NON SURGICAL TREATMENT OF CARCINOID LUNGS TUMORS

Typical carcinoid lungs tumors are neuroendocrine bronchopulmonary tumors with a low-grade malignancy, and an atypical carcinoid is an intermediate form of these tumors. Their systemic treatment is greatly influenced by therapeutic evidence derived from the more frequent gastroenteropancreatic neuroendocrine neoplasms. Currently, systemic therapies for lung carcinoids, aiming at controlling tumor growth include long acting somatostatin analogues (SSAs), peptide receptor radionuclide therapy, chemotherapy and molecular-targeted therapy.

Follicular dendritic cell sarcoma of liver: a rare case report

Follicular dendritic cell sarcoma (FDCS) most commonly involves lymph nodes but also affects extranodal sites like liver, spleen, pancreas less commonly. It is a low to intermediate grade malignancy. Unusual presentation, morphology or immunophenotyping makes the diagnosis of FDCS very challenging. Immunohistochemistry plays a major role in confirming the diagnosis of FDCS. A 40 year male presented with dull abdominal pain. Ultrasonography revealed two hypoechoiec lesions in liver. Cytomorphology, later histological and immunohistochemistry (IHC) diagnosis of FDCS was established. Patient was put on chemotherapy.

Secretory Carcinoma in Children and Young Adults: A Case Series

Secretory carcinoma (SC), previously known as mammary analogue secretory carcinoma, is a rare salivary gland neoplasm that typically presents as a slow-growing painless lesion in the head and neck. SC occurs mainly in adults but has been described in children with the youngest reported patient diagnosed at five years of age. In children the gender distribution has been reported as female to male ratio of 1:1.2. SC is generally considered a low-grade malignancy with characteristic morphological features and immunological profile. SC also harbors ETV6-NTRK3 fusion (t(12;15)(p13:q25)). Surgical resection with or without lymph node dissection is the standard treatment, with generally favorable clinical outcomes. Here we present a single institution case series of six patients (ages 9-21) with SC and a review of the previously described pediatric cases. Our small series showed male predominance in pediatric patients with predominantly low-grade and stage tumors. All cases underwent complete surgical resections and when follow up is available there was no evidence of recurrences or metastases. To the best of our knowledge, this is the only SC case series comprised exclusively of pediatric and youth patients.

circSMARCA5-SRSF1 interaction is functionally involved in GBM pathogenesis

Glioblastoma multiforme (GBM) is the most aggressive human brain tumor with a median survival of 15 months. The standard treatments of GBM and the total medical resection are unable to contrast this mortal cancer. For these reason new diagnostic approaches and therapies are needed. The identification of molecular features of this cancer may allow to create a personalized therapy. Circular RNAs (circRNAs) are a new class of non-coding RNAs (ncRNAs) highly enriched in brain, stable within the cells, detectable in body fluids and having a potential role and biological importance still object of debate. This thesis investigated the putative involvement of circRNAs in GBM pathogenesis. It has been demonstrated that circSMARCA5 is downregulated in GBM biopsies, its expression is associated to the glioma grade malignancy and it negatively regulates migration of U87MG cells. Moreover, it has been proved the physical interaction between circSMARCA5 and one of its predicted interactor Serine/arginine-rich splicing factor 1 (SRSF1). Interesting splicing targets of SRSF1 are the serine and arginine rich splicing factor 3 (SRSF3) and the vascular endothelial growth factor A (VEGFA). It has been proposed that circSMARCA5 may regulate the alternative splicing of SRSF3 in favor of the formation of a stable oncoprotein in GBM. It also regulates the alternative splicing of VEGFA mRNA through the binding to SRSF1. In addition, blood vascular microvessel density evaluated in GBM negatively correlates with the expression of circSMARCA5, while positively correlates with that of SRSF1 and pro-to-anti-angiogenic VEGFA isoform ratio. GBM patients with low circSMARCA5 expression have lower overall and progression free survival rates. Based on these findings, CircSMARCA5 could be considered a promising druggable tumor suppressor in GBM. Moreover, its interaction with SRSF1 makes circSMARCA5 an upstream regulator of pro- to anti-angiogenic VEGFA isoforms ratio within GBM cells and a highly promising GBM prospective anti-angiogenic molecule.

Mass-forming chronic invasive fungal sinusitis; an uncommon mimicker of head & neck malignancy

Introduction/Objective Invasive fungal sinusitis is a progressive infection affecting the nasal cavity and associated sinus spaces. Acute forms affect immunocompromised patients with debilitating systemic diseases while chronic forms usually occur in immunocompetent individuals. Methods/Case Report A 26-year-old immunocompetent male with daily Marijuana smoking consulted for acute swelling of the right eye with dull pain, photophobia, loss of vision and pressure of 4 day duration. He reported nasal congestion for approximately one year and over the previous 2 months developed exophthalmos. Imaging demonstrated a 4.9 x 4.3 x 4.1 cm invasive soft tissue mass in the right lamina papyracea with involvement of the paranasal sinus, right orbit, and intracranial space. The mass was presumed to represent a high-grade malignancy. Biopsy was performed and demonstrated granulomatous invasive fungal sinusitis. The patient underwent right orbitotomy and right extranasal ethmoidectomy. He was begun on empiric amphotericin B. After culture and sequencing identification of Curvularia species, voriconazole was added. Follow up imaging studies after two month of treatment still demonstrated residual mass forming lesion with 10-20% decrease in size. The patient has regained his vision after 4 month of treatment and he is expected to complete a six months course. If the mass forming lesion persists, a second round of surgical debridement will be performed. Results (if a Case Study enter NA) N/A Conclusion Curvularia is a dematiaceous fungus most commonly associated with allergic sinusitis, but can rarely become invasive in immunocompetent individuals. Histopathologically large fungal elements are easily recognizable but specific identification requires culture or ribosomal DNA sequencing by PCR. Mass-forming chronic fungal sinusitis overlaps clinically and radiologically with head and neck malignancies. Our report brings attention to this uncommon clinical variant of invasive fungal sinusitis that affects immunocompetent individuals.

