Search

Sclerotic fibroma (storiform collagenoma) is a rare benign skin tumor. A solitary tumor, as well as multifocal lesions, are found either sporadically, or associated with Cowden syndrome. The tumor usually presents as clinically asymptomatic, slowly growing papule or nodule on the skin of the head, neck, and upper extremities. Microscopically the lesion is sharply demarcated, composed of hyalinized bands of collagen with low cellularity and a distinctive irregularly whorled or storiform pattern. We describe a case of a unique variant of this tumor in the scalp of a 33-year-old male. The tumor was microscopically composed of concentrically arranged collagen bundles with prevailing type III collagen, which resembled an enlarged Vater-Pacini corpuscle, with low density of CD34-positive and glucose transporter 1-negative spindle shaped cells. The specific microscopic appearance is suggestive of the term "Pacinian collagenoma" for this unique benign tumor.

A thorough understanding of the anatomy, physiology, and development of the spleen is essential for determining the pathophysiological mechanisms underpinning splenic diseases and congenital variations. The aim of this review is to briefly summarize current knowledge regarding the normal development of the spleen, and to provide an overview of clinically relevant congenital splenic variations. These include such variations as asplenia, polysplenia, hyposplenia, lobulation of spleen, accessory spleens, accessory splenic nodules, wandering spleen, splenogonadal and splenopancreatic fusion, splenic cysts, and cavernous haemangioma of the spleen. All of these congenital variations are also mentioned in internationally accepted embryological nomenclature, known as the Terminologia Embryologica. Interestingly, most patients who have these diseases are asymptomatic, and are often diagnosed only after an injury or during unrelated medical procedures. Using examples from published case reports, we highlight how an understanding of the embryology of the spleen and the etiology of its disease states would improve clinical practice.

Ectopic accessory thymic tissue usually presents as an asymptomatic neck mass found at any level corresponding to the embryonic descent of the thymus. This tissue may contain smaller or larger cysts. However, the exact pathogenesis of “enigmatic” cervical thymic cysts remains controversial. A 7-year-old boy was referred to our workplace for the evaluation of a cervical mass. An ultrasound suggested a multi-loculated cystic mass, while CT and MRI indicated a left-sided, anteriorly located cervical mass beneath the sternocleidomastoid muscle. Following the radiological findings, surgical excision revealed a cystic mass. The mass of tissue was covered by a capsule. In H&E staining, the cervical mass had the same structure as normal thymus. Additionally, immunohistochemical findings suggest that the cellular microenvironment of cervical thymus also displays a place for development of T-lymphocytes. Within the parenchyma multiple cysts lined with cytokeratin-positive thymic epithelial cells were found. Inside the cysts, there were CD68-positive multinucleated giant cells and cholesterol clefts. A tendency to cystic degeneration inside the thymic tissue occurs more often in cervical thymuses than in normally located ones. The reason for the formation of cysts is unknown. We summarized seven possible histological, embryological and evolutional backgrounds for the development of these thymic cysts.

Introduction Thyroid cancer in children is a hot topic because of the large clinical heterogeneity and the risk of severe complications. We aimed to study 1. The frequency, 2. Etiology, and 3. Risk factors of post-surgery complications of thyroid cancer. Material and methods A retrospective analysis including risk factors for post-surgery complications of patients treated for thyroid malignancies in years 2006–2018 was performed. Results Over a period of 12 years 22 patients with thyroid malignancy (68% female; 12.6 ± 4.0 years of age, median follow-up 6 years) were identified. Histologically, 12 (55%) patients had papillary carcinoma. Six patients (27.3%) had multiple endocrine neoplasia type 2 (MEN2) syndrome, 3 (13.7%) patients had medullary carcinoma and 1 patient had follicular carcinoma. Neck lymph node metastases were diagnosed in 8 (36.4%), distant metastases in 6 (27.3%), and both locations were involved in 4 (18.2%) patients. Six (27.3%) children had surgical complications: 1 child had unilateral vocal cord paralysis and transient hypoparathyroidism and 5 had transient hypoparathyroidism. The higher risk of surgery complications in forward stepwise logistic regression was associated in with distant metastases (R2 = 0.584, OR 52.63, p = 0.010). Conclusions Postoperative complications were significantly associated with presence of distant metastases. Favorable results were observed in with children with MEN2 syndrome.