describes an unusual combination of two diseases: extramammary Paget’s disease manifested by intraepithelial adenocarcinoma of the vulva and anus combined with i...The Object of the Study: The author of the given paper describes an unusual combination of two diseases: extramammary Paget’s disease manifested by intraepithelial adenocarcinoma of the vulva and anus combined with invasive adenocarcinoma of the ampullary part of the rectum and describes the atypical manifestations of these diseases. The Content: The content of this research paper includes a description of the patient, an analysis of the clinical picture, diagnostic methods and therapeutic interventions used, a report of the following disease, and the result of the presented case. The Result of the Research Work: The result of the research work is the analysis of a clinical case with two different tumors, where such a combination of tumors is rarely described in the literature. Moreover, no large specific sample with this combination of diseases is available. Patient Characteristics: The given case report describes a patient of the Palliative Care Unit of the Gerontology Clinic with a primary diagnosis of C20-rectal adenocarcinoma in the background of the anal canal, perineal skin Paget’s disease, stage IV. The presented complications of the patient’s primary diagnosis are multiple metastases in the liver;status post palliative chemotherapy;hepatomegaly;metastases to abdominal lymph nodes, inguinal lymph nodes;metastases at Th12, L4 level;pain syndrome. The presented above combination of diagnosed diseases is very rare. Applied Diagnostics: In October 2021, it was performed diagnostic manipulation: biopsy and the pathologist have provided a microscopic description. The first tissue fragment had a pronounced electrothermal lesion and the epithelial structures were not valuable. The second skin tissue fragment was covered with hyperplastic and acanthotic epithelium;its basal and middle layers contained multiple large cells proliferates extending into the medial epidermis, and the cytoplasm of these cells reacted positively with PAS (Periodic Acid Schiff reaction). It needs to be noted that the patient had previously had several years of biopsies from the perineal and anal epidermis, where Paget’s disease had also been diagnosed. The performed immunohistochemistry showed these cells to be CK20 positive, CK7 rare positive and p16 negative. The following pathohistological findings were made: morphological and immunohistochemical picture is consistent with Paget’s disease. According to the ICD-10, the patient was diagnosed with C51 malignant neoplasm of the female external genitalia. Using imaging diagnostics, it became clear that the patient’s rectal adenocarcinoma had progressed to metastatic stage with distant liver metastases in the background of anal canal, perineal skin Paget’s disease. Therapeutic Plan of the Patient: Based on the patient’s main diagnoses, the complications of the principal diagnosis, the patient’s overall severe condition, pain syndrome, age and comorbidities, palliative chemotherapy was approved as a therapeutic option in council of doctors. Monitoring and Outcome of the Patient: The patient’s general condition was becoming worse over time, and she was diagnosed with exitus latalis in December 2022. At that time, the patient was discharged from hospital and was on palliative care at home under the control of her family physician.展开更多
Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease first named by Anhalt, et al. in 1990. The disease is characterized by such distinctive clinical symptoms and signs as severe, painful mucosal eros...Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease first named by Anhalt, et al. in 1990. The disease is characterized by such distinctive clinical symptoms and signs as severe, painful mucosal erosions, polymorphous skin lesions, histopathology hallmarks, and immunological findings. The situation typically presents in patients with lymphoproliferative diseases and primarily malignancies. A main challenge of the study is the relationship between the existence of associated tumors and the autoimmune reaction to the skin. Some researchers suspected that the possible expression of foreign antigens on the tumor can cross react with epidermal antigens inducing the auto-reactive clones of T-lymphocytes. Some speculated that the type of tumors associated with PNP may produce plakin proteins that result in initiation of the immune response. Other reports believed the autoimmune reaction is related to the epitope spreading or to the changing of cytokines. We analyzed 12 PNP patients diagnosed in our department in the past few years. An intensive study to the B cells in the PNP associated with tumors demonstrated that the tumors have structural basis to produce antibody. The similar immunoglobulin heavy chain genes of tumor B-cells in 7 patients strongly suggested that the B cell clones were functional and recognized the same antigen epitope. The autoantibodies secreted by the tumor can react against specific plakin proteins in epidermis, lead to the impairment of cell-cell adhesion, and cause the mucocutanous lesions. The clinical significance of the results indicates the importances of early finding and total resection of the associated tumors, and the usage of IVIG pre or during operation to prevent Bronchitis Obliterans. The new finding is also important for the study of other antibody mediated autoimmune diseases.展开更多
