Novel role of fluorescent in situ hybridization technique (FISH) in recommended surgical margins of dermatofibrosarcoma protuberans: A preliminary study.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is a rare skin tumor. There is no standard recommendation for its surgical management. The currently used histological analysis are HES (hematoxylin eosin saffron) staining and immunohistochemistry for CD34 expression in particular cases. Fluorescent in situ hybridization (FISH) technique is only used to qualify the DFSP as translocated or non-translocated and is not used as a diagnostic method. The aim of our study was to determine by FISH (as a diagnostic method) whether cancerous cells that could not be identified through HES staining±immunohistochemistry were present at the two-centimeter margins that were found to be tumor-free. METHODS: Samples from patients who underwent surgery between 2010 and 2018 were collected. Intralesional and peripheral (at 2cm margins) paraffin slides were included. An average of 7.4 slides per specimen was analyzed. Firstly, the preselected slides were reread by a senior pathologist to confirm the absence of microscopic findings of DFSP at 2cm margins. Secondly a FISH analysis was used as a quantitative diagnostic approach, in order to find the t(17;22) translocation. RESULTS: Among the seven specimens that included 2cm margins, two samples presented one or more translocations, which were not visible in standard morphology assessments at two centimeters tumor-free margins. CONCLUSIONS: FISH analysis can have a new role in defining tumor-free margins. This would reduce the incidence of disease recurrence after resection and improve the post-operative complementary care.

Reverse Flow Shunt Restricted Arterialized Venous Free Flap.

Several technical modifications have been described to avoid complications of venous flaps. The authors describe a technical variation of the venous flap to reduce the risk of venous congestion and the likelihood of shunting, thus increasing venous flap reliability.

OUR PRELIMINARY EXPERIENCE WITH A NEW METHOD OF DIEAp FLAP DISSECTION.

BACKGROUND: The abdominal tissue is an ideal source for autologous breast reconstruction. We propose a new approach for intramuscular dissection of a DIEP flap in this paper. METHODS: A total of 84 women underwent breast reconstruction after mastectomy. From this group, 49 patients were treated with traditional DIEP flap of which 21 had unilateral procedure and 28 had bilateral procedure. This new type of dissection was performed in 35 women, with unilateral approach in 14 cases and bilateral approach in 21 cases. RESULTS: The statistical differences are not significant in the two groups with regards to complications (p > 0.1). Mean operative time in this new approach was 3 hours and 10 minutes per flap. Mean operative time in the traditional dissection of DIEP was 3 hours and 41 minutes per flap. The operative time of the new approach is significantly shorter than the dissection of the traditional DIEP flap (p < 0.01). CONCLUSION: The approach to DIEP flap dissection proposed by the authors is a new concept in autologous breast reconstruction. In this type of dissection no fascia is resected and it is more reliable than a traditional DIEP flap for the ease of dissection and for the presence of a small protective cuff of muscle around the vessel with a lower risk of perforator injury.It is a reproducible option of dissection, useful also in less experienced hands and it is a time reducing technique compared with the traditional DIEP flap.

TRIDIMENSIONAL DOPPLER ASSESSMENT: A RELIABLE, NON-INVASIVE AND COST-EFFECTIVE METHOD FOR PREOPERATIVE PERFORATOR ASSESSMENT IN DIEP FLAP.

BACKGROUND: The preoperative perforator mapping is an important step in autologous breast reconstruction, making the flap raising safer, more predictable and time-saving. Although the Doppler exam has proven to be less accurate in locating perforators compared with colour duplex sonography and CTA, it will probably remain of importance in clinical practice. The aim of this paper is to share some advices on how to perform a Doppler exam in preoperative evaluation of a DIEAp flap, increasing its reliability in location of the perforators. METHODS: The study was carried-out preoperatively on 26 consecutive patients. For the evaluation of the matching between Doppler Dot and operative finding was used a Cartesian coordinate systemResults: We have marked preoperatively 145 perforators in 26 patients for a total of 52 semi-abdomens. An average of 5.6 vessels per patient were marked. Of these, 80 (55.17%) were found between 0-1 cm, 36 (24.82%) between 1-2 cm and 5 (3.4%) of these more than 2 cm from each other. We had 24 (16.55%) false positives in which there was no correspondence between the signal and the intraoperative finding. CONCLUSION: Although the Doppler exam may not provide the same anatomic details as the other newer modalities, such as CTA and MRA, the HHD remains a very useful and important tool for autologous reconstruction. We recommend performing this exam in our standardized and reproducible method to improve the reliability..

