Skeletal cystic angiomatosis:A rare cause of unilateral lytic bone lesions

Cystic angiomatosis is a rare, benign, multifocal disorder of bone and viscera. Angiomatous deposits result in bone lysis and organ dysfunction. Bony cystic lesions occur in the axial and proximal appendicular skeleton. Lesions may cause bone pain or pathological fracture. Diagnosis is difficult, of exclusion and demands a biopsy. The prognosis varies upon whether the lesions are solely skeletal or there is visceral involvement. A 71-year-old man reports increasing symptoms of painful swelling in the right thoracic wall for over a month. The swelling was bony hard in consistency. Except for his bony swelling, the patient’s physical examination was within normal limits, as were all his laboratory studies. X-ray imagery showed multicystic expansive lytic areas involving the right ribs. Computerized tomography, magnetic resonance imagery and gallium bone scan revealed lytic lesions of multiple right ribs, and cervical, dorsal, lumbar and sacrum iliac spine. A right rib biopsy has shown a cystic formation with endothelial walls. Five years later, the patient remained stable, with no clinical, laboratory or imagilogic progression of disease and without visceral involvement. This case is presented in his rarity and differential diagnosis challenge.

Expect the unexpected:Brown tumor of the mandible as the first manifestation of primary hyperparathyroidism

Hyperparathyroidism(HPT)is a condition in which one or more parathyroid glands produce increased levels of parathyroid hormone(PTH),causing disturbances in calcium homeostasis.Most commonly HPT presents with asymptomatic hypercalcemia but the clinical spectrum may include disturbances reflecting the combined effects of increased PTH secretion and hypercalcemia.Brown tumors are rare,benign,tumor-like bone lesions,occurring in 1.5%to 4.5%of patients with HPT,as a complication of an uncontrolled disease pathway,and are nowadays rarely seen in clinical practice.The tumor can appear either as a solitary or multifocal lesion and usually presents as an asymptomatic swelling or a painful exophytic mass.Furthermore,it can cause a pathological fracture or skeletal pain and be radiologically described as a lytic bone lesion.The diagnosis of a brown tumor in HPT is typically confirmed by assessing the levels of serum calcium,phosphorus,and PTH.Although when present,brown tumor is quite pathognomonic for HPT,the histologic finding often suggests a giant cell tumor,while clinical presentation might suggest other more frequent pathologies such as metastatic tumors.Treatment of brown tumors frequently focuses on managing the underlying HPT,which can often lead to regression and resolution of the lesion,without the need for surgical intervention.However,in refractory cases or when dealing with large symptomatic lesions,surgical treatment may be necessary.