Peroneus longus tendon transfer; prospective study of treatment outcome of recurrent fixed clubfoot deformities in children after failed surgical treatment

BackgroundIn childhood, for flexible clubfoot deformity, the transfer of the tendon of the tibialis anterior muscle is widely used. In contrast, extensive surgical procedures are required for fixed clubfoot deformities.MethodsWe describe the peroneus longus tendon transfer to the peroneus brevis tendon, additionally to full surgical release, in cases of recurrent fixed clubfoot deformities. The purpose of this surgical technique was to restore and maintain the dynamic balance of foot inversion-eversion during the gait cycle by augmenting the muscular strength of the weak peroneus brevis tendon. We report the prospective study of treatment outcome of twenty recurrent fixed clubfoot deformities in twelve children (20 feet) after failed surgical treatment they had. Anteroposterior and lateral radiographs under full-body weight-bearing and the AOFAS score pre-and postoperatively were used in all patients. For the estimation of the severity of the recurrent clubfoot deformity in each child and to increase the credibility of the AOFAS rating scale, we additionally used a clubfoot sheet score preoperatively and postoperatively (maximum score 100 points for normal foot appearance clinically and radiologically).ResultsThe mean age at surgery was 6,85 (±1,81; 5–11) years. The mean follow-up time was 5,4 (±1,7; 2–8) years. The mean AOFAS ankle-hindfoot rating score increased from 69,85 (±9,51; 53–82) points preoperatively to 94,4 (±2,43; 91–97) points postoperatively. The mean clubfoot sheet rating score increased from 43,00 (±12,18; 15–55) points, preoperatively to 90,0 (±4,58; 80–95) points postoperatively. The two-tailed p-value was < 0,0001.ConclusionsThe transfer of the peroneus longus tendon to the peroneus brevis tendon is a minimal surgical procedure that acts collaboratively in maintaining the correction of foot deformity, achieved by the complete surgical release. Level of Evidence: IV.     

Cyto‐histological correlation of Xp11.2 translocation/TFE3 gene fusion associated renal cell carcinoma: Report of a case with review of literature

The MiT family translocation renal cell carcinomas (RCCs) are relatively rare in comparison to the conventional RCC. The cytologic features overlap with conventional clear cell RCC and papillary RCCs, thereby making the diagnosis extremely challenging. Here, we describe a case of TFE3 translocation associated RCC in a 58‐year‐old patient, with emphasis on cytomorphologic features and clues toward this diagnostic entity. Correlating the cytohistologic findings and review of touch imprints revealed that presence of hyaline nodules resembling leisegang rings and psammoma bodies in cytologic smears from kidney tumors serve as an important clue in raising a suspicion for the diagnosis of MiT family translocation RCCs.     

Mouse models for the study of fate and function of innate lymphoid cells

Natural killer (NK) cells and lymphoid tissue inducer (LTi) cells were discovered more than 40 and 20 years ago, respectively. These two cell types were initially studied for their unique functions in the elimination of infected or transformed cells, and in the development of lymphoid tissues. It took an additional 10 years to realize that NK cells and LTi cells were members of a larger family of innate lymphoid cells (ILCs), whose phenotypes and functions mirror those of T cells. Many mouse models have since been developed to identify and isolate ILCs, map their developmental pathways and characterize their functions. Because of the similarity between ILCs and T cells, this exploration remains a challenge. In spite of this, a broad range of mouse models available to researchers has lead to significant progress in untangling the unique roles of ILCs early in defense, regulation of adaptive immunity and homeostasis. Here, we review these mouse models, and discuss their strengths and limitations.     

Concurrent rearrangements of BCL2, BCL3, and BCL11A genes in atypical chronic lymphocytic leukemia

The most frequent chromosomal aberrations with the well established prognostic meaning in chronic lymphocytic leukemia (CLL) are +12, del(11q), del(13q), and del(17p). Less common translocations lead to deregulation of genes primarily due to juxtaposition with IGH gene.

We present a case of CLL patient with atypical morphology and an aggressive course of disease. In spite of aggressive treatment including allogeneic hematopoietic stem cell transplantation disease progressed into a rare cutaneous Richter's syndrome. Trisomy 12 was found as a sole chromosomal change at initial cytogenetic analysis of lymphoma cells. At progression, besides trisomy 12 three concomitant balanced translocations t(2;14)(p13;q32), t(14;19)(q32;q13), and t(18;22)(q21;q11) were found. The same karyotype was confirmed in cells aspirated from skin infiltrates at Richter transformation.

Atypical cytological features, trisomy 12, and a progressive course of disease observed in our case are typical for CLL with each of particular Ig translocations that were concomitantly found in CLL for the first time. Similar to “double hit” lymphoma concurrent rearrangements may be relevant also in CLL.