RESUMO
We presented a case of diffuse-type tenosynovial giant cell tumour (DTSGCT) of foot masquerading as Langerhans cell histiocytosis. Preliminary diagnosis by needle biopsy was difficult due to the major involvement of bones and the overshadowing effect of the accompanying Langerhans cells. The complete curettage specimen with relevant immunohistochemistry and molecular tests made the final diagnosis of DTSGCT possible. The biomolecular mechanism for the masquerading phenomenon was explained by CSF1 overexpression in the neoplastic cells attracting migration and proliferation of CSF1R-positive Langerhans cells.
RESUMO
Dermatofibrosarcoma Protuberans (DFSP) is a rare malignant soft-tissue neoplasm which is often misdiagnosed due to its indolent clinical course and non-specific radiological appearances. An observation case series was conducted with retrospective review of clinical and radiological data of DFSP patients presenting to a major tertiary hospital in Hong Kong for radiological assessment between November 2006 and February 2016. Seven patients with confirmed histological diagnosis of DFSP were included. Tumour sizes at presentation ranged from 1 to 5 cm, most commonly (n = 6) occurred over chest wall and abdominal wall. History of previous local trauma or surgery was identified in the majority of cases (n = 4). There was poor correlation between pre-imaging clinical diagnoses and pathological diagnoses. Local recurrence and tumour de-differentiation with sarcomatous changes occurred in the minority of cases (n = 2). A common radiological 'claw' sign at the lesion/skin interface formed by elongated appendages of the tumour superficially was appreciated in most cases (n = 6). A history of previous local trauma or surgery serves as a possible etiological factor for the development of DFSP. High clinical suspicion for the entity is essential in its detection and differentiation from simple wound complications and local recurrence of other benign lesions. The radiological 'claw' sign at the lesion/skin interface might serve as a tell-tale sign for cutaneous tumour involvement. A comprehensive analysis of imaging findings in conjunction with individual clinical presentations is the key to accurate diagnoses and proper management.
Assuntos
Dermatofibrossarcoma/diagnóstico por imagem , Dermatofibrossarcoma/patologia , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/patologia , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Pele/diagnóstico por imagem , Pele/patologiaRESUMO
OBJECTIVE: To compare the safety, effectiveness, and outcomes of primary stenting and salvage stenting for malignant superior vena cava obstruction. DESIGN: Case series with internal comparison. SETTING: Regional hospital, Hong Kong. PATIENTS: A total of 56 patients with malignant superior vena cava obstruction underwent 59 stentings from 1 May 1999 to 31 January 2014. Patients' characteristics, procedural details, and outcomes were retrospectively reviewed. Of the 56 patients, 33 had primary stenting before conventional therapy and 23 had salvage stenting after failure of conventional therapy. Statistical analyses were made by Fisher's exact test and Mann-Whitney U test. RESULTS: Primary lung carcinoma was the most common cause of malignant superior vena cava obstruction (primary stenting, 22 patients; salvage stenting, 16 patients; P=0.768), followed by metastatic lymphadenopathy. Most patients had superior vena cava obstruction only (primary stenting, 16 patients; salvage stenting, 15 patients; P=0.633), followed by additional right brachiocephalic vein involvement. Wallstents (Boston Scientific, Natick [MA], US) were used in all patients. Technical success was achieved in all but two patients, one in each group (P=1.000). Only one stent placement was required in most patients (primary stenting, 28 patients; salvage stenting, 20 patients; P=0.726). Procedure time was comparable in both groups (mean time: primary stenting, 89 minutes; salvage stenting, 84 minutes; P=0.526). Symptomatic relief was achieved in most patients (primary stenting, 32 patients; salvage stenting, 23 patients; P=0.639). In-stent restenosis and bleeding were the commonest complications (primary stenting, 6 and 1 patients, respectively; salvage stenting, 2 and 2 patients, respectively). Nine patients required further treatment for symptom recurrence (primary stenting, 6 patients; salvage stenting, 3 patients; P=0.725). CONCLUSION: Endovascular stenting is safe and effective for relieving malignant superior vena cava obstruction. No statistically significant differences in number of stents, success rates, procedure times, symptom relief rates, complication rates, and re-procedure rates were found between primary stenting and salvage stenting.
Assuntos
Carcinoma/complicações , Neoplasias/complicações , Tumores Neuroendócrinos/complicações , Terapia de Salvação , Stents , Síndrome da Veia Cava Superior/terapia , Idoso , Idoso de 80 Anos ou mais , Procedimentos Endovasculares/efeitos adversos , Feminino , Humanos , Linfoma/complicações , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Stents/efeitos adversos , Síndrome da Veia Cava Superior/etiologia , Resultado do TratamentoRESUMO
Isolated spinal artery aneurysm is a rare lesion which could be accountable for spontaneous spinal subarachnoid haemorrhage. We describe the case of a 74-year-old man presenting with sudden onset of chest pain radiating to the neck and back, with subsequent headache and confusion. Initial computed tomography aortogram revealed incidental finding of subtle acute spinal subarachnoid haemorrhage. A set of computed tomography scans of the brain showed further acute intracranial subarachnoid haemorrhage with posterior predominance, small amount of intraventricular haemorrhage, and absence of intracranial vascular lesions. Subsequent magnetic resonance imaging demonstrated a thrombosed intradural spinal aneurysm with surrounding sentinel clot, which was trapped and excised during surgical exploration. High level of clinical alertness is required in order not to miss this rare but detrimental entity. Its relevant aetiopathological features and implications for clinical management are discussed.
Assuntos
Aneurisma/complicações , Hemorragia Subaracnóidea/etiologia , Artéria Vertebral/diagnóstico por imagem , Idoso , Aneurisma/diagnóstico , Aneurisma/cirurgia , Dor no Peito/diagnóstico , Dor no Peito/etiologia , Serviço Hospitalar de Emergência , Seguimentos , Humanos , Laminectomia/métodos , Angiografia por Ressonância Magnética/métodos , Masculino , Doenças Raras , Índice de Gravidade de Doença , Hemorragia Subaracnóidea/diagnóstico , Hemorragia Subaracnóidea/cirurgia , Tomografia Computadorizada por Raios X/métodos , Resultado do Tratamento , Procedimentos Cirúrgicos Vasculares/métodos , Artéria Vertebral/fisiopatologiaRESUMO
Congenital pseudarthrosis of the clavicle is a rare clinical entity, first described in 1910. We report on a newborn baby girl who presented with a painless lump over mid-portion of right clavicle at her routine newborn examination, which was subsequently diagnosed as a congenital pseudarthrosis. Here we explore its pathogenesis, elaborate on its differential diagnoses in paediatric patients, and comment on its distinct radiological features.
Assuntos
Clavícula/patologia , Pseudoartrose/congênito , Clavícula/diagnóstico por imagem , Diagnóstico Diferencial , Feminino , Humanos , Recém-Nascido , Pseudoartrose/diagnóstico , Pseudoartrose/diagnóstico por imagem , Pseudoartrose/patologia , RadiografiaRESUMO
Abstract Papillary thyroid carcinoma is rare in children. Its clinical manifestation may deviate from that observed in adult patients. We present a 14-year-old girl with papillary thyroid carcinoma. We then discuss various distinctive sonographic signs that may aid diagnosis, and we discuss differences in clinical features between children and adults [added].