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An unusual cause of tremor in an elderly man

Fadilah SA, Raymond AA, Cheong SK

Postgrad Med J, 2001 Apr;77(906):268-269; discussion 277-8.
PMID: 11264499
Matched MeSH terms: Paraneoplastic Syndromes/diagnosis
POEMS syndrome: A rare paraneoplastic presentation of spinal plasmacytoma

Low JM, Basiam S, Ahlam Naila K

Med J Malaysia, 2019 08;74(4):335-337.
PMID: 31424045

The acronym POEMS syndrome was coined for a unique multisystem disorder characterised by peripheral neuropathy, organomegaly, endocrinopathies, monoclonal gammopathy and skin changes. We report a male patient presenting to us with spinal plasmacytoma complicated with paraplegia. He was subsequently diagnosed to have POEMS syndrome and successfully treated with thalidomide and dexamethasone. Post treatment, he is able to ambulate independently.

Matched MeSH terms: Paraneoplastic Syndromes/diagnosis
Fulltext Arthritis as an initial presentation of malignancy: two case reports

Sachdev Manjit Singh B, Wan SA, Cheong YK, Chuah SL, Teh CL, Jobli AT

J Med Case Rep, 2021 Feb 23;15(1):94.
PMID: 33618728 DOI: 10.1186/s13256-020-02642-z

BACKGROUND: Arthritis is rarely reported as a paraneoplastic manifestation of occult malignancy. We report herein two cases of paraneoplastic arthritis due to occult malignancy. CASE 1: The patient was a 65-year-old woman of asian descent who was a former smoker with a history of spine surgery performed for L4/L5 degenerative disc disease. She presented with a 1-month history of oligoarthritis affecting both ankle joints and early morning stiffness of about 3 hours. Laboratory tests were positive for antinuclear antibody at a titer of 1:320 (speckled) but negative for rheumatoid factor. She was treated for seronegative spondyloarthritis and started on prednisolone without much improvement. A routine chest radiograph incidentally revealed a right lung mass which was found to be adenocarcinoma of the lung. She was treated with gefitinib and her arthritis resolved. CASE 2: The patient was a 64-year-old woman of asian descent, nonsmoker, who presented with a chief complaint of asymmetrical polyarthritis involving her right wrist, second and third metacarpophalangeal joints, and first to fifth proximal interphalangeal joints. She was treated for seronegative rheumatoid arthritis (RA) and started on sulfasalazine, with poor clinical response. Six months later, she developed abdominal pain which was diagnosed as ovarian carcinoma by laparotomy. Her arthritis resolved following treatment of her malignancy with chemotherapy.
CONCLUSION: In summary, paraneoplastic arthritis usually presents in an atypical manner and responds poorly to disease-modifying antirheumatic drugs. Accordingly, we recommend screening for occult malignancy in patients presenting with atypical arthritis.

Matched MeSH terms: Paraneoplastic Syndromes/diagnosis*
Paraneoplastic relapsing minimal change disease associated with type A thymoma in an elderly patient: A case report and literature review

Ngoh CLY, Goh GHS, Wong WK

Med J Malaysia, 2019 02;74(1):97-98.
PMID: 30846674

Thymoma is a rare mediastinal tumour that can be accompanied by different paraneoplastic syndromes. Here we report a case of Type A thymoma associated with relapsing minimal change disease (MCD). This case highlights: (1) The need to balance rapid prednisolone weaning against risk for relapse in an elderly patient at risk for steroid-induced complications. (2) The addition of calcineurin inhibitor in relapsed thymoma-related MCD, to achieve steroid sparing effects. Resection of the offending tumour and prompt immunosuppressive therapy are critical in getting best renal and overall outcomes in this rare entity.

Matched MeSH terms: Paraneoplastic Syndromes/diagnosis
Fulltext Paraneoplastic Secretion of Multiple Phosphatonins From a Deep Fibrous Histiocytoma Causing Oncogenic Osteomalacia

Leow MKS, Dogra S, Ge X, Chuah KL, Liew H, Loke KSH, et al.

J Clin Endocrinol Metab, 2021 04 23;106(5):e2299-e2308.
PMID: 33462615 DOI: 10.1210/clinem/dgaa964

CONTEXT: Literature suggests that oncogenic osteomalacia is usually caused by a benign mesenchymal tumor secreting fibroblast growth factor subtype-23 (FGF-23), but the involvement of other phosphatonins has only been scarcely reported. We have previously published a seemingly typical case of oncogenic osteomalacia. Following curative neoplasm resection, we now report unique molecular characteristics and biology of this tumor.
CASE DESCRIPTION: A 25-year-old man had been diagnosed with severe oncogenic osteomalacia that gradually crippled him over 6 years. 68Ga-DOTA-TATE positron emission tomography/computed tomography scan localized the culprit tumor to his left sole, which on resection revealed a deep fibrous histiocytoma displaying a proliferation of spindle cells with storiform pattern associated with multinucleated giant cells resembling osteoclasts. Circulating FGF-23, which was elevated more than 2-fold, declined to undetectable levels 24 h after surgery. Microarray analysis revealed increased tumor gene expression of the phosphatonins FGF-23, matrix extracellular phosphoglycoprotein (MEPE) and secreted frizzled-related protein subtype 4, with elevated levels of all 3 proteins confirmed through immunoblot analysis. Differential expression of genes involved in bone formation and bone mineralization were further identified. The patient made an astonishing recovery from being wheelchair bound to fully self-ambulant 2 months postoperatively.
CONCLUSION: This report describes oncogenic osteomalacia due to a deep fibrous histiocytoma, which coincidentally has been found to induce profound muscle weakness via the overexpression of 3 phosphatonins, which resolved fully upon radical resection of the tumor. Additionally, genes involved in bone formation and bone remodeling contribute to the molecular signature of oncogenic osteomalacia.

Matched MeSH terms: Paraneoplastic Syndromes/diagnosis