Immunohistochemical patterns in the differential diagnosis of rhinopharyngeal granulocytic sarcoma

Granulocytic sarcoma (GS) is a rare extramedullary manifestation of acute myeloid leukemia (AML). GS may develop simultaneously to AML or as a relapse of leukemia, particularly following allogeneic hematopoietic stem cell transplant. Subperiosteal bone, lymph nodes and skin are commonly involved, whereas rhinopharyngeal involvement is less common, with only 14 cases reported in the literature. Due to its rarity, rhinopharyngeal GS may lead to diagnostic pitfalls, particularly when it is poorly differentiated or is without concomitant marrow involvement. Thus, immunohistochemical findings play a key role in diagnosis. The current report describes a case of a 53-year-old female suffering from rhinopharyngeal GS and with a history of AML treated with chemotherapy and radiotherapy, focusing on the importance of the immunohistochemical pattern to assess the right diagnosis. Recent studies have demonstrated that the immunophenotype is of utmost importance for the diagnosis of GS. The high expression of myeloperoxidase (MPO) is common in GS; however, ~30% of GSs do not contain MPO. Therefore, the presence of other markers is required to confirm the diagnosis of GS

Epidermal cyst of temporal bone as a delayed complication of myringoplasty

Epidermal cysts are benign tumors derived from the epidermis or the epithelial hair follicle filled with keratin and lipid-rich debris, typically occurring in areas with a high-density of sebaceous glands. These cysts commonly occur on the face, scalp, neck and trunk, where the sebaceous glands are more active. Their localization within the bone is extremely uncommon. The current study details the case of a 24-year-old male who presented with right otorrhea and ipsilateral hypoacusia having undergone right overlay myringoplasty for subtotal eardrum perforation. This patient represents a rare case of an epidermal cyst localized in the temporal bone (the fifth described in English-language literature), which may be considered as a complication of myringoplasty

Laparoscopic patch repair of recurrent anterior diaphragmatic hernia in a child with SMA

An anterior congenital diaphragmatic hernia (CDH) is a diaphragmatic defect that allows the passage of abdominal organs into the thorax. It is typically asymptomatic (the diagnosis is incidental) and it requires surgical correction. In this paper we present a 6 year-old girl affected by spinal muscular atrophy (SMA) who was diagnosed with anterior CDH. Four years after laparoscopic closure of the defect by interrupted suture the girl returned for hernia recurrence. Another laparoscopic procedure was performed and the defect was closed using a GORE-TEX patch. We postulate a mechanism of altered respiratory dynamic and increased abdominal pressure related to scoliosis favouring CDH recurrence in patients with neuromuscular pathologies such as SMA. In these patients patch interposition should be considered in the first place in order to reduce tension over margins. Laparoscopy is a safe and feasible procedure for CDH correction also in case of recurrence and when the interposition of a patch is required

Skull Base Fungal Osteomyelitis: A Case Report and Review of the Literature

Skull base osteomyelitis (SBO) is an invasive infection refractory to therapy, closely linked with malignant otitis externa (MOE). It is characterized by a mild clinical presentation that can delay cross-sectional imaging considered as the key to revealing it. Skull base osteomyelitis typically affects elderly diabetics and immunocompromised patients (>70 years). It most commonly has an otogenic origin due to an extension of MOE. The prognosis can be very poor without the administration of adequate and timely therapy at an early disease stage. Nowadays, Pseudomonas aeruginosa remains the most common pathogen associated with SBO. Fungi are a rare cause of MOE. This report documents a rare case of otogenic SBO caused by Candida parapsilosis in a diabetic patient, with persistent otologic symptoms as clinical onset and resistance to medical treatment. Fungal MOE has more subtle symptoms and is more aggressive than its bacterial counterpart. When MOE is resistant to antibacterial drugs, this should raise the suspicion of a fungal etiology of MOE. The current guidelines do not exhaustively describe the diagnosis, antifungal drugs of choice, and optimum duration of treatment. The description of these rare clinical cases should help with the multidisciplinary management of this disease in order to optimize the diagnosis and therapeutic protocol

Modeling Lung Carcinoids with Zebrafish Tumor Xenograft

Lung carcinoids are neuroendocrine tumors that comprise well-differentiated typical (TCs) and atypical carcinoids (ACs). Preclinical models are indispensable for cancer drug screening since current therapies for advanced carcinoids are not curative. We aimed to develop a novel in vivo model of lung carcinoids based on the xenograft of lung TC (NCI-H835, UMC-11, and NCI-H727) and AC (NCI-H720) cell lines and patient-derived cell cultures in Tg(fli1a:EGFP)(y1) zebrafish embryos. We exploited this platform to test the anti-tumor activity of sulfatinib. The tumorigenic potential of TC and AC implanted cells was evaluated by the quantification of tumor-induced angiogenesis and tumor cell migration as early as 24 h post-injection (hpi). The characterization of tumor-induced angiogenesis was performed in vivo and in real time, coupling the tumor xenograft with selective plane illumination microscopy on implanted zebrafish embryos. TC-implanted cells displayed a higher pro-angiogenic potential compared to AC cells, which inversely showed a relevant migratory behavior within 48 hpi. Sulfatinib inhibited tumor-induced angiogenesis, without affecting tumor cell spread in both TC and AC implanted embryos. In conclusion, zebrafish embryos implanted with TC and AC cells faithfully recapitulate the tumor behavior of human lung carcinoids and appear to be a promising platform for drug screening

