Cranial Fasciitis in Children: Expanding the Spectrum of USP6-Associated Clonal Transient Neoplasms

Background: Cranial fasciitis (CF) is a benign (myo)fibroblastic proliferation of children. Typical presentation consists of a rapidly growing solitary mass on the temporal or parietal cranium in the first 2 years of age. CF is characterized by a rapid growth followed by a relative slowdown and even growth arrest. The finding of somatic USP6 gene rearrangements demonstrating clonality in CF together with its clinical behavior places it in the category of diseases recently termed “transient neoplasia.” Methods: Histological, immunohistochemical, and molecular findings of 18 patients with CF were retrospectively studied. Results: The tumor typically presented as a painless rapidly enlarging mass in the temporal region. Sixty-six percent of the cases harbored USP6 gene rearrangement. Nine patients were treated with gross total resection (GTR) and 9 with subtotal tumor resection (STR). Two patients treated with GTR had recurrence. Five patients treated with STR had progression-free disease for at least 10 months after surgery and in four patients the tumor regressed spontaneously a median 16 months after surgery. Conclusions: In this largest series to date, we reported the clinicopathological, immunohistochemical, and molecular findings of 18 pediatric cases of CF with emphasis on the clinical growth pattern of these tumors.

Keloid-like dermatofibroma

Dermatofibroma is a common benign skin tumor, mainly occurring in young to middle-aged females. It is frequently localized in the lower extremities. A typical dermatofibroma usually presents itself as a single firm papule or nodule, of variable color, bluish, brownish, or pinkish. Its clinical, dermoscopic, and histological features usually allow easy diagnosis [1]. However, it is possible to observe some variations of these typical features. Keloid-like dermatofibroma is one of these atypical presentations rarely reported in the literature [2]. A 40-year-old patient with no previous medical history presented to our dermatology department with a lumbosacral lesion evolving for several months. A physical examination revealed a firm, well-demarcated, asymptomatic erythematous nodule, 5 × 12 mm in size, localized in the lumbosacral area (Fig. 1). The patient denied any trauma preceding the onset of the lesion. There was no personal or familial history of keloidal scars. A dermoscopic examination revealed erythema, telangiectatic vessels, a shiny white streak, and a brownish-yellow pigmentation (Fig. 2). A biopsy was performed. A histological examination revealed an atrophic epidermis. The dermis contained a fibroblastic proliferation of low cell density haphazardly arranged, located on a fibromatous background (Figs. 3 and 4). Dermatofibroma with a keloidal presentation was the diagnosis.

Clinical Effects of Microwave Ablation in the Treatment of Low-Risk Papillary Thyroid Microcarcinomas and Related Histopathological Changes

ObjectiveThis study aimed to evaluate the feasibility and efficacy of ultrasound-guided percutaneous microwave ablation (MWA) in the treatment of low-risk papillary thyroid microcarcinoma (PTMC), and to observe the histopathological changes after MWA.MethodsMWA was performed under ultrasound guidance for 73 unifocal PTMC patients without clinically cervical or distant metastasis. The target ablation zone exceeded the tumor edge (judged by contrast-enhanced US) to avoid marginal residue and recurrence. Ultrasound evaluation was performed at 1 day, 1, 3, 6, 12 and 24 months after treatment, and thyroid function evaluation at the first 6 months. Repeated fine needle aspiration cytology or core needle biopsy pathology was performed at 3 or 6 months after MWA to evaluate residual tumors. Any adverse event associated with MWA was evaluated.ResultsThe follow-up after MWA lasted 6 (6, 12) months. Tumor volume decreased significantly from 0.06 mm3 (0.04, 0.11 mm3) to 0.03 mm3 (0.00, 0.06 mm3) at 12 months after MWA (P< 0.001), with a median volume reduction ratio of 80.28% (-7.43, 100%) and 16 cases (21.92%) presenting complete remission. The largest diameter, volume and ablation energy were found to be different in patients with and without complete remission 12 months after MWA. On histopathological examinations, no atypical or malignant follicular cells were identified after thermal ablation. The most common pathological characteristics were fibroblastic proliferation (34/39, 87.18%) and chronic inflammation (32/39, 82.05%), followed by infarction (21/39, 53.85%). Five patients were transferred to thyroidectomy and 4 of them were confirmed with local recurrence and/or lymph node metastasis. Serum thyrotropin decreased transiently after MWA (P< 0.01) but normalized thereafter. No serious and permanent complications were reported.ConclusionsMWA is a safe and effective treatment for low-risk PTMC. Fibroblastic proliferation and chronic inflammation are the most common pathological changes after MWA of PTMC.

