Search

Resumen El bocio amiloide es una entidad benigna poco frecuente que se caracteriza por el aumento brusco de tamano de la glandula, condicionado por el deposito de amiloide en el parenquima tiroideo. Este aumento de tamano, que se produce en el espacio de meses, puede producir en el paciente sintomas como disnea, disfagia o disfonia. Presentamos tres casos diagnosticados en el mismo centro y estimamos la prevalencia de esta entidad. Todos los casos mostraron una morfologia similar, consistente en la presencia de foliculos tiroideos, tejido adiposo maduro y amiloide en proporciones variables. Se constato acumulo de amiloide AA y su asociacion con patologias cronicas (enfermedad de Crohn, artritis reumatoide juvenil y enfermedad renal cronica). Uno de los casos presentaba incidentalmente un microcarcinoma papilar. Es una entidad poco frecuente que debe tenerse en cuenta en el diagnostico diferencial de pacientes con bocio, sobre todo en aquellos que presentan enfermedades cronicas de base.

BackgroundSecondary haemophagocytic lymphohistiocytosis (sHLH) is characterised by a hyper activation of immune system that leads to multiorgan failure. It is suggested that excessive immune response in patients with COVID-19 could mimic this syndrome. Some COVID-19 autopsy studies have revealed the presence of haemophagocytosis images in bone marrow, raising the possibility, along with HScore parameters, of sHLH.AimOur objective is to ascertain the existence of sHLH in some patients with severe COVID-19.MethodsWe report the autopsy histological findings of 16 patients with COVID-19, focusing on the presence of haemophagocytosis in bone marrow, obtained from rib squeeze and integrating these findings with HScore parameters. CD68 immunohistochemical stains were used to highlight histiocytes and haemophagocytic cells. Clinical evolution and laboratory parameters of patients were collected from electronic clinical records.ResultsEleven patients (68.7%) displayed moderate histiocytic hyperplasia with haemophagocytosis (HHH) in bone marrow, three patients (18.7%) displayed severe HHH and the remainder were mild. All HScore parameters were collected in 10 patients (62.5%). Among the patients in which all parameters were evaluable, eight patients (80%) had an HScore >169. sHLH was not clinically suspected in any case.ConclusionsOur results support the recommendation of some authors to use the HScore in patients with severe COVID-19 in order to identify those who could benefit from immunosuppressive therapies. The presence of haemophagocytosis in bone marrow tissue, despite not being a specific finding, has proved to be a very useful tool in our study to identify these patients.

Lymphoplasmacytic lymphoma (LPL) and marginal zone lymphoma (MZL) frequently infiltrate the bone marrow with similar histologic and immunohistochemical characteristics posing diagnostic problems. Bone marrow biopsy specimens from 25 LPL and 16 MZL have been studied, correlating with clinical, laboratory parameters and the MYD88_p.L265P mutation. Paratrabecular and interstitial infiltration pattern, serum IgM paraprotein levels, and MYD88_p.L265P mutation were significantly more frequent in LPL. Nodular or intrasinusoidal pattern with lymphocytosis and splenomegaly were associated with MZL diagnosis. Different clinical and histological parameters should be collected when LPL or MZL is suspected in bone marrow biopsy specimens.

Perineal carcinoma of unknown primary origin (CUP) is a rare entity and represents a diagnostic and therapeutic challenge. These tumors may respond well to a combination of surgical resection, when feasible, local radiotherapy, and platinum-based systemic chemotherapy. A 67-year-old male patient consulted for urinary discomfort associated with perineal abscess. The perineal abscess was drained, and the patient was diagnosed with perineal carcinoma of unknown origin. Computed tomography (CT) scan shows a large perineal mass that involves both corpora cavernosa and corpus spongiosum. CT and bone scan excluded the metastatic spread of the disease. Inguinal lymph nodes were not identified. The surgical treatment consisted of two different phases. Phase I: the perineal mass exeresis with total penectomy. Phase II: perineal neourethrostomy with double oral mucosa graft. Coverage of the perineal defect and morphological reconstruction of the penis with fasciocutaneous and gracilis flap. The surgical intervention lasted for nine hours and the estimated blood loss was 500cc. The patient was discharged twelve days after the intervention. Later he received adjuvant treatment with immunotherapy (Cemiplimab) because was not subsidiary to treatment with platinum for renal failure. The patient died after nine months of follow-up due to pulmonary and abdominal metastatic spread.

Immune checkpoint inhibitors have revolutionized cancer treatment due to their undeniable efficacy, but a range of new adverse events (AE) has emerged. In particular, cardiac toxicity is a potentially fatal AE, and introduces new challenges regarding its underlying molecular mechanisms of occurrence, optimal treatment and follow up, and prevention. We present a clinical case of a patient with advanced kidney cancer treated with nivolumab as a third line treatment. After four cycles, the patient developed nonspecific symptoms and was hospitalized, identifying a set of clinical, analytical and electrocardiographic alterations compatible with myocarditis. Despite the intensive support, the patient died and a necropsy study was performed. We present a detailed description of the clinical case including the pathological and molecular findings, and we conduct a review of the available evidence related to immune-mediated cardiac toxicity to offer some new highlights in the management of this AE.

Made available in DSpace on 2022-04-29T08:31:19Z (GMT). No. of bitstreams: 0 Previous issue date: 2021-01-01 Purpose: To describe the characteristics and recurrence rates of primary periocular squamous cell carcinoma (SCC) in Spain. Methods: This retrospective study investigates the characteristics of primary periocular SCC at two tertiary centers in Spain from 2000 to 2020. Data were collected on demographics, skin phenotype, location, clinical and histological diagnosis, the commitment of surgical margins, recurrence, and risk factors. Multivariate analysis and risk factors were used to investigate recurrence rates, considering p < 0.05 as statistically significant. Results: Over the 20-year studied period, 107 patients with primary periocular SCC were assessed. The mean age of SCC was 76.8 ± 12.8 years, 55 (50.9%) were females, and 105 (98.1%) had Fitzpatrick skin phenotype type II or III. SCC lesions affected less than 1/3 of the eyelid (56/52.3% cases), mainly the lower lid (42/39.3% lesions). Sixty (56.1%) cases were SCC differentiated, 76 (71%) had clear margins. Clinical diagnosis of SCC corroborated with histological in 84 (78.5%) cases and orbital involvement occurred in 18 (16.8%) cases. Recurrence was observed in 13 (12.1%) cases, occurring more in large tumors and undifferentiated subtype (53.8%), with committed margins (69.2%) (p = 0.001), being the commitment of margins a significant predictor for recurrence, although thickness was an independent predictor. Conclusion: Periocular SCC in Spain showed a similar pattern as in the world. The risk of recurrence is increased in undifferentiated and large periocular SCC, partially removed with committed margins. Department of Ophthalmology Rio Hortega University Hospital Department of Pathology Rio Hortega University Hospital Department of Pathology Hospital Ramón y Cajal Department of Ophthalmology Oculoplastic Unit Hospital Ramón y Cajal Department of Oculoplastic Instituto de Microcirugía Ocular Madrid Department of Ophthalmology Faculdade de Medicina de Botucatu − UNESP Department of Ophthalmology Faculdade de Medicina de Botucatu − UNESP

Background Primary cardiac lymphomas are extremely rare entities ( Case presentation We describe a case of FA-DLBCL arising in a cardiac myxoma, with plasmacytic differentiation and type I EBV latency. Conclusions Although they are very rare, FA-DLBCLs should be known for their diagnostic difficulty, due to its unspecified clinical manifestations, and for their more favourable prognosis, sometimes even without additional treatment after surgical resection.

Amyloid goitre is an infrequent benign entity characterized by a rapid increase in the size of the thyroid gland due to amyloid deposit in the parenchyma. Such an increase in size, which takes place over only a few months, can cause symptoms such as dyspnea, dysphagia and dysphonia. We present three cases diagnosed in the same centre and estimated its incidence. All cases showed similar morphology, with varying proportions of thyroid follicles, mature adipose tissue and amyloid. AA Amyloid accumulation was verified and associated to previous pathologies (Crohn's disease, juvenile rheumatoid arthritis and chronic renal disease). One case showed a papillary microcarcinoma. Amyloid goitre is an infrequent entity which should be considered in the differential diagnosis of all patients with goitre, especially those with chronic underlying diseases.

Immune checkpoint inhibitors have revolutionized cancer treatment due to their undeniable efficacy, but a range of new adverse events (AE) has emerged. In particular, cardiac toxicity is a potentially fatal AE, and introduces new challenges regarding its underlying molecular mechanisms of occurrence, optimal treatment and follow up, and prevention. We present a clinical case of a patient with advanced kidney cancer treated with nivolumab as a third line treatment. After four cycles, the patient developed nonspecific symptoms and was hospitalized, identifying a set of clinical, analytical and electrocardiographic alterations compatible with myocarditis. Despite the intensive support, the patient died and a necropsy study was performed. We present a detailed description of the clinical case including the pathological and molecular findings, and we conduct a review of the available evidence related to immune-mediated cardiac toxicity to offer some new highlights in the management of this AE., Video abstract Point your SmartPhone at the code above. If you have a QR code reader the video abstract will appear. Or use: https://youtu.be/ItopEuGMDuI