A rare case of asymptomatic solitary cutaneous mastocytoma in a child

Mastocytosis is a rare group of disorders occurring when mast cells diffusely infiltrate the skin or internal organs. It has two forms: Cutaneous and systemic mastocytosis. We present a case of a 12-year-old male child presenting with a reddish-brown cutaneous nodule on the anterior chest wall for 1 month. No other similar lesions were noted elsewhere. Routine hematological and radiological investigations did not reveal any abnormality. The lesion was excised, and a diagnosis of solitary cutaneous mastocytoma was made based on the clinical, histopathological, and immunohistochemical features. This case highlights the importance of diagnosing this rare tumor which can present as an asymptomatic cutaneous lesion in a young child.

Redefining WILD syndrome: a primary lymphatic dysplasia with congenital multisegmental lymphoedema, cutaneous lymphovascular malformation, CD4 lymphopaenia and warts

BackgroundPrimary lymphoedema (PL) syndromes are increasingly recognised as presentations of complex genetic disease, with at least 20 identified causative genes. Recognition of clinical patterns is key to diagnosis, research and therapeutics. The defining criteria for one such clinical syndrome, ‘WILD syndrome’ (Warts, Immunodeficiency, Lymphoedema and anogenital Dysplasia), have previously depended on a single case report.Methods and resultsWe present 21 patients (including the first described case) with similar clinical and immunological phenotypes. All had PL affecting multiple segments, with systemic involvement (intestinal lymphangiectasia/pleural or pericardial effusions) in 70% (n=14/20). Most (n=20, 95%) had a distinctive cutaneous lymphovascular malformation on the upper anterior chest wall. Some (n=10, 48%) also had hyperpigmented lesions resembling epidermal naevi (but probably lymphatic in origin). Warts were common (n=17, 81%) and often refractory. In contrast to the previous case report, anogenital dysplasia was uncommon—only found in two further cases (total n=3, 14%). Low CD4 counts and CD4:CD8 ratios typified the syndrome (17 of 19, 89%), but monocyte counts were universally normal, unlike GATA2 deficiency.ConclusionWILD syndrome is a previously unrecognised, underdiagnosed generalised PL syndrome. Based on this case series, we redefine WILD as ‘Warts, Immunodeficiency, andLymphatic Dysplasia’ and suggest specific diagnostic criteria. The essential criterion is congenital multisegmental PL in a ‘mosaic’ distribution. The major diagnostic features are recurrent warts, cutaneous lymphovascular malformations, systemic involvement (lymphatic dysplasia), genital swelling and CD4 lymphopaenia with normal monocyte counts. The absence of family history suggests a sporadic condition, and the random distribution of swelling implicates mosaic postzygotic mutation as the cause.

Solitary Plasmacytoma of The Sternal Stalk: A Case Report and Literature Review

Background: It has been reported that sternal neoplasms are a rare disease, accounting for about 1% of primary bone neoplasms, of which about 60% are malignancy, mostly occurring in the manubrium sternum. Case presentation: We reviewed a 77-year-old man with a solitary plasmacytoma of the sternal stalk presenting with pain in the anterior chest wall by examining a preoperative diagnosis of sternal manubrium malignant bone tumor. We performed extensive sternal manubrium tumor resection + bilateral partial costal cartilage resection + bilateral clavicular head resection + cervical lymph node dissection + thoracic reconstruction, and the postoperative recovery was perfect. Conclusion: We report a rare case of solitary plasmacytoma of the manubrium sternum successfully treated by extensive radical surgery for the manubrium tumor. Although rare, the disease should be identified, examined, and treated early to avoid serious complications.

Chest Imaging in the Diagnosis and Management of Pulmonary Tuberculosis: The Complementary Role of Thoraci Ultrasound

Tuberculosis (TB) is a severe infectious disease that still represents a major cause of mortality and morbidity worldwide. For these reasons, clinicians and radiologists should use all the available diagnostic tools in the assessment of the disease in order to provide precise indications about starting an anti-tubercular treatment and reduce risk of TB transmission and complications especially in developing countries where the disease is still endemic. As TB mycobacteria are mainly transmitted through respiratory droplets, the pulmonary parenchyma is usually the first site of infection. As a result, chest imaging plays a central role in the diagnostic process. Thoracic ultrasound (TUS) is a portable, non-invasive, radiation-free, and cost-contained technology which could be easily available in resource-limited settings. This perspective article focuses on the potential role of TUS in the diagnosis and management of patients with pulmonary TB. Unfortunately, there are still insufficient evidence and too contrasting data to judge TUS as an appropriate diagnostic method for the screening of the disease. Despite this, TUS may have a useful role in identifying pleural and anterior pericardial effusions or in the identification of abscesses of the anterior chest wall and paraspinal collections in low- and middle-income settings. In addition, TUS seems to have a milestone role in guiding minimally invasive interventional procedures, such as placement of chest tubes, drainage of loculated collections, thoracentesis and pericardiocentesis, and percutaneous biopsy of subpleural pulmonary consolidations or pleural plaques.

