Unilateral Multiple Extramedullary Plasmacytoma of the Choroid and Conjunctiva: A Case Report.

Purpose: We report a case of multiple extramedullary plasmacytoma (EMP) of the choroid and conjunctiva as a sign of multiple myeloma (MM) relapse. Methods: An observational case report with clinicopathologic correlation is presented. Results: A 45-year-old man with a 2.5-year history of MM presented with left eye pain. Examination revealed a conjunctival nodule and choroidal infiltrate in the left eye. Excisional biopsy demonstrated plasmacytoma of the conjunctiva and choroid. Despite radiotherapy and chemotherapy, multiple EMP recurred and the patient died 8 months after initial presentation. Conclusions: Multiple EMP of the choroid and conjunctiva has a poor prognosis and can present as a sign of MM relapse. Our imaging demonstrates the clinical manifestations of this tumor and can aid future diagnoses. Our radiation dose (20 Gy) was inadequate, which is an important reportable factor for future patients.

Integration and Diagnostic Accuracy of 3T Nonendorectal coil Prostate Magnetic Resonance Imaging in the Context of Active Surveillance.

OBJECTIVE: To evaluate the integration of 3T nonendorectal coil multiparametric prostate magnetic resonance imaging (mpMRI) at 2 high-volume practices that routinely use mpMRI in the setting of active surveillance. MATERIALS AND METHODS: This was an institutional review board-approved, Health Insurance Portability and Accountability Act-compliant, and dual-institution retrospective cohort study. Subjects undergoing 3T mpMRI without endorectal coil at either study institution over a 13-month period (August 1, 2015-August 31, 2016) were selected based on predefined criteria: clinical T1/T2 Gleason 6 prostate cancer, prostate-specific antigen <15 ng/mL, ≥40 years old, mpMRI within 2 years of prostate biopsy, and Prostate Imaging Reporting and Data System (PI-RADS) v2 score assigned. Subjects surveilled for Gleason ≥3 + 4 prostate cancer were excluded. The primary outcome was detection of Gleason ≥3 + 4 prostate cancer on magnetic resonance-ultrasound fusion biopsy, standard biopsy, or prostatectomy within 6 months following mpMRI. Positive predictive values (PPVs) were calculated. RESULTS: A total of 286 subjects (N = 193 from institution 1, N = 93 from institution 2) met the criteria. Most (87% [90 of 104]) with maximum PI-RADS v2 scores of 1-2 did not receive immediate biopsy or treatment and remained on active surveillance. Incidence and PPVs for PI-RADS v2 scores of ≥3 were the following: PI-RADS 3 (n = 57 [20%], PPV 21% [6 of 29]), PI-RADS 4 (n = 96 [34%], PPV 51% [39 of 77]), and PI-RADS 5 (n = 29 [13%], PPV 71% [20 of 28]). No Gleason ≥4 + 3 prostate cancer was identified for PI-RADS v2 scores of 1-3 (0 of 43 with histology). Following mpMRI and subsequent biopsy, 21% (61 of 286) of subjects were removed from active surveillance and underwent definitive therapy. CONCLUSION: The 3T nonendorectal coil mpMRI has been integrated into the care of patients on active surveillance and effectively stratifies risk of Gleason ≥3 + 4 prostate cancer in this population.

An unusual case of swelling--Clarkson's syndrome.

Clarkson's syndrome, also called idiopathic systemic capillary leak syndrome is a rare condition characterised by vascular hyper permeability resulting in extreme intravascular volume depletion. The syndrome is unique and almost paradoxical in its presentation, with findings initially suggesting overwhelming heart failure, but in reality the extra vascular fluid represents overt capillary leak, with ultimate intravascular volume depletion, a low output state and hypovolemic shock. Previously described characteristics have classically included severe oedema and anasarca with rapid, profound shock, typically accompanied by haemoconcentration. The authors describe a patient, initially seeming benign in presentation, who rapidly progressed with confusing findings of fluid overload by examination and imaging, ultimately manifesting these findings by severe capillary leak rather than hydrostatic oedema, with ultimate hypovolaemic shock, multisystem organ failure and death. Our aim is that by describing clinical, haemodynamic and pathologic descriptors of the disease, the authors can aid in increasing physician awareness of this unusual syndrome.

Dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features.

The dedifferentiated component of dedifferentiated liposarcoma shows wide histologic variation including tumors with heterologous differentiation. Myofibroblastic differentiation has been recognized in dedifferentiated liposarcoma. However, tumors closely resembling inflammatory myofibroblastic tumor have not. We report the clinicopathologic, immunohistochemical, and molecular finding in 6 cases of dedifferentiated liposarcoma with inflammatory myofibroblastic tumor-like features treated at our institution. The tumors occurred mostly in middle age or elderly men, involved mostly the inguinal/scrotal region or retroperitoneum, and behaved aggressively. Microscopically, the dedifferentiated component closely resembled or, if taken out of context, was indistinguishable from inflammatory myofibroblastic tumor. All 3 major patterns seen in inflammatory myofibroblastic tumor (myxoid, cellular, and hypocellular fibrous) were represented. Areas resembling nodular fasciitis or desmoid fibromatosis were frequent findings. One tumor had heterologous osseous differentiation. In 4 tumors the inflammatory myofibroblastic tumor-like areas were diffuse, whereas in 2 they were combined with noninflammatory myofibroblastic tumor-like high-grade sarcoma. Five tumors stained for smooth muscle actin or desmin, none stained for ALK-1, 5 stained for MDM2, and 5 had amplified MDM2 by fluorescence in situ hybridization. Well-differentiated liposarcomatous components were present in every tumor. All patients developed locally recurrent or metastatic disease. At last follow-up 2 patients had died of disease and 2 were alive with disease. Dedifferentiated liposarcoma can have prominent inflammatory myofibroblastic tumor-like features, a finding that further expands its histologic spectrum. Awareness of this finding can prevent one from misdiagnosing dedifferentiated liposarcoma as inflammatory myofibroblastic tumor, a much less aggressive neoplasm.