文摘The Object of the Study: The author of the given paper describes an unusual combination of two diseases: extramammary Paget’s disease manifested by intraepithelial adenocarcinoma of the vulva and anus combined with invasive adenocarcinoma of the ampullary part of the rectum and describes the atypical manifestations of these diseases. The Content: The content of this research paper includes a description of the patient, an analysis of the clinical picture, diagnostic methods and therapeutic interventions used, a report of the following disease, and the result of the presented case. The Result of the Research Work: The result of the research work is the analysis of a clinical case with two different tumors, where such a combination of tumors is rarely described in the literature. Moreover, no large specific sample with this combination of diseases is available. Patient Characteristics: The given case report describes a patient of the Palliative Care Unit of the Gerontology Clinic with a primary diagnosis of C20-rectal adenocarcinoma in the background of the anal canal, perineal skin Paget’s disease, stage IV. The presented complications of the patient’s primary diagnosis are multiple metastases in the liver;status post palliative chemotherapy;hepatomegaly;metastases to abdominal lymph nodes, inguinal lymph nodes;metastases at Th12, L4 level;pain syndrome. The presented above combination of diagnosed diseases is very rare. Applied Diagnostics: In October 2021, it was performed diagnostic manipulation: biopsy and the pathologist have provided a microscopic description. The first tissue fragment had a pronounced electrothermal lesion and the epithelial structures were not valuable. The second skin tissue fragment was covered with hyperplastic and acanthotic epithelium;its basal and middle layers contained multiple large cells proliferates extending into the medial epidermis, and the cytoplasm of these cells reacted positively with PAS (Periodic Acid Schiff reaction). It needs to be noted that the patient had previously had several years of biopsies from the perineal and anal epidermis, where Paget’s disease had also been diagnosed. The performed immunohistochemistry showed these cells to be CK20 positive, CK7 rare positive and p16 negative. The following pathohistological findings were made: morphological and immunohistochemical picture is consistent with Paget’s disease. According to the ICD-10, the patient was diagnosed with C51 malignant neoplasm of the female external genitalia. Using imaging diagnostics, it became clear that the patient’s rectal adenocarcinoma had progressed to metastatic stage with distant liver metastases in the background of anal canal, perineal skin Paget’s disease. Therapeutic Plan of the Patient: Based on the patient’s main diagnoses, the complications of the principal diagnosis, the patient’s overall severe condition, pain syndrome, age and comorbidities, palliative chemotherapy was approved as a therapeutic option in council of doctors. Monitoring and Outcome of the Patient: The patient’s general condition was becoming worse over time, and she was diagnosed with exitus latalis in December 2022. At that time, the patient was discharged from hospital and was on palliative care at home under the control of her family physician.
文摘Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease first named by Anhalt, et al. in 1990. The disease is characterized by such distinctive clinical symptoms and signs as severe, painful mucosal erosions, polymorphous skin lesions, histopathology hallmarks, and immunological findings. The situation typically presents in patients with lymphoproliferative diseases and primarily malignancies. A main challenge of the study is the relationship between the existence of associated tumors and the autoimmune reaction to the skin. Some researchers suspected that the possible expression of foreign antigens on the tumor can cross react with epidermal antigens inducing the auto-reactive clones of T-lymphocytes. Some speculated that the type of tumors associated with PNP may produce plakin proteins that result in initiation of the immune response. Other reports believed the autoimmune reaction is related to the epitope spreading or to the changing of cytokines. We analyzed 12 PNP patients diagnosed in our department in the past few years. An intensive study to the B cells in the PNP associated with tumors demonstrated that the tumors have structural basis to produce antibody. The similar immunoglobulin heavy chain genes of tumor B-cells in 7 patients strongly suggested that the B cell clones were functional and recognized the same antigen epitope. The autoantibodies secreted by the tumor can react against specific plakin proteins in epidermis, lead to the impairment of cell-cell adhesion, and cause the mucocutanous lesions. The clinical significance of the results indicates the importances of early finding and total resection of the associated tumors, and the usage of IVIG pre or during operation to prevent Bronchitis Obliterans. The new finding is also important for the study of other antibody mediated autoimmune diseases.