DELAY PROCEDURE IN THE PERFORASOME ERA: A CASE IN A DIEAP FLAP.

The deep inferior epigastric artery perforator (DIEAp) flap is becoming a widely used method of autologous breast reconstruction. Despite the huge use of the DIEAp flap in reconstructive field, an evidenced based approach in perforator selection has not yet been developed. Unfortunately there is no clear evidence about the relation between the number and dimension of the perforator vessel and the prediction of flap survival in a living model. An old technique like the vascular delay could be extremely useful as a lifeboat procedure when the vascularization of the flap after the dissection is inadequate.

Mycosis fungoides: disease evolution of the "lion queen" revisited.

Mycosis fungoides (MF), which represents the most common subtype of primary cutaneous T-cell lymphoma (CTCL), is an epidermotropic lymphoma included as an indolent form in the recent WHO/EORTC classification. From a clinical point of view, the classic disease progression usually is slow and takes over years or even decades, and characterized by the evolution from patches to more infiltrated plaques and eventually to tumours or erythroderma. However, the analysis of the MF disease course has been greatly impaired by the rarity of the disease, thus data about the time course of disease progression and pattern of relapse during time are not well known. In this review, a summary of published data on MF large patients cohorts will be presented, together with the results obtained by a retrospective analysis of clinical features and follow-up data of 1,422 MF patients diagnosed and followed-up from 1975 to 2010 in 27 Italian Centres (Italian Study Group for Cutaneous Lymphoma). From a clinical perspective, the amount of data support the relevance of a stage-tailored, differentiated follow-up strategy, in as much as the TNMB staging appears not only to be associated with different progression rates, but also shows as a new finding a relationship with different patterns of disease progression. From a biological point of view, there is the need to understand the molecular basis of the different clinical pathways of disease progression, to be able to potentially identify at an earlier phase of disease evolution, the patients who are more likely to develop erythroderma or tumour-stage progression. In conclusion, if MF is indeed a true "lion queen", as dermatologists we need to be expert and wise tamers to keep it under control.

MicroRNA profiling reveals that miR-21, miR486 and miR-214 are upregulated and involved in cell survival in Sézary syndrome.

Sézary syndrome (SS) is an incurable leukemic variant of cutaneous T-cell lymphoma and its pathogenesis is still unknown. Diagnosis/prognosis may strongly ameliorate the management of SS individuals. Here, we profiled the expression of 470 microRNAs (miRNAs) in a cohort of 22 SS patients, and we identified 45 miRNAs differentially expressed between SS and controls. Using predictive analysis, a list of 19 miRNAs, including miR-21, miR-214, miR-486, miR-18a, miR-342, miR-31 and let-7 members were also found. Moreover, we defined a signature of 14 miRNAs including again miR-21, potentially able to discriminate patients with unfavorable and favorable outcome. We validated our data for miR-21, miR-214 and miR-486 by qRT-PCR, including an additional set of array-independent SS cases. In addition, we also provide an in vitro evidence for a contribution of miR-214, miR-486 and miR-21 to apoptotic resistance of CTCL cell line.

Primary cutaneous B-cell lymphoma is associated with somatically hypermutated immunoglobulin variable genes and frequent use of VH1-69 and VH4-59 segments.

BACKGROUND: Accurate assessment of the somatic mutational status of clonal immunoglobulin variable region (IgV) genes is relevant in elucidating tumour cell origin in B-cell lymphoma; virgin B cells bear unmutated IgV genes, while germinal centre and postfollicular B cells carry mutated IgV genes. Furthermore, biases in the IgV repertoire and distribution pattern of somatic mutations indicate a possible antigen role in the pathogenesis of B-cell malignancies. OBJECTIVES: This work investigates the cellular origin and antigenic selection in primary cutaneous B-cell lymphoma (PCBCL). METHODS: We analysed the nucleotide sequence of clonal IgV heavy-chain gene (IgVH) rearrangements in 51 cases of PCBCL (25 follicle centre, 19 marginal zone and seven diffuse large B-cell lymphoma, leg-type) and compared IgVH sequences with their closest germline segment in the GenBank database. Molecular data were then correlated with histopathological features. RESULTS: We showed that all but one of the 51 IgVH sequences analysed exhibited extensive somatic hypermutations. The detected mutation rate ranged from 1.6% to 21%, with a median rate of 9.8% and was independent of PCBCL histotype. Calculation of antigen-selection pressure showed that 39% of the mutated IgVH genes displayed a number of replacement mutations and silent mutations in a pattern consistent with antigenic selection. Furthermore, two segments, VH1-69 (12%) and VH4-59 (14%), were preferentially used in our case series. CONCLUSIONS: Data indicate that neoplastic B cells of PBCBL have experienced germinal centre reaction and also suggest that the involvement of IgVH genes is not entirely random in PCBCL and that common antigen epitopes could be pathologically relevant in cutaneous lymphomagenesis.