Minimally invasive repair of pectus carinatum using the Abramson technique

In the past, the treatment of pectus carinatum has been managed by open, invasive surgical procedures, which involved the resection of cartilage growth plates (Ravitch procedure). By preventing normal bony growth and maturity, this technique often led to postoperative complications, such as acquired thoracic dystrophy, chronic pain and scarring, and stiffness of the whole anterior chest. Dyspnea and exercise intolerance due to restricted thoracic space and cardiac compression were not uncommon as well. Over the last 2 decades, nonsurgical and minimally invasive approaches have gained ground because it was recognized that simple sternal compression was able to remodel the elastic anterior chest wall and therefore correct pectus carinatum adequately/efficiently, at least in children. However, failure of this compressive brace treatment is not uncommon in adolescents and older patients. Abramson therefore developed a minimally invasive technique for the correction of pectus carinatum using a pectus bar that is placed anteriorly to the sternum. The procedure is less invasive and less risky than a pectus bar inserted for pectus excavatum, but the lateral fixation of the pectus bar in the Abramson procedure remains a challenge. We demonstrate the technical aspects of the procedure step by step including our solution for fixation of the stabilizers.

Endoscopic Cervical Lymph Node Dissection Using the Extra-cervical Anterior Chest Wall Approach: A New Technique

Introduction This study aims to demonstrate the safety, surgical feasibility, and esthetic features of total endoscopic neck dissection (END) through anterior chest wall approach (ACWA) without creation of any neck incisions. Resection of their primary tumors followed by selective total END through ACWA using 3 ports (one 10-mm port for the camera and two 5-mm ports for the working instruments). Methods From January 2020 to August 2020, 6 patients with a biopsy proven head and neck carcinoma underwent resection of their primary tumors followed by selective total END through ACWA using 3 ports (one 10-mm port for the camera and two 5-mm ports for the working instruments). Results The selective neck dissection was successfully performed endoscopically in all cases with no conversion to open approach and with good visualization of the major neurovascular structures. The operative time for the END ranged from 120 to 170 minutes, with 10–50 mL estimated blood loss. No significant perioperative complications were encountered. The mean total number of cervical LN retrieved was 13.67 + 2.42, and the mean LNR was .01 + .13. All patients were discharged in the third postoperative day, and they were satisfied with the cosmetic outcome. Conclusion Selective total END through ACWA is technically feasible and safe with satisfactory cosmetic results. The absence of neck scars and magnification of the important neurovascular structures are the most obvious advantages of this innovative technique. It may be a valid alternative to conventional surgery when performed in selected patients. However, further research with longer follow up is needed to clarify the oncological safety and the real benefits of END in head and neck cancer patients.

Unusual presentation of lung carcinoma as nasal tip metastasis: A review of literature

Lung carcinoma is the most common type of carcinoma seen in males and 4 most common in females. Skin metastasis from lung carcinoma is frequent with an incidence of 1%-12%, with most common site being the anterior chest wall. Skin metastasis from lung carcinoma to the tip of nose is very rare with only 12 cases being reported in literature(Table 1). It can be confused with other benign and malignant conditions, such as infection, lymphoma, hemangioma, rhinophyma, sarcoidosis, tuberculosis and carcinomas, making its diagnosis difficult. Less than 20 cases of cutaneous nasal tip metastasis are reported in literature out of which 12 are from malignancies of lung. Sometimes it can appear earlier than the primary lesion and thus delays the diagnosis of primary lesion. = In this case report, we report a case of nasal tip cutaneous metastasis from squamous cell carcinoma of lung.

Ectopia cordis in an adult patient with COVID-19: A case report and literature review

Ectopia cordis (EC) is a rare congenital condition characterized by a partial or complete defect of the anterior chest wall. It is associated with ventricular and atrial septal defects (ASD),. This study aimed to explore the cardiac manifestations of EC complