Propranolol and infantile hemangiomas: Outcome from a tricentric study.

INTRODUCTION: The introduction of propranolol as systemic therapy for infantile hemangiomas (IH) has changed the natural history of these tumors. Cases presenting with ulceration, functional limitation, and visceral or life-threatening localization are excellent indications to medical therapy. The aim was to report the medium term follow-up after the introduction of propranolol in 3 referral centers with particular attention to outcome and surgical treatments. MATERIALS AND METHODS: In the period 2011-2018 348 patients underwent systemic therapy with propanolol at a mean age of 3.5 months (range 1-10). The indications to begin the treatment were: visceral localization (10,6%), ulceration (20%), risk of severe esthetic impairment (27%), and peri­orifices localization (42.4%). RESULTS: The minimum follow-up was 1 year. Propranolol was administered for a mean of 11 months (range: 6-19) and in 22 cases it was resumed for extra 4.5 months due to rebound. Overall, 97.4% responded to therapy without severe side effects. Seven patients underwent surgery and 9 laser therapy . Twenty-four cases are scheduled for surgery for evident inesthetisms and 4 will undergo laser therapy to complete the treatment. CONCLUSIONS: The use of propranolol has considerably decreased the surgical indications for IH, improved the esthetic and functional outcome, and simplified the definitive surgical treatment. The response rate to therapy is very high and in case of failure both laser and surgery are still very effective. The correct diagnosis, early starting, and adequate duration of therapy are key factors for the success of treatment.

In transit metastases in children, adolescents and young adults with localized rhabdomyosarcoma of the distal extremities: Analysis of the EpSSG RMS 2005 study.

In-transit metastases (ITM) are defined as metastatic lymph nodes or deposits occurring between the primary tumor and proximal draining lymph node basin. In extremity rhabdomyosarcoma (RMS), they have rarely been reported. This study evaluates the frequency, staging and survival of patients with ITM in distal extremity RMS. METHODS: Patients with extremity RMS distal to the elbow or knee, enrolled in the EpSSG RMS 2005 trial between 2005 and 2016 were eligible for this study. RESULTS: One hundred and nine distal extremity RMS patients, with a median age of 6.2 years (range 0-21 years) were included. Thirty seven of 109 (34%) had lymph node metastases at diagnosis, 19 of them (51%) had ITM, especially in lower extremity RMS. 18F-FDG-PET/CT detected involved lymph nodes in 47% of patients. In patients not undergoing 18F-FDG-PET/CT lymph node involvement was detected in 22%. The 5-yr EFS of patients with ITM vs proximal lymph nodes vs combined proximal and ITM was 88.9% vs 21.4% vs 20%, respectively (p = 0.01) and 5-yr OS was 100% vs 25.2% vs 15%, respectively (p = 0.003). CONCLUSION: Our study showed that in-transit metastases constituted more than 50% of all lymph node metastases in distal extremity RMS. 18F-FDG-PET/CT improved nodal staging by detecting more regional and in-transit metastases. Popliteal and epitrochlear nodes should be considered as true (distal) regional nodes, instead of in-transit metastases. Biopsy of these nodes is recommended especially in distal extremity RMS of the lower limb. Patients with proximal (axillary or inguinal) lymph node involvement have a worse prognosis.

Fine-needle aspiration cytology in children with superficial lymphadenopathy.

INTRODUCTION: In pediatric population Fine-Needle Aspiration Citology (FNAC) is slowly gaining acceptance in clinical management of Superficial Lymphadenopathy (SL). Our experience adds some data about the usefulness of this technique in diagnosing the cause of a SL and therefore guiding further treatment. PATIENTS AND METHODS: 238 FNAC were performed in 217 patients with SL, observed at our Institution from 2002 to 2006. The neck was the most frequent localization. The results were available within few hours. In cases of granulomatous findings, the samples were processed for microbiological and PCR test, in order to identify Mycobacteria. RESULTS: 174 were reactive lesions, 38 granulomatous lymphadenopathies, 24 malignant lesions, 2 specimens inadequate for diagnosis. Among the 174 reactive SL, 22 required an incisional biopsy after 1 month follow-up. Among the granulomatous lymphadenopathies, 13 children with Cat-Scratch Disease recovered, 25 with Mycobacteria infection underwent surgical excision. For 24 malignant lesions, the diagnosis was confirmed by further biopsy. Two false negative and no false positive were detected (sensitivity 92%, specificity 100%). No complications were encountered. CONCLUSIONS: In our experience FNAC, performed by experienced cytopathologist, has revealed to be a fast, safe, non invasive and inexpensive method to achieve diagnosis in persistent SL. The use of FNAC gave us the possibility to select patients for further investigation and/or surgical treatment. Incisional biopsy remains necessary to confirm the diagnosis in case of malignancy or doubtful lesions.

Hematometra in uterus didelphis: a pitfall in teenagers with lower abdominal mass. Report of two cases.

Müllerian malformations represent a wide spectrum of anomaly of the female reproductive tract, usually detected at birth or at puberty. The presence of uterus didelphys and obstructed hemivagina, associated with ipsilateral renal agenesis, is known as Herlyn-Werner-Wunderlich syndrome, a rare congenital anomaly. Two cases of uterus didelphic and hematometra caused by vaginal septum are reported; the girls had a delayed diagnosis due to absence of a specific clinical feature. Clinicians must be aware of the existence of these malformations. An adequate radiological study is mandatory to assess the anatomic conformation, and to optimize the therapeutic procedures.