Low incidence of pulmonary metastases in vulvar cancer patients: limited value of routine chest imaging based on a cohort study

OBJECTIVE: To evaluate the incidence of pulmonary metastases in the preoperative work-up of patients with primary vulvar squamous cell carcinoma (SCC). DESIGN: Retrospective cohort study. SETTING: Tertiary referral centre. POPULATION: Patients treated for primary vulvar SCC from 2000 to 2018. METHODS: The pre-operative chest imaging of 452 consecutively treated patients was documented with a minimal follow-up period of 2 years. MEAN OUTCOME MEASURES: Incidence of pulmonary metastases, frequency of chest imaging and subsequent coincidental findings. RESULTS: In total, 80.8% of patients underwent pre-operative chest imaging. Seven patients (1.9%), with a median tumour size of 80 mm, presented with pulmonary metastases. None of the patients with early stage disease and tumour size <40 mm who underwent radical local excision (RLE) with sentinel node (SN)-procedure, was diagnosed with pulmonary metastasis. Chest imaging was performed by radiography (58.9%) and computerised tomography (CT) (41.1%). Coincidental findings were reported in 40.7% of patients who underwent CT, compared with 15.8% of patients undergoing radiography, resulting in additional diagnostics in 14.7 and 19.7% and being of limited consequence for outcome in 2.9 and 3.3%, respectively. CONCLUSIONS: The incidence of pulmonary metastases in patients with primary vulvar SCC is extremely low, and none in patients with early stage disease undergoing the SN procedure. Chest imaging was performed in the majority of patients and was associated with frequent coincidental findings leading to clinically irrelevant diagnostic procedures. Therefore, we recommend omitting chest imaging in patients with early stage disease and tumours <40 mm, considering chest CT only in patients with large tumours and/or advanced stage disease. TWEETABLE ABSTRACT: The incidence of pulmonary metastases is 1.9%, none in early stage disease planned for SN. Omitting chest imaging in this group is advised

Recurrent Fusions Between YAP1 and KMT2A in Morphologically Distinct Neoplasms Within the Spectrum of Low-grade Fibromyxoid Sarcoma and Sclerosing Epithelioid Fibrosarcoma

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Waardendiscussie nodig in stikstofcrisis

Item does not contain fulltextRadboud Recharge, 22 november 201

Stikstofreductie en duurzaam natuurherstel kunnen samengaan

Item does not contain fulltext11 september 201

Ultrasound-guided diagnosis of fractures of the distal forearm in children

AbstractPurposeDistal radius and forearm fractures are injuries that are frequently seen in trauma surgery outpatient clinics. Usually, the wrist is X-rayed in 2 planes as standard diagnostic procedure. In contrast, we evaluate in our study the accuracy of ultrasonography (US) in diagnosing these fractures.MethodsThis prospective study includes the patients who presented at two trauma surgery clinics with a presumptive diagnosis of distal radius or forearm fracture between January and December 2012. After a clinical examination, US imaging of the distal forearm was first carried out on 6 standardized planes followed by radiographs of the wrist made in two planes. The age limit was set at the end of 11years.ResultsIn total, 201 patients between 4 and 11years of age were recruited with an average age of 9.5years at the time of the trauma. There were 104 (51.7%) fractures distributed as follows: 89 (85.9%) injuries of the distal radius, 9 (8.7%) injuries of the distal ulna, and 6 (5.8%) combined injuries (radius and ulna). Sixty-five greenstick fractures were detected. Surgery was necessary in 34 cases. Specificity and sensitivity of ultrasound diagnosis were 99.5%.ConclusionUltrasound imaging is suitable to demonstrate fractures of the distal forearm. It is a highly sensitive procedure in detecting distal forearm fractures. In our opinion, a negative result in ultrasound may reduce the need for further radiographs in children with distal forearm lesions. But in any doubtful situation the need for conventional radiographs remains.Level of evidenceLevel III. Prospective study

Clinicopathologic and molecular features of intracranial desmoplastic small round cell tumors

Contains fulltext : 219243.pdf (Publisher’s version ) (Closed access)Desmoplastic small round cell tumors (DSRCTs) are highly aggressive sarcomas that most commonly occur intra-abdominally, and are defined by EWSR1-WT1 gene fusion. Intracranial DSRCTs are exceptionally rare with only seven previously reported fusion-positive cases. Herein, we evaluate the clinical, morphologic, immunohistochemical and molecular features of five additional examples. All patients were male (age range 6-25 years; median 11 years), with four tumors located supratentorially and one within the posterior fossa. The histologic features were highly variable including small cell, embryonal, clear cell, rhabdoid, anaplastic and glioma-like appearances. A prominent desmoplastic stroma was seen in only two cases. The mitotic index ranged from <1 to 12/10 HPF (median 5). While all tumors showed strong desmin positivity, epithelial markers such as EMA, CAM 5.2 and other keratins were strongly positive in only one, focally positive in two and negative in two cases. EWSR1-WT1 gene fusion was present in all cases, with accompanying mutations in the TERT promoter or STAG2 gene in individual cases. Given the significant histologic diversity, in the absence of genetic evaluation these cases could easily be misinterpreted as other entities. Desmin immunostaining is a useful initial screening method for consideration of a DSRCT diagnosis, prompting confirmatory molecular testing. Demonstrating the presence of an EWSR1-WT1 fusion provides a definitive diagnosis of DSRCT. Genome-wide methylation profiles of intracranial DSRCTs matched those of extracranial DSRCTs. Thus, despite the occasionally unusual histologic features and immunoprofile, intracranial DSRCTs likely represent a similar, if not the same, entity as their soft tissue counterpart based on the shared fusion and methylation profiles