Response of Aneurysmal Bone Cyst to Denosumab.

STUDY DESIGN: Case report and literature review. OBJECTIVE: To describe a unique case of large sacral aneurysmal bone cysts (ABCs) treated with denosumab and review the literature on this rare entity. SUMMARY AND BACKGROUND DATA: ABCs are expansile osteolytic lesions that typically contain blood-filled spaces separated by fibrous septa. Standard treatment includes surgical resection or curettage and packing; however, for some spinal lesions, the standard approach is not optimal. One therapeutic strategy is to treat spinal ABC with an agent that targets a pathway that is dysregulated in a disease with similar pathophysiology. The bone destruction in both giant cell tumors of bone and ABCs is mediated by RANK ligand (RANKL) produced by the tumor cells. Denosumab, a human monoclonal antibody to RANKL, is effective in the treatment of giant cell tumors of bone. METHODS: We report a case of a large sacral ABC that responded to denosumab. A 27-year-old male developed increasing back pain. Imaging revealed a lytic lesion in the sacrum with no clear solid component and regions where the cortex was difficult to identify. ABC was diagnosed on biopsy. Surgery or radiation treatment was expected to be associated with serious morbidity; therefore, denosumab was given using the regimen for giant cell tumors of bone (120  mg monthly with a loading dose). RESULTS: The patient's pain gradually resolved after 2 months of treatment. New bone formation with a more clearly defined cortex was evident on computed tomographic scan at 16 weeks and continued to show evidence of improvement at 7 and 12 months. Biopsy at 12 months revealed a hypocellular fibrous stroma with new bone formation and no giant cells. CONCLUSION: We conclude that denosumab can result in symptomatic and radiological improvement in ABC and may be useful in select cases. LEVEL OF EVIDENCE: 5.

Endobronchial ultrasound-guided transbronchial needle aspiration diagnosis of mediastinal lymph node metastasis of mucinous adenocarcinoma: arborizing stromal meshwork fragments as a diagnostic clue.

Endobronchial Ultrasound-Guided Transbronchial Needle Aspiration (EBUS-TBNA) is a reliable and accurate method for the diagnosis of mediastinal metastases in patients with pulmonary and extrathoracic neoplasms. We report the cytopathologic findings of a case of metastatic signet-ring cell carcinoma with abundant extracellular mucin production in the mediastinal lymph nodes of a 41-year-old woman, who presented with nausea, abdominal pain, and weight loss. Imaging studies showed a renal mass, numerous lung nodules, and mediastinal and retroperitoneal lymphadenopathy. EBUS-TBNA of level 4R and 7 lymph nodes showed abundant, thick, "clean" mucus with entrapped ciliated bronchial cells, rare histiocytes, and fragments of cartilage. No neoplastic cells could be identified in Diff-Quik®-stained smears during the rapid on-site evaluation, but rare signet-ring cells were identified in the Papanicolaou-stained smears and cellblock sections. A distinctive feature of the aspirates was the presence of large branching (arborizing), "spidery" stromal fiber meshwork fragments. These stained metachromatically (magenta) with Romanowsky-type stains and cyanophilic to orangeophilic with Papanicolaou stains and showed occasional attached bland spindle cells, but had no capillary lumina or CD31-staining endothelial cells. The tumor cells were strongly and diffusely positive for CEA, CDX2, CK7, CK20, and MUC2, supporting the diagnosis of a metastatic signet-ring cell adenocarcinoma, most likely of gastrointestinal origin. We believe that the presence of the large spidery stromal fiber fragments is a useful clue to the presence of a mucinous neoplasm in EBUS-TBNA and allows the differentiation of the neoplastic mucus from contaminating endobronchial mucus.