To compare magnetic resonance (MR) imaging, computed tomography (CT) and radiography for the detection of arthropathies in patients with haemophilia. Forty-one symptomatic joints in 14 men with haemophilia, ages 11-24 years (mean age 17.5 +/- 3.9 years) were examined with radiography, CT and MR. Images of each joint were acquired on the same day. The precontrast MR examination obtained coronal spin-echo T1 images and gradient echo with rephasing T2* images, as well as sagittal, axial gradient echo with rephasing T2* images using a low-field-strength 0.3-T MR imager. For the postcontrast MR examination, coronal, sagittal and axial images were acquired using the same precontrast T1 sequence. Thirteen joints were also examined on enhanced MR. The severity of damage was classified using conventional radiographical staging. Severely affected haemophilic arthropathy (HA) patients (stage 5) were excluded. Findings of soft tissue swelling, osteoporosis, epiphyseal overgrowth, joint erosion, cysts, joint space narrowing, bone marrow oedema, joint effusion, haemorrhage, synovial hypertrophy and widened intercondylar notches as well as anterior and posterior cruciate ligaments (for the knee) were used in all imaging comparisons. The joints were classified by radiographical criteria into stage 0 (n = 5), stage 1 (n = 7), stage 2 (n = 6), stage 3 (n = 8) and stage 4 (n = 15). Soft tissue swelling or joint effusion was observed in 33 joints on radiographs, in 34 on both CT and MR; joint erosions were observed in 34 joints on MR, 33 on CT and 20 on radiographs. Joint cysts appeared in 21 joints on MR, 18 on CT and 9 on radiographs. Significant differences in detection of erosion and cysts were found between radiography and CT (P < 0.05) and radiography and MR imaging (P < 0.05), not between CT and MR (P > 0.05). MR was better for detecting foci of both erosion and cysts than CT and radiography, and CT was better than radiography. MR imaging, CT and radiography were equally effective in showing the changes of epiphyseal overgrowth in 26 joints, joint space narrowing in 14 joints and widened intercondylar notches in 20 knee joints. However, only MR imaging detected tears in 17 anterior and 13 posterior cruciate ligaments in the 20 knee joints with widened intercondylar notches. Bone marrow oedema in 14 joints, haemorrhage in 34 joints and synovial hypertrophy in 27 joints were seen on MR images, but not on CT or radiography. MR imaging is superior to CT and conventional radiography for detecting abnormal changes and should be considered the first choice among imaging modalities in evaluating HAs.

Desmoplastic small round cell tumor (DSRCT) is a rare aggressive sarcoma with characteristic clinical and pathologic features. It typically involves pelvic and abdominal organs of young male patients, and patients usually present at advanced stage with poor prognosis. A few reports are available describing the cytopathologic features of DSRCT in serous effusions, with the majority of published cases depicting undifferentiated small blue cells that need to be distinguished from other small blue cell tumors. We report an interesting case of DSRCT involving a pleural effusion with a “floating island” pattern that has been described in hepatocellular carcinoma, renal cell carcinoma, and adrenal cortical carcinoma. In our case, the epithelioid tumor cells form cohesive aggregates surrounded by a single layer of spindle cells, mimicking the “endothelial wrapping” in other tumors with “floating island” patterns. We demonstrate, by ancillary testing, that these peripheral spindle cells are tapered/flattened DSRCT cells, in contrast to endothelial wrapping cells, as seen in other tumors with this unique cytomorphology. To our knowledge, this is the first case report describing DSRCT showing a “floating island” pattern that needs to be differentiated from metastatic hepatocellular carcinoma, renal cell carcinoma, and adrenal cortical carcinoma in effusion cytology.

Sinonasal hamartomas are uncommon lesions of nasal and sinus cavities. Based on indigenous cellular components and characteristic histologic features, they are further classified into four entities: respiratory epithelial adenomatoid hamartoma (REAH), seromucinous hamartoma (SH), chondro-osseous and respiratory epithelial hamartoma (CORE), and nasal chondromesenchymal hamartoma (NCH). REAH, SH, and CORE are seen in adult patients, while NCH predominantly occurs in newborns and infants. Morphologically REAH and SH are composed of respiratory epithelium and seromucinous glands, CORE is related to REAH but with additional feature of chondroid and/or osseous tissue, and NCH is composed of chondroid and stromal elements but devoid of epithelial component. All four lesions can present as sinonasal mass lesions and with associated obstructive symptoms. Given the rarity of these lesions, diagnosis can be challenging, especially in unusual clinical scenario. In this study, we report six cases of sinonasal hamartoma, including one case of NCH, one case of CORE, two cases of SH, and two cases of REAH. All cases were from adult patients including four men and two women. We also review the literature of the clinical and pathologic features of these rare lesions.

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