Rosai-Dorfman Disease Presenting as Peripheral Vascular Insufficiency.

Rosai-Dorfman (-Destombes) disease (RDDD) is a rare idiopathic disorder of histiocyte proliferation, usually involving lymph node stations. The most common clinical finding is a bilateral cervical lymphadenopathy, fever, and weight loss. Arterial or venous structures are notably not involved. We hereby present a case of a 78-year-old Caucasian man, presenting with symptoms of progressive arterial insufficiency and right lower-limb edema, along with a nonpulsatile mass at the middle third of the thigh. Initial diagnostic hypothesis was a superficial femoral artery aneurysm thrombosis with a secondary postcompressive superficial femoral vein thrombosis. Duplex examination showed right superficial femoral arterial and venous thrombosis, along with a hypoechogenic mass causing compression of the neurovascular bundle. Suspecting a connective tissue sarcoma, computed tomography scan was performed after combined en bloc removal of the mass along with femoral artery and vein and prosthetic reconstruction of vascular continuity. Histopathology diagnosis was connective tissue RDDD. The atypical presentation of this rare syndrome induces us to include in differential diagnosis, among other more common forms of external compression of the neurovascular bundles, even rare conditions such as these, which generally only involve lymphatic stations.

Thyroglossal duct cyst cancer most likely arises from a thyroid gland remnant.

Thyroglossal duct cancer is a rare entity, occurring in 1.5 % of all thyroglossal duct cysts (TDC). A definitive consensus about its neoplastic origin has not been established as two contrasting theories exist, one proposing an origin in extra-thyroid remnants and the other a metastatic localization of a primary thyroid cancer. We compare morphological and molecular characteristics of both thyroglossal and thyroid carcinomas in a case series from our institute. We evaluated histology of 80 TDC. In 12 cases, prior cytological evaluation had been performed by liquid-based cytology (LBC). The BRAF gene was examined for mutations, and the histology of both thyroglossal duct and synchronous thyroid carcinoma was reevaluated. In 9 out of 80 (11 %) TDC cases, a papillary thyroid cancer (PTC) was diagnosed. In five out of nine (56 %) thyroglossal carcinomas, a synchronous thyroid cancer was diagnosed: 3 PTC and 2 follicular variant PTC (FVPC). In five thyroglossal carcinomas, mutated BRAF (V600E) was found, three in PTC and in thyroglossal as well as in the synchronous tumor in the thyroid. All the patients are in a disease-free status and still alive. Our results suggest that the majority of thyroglossal carcinomas most likely develop as a primary malignancy from a thyroid remnant. Neither the presence of V600E BRAF mutations nor that of a well-differentiated thyroid carcinoma changed the outcome or disease-free survival. We suggest that a diagnosis of thyroglossal carcinoma should be followed by a detailed evaluation of the thyroid gland. In the absence of clinical and radiological thyroid alterations, follow-up as for thyroid cancer is the correct management.

Spiral hydro-CT in the evaluation of colo-sigmoideal cancer.

PURPOSE: To evaluate the accuracy of spiral hydro-CT in the diagnosis and preoperative staging of colo-sigmoideal neoplasms and the impact of this technique on therapeutic decisions. MATERIALS AND METHODS: Sixty patients with colo-sigmoideal neoplasms, 16 in caecum-ascending colon, 2 in transverse colon, 14 in descending colon, and 28 in sigmoid colon, diagnosed with fiberoptic colonoscopy and histopathological specimen, under-went spiral hydro-CT with IV contrast medium administration. Local invasion (T factor) and nodal involvement (N factor) were evaluated. In all cases, the CT reports were compared with the result of the histopathological examination of the surgical specimen. TNM and Dukes staging classifications were applied. RESULTS: Local invasion was correctly identified in 80% of cases. There was overstaging in 18.3% and understaging in 1.7% of the patients. Sensitivity, specificity and accuracy for nodal involvement were 57.9%, 67.5% and 63.3%, respectively. There was overstaging in 23.3% and understaging in 15% of patients. With Dukes classification, CT correctly staged 38.8% of patients. Significant differences were detected in different stages. The stage with the highest concordance was A/B1 with 58.8% (10/17), whereas the one with the lowest concordance was B2 with 9.1% (2/22). All of the three patients in stage D were correctly staged and none of the patients with stage C was overstaged. CONCLUSIONS: Spiral hydro-CT with intravenous contrast medium administration, despite the limits in the correct evaluation of local depth invasion and nodal involvement, proved useful for selecting patients likely to benefit from surgery.