Nodular fasciitis on the arm

A 20-year old female with no trauma history presented to our department of dermatology with a 6-month history of a painful tumor of the arm. Physical examination revealed a 5-cm hard-elastic and friable tumor, which were movable and unattached to the underlying tissues. Surgical excision of the tumor was performed. Histopathologic examination concluded to nodular fasciits. Nodular fasciitis is a benign pseudosarcomatous tumour composed of a vascular and fibroblastic proliferation. Surgical excision is the gold standard treatment and recurrence is rare.

A Novel Therapeutic Approach for Cranial Fasciitis Utilizing Frameless Stereotaxy

Cranial fasciitis is a rare cause of a painless, progressive skull mass in the pediatric population, having been described in only 80 cases in the literature to-date. While the etiology remains unknown, the pathogenesis is thought to be a reactive process causing a non-neoplastic fibroblastic proliferation arising from the deep fascia or periosteum of the cranium. Accurate diagnosis may be delayed due to the lack of physical signs and symptoms on presentation, as well as equivocal imaging for the other lesions included in the differential diagnosis. Importantly, in the case of cranial fasciitis, involved tissue is hard to decipher from normal tissue in the cranium, and margin recommendations have not yet been made clear in the current literature. Herein, we present a case of confirmed cranial fasciitis in a 2-year-old female patient in whom frameless stereotaxy was used intraoperatively to determine the extent of the lesion and ensure adequate resection and minimize recurrence risk.

Spontaneous Remission of Retroperitoneal Fibrosis in a Patient with Inherited Thrombophilia

Retroperitoneal fibrosis (RPF) is a rare condition characterized by a diffuse or localized fibroblastic proliferation associated with chronic inflammation. RPF is generally idiopathic, but can also be secondary to the use of certain drugs, malignant diseases, infections, and surgery. Treatment of RPF aims to relieve ureteral obstruction and to induce disease regression, and includes the use of steroids combined or not with other immunosuppressive agents. We present the case of a 35-years old female with a medical history of transient ischemic stroke, myocardial infarction, miscarriage and inherited thrombophilia, who was diagnosed in our Department with idiopathic RPF. Due to the mentioned associated comorbidities, no immuno-active treatment could be initiated. After one year, MRI exam showed significant spontaneous decrease of RPF mass. Although an uncommon event, the spontaneous resolution of idiopathic RPF could encourage in some selected cases a conservative management. By our knowledge, no previously cases of spontaneously remitted RPF in patients with inherited thrombophilia have been reported.

Effect of local use of l-carnitine after myringotomy on myringosclerosis development in rats

Objectives:This study aimed to investigate the effect of local and intraperitoneal administration of l-carnitine on the prevention of experimentally induced myringosclerosis, and to compare treatment efficiency.Methods:Twenty-four Albino-Wistar rats (48 ears) were bilaterally myringotomised and divided randomly into four groups: group one received no treatment, group two received intraperitoneal l-carnitine, group three received local l-carnitine, and group four received both intraperitoneal and local l-carnitine. On the 15th day after treatment, tympanic membranes were harvested and evaluated histopathologically for myringosclerotic plaque formation, fibroblastic proliferation, tympanic membrane thickness and new vessel formation.Results:The group one tympanic membranes showed extensive thickness, and the incidence of myringosclerosis and fibroblast proliferation were greater than in groups two and four. There were statistically significant differences in tympanic membrane thickness between groups three and four, and in myringosclerosis incidence and fibroblast proliferation, comparing groups two, three and four.Conclusion:Myringosclerosis development was significantly reduced in rats receiving myringotomy plus intraperitoneal l-carnitine. Intraperitoneal l-carnitine administration prevented fibroblastic proliferation and tympanic membrane thickening (both of which cause further tympanic membrane destruction), thus reducing myringotomy-associated morbidity. Local l-carnitine administration had limited effectiveness in this experimental setting.