Metastatic Granular Cell Tumor: A Rare Entity.

Granular cell tumor, which is thought to recapitulate a Schwann cell phenotype, is a very rare neoplasm that belongs to soft tissue tumors. It can be classified as benign, atypical or malignant, based on specific histological criteria, with the majority of cases exhibiting an indolent behavior. Its biology and clinical course are poorly understood and its optimal management is yet to be defined, given the rarity of cases. Here we describe an atypical granular cell tumor in the upper middle back skin that evolved after a thirty-year indolent period. Despite complete surgical removal, the patient experienced a recurrence, both local and in the lungs, following an aggressive clinical course. Data on management of metastatic disease are extremely scarce, comprised exclusively of case reports. Therefore, we administered to the patient systemic therapy according to soft tissue sarcoma guidelines, which led to disease progression, with fatal outcome. In conclusion, recurrent and/or metastatic granular cell tumor is a rare disease that can be life-threatening, for which response to different therapies is unknown. The biologic behavior of atypical and malignant granular cell tumor is quite different from its benign counterpart, evoking soft tissue sarcomas, and its diagnosis should alert clinicians. The role of adjuvant chemotherapy and radiation therapy in this setting should be explored, to limit disease recurrence.

Rapid and reliable testing for clinically actionable EGFR mutations in non-small cell lung cancer using the IdyllaTM platform: a real-world two-center experience in Greece.

BACKGROUND: Limited information exists on epidermal growth factor receptor (EGFR) molecular epidemiology in Greece. Next-generation sequencing (NGS) is the recommended method for EGFR genotyping in NSCLC. The Idylla Biocartis platform is a fully automated system for actionable EGFR mutation detection. RESEARCH DESIGN AND METHODS: We describe the prevalence of EGFR mutations in NSCLC patients in two high-volume clinical centers in Greece and compare key methods used for their determination. Eight hundred and fifty-seven FFPE samples from NSCLC patients were tested for EGFR mutations at University of Crete (UoC; n = 324) and at Evangelismos Hospital, Athens (Evangelismos; n = 503). RESULTS: The prevalence of EGFR mutations was 11.1% in the whole cohort (11.5% in non-squamous). The detection rate was 11.0% by NGS, 9.8% by Sanger and 11.3% by Idylla for the whole cohort (12.0% in non-squamous). The agreement between Idylla and Sanger was 93.2%. A targetable EGFR mutation was detected in 10.0% using tissue NGS alone, and in 16.0% using concurrent Idylla ctEGFR testing. CONCLUSION: The frequency of EGFR mutations was as expected for a Caucasian population. The Idylla EGFR test performance is comparable to reference methods and with a shorter TAT. Adding a concurrent plasma Idylla test to tissue NGS testing increases the detection rate of EGFR mutations in NSCLC.

Cutaneous Stevens Johnson - Toxic Epidermal Necrolysis Immunotherapy related Toxicities in Lung Cancer Patients.

INTRODUCTION: New Immuno- Checkpoint inhibitors (ICIs) functioning as PD-1- PDL-1 blockers are nowadays used in a majority of anticancer treatments. Many immune- related Adverse Events (irAEs) are published daily; severe skin toxicities, Stevens Johnson/ Toxic Epidermal Necrolysis (SJS/TEN) are seldom reported. CASE REPORT: Herein, we present two interesting skin sever toxicity cases of lung cancer patients, undergoing PD-1- PDL-1 Immunotherapy. In both cases, a morbilliform rash with documented histological Toxic Epidermal Necrolysis Pattern /Stevens Johnson findings, was thoroughly studied. MANAGEMENT & OUTCOME: Both cases were therapeutically managed according to guidelines with different outcome. DISCUSSION: Two focused cases of irAEs, is the rationale, to briefly review mechanisms of major toxicities caused by PD-1/PD-L1 blockade, and present all new data in their precise management. ICIs' association with SJS/ TEN still remains unclear; underlying urgent need for further studies. It is important to alert physicians to promptly identify life threatening irAEs. Being familiar, provides management efficacy, safe resolve and encourage beneficial balanced cost effective treatments.

Cutaneous Vasculopathy in a COVID-19 Critically Ill Patient: A Histologic, Immunohistochemical, and Electron Microscopy Study.

We describe a critically ill, SARS-CoV-2 positive patient with respiratory failure and thrombotic/livedoid skin lesions, appearing during the course of the disease. The biopsy of the lesions revealed an occlusive, pauci-inflammatory vasculopathy of the cutaneous small vessels characterized by complement and fibrinogen deposition on vascular walls, pointing to a thrombotic vasculopathy. Transmission electron microscopy of the affected skin failed to reveal any viral inclusions. Clinical evaluation and laboratory findings ruled out systemic coagulopathies and disseminated intravascular coagulation, drug-induced skin reaction, and common viral rashes. Our hypothesis is that the, herein evidenced, microvascular occlusive injury might constitute a significant pathologic mechanism in COVID-19, being a common denominator between cutaneous and pulmonary manifestations.

An unusual cause of large bowel obstruction: are we aware of this?

Primary lymphomas of the colon account for 0.5% of all primary colon malignancies. Burkitt´s lymphoma is a B-cell lymphoma with aggressive clinical behavior. Herein, we describe a case of a male patient who presented with signs of large bowel obstruction, underwent surgery and found to suffer from Burkitt´s lymphoma of the ileocecal region. The histopathological examination was indicative for Burkitt´s lymphoma. To the best of our insight this is one of the few reported cases of such type of lymphoma in an adult patient presenting with bowel obstruction. Burkitt´s lymphoma is a rare malignancy in adults affecting gastrointestinal tract. It has a high proliferation potential and can rapidly progress to advanced disease. Early diagnosis is necessary to prevent complications and improve overall prognosis.

Simultaneous pleomorphic adenoma of the left parotid gland and adenoid cystic carcinoma of the contralateral sublingual salivary gland: a case report.

INTRODUCTION: Sublingual salivary gland neoplasms are extremely rare, accounting for only 0.3-1% of all epithelial salivary gland tumors. Most of the sublingual tumors are malignant, adenoid cystic carcinoma (ACC) and mucoepidermoid carcinoma (MEC) being the most common histological types. The coexistence of two salivary gland tumors located in different major salivary glands is uncommon. CASE REPORT: A rare case of two simultaneous tumors of the major salivary glands, one in the sublingual and the other in the contralateral parotid gland in a female patient is reported. DISCUSSION: The diagnostic procedure followed, and the management of the patient, is outlined in the paper.

Surgical management of a large median dermoid cyst of the neck causing airway obstruction. A case report.

INTRODUCTION: Dermoid cysts of the floor of the mouth and submandibular space represent an unusual clinical entity. CASE REPORT: A case of an enlarged median dermoid cyst in a young female adult causing obstruction of the airway is reported. DISCUSSION: The need of an extraoral and intraoral incision for the removal of the cyst is discussed, along with a review of the literature.