CHRONIC RHINOSINUSITIS IN CYSTIC FIBROSIS PATIENTS: SMELL EVALUATION

Cystic Fibrosis (CF) involves the upper airways with chronic rhinosinusitis (CRS) causing nasal congestion, rhinorrhea, mouth breathing, facial pain, and olfactory dysfunction. Twelve to 71% of CF patients report smelling alterations impacting nutrition and quality of life. The aim was to study olfaction performance in CF patients with CRS that worsens quality of life. One hundred and twenty-one subjects were enrolled in this study. Seventy-one had CF and underwent ear, nose, and throat evaluation with nasal endoscopy, SNOT-22, VAS and “Sniffin’ Sticks”. Fifty subjects were age-matched with healthy controls. All 71 CF patients were affected by CRS; 59/71 (83.1%) had CRS without nasal polyps and 12/71 (16.9%) had CRS with early nasal polyps. None of the 50 controls had CRS. Total SNOTT-22 mean values in the 71 CF patients was 38.10 ± 21.08 pts. If considering only the 59 CF patients without nasal polyps the SNOTT-22 mean value was 36.76 ± 21.52 pts. Moreover, based on the VAS scores, the degree of nasal symptoms was classified as mild for facial pain, smell alteration, nasal discharge, and sneezing and resulted in moderate symptoms for nasal blockage and headache. Among the CF patients, 55/71 (76.5%) declared normosmia while the smelling ability assessed by “Sniffin’ Sticks” showed that only 4/71 (5.63%) were normosmic, 58 (81.69%) were hyposmic, and 9 (12.68%) were anosmic. In the controls 41(82%) were normosmic, 9 (18%) were hyposmic, and none were reported anosmia (p < 0.001). The study confirms that most CF patients have a relevant olfactory impairment, although only a low percentage declare it. A careful evaluation with simple and rapid tests helps to select the patients that may benefit from specific therapies

Assessing the extent and timing of chemosensory impairments during COVID-19 pandemic

Chemosensory impairments have been established as a specific indicator of COVID-19. They affect most patients and may persist long past the resolution of respiratory symptoms, representing an unprecedented medical challenge. Since the SARS-CoV-2 pandemic started, we now know much more about smell, taste, and chemesthesis loss associated with COVID-19. However, the temporal dynamics and characteristics of recovery are still unknown. Here, capitalizing on data from the Global Consortium for Chemosensory Research (GCCR) crowdsourced survey, we assessed chemosensory abilities after the resolution of respiratory symptoms in participants diagnosed with COVID-19 during the first wave of the pandemic in Italy. This analysis led to the identification of two patterns of chemosensory recovery, partial and substantial, which were found to be associated with differential age, degrees of chemosensory loss, and regional patterns. Uncovering the self-reported phenomenology of recovery from smell, taste, and chemesthetic disorders is the first, yet essential step, to provide healthcare professionals with the tools to take purposeful and targeted action to address chemosensory disorders and their severe discomfort

Assessing the extent and timing of chemosensory impairments during COVID-19 pandemic

Chemosensory impairments have been established as a specific indicator of COVID-19. They affect most patients and may persist long past the resolution of respiratory symptoms, representing an unprecedented medical challenge. Since the SARS-CoV-2 pandemic started, we now know much more about smell, taste, and chemesthesis loss associated with COVID-19. However, the temporal dynamics and characteristics of recovery are still unknown. Here, capitalizing on data from the Global Consortium for Chemosensory Research (GCCR) crowdsourced survey, we assessed chemosensory abilities after the resolution of respiratory symptoms in participants diagnosed with COVID-19 during the first wave of the pandemic in Italy. This analysis led to the identification of two patterns of chemosensory recovery, partial and substantial, which were found to be associated with differential age, degrees of chemosensory loss, and regional patterns. Uncovering the self-reported phenomenology of recovery from smell, taste, and chemesthetic disorders is the first, yet essential step, to provide healthcare professionals with the tools to take purposeful and targeted action to address chemosensory disorders and their severe discomfort

Velocity of Ultrasonic Waves in Solutions of Electrolytes

List of the genes involved in CVDs according to DisGeNET database. For each gene all the related diseases and the putative EV-MiRNAs targeting it are indicated both as list and as number of occurrences. (PDF 1206 kb