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High-dose-rate intraluminal brachytherapy for paraneoplastic autoimmune multiorgan syndrome

View Article: PubMed Central - PubMed

ABSTRACT

Paraneoplastic autoimmune multiorgan syndrome (PAMS), also known as paraneoplasic pemphigus, involves the skin, internal organs and mucosa. PAMS-associated mortality may occur as a result of autoantibody formation against internal tumors and their infiltration into organs other than the skin lesions that characterize PAMS. The most common symptoms of PAMS include pain associated with continuous oral ulceration and resistance to pharmacological treatment. The present study reports the case of a 42-year-old female patient who was admitted with an 8-month history of erosive skin lesions within the trunk region, oral mucosa and vaginal mucosa. The patient was diagnosed with PAMS based on computed tomography scans and histological analyses of the lesions. The lymphoid hyperplasia in the retroperitoneum and lesions in the vaginal mucosa and trunk area were improved following pharmacological treatment and resection of the lymph node showing hyperplasia. However, the oral lesion was treated with intraluminal brachytherapy due to its resistance to long-term pharmacological treatment. The majority of the lesions were improved following treatment, in the absence of any severe side effects. In addition, neither worsening nor progression of the oral lesion was observed during the 4-year follow-up period.

No MeSH data available.


Related in: MedlinePlus

Axial abdominal computed tomography scan. A lobulated hypovascular mass of 12 cm was observed between the stomach and left kidney of the patient, which was accompanied by central dense calcification.
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f1-etm-0-0-3458: Axial abdominal computed tomography scan. A lobulated hypovascular mass of 12 cm was observed between the stomach and left kidney of the patient, which was accompanied by central dense calcification.

Mentions: A 42-year-old female patient visited Chonbuk National University Hospital (Jeonju, Republic of Korea) with an 8-month history of erosive skin lesions occurring in the trunk region, oral mucosa and vaginal mucosa. The patient's medical history and family history were normal. An abdominal computed tomography (CT) scan (date, 18/6/2008) was performed following the detection of elevated CA125 (86.4 U/ml; normal, 0–37 U/ml) and CA19-9 (185.46 U/ml; normal, <37 U/ml) levels in a blood examination. A retroperitoneal mass identified in the abdominal CT scan was removed (date, 25/6/2008) and the patient was diagnosed with Castleman's disease according to this biopsy (date, 30/6/2008) (Fig. 1). The biopsy was a large (12×6.5×6.8 cm) mass with multicentric, hyaline vasular lymph node hyperplasia. The study was approved by the Institutional Review Board of Chonbuk National University Hospital and was conducted according to the Declaration of Helsinki regarding biomedical research involving human subjects. A detailed explanation of the study was provided to the patient, and written informed consent was obtained from the patient.


High-dose-rate intraluminal brachytherapy for paraneoplastic autoimmune multiorgan syndrome
Axial abdominal computed tomography scan. A lobulated hypovascular mass of 12 cm was observed between the stomach and left kidney of the patient, which was accompanied by central dense calcification.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4998192&req=5

f1-etm-0-0-3458: Axial abdominal computed tomography scan. A lobulated hypovascular mass of 12 cm was observed between the stomach and left kidney of the patient, which was accompanied by central dense calcification.
Mentions: A 42-year-old female patient visited Chonbuk National University Hospital (Jeonju, Republic of Korea) with an 8-month history of erosive skin lesions occurring in the trunk region, oral mucosa and vaginal mucosa. The patient's medical history and family history were normal. An abdominal computed tomography (CT) scan (date, 18/6/2008) was performed following the detection of elevated CA125 (86.4 U/ml; normal, 0–37 U/ml) and CA19-9 (185.46 U/ml; normal, <37 U/ml) levels in a blood examination. A retroperitoneal mass identified in the abdominal CT scan was removed (date, 25/6/2008) and the patient was diagnosed with Castleman's disease according to this biopsy (date, 30/6/2008) (Fig. 1). The biopsy was a large (12×6.5×6.8 cm) mass with multicentric, hyaline vasular lymph node hyperplasia. The study was approved by the Institutional Review Board of Chonbuk National University Hospital and was conducted according to the Declaration of Helsinki regarding biomedical research involving human subjects. A detailed explanation of the study was provided to the patient, and written informed consent was obtained from the patient.

View Article: PubMed Central - PubMed

ABSTRACT

Paraneoplastic autoimmune multiorgan syndrome (PAMS), also known as paraneoplasic pemphigus, involves the skin, internal organs and mucosa. PAMS-associated mortality may occur as a result of autoantibody formation against internal tumors and their infiltration into organs other than the skin lesions that characterize PAMS. The most common symptoms of PAMS include pain associated with continuous oral ulceration and resistance to pharmacological treatment. The present study reports the case of a 42-year-old female patient who was admitted with an 8-month history of erosive skin lesions within the trunk region, oral mucosa and vaginal mucosa. The patient was diagnosed with PAMS based on computed tomography scans and histological analyses of the lesions. The lymphoid hyperplasia in the retroperitoneum and lesions in the vaginal mucosa and trunk area were improved following pharmacological treatment and resection of the lymph node showing hyperplasia. However, the oral lesion was treated with intraluminal brachytherapy due to its resistance to long-term pharmacological treatment. The majority of the lesions were improved following treatment, in the absence of any severe side effects. In addition, neither worsening nor progression of the oral lesion was observed during the 4-year follow-up period.

No MeSH data available.


Related in: MedlinePlus