Distant lung metastases caused by a histologically benign phosphaturic mesenchymal tumor

Επιστημονική δημοσίευση - Άρθρο Περιοδικού uoadl:3179683 16 Αναγνώσεις

Μονάδα:
Ερευνητικό υλικό ΕΚΠΑ
Τίτλος:
Distant lung metastases caused by a histologically benign phosphaturic
mesenchymal tumor
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Tumor-induced osteomalacia (TIO) is a rare form of hypophosphatemia
usually caused by phosphaturic mesenchymal tumors (PMTs); the biologic
behavior of PMTs is under investigation. Herein we present a case of TIO
with a protracted course over 12 years leading to a fatal outcome. A
39-year-old man presented with weakness in 2004 and was found to have
decreased serum phosphorus, phosphaturia and low levels of
1,25-dihydroxyvitamin D3. Four years later he developed a painful left
calf mass. The lesion was resected, but recurred causing extreme pain
and dysfunction. Radiological examination showed a large cluster of soft
tissue tumors affecting all the muscle compartments of the calf and a
smaller lesion inside the metaphysis of the tibia. Above-knee amputation
was performed. Histological examination of all lesions showed a cellular
spindle cell neoplasm with variously sized vessels, wide vessel-like
spaces and scattered deposits of calcified extracellular material. The
tumor infiltrated skeletal muscles, subcutaneous fat and the proximal
end of the fibula. The tibial lesion had identical histology. Three
years after the amputation the patient presented with cough and dyspnea.
Radiological examination, followed by an open biopsy, showed that there
were multiple metastatic nodules of PMTs in both lungs. Shortly after
the diagnosis the patient died. This case illustrates that even benign
cases of PMTs may lead to a fatal outcome and the classification of PMTs
into benign and malignant should be reassessed in order to correspond to
its biological behavior.
Έτος δημοσίευσης:
2018
Συγγραφείς:
Yavropoulou, Maria P.
Poulios, Christos
Foroulis, Christoforos
and Tournis, Symeon
Hytiroglou, Prodromos
Kotsa, Kalliopi and
Kessisoglou, Isaak
Zebekakis, Pantelis
Περιοδικό:
Endocrinology, Diabetes and Metabolism Case Reports
Εκδότης:
BioScientifica Ltd
Επίσημο URL (Εκδότης):
DOI:
10.1530/EDM-18-0023
Το ψηφιακό υλικό του τεκμηρίου δεν είναι διαθέσιμο.