Quality of life and psychological distress in patients with cutaneous lymphoma.

BACKGROUND: Cutaneous lymphomas may have a profound impact on patients' health-related quality of life (HRQoL) and psychological well-being. OBJECTIVES: To evaluate HRQoL and psychological distress in patients with cutaneous lymphoma, and to evaluate them in relation to personal and clinical characteristics. METHODS: Patients with cutaneous T-cell lymphoma or cutaneous B-cell lymphoma (CBCL) were consecutively recruited in a dermatological hospital. Data on HRQoL were collected using a dermatology-specific questionnaire, the Skindex-29, and an oncology-specific questionnaire, the EORTC QLQ-C30. RESULTS: Of 95 patients, there were 24 with CBCL, 59 with mycosis fungoides (MF) and 12 with Sézary syndrome (SS). The most frequent items reported in Skindex-29 were itching and sensitive skin, being annoyed by the disease, worry that it could get worse, affected interactions, and impairment in sexual life. The most frequent problems appearing from the EORTC QLQ-C30 analysis were fatigue, pain and insomnia. A worse HRQoL was observed for all the scales in patients with SS, followed by MF, and CBCL. HRQoL impairment in all histotypes was higher in women than in men, in patients with probable anxiety or depression, and when the disease worsened. The highest prevalence of probable anxiety or depression was observed in patients treated with systemic steroids (60%) and interferon (50%). CONCLUSIONS: The detailed evaluation of HRQoL and psychological problems in patients with cutaneous lymphomas, and their relationship with clinical variables, may give important information on the burden of the disease for patients, and thus improve communication and satisfaction with care.

Keratosis lichenoides chronica. Report of a case associated with B-cell lymphoma and leg panniculitis.

Keratosis lichenoides chronica (KLC) is a rare chronic dermatosis characterized by lichenoid hyperkeratotic papules arranged in a linear and reticular pattern, and seborrheic-dermatitis-like lesions on the face. Less frequently, palmoplantar keratoderma, nail dystrophies, mucosal as well as eye lesions are present. KLC affects adults and very few cases have been reported in childhood. Although infrequently, KLC has been associated with systemic diseases, including chronic infectious diseases, kidney disorders and lymphoma. Here we report the case of an adult KLC patient with skin, nail and mucosal involvement, and onset in the first year of life who developed a leg panniculitis and a mantle cell lymphoma. Following chemotherapy for the lymphoma, panniculitis resolved completely, and skin and mucosal KLC lesions ameliorated.

The impact of clinical practice patterns on hospital charges for orthognathic surgery.

To assess the impact of changes in practice patterns, hospital charges (overall and component charges excluding professional fees) for 1992 were compared to 1985 data for three orthognathic surgical procedures: bilateral sagittal split osteotomy (BSSO), Le Fort I osteotomy (LFI), and a combination of both (BSSO/LFI). Data for 1992 were obtained for 59 patients (26 BSSO, 18 LFI, 15 BSSO/LFI). Secondary analysis of similar data for 1985 included 77 patients (27 BSSO, 16 LFI, 34 BSSO/LFI). Changes in clinical practice from 1985 to 1992 reduced mean length of stay (days): BSSO 3.2 to 1.2, LFI 3.7 to 1.4, BSSO/LFI 4.1 to 1.8. Mean total charges (dollars) increased: BSSO 3,086 to 5,023, LFI 3,538 to 6,784, BSSO/LFI 4,778 to 8,816. If 1992 charges are expressed in 1985 dollars, ancillary, pharmacy, and room charges all are reduced. Charges attendant to the surgical procedure (anesthesia, operating room, supply, and recovery) were just over half of total charges in 1985. By 1992, these components escalated to 80% of total charges. Most notable was the increase in supply charges. Charges for rigid fixation were a major component of increased supply charges in 1992. Surgeons minimally influence charges attendant to the operating room and postanesthesia recovery, the major components of total charges in 1992. Quality can be maintained with reduction in some charge categories, but these components total less than 25% of the patient's bill. Technology changes benefit patients, but a judgment must be made weighing benefits against increased health care costs.