scholarly journals Tripe Palms: A Rare Cutaneous Paraneoplastic Disorder

2020 ◽  
Vol 33 (13) ◽  
Author(s):  
Alexandre Miroux-Catarino ◽  
Leandro Silva ◽  
Goreti Catorze

N/a.

2017 ◽  
Vol 1 (6) ◽  
pp. 420-423
Author(s):  
Matthew S. J. Katz ◽  
Henry Alexander Leder ◽  
Tahsin Choudhury

Introduction: Bilateral diffuse uveal melanocytic proliferation (BDUMP) is a rare paraneoplastic disorder that bears grave ocular prognosis. No consensus exists regarding treatment regimen and very little has been achieved in the way of successful treatment. We present here a case of BDUMP associated with metastatic non–small cell lung carcinoma treated successfully with plasmapheresis. Methods: This is a nonmasked, retrospective interventional case study conducted at a tertiary care center, characterizing bilateral disease in 1 patient, and describing response to plasmapheresis. Outcome measures were resolution of optical coherence tomography (OCT) findings, as well as visual response. Results: Resolution of bilateral exudative retinal detachments was documented with both ophthalmoscopic photography and OCT and accompanied by concurrent improvement in visual function. Conclusion: Bilateral diffuse uveal melanocytic proliferation has been treated with plasmapheresis in only 3 previously reported cases. This case demonstrates and reinforces the great promise in the treatment of this rare and devastating condition.


2016 ◽  
Vol 2016 ◽  
pp. 1-10 ◽  
Author(s):  
Fangke Hu ◽  
Chengying Jiang ◽  
Qiang Zhang ◽  
Huaiyin Shi ◽  
Lixin Wei ◽  
...  

Tumor-induced osteomalacia (TIO) is a rare acquired paraneoplastic disorder and fibroblast growth factor 23 (FGF23) plays a key role in its pathogenesis. This study was conducted to describe a novel FGF23 detecting procedure and describe clinical features of the disease. Fourteen TIO cases were retrieved and FGF23 expression was measured by quantitative ELISA-like immunohistochemistry using formalin-fixed and paraffin-embedded tissues. As summarized from 14 TIO cases, clinical features of TIO were long-standing history of osteomalacia, hypophosphatemia, and urinary phosphate wasting. The associated tumors were mostly benign phosphaturic mesenchymal tumors mixed connective tissue variant (PMTMCT) which could be located anywhere on the body, and most of them could be localized by conventional examinations and octreotide scanning. By quantitative ELISA-like immunohistochemistry, all the 14 TIO cases had high FGF23 expression (median 0.69, 25%–75% interquartile 0.57–1.10, compared with 26 non-TIO tumors of median 0.07, 25%–75% interquartile 0.05–0.11,p<0.001). The quantitative ELISA-like immunohistochemistry was a feasible and reproducible procedure to detect the high FGF23 expression in formalin-fixed and paraffin-embedded biopsies or specimens. Since TIO was often delay-diagnosed or misdiagnosed, clinicians and pathologists should be aware of TIO and PMTMCT, respectively.


The Lancet ◽  
2017 ◽  
Vol 389 (10076) ◽  
pp. 1367-1368 ◽  
Author(s):  
Thiago Cardoso Vale ◽  
José Luiz Pedroso ◽  
Lívia Almeida Dutra ◽  
Lyamara Azevedo ◽  
Lucio Huebra Pimentel Filho ◽  
...  

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Zong Fei Ji ◽  
Dan Ying Zhang ◽  
Shu Qiang Weng ◽  
Xi Zhong Shen ◽  
Hou Yu Liu ◽  
...  

POEMS syndrome is a rare paraneoplastic disorder associated with an underlying plasma cell dyscrasia presenting polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. This study reviewed the clinical characteristics of 14 POEMS patients in Zhongshan hospital. The ratio of male to female was 9 : 5, and the average age was 47.1 years. The clinical manifestations were various, including motorial symptoms (weakness), sensory symptoms (numbness), lymphadenopathy, edema, abdominal distention, and skin hyperpigmentation. Imaging studies and laboratory tests also exhibited hepatomegaly, splenomegaly, thrombocytosis, endocrinopathy, and positive serum immunofixation in most patients. In addition, increased plasma cells in bone marrow and Castleman Disease were found in bone marrow and lymph nodes biopsies. All the eight follow-up patients were treated with alkylator-based combination chemotherapy or corticosteroids and thalidomide, with or without autologous stem cell transplantation. Unfortunately, two patients died three or four years after diagnosis of POEMS syndrome. The others showed response to therapy to some extent, but not completely remission. Currently, treatments for POEMS include radiation to the plasmacytoma, and systemic therapy is indicated. Low-dose alkylators with or without corticosteroids are effective in some patients. However, high-dose chemotherapy with auto-SCT dramatically improved symptoms and outcomes for POEMS patients.


Sign in / Sign up

Export Citation Format

Share Document