Tratamiento quirúrgico del carcinoma de Merkel facial avanzado. Presentación de un caso / Surgical treatment of advanced facial Merkel cell carcinoma. A case report

Introducción. El carcinoma de Merkel es un tumor maligno poco frecuente, que afecta principalmente a la población caucásica y cuya etiología guarda relación con el poliomavirus de las células de Merkel. Conlleva mal pronóstico, especialmente en estadios finales. Caso clínico. Se expone el caso de una paciente que presentaba un tumor primario facial de grandes dimensiones, con avanzado grado de extensión, afectación linfática cervical y metástasis parotídea derecha. Fue tratada mediante exéresis de la lesión primaria y cobertura con injerto de piel parcial, linfadenectomía cervical y parotidectomía ipsilateral. Resultados. Se logró mejoría importante en la calidad de vida de la paciente y sobrevida de al menos seis meses. Conclusión. Aunque no está claro el manejo óptimo del carcinoma de Merkel avanzado debido a su mal pronóstico, la cirugía favorece una mejoría en la calidad de vida del paciente y puede tener un papel clave en el manejo del carcinoma de Merkel en los estadios avanzados.

Introduction. Merkel carcinoma is a rare malignant tumor that mainly affects the Caucasian population and whose etiology is related to the Merkel cell polyomavirus. It has a poor prognosis, especially in the final stages. Clinical case. The case of a patient who presented a large primary facial tumor, with an advanced degree of extension, cervical lymphatic involvement and right parotid metastasis is described. She was treated surgically by excision of the primary lesion and coverage with partial skin graft, cervical lymphadenectomy, and ipsilateral parotidectomy. Results. A significant improvement was achieved in the patient's quality of life and survival of at least six months.Conclusion. Although the optimal management of advanced Merkel carcinoma is unclear due to its poor prognosis, surgery improves the patient's quality of life and it can play a key role in the management of Merkel carcinoma in advanced stages.

Lung Transplantation for Multiple Giant Hamartomas: First Case Report and Review of the Literature.

BACKGROUND: To assess lung transplant as a solution for a rare complication of giant lung hamartoma, in which surgical resection was discouraged because of severe underlying chronic lung disease. METHODS: A 54-year-old woman had an episode of severe acute hemoptysis. Imaging techniques showed 2 masses in the right lung as hamartomas and severe pulmonary emphysema. After resolution of the acute episode of hemoptysis, the case could have been solved with a right pneumonectomy; however, a single-lung transplant was performed because of the underlying chronic obstructive pulmonary disease. RESULTS: The outcome of the surgery was satisfactory, although a second operation was required because of bleeding. The patient was discharged and currently has a good quality of life, with no recurrence of the disease. The pathologic anatomy of the surgical specimen confirmed the diagnosis of multiple giant hamartomas. CONCLUSIONS: Lung transplant could be a feasible procedure to treat life-threatening complications of benign tumors in selected patients with pulmonary end-stage disease.

Pathogenic mechanisms involving the interplay between adipose tissue and auto-antibodies in rheumatoid arthritis.

We aimed to evaluate the association between adipose tissue (AT) dysfunction, autoimmunity, and disease activity in rheumatoid arthritis (RA). A cross-sectional study including 150 RA patients and 50 healthy donors and longitudinal study with 122 RA patients treated with anti-tumor necrosis factor (TNF)-α, anti-interleukin 6 receptor (IL6R) or anti-CD20 therapies for 6 months were carried out. In vitro experiments with human AT and adipocyte and macrophage cell lines were performed. A collagen-induced arthritis mouse model was developed. The insulin resistance and the altered adipocytokine profile were associated with disease activity, the presence of anti-citrullinated proteins anti-bodies (ACPAs), and worse response to therapy in RA. AT in the context of arthritis is characterized by an inflammatory state alongside the infiltration of macrophages and B/plasmatic cells, where ACPAs can have a direct impact, inducing inflammation and insulin resistance in macrophages and promoting a defective adipocyte differentiation, partially restored by biologicals.

Prognosis and survival of patients diagnosed with well-differentiated and dedifferentiated retroperitoneal liposarcoma.

OBJECTIVES: The main objective is to establish the overall survival and disease-free survival profiles regarding the patients with retroperitoneal liposarcoma, making a comparison based on the well-differentiated and dedifferentiated histological subtypes. The secondary objectives are to descriptively analyze the clinical characteristics of said patients and to identify and analyze other independent variables that might modify these survival profiles significantly. METHODS: An observational and analytical study was performed using a retrospective historical cohort that was followed prospectively. The inclusion criteria consisted of: the procedure of liposarcoma located in the retroperitoneum, the well-differentiated and dedifferentiated histological subtypes, between January of 2002 and May of 2019. As a result, 32 patients took part in this study's sample. Kaplan-Meier estimator was used to summarise the results and log-rank test was used in the comparative analysis. RESULTS: The overall survival at 5 years was around 59%. No differences were found between the patients with a well-differentiated subtype compared to the dedifferentiated ones (p = 0.834). The disease-free survival at 2 years was 59% regarding the well-differentiated and 26% regarding the dedifferentiated, with these differences being statistically significant (p = 0.005). None of the other studied variables modified these survival profiles significantly. CONCLUSIONS: Dedifferentiated retroperitoneal liposarcomas show less disease-free survival than well-differentiated liposarcomas. However, regarding overall survival no differences can be claimed.

First Report in the Literature of Biopsy-Proven Noncollapsing Focal Segmental Glomerulosclerosis Relapse in a Second Renal Transplant Presenting With Thrombotic Microangiopathy: A Case Report.

Primary focal segmental glomerulosclerosis (FSGS) is a podocytopathy with an irregular response to immunosuppressive therapies. FSGS relapse occurs in 30% to 80% of kidney grafts, and poor survival outcomes include large proteinuria and the nephrotic syndrome's cardinal clinical features. Thrombotic microangiopathy (TMA) is caused by endothelial injury due to complement dysregulation including acute kidney injury, proteinuria, and severe hypertension common renal presentations. Both pathologies have well-described genetic forms, but their relationship remains uncertain. FSGS lesions can be found in kidney biopsy specimens in patients with TMA, and TMA has been reported in patients with collapsing glomerulopathy. However, this combination has not been clearly described in renal transplant recipients. We present the case of a 22-year-old man who received his second kidney allograft and developed an early graft disfunction with nephrotic syndrome and clinical TMA. His background was remarkable for primary, biopsy-confirmed FSGS in childhood, and he started hemodialysis in 2006 and received a living donor kidney graft the same year. He presented with a FSGS relapse with malignant hypertension and seizures in the first posttransplant month and had an irregular response to plasma exchange and rituximab, and dialysis was reinitiated 10 years later. A total of 3 biopsies were performed after his second kidney transplant showing the evolution of a FSGS relapse with histologic and clinical TMA in the absence of identified genetic mutations. Partial responses to treatments with plasma exchange, eculizumab, and rituximab were obtained, but the allograft was lost after 26 months. This case is the first report of concomitant FSGS and TMA in a renal transplant recipient.

Surgical management of Miescher's cheilitis: a case report.

Miescher's cheilitis, also known as cheilitis granulomatosa, is an infrequent disease characterized by chronic recurrent swelling of one lip or both lips. It is considered as one of the three main symptoms of the triad of the Melkersson-Rosenthal syndrome, although in many cases it may develop monosymptomatically. The initial management is based on the administration of corticoids, followed in many cases by the use of other systemic treatments. Nevertheless, because recurrence is quite frequent, surgery remains in many cases as the only definitive treatment. In this report we present the case of a Caucasian woman with Miescher's cheilitis who was successfully surgically managed.

Prognosis and survival of patients diagnosed with well-differentiated and dedifferentiated retroperitoneal liposarcoma. / Pronóstico y supervivencia de pacientes diagnosticados de liposarcoma retroperitoneal bien diferenciado y desdiferenciado.

OBJECTIVES: The main objective is to establish the overall survival and disease-free survival profiles regarding the patients with retroperitoneal liposarcoma, making a comparison based on the well-differentiated and dedifferentiated histological subtypes. The secondary objectives are to descriptively analyze the clinical characteristics of said patients and to identify and analyze other independent variables that might modify these survival profiles significantly. METHODS: An observational and analytical study was performed using a retrospective historical cohort that was followed prospectively. The inclusion criteria consisted of: the procedure of liposarcoma located in the retroperitoneum, the well-differentiated and dedifferentiated histological subtypes, between January 2002 and May 2019. As a result, 32 patients took part in this study's sample. Kaplan-Meier estimator was used to summarize the results and log-rank test was used in the comparative analysis. RESULTS: The overall survival at 5 years was around 59%. No differences were found between the patients with a well-differentiated subtype compared to the dedifferentiated ones (p=0.834). The disease-free survival at 2 years was 59% regarding the well-differentiated and 26% regarding the dedifferentiated, with these differences being statistically significant (p=0.005). None of the other studied variables modified these survival profiles significantly. CONCLUSIONS: Dedifferentiated retroperitoneal liposarcomas show less disease-free survival than well-differentiated liposarcomas. However, regarding overall survival no differences can be claimed.

Ultrasonographic and Histological Correlation after Experimental Reconstruction of a Volumetric Muscle Loss Injury with Adipose Tissue.

Different types of scaffolds are used to reconstruct muscle volume loss injuries. In this experimental study, we correlated ultrasound observations with histological findings in a muscle volume loss injury reconstructed with autologous adipose tissue. The outcome is compared with decellularized and porous matrix implants. Autologous adipose tissue, decellularized matrix, and a porous collagen matrix were implanted in volumetric muscle loss (VML) injuries generated on the anterior tibial muscles of Wistar rats. Sixty days after implantation, ultrasound findings were compared with histological and histomorphometric analysis. The muscles with an autologous adipose tissue implant exhibited an ultrasound pattern that was quite similar to that of the regenerative control muscles. From a histological point of view, the defects had been occupied by newly formed muscle tissue with certain structural abnormalities that would explain the differences between the ultrasound patterns of the normal control muscles and the regenerated ones. While the decellularized muscle matrix implant resulted in fibrosis and an inflammatory response, the porous collagen matrix implant was replaced by regenerative muscle fibers with neurogenic atrophy and fibrosis. In both cases, the ultrasound images reflected echogenic, echotextural, and vascular changes compatible with the histological findings of failed muscle regeneration. The ultrasound analysis confirmed the histological findings observed in the VML injuries reconstructed by autologous adipose tissue implantation. Ultrasound can be a useful tool for evaluating the structure of muscles reconstructed through tissue engineering.

Histology of skeletal muscle reconstructed by means of the implantation of autologous adipose tissue: an experimental study.

The purpose of this study was to determine the histological characteristics of a skeletal muscle reconstructed by means of the implantation of autologous adipose tissue following an experimentally-induced volumetric muscle loss. A cylindrical piece in the belly of the rat anterior tibial muscle was removed. In the hole, inguinal subcutaneous adipose tissue of the same rat was grafted. Animals were sacrificed 7, 14, 21, 28 and 60 days posttransplantation. Histological, histochemical, immunohistochemical and morphometric techniques were used. At all times analyzed, the regenerative muscle fibers formed from the edges of the muscle tissue showed histological, histochemical and immunohistochemical differences in comparison with the control group. These differences are related to delays in the maturation process and are related to problems in reinnervation and disorientation of muscle fibers. The stains for MyoD and desmin showed that some myoblasts and myotubes seem to derive from the transplanted adipose tissue. After 60 days, the transplant area was 20% occupied by fibrosis and by 80% skeletal muscle. However, the neo-muscle was chaotically organized showing muscle fiber disorientation and centronucleated fibers with irregular shape and size. Our results support the hypothesis that, at least from a morphological point of view, autologous adipose tissue transplantation favors reconstruction following a volumetric loss of skeletal muscle by combining the inherent regenerative response of the organ itself and the myogenic differentiation of the stem cells present in the adipose tissue. However, in our study, the formed neo-muscle exhibited histological differences in comparison with the normal skeletal muscle.