TY - JOUR
T1 - Neoplasms associated with paraneoplastic pemphigus
T2 - A review with emphasis on non-hematologic malignancy and oral mucosal manifestations
AU - Kaplan, Ilana
AU - Hodak, Emmilia
AU - Ackerman, Lehavit
AU - Mimouni, Daniel
AU - Anhalt, Grant J.
AU - Calderon, Shlomo
PY - 2004/7
Y1 - 2004/7
N2 - The review included 163 cases of paraneoplastic pemphigus (PNP) reported between 1990 and 2003, including a new unique case of PNP associated with occult breast cancer and an ovarian cyst of borderline malignancy. Hematologic-related neoplasms or disorders were associated with 84% of the cases, with non-Hodgkin lymphoma (38.6%) as the most frequent, followed by chronic lymphocytic leukemia (18.4%) and Castleman's disease (18.4%). The non-hematologic neoplasms comprised 16% of all cases: epithelial origin-carcinoma (8.6%), mesenchymal origin-sarcoma (6.2%), and malignant melanoma (0.6%). Carcinoma cases comprised 58% of the non-hematologic neoplasms. Carcinoma cases (n=14) consisted of adenocarcinoma (n=7), squamous cell carcinoma (n=2), multiple skin tumors probably basal cell carcinoma (n=1), and bronchogenic carcinoma (n=1). Of the 10 (6.2%) sarcoma cases, there was one case each of leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, poorly differentiated sarcoma, reticulum cell sarcoma, dendritic cell sarcoma and inflammatory myofibroblastic tumor. The oral mucosa was involved in all of cases. Isolated oral ulcerations were the first sign in 45% of the cases. Diffuse and persistent oral ulcerations with a progressive course could be a sign of malignancy, either recognized or occult. In the absence of a clear diagnosis, malignancy should be suspected and extensive work-up performed. The full spectrum of signs of PNP may not be present initially. Repeated biopsies, direct and indirect immunofluorescence as well as screening indirect immunofluorescence on murine bladder are required for diagnosis. Clinicians should be highly suspicious when signs and symptoms suggestive of PNP are present in cancer patients, of hematologic and non-hematologic origin.
AB - The review included 163 cases of paraneoplastic pemphigus (PNP) reported between 1990 and 2003, including a new unique case of PNP associated with occult breast cancer and an ovarian cyst of borderline malignancy. Hematologic-related neoplasms or disorders were associated with 84% of the cases, with non-Hodgkin lymphoma (38.6%) as the most frequent, followed by chronic lymphocytic leukemia (18.4%) and Castleman's disease (18.4%). The non-hematologic neoplasms comprised 16% of all cases: epithelial origin-carcinoma (8.6%), mesenchymal origin-sarcoma (6.2%), and malignant melanoma (0.6%). Carcinoma cases comprised 58% of the non-hematologic neoplasms. Carcinoma cases (n=14) consisted of adenocarcinoma (n=7), squamous cell carcinoma (n=2), multiple skin tumors probably basal cell carcinoma (n=1), and bronchogenic carcinoma (n=1). Of the 10 (6.2%) sarcoma cases, there was one case each of leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, poorly differentiated sarcoma, reticulum cell sarcoma, dendritic cell sarcoma and inflammatory myofibroblastic tumor. The oral mucosa was involved in all of cases. Isolated oral ulcerations were the first sign in 45% of the cases. Diffuse and persistent oral ulcerations with a progressive course could be a sign of malignancy, either recognized or occult. In the absence of a clear diagnosis, malignancy should be suspected and extensive work-up performed. The full spectrum of signs of PNP may not be present initially. Repeated biopsies, direct and indirect immunofluorescence as well as screening indirect immunofluorescence on murine bladder are required for diagnosis. Clinicians should be highly suspicious when signs and symptoms suggestive of PNP are present in cancer patients, of hematologic and non-hematologic origin.
KW - Carcinoma
KW - Occult tumor
KW - Paraneoplastic pemphigus
KW - Sarcoma
UR - http://www.scopus.com/inward/record.url?scp=1842582053&partnerID=8YFLogxK
U2 - 10.1016/j.oraloncology.2003.09.020
DO - 10.1016/j.oraloncology.2003.09.020
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AN - SCOPUS:1842582053
SN - 1368-8375
VL - 40
SP - 553
EP - 562
JO - Oral Oncology
JF - Oral Oncology
IS - 6
ER -