Isolated High-Grade Malignancy of the Spinal Cord Presenting as Longitudinally Extensive Transverse Myelitis

This article characterizes 2 cases of longitudinally extensive transverse myelitis (LETM) that did not respond to immunotherapy and were diagnosed by biopsy as primary central nervous system (CNS) malignancies. Diffuse H3 K27M-mutant glioma is a recently described entity with very few cases of isolated spinal disease described in adults. Primitive neuroectodermal tumor is similarly uncommon in the spinal cord. Malignancies should be considered in patients who fail to improve with immunomodulatory therapy. We believe the experiences of our center will raise awareness about that point, broaden the existing understanding of the diagnostic approach to LETM, and highlight the need for additional studies.

High Rate of Epidermal Growth Factor Receptor-Mutated Primary Lung Cancer in Patients with Primary Breast Cancer

Background With increased survival in breast cancer, resulting from advances in treatment, patients incur the possibility of subsequent primary malignancies, especially lung cancer. The aim of this study was to assess the frequency of CT-detected pulmonary ground-glass nodules and lung cancer following breast cancer diagnosis, the associations between breast cancer and lung cancer, the pathological features of double primary cancer, and the status of epidermal growth factor receptor (EGFR) mutations in second primary lung cancer. Methods Clinical data from more than 8000 individuals who were diagnosed with primary breast cancer at Jiangsu Province Hospital (Jiangsu, China) between January 2008 and December 2020 were retrospectively analyzed. Results Of the 8048 patients, 55 (0.7%) were diagnosed with a second primary lung cancer, which accounted for approximately 14.8% of the pulmonary ground-glass opacity (GGO) detected. The incidence was higher than in the general female population (standardized incidence ratio 1.4 [95% confidence interval (CI): 1.25–1.55]). Patients who experienced a second primary lung cancer exhibited a significantly higher rate of EGFR mutation (78.5%) than those with lung adenocarcinoma alone, with most exhibiting low-grade malignancy, older age, estrogen receptor negativity, low Ki67, and no lymph node metastasis. Conclusions Breast cancer patients, especially those with low-grade malignancy, were at high risk for developing primary lung cancer. For isolated GGO in patients with high-risk factors, clinicians should insist on close follow-up. Furthermore, EGFR may play an important role in primary lung adenocarcinomas and breast cancer.

Predictors of laboratory spontaneous tumour lysis syndrome in children with high-grade tumours in Uganda

High-grade malignancy is endemic in sub-Saharan Africa and is prone to the spontaneous tumour lysis syndrome. However, data on spontaneous tumour lysis syndrome remain scanty in our setting. We sought to determine the prevalence and factors associated with laboratory spontaneous tumour lysis syndrome in children in Uganda. We conducted a cross-sectional study among children <18 years old with histologically confirmed high-grade malignancy between October 2013 and April 2014. Laboratory spontaneous tumour lysis syndrome was defined as the presence of ≥2 of each of hyperkalaemia, hypocalcaemia, hyperuricaemia and hyperphosphatemia prior to administration of chemotherapy when alternative diagnoses had been excluded. A p < 0.05 was considered statistically significant. Of 108 children, of median age 7.7 years, where boys outnumbered girls 2:1, high-grade, malignancy included Burkitt’s lymphoma, acute lymphoblastic leukaemia, non-Hodgkin’s lymphoma, acute myeloid leukaemia and Burkitt’s leukaemia, with 14 suffering with laboratory spontaneous tumour lysis syndrome. Hypocalcaemia was its most common electrolyte imbalance; and four children died prior to commencement of chemotherapy. Bulky disease, lactate dehydrogenase levels ≥500 iu/l and serum creatinine levels >1.2 mg/dl were associated with laboratory spontaneous tumour lysis syndrome. However, only bulky disease was significantly predictive of laboratory spontaneous tumour lysis syndrome. Such children would benefit from routine screening.

A Delicate Surgical Resection to Treat a Proximal Pulmonary Adenoid Cystic Carcinoma

Pulmonary adenoid cystic carcinoma (PACC) is an exceedingly rare tumor of low-grade malignancy. Diagnosis is often late, so the tumoral process may be huge at presentation. Surgical resection could be challenging, and the recurrence rate would be greater. We report, hereby, a case of proximal PACC with involvement of the carina in a young male adult, without respiratory distress. Surgical resection was performed through a left pneumonectomy followed by a complex trachea-bronchoplastic procedure. During the operative time, the assisted ventilatory mode was carefully chosen. No adjuvant treatment was needed. Our patient is still under clinicoradiological surveillance and remains disease-free.

Dermoscopy of Dermatofibrosarcoma Protuberans

ermatofibrosarcoma protuberans (DFSP), also known as dermatofibrosarcoma of Darier and Ferrand, is a soft-tissue neoplasm of intermediate to low-grade malignancy, which accounts for 0.1–1% of all cutaneous malignant tumours [1]. The diagnosis of this entity is based on physical examination and a confirmative biopsy sample [2-5]. However, the lack of reliable clinical criteria suggestive of DFSP results in delayed diagnosis and poorer patient outcome.