Summary Dendritic cell sarcomas (DCS) are rare tumours of antigen presenting cells. Data regarding their biology, management and outcomes are sparse. We analysed 66 patients with follicular dendritic cell sarcoma (FDCS). Six patients also had Castleman disease, 9 had another malignancy and 13 had an autoimmune disease. Fifty-four per cent of patients presented with localized disease and 46% with systemic involvement. The median progression-free (PFS) and overall survival (OS) following frontline therapy was 21 months and 50 months, respectively. Survival outcomes were significantly inferior in patients with extranodal, bulky or intra-abdominal disease at presentation. Stage was not associated with survival. Management approaches were heterogeneous. Patients who underwent an upfront gross total resection (GTR) experienced better PFS and OS (both P<0.0001). In patients who underwent a GTR, consolidative radiotherapy was associated with improved local control (P = 0.03), PFS (P = 0.04) and OS (P = 0.05). In patients with measureable disease, gemcitabine with a taxane yielded an overall response rate of 80%. The pattern of relapse was predominantly locoregional. Salvage rates after recurrence were poor. Studies are underway at our institution to define the genomic profile in FDCS and identify potential novel therapeutic targets.

Priapism associated with sickle cell disease is classically described as a low flow state. We report 2 cases of high flow priapism associated with sickle cell disease. High flow priapism has previously been reported almost exclusively in patients with traumatic rupture of the cavernous artery. Neither of our patients had historical or radiographic findings consistent with injury to the penile vasculature. One patient was treated unsuccessfully with intracorporeal injection of methylene blue and 1 underwent successfully bilateral pudendal artery embolization. The pathophysiological mechanism(s) responsible for the production of high flow priapism in patients with sickle cell disease is not known.

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Priapism associated with sickle cell disease is classically described as a low flow state. We report 2 cases of high flow priapism associated with sickle cell disease. High flow priapism has previously been reported almost exclusively in patients with traumatic rupture of the cavernous artery. Neither of our patients had historical or radiographic findings consistent with injury to the penile vasculature. One patient was treated unsuccessfully with intracorporeal injection of methylene blue and 1 underwent successfully bilateral pudendal artery embolization. The pathophysiological mechanism(s) responsible for the production of high flow priapism in patients with sickle cell disease is not known.

True thymic hyperplasia (enlarged gland composed of histologically unremarkable cortical and medullary parenchyma) and lymphoid hyperplasia (medullary lymphoid follicles in the clinical setting of autoimmunity) usually develop as independent pathologic processes. We reviewed the clinical features and gross and microscopic pathology of 2 hyperthyroid patients with features of both thymic hyperplasia and lymphoid hyperplasia. The diagnosis of thymic hyperplasia was supported by thymic weights greater than two standard deviations above the mean weight for age and histologic evidence of expanded cortical and medullary parenchyma. The diagnosis of lymphoid hyperplasia was supported by the increased number and size of medullary lymphoid follicles and the association with Graves' disease. This unusual combination results from two separate pathogenic mechanisms operating simultaneously in hyperthyroid patients. Elevated thyroid hormones directly stimulate the proliferation of thymic epithelium, producing thymic hyperplasia. The immune abnormalities underlying Graves' disease can also result in lymphoid hyperplasia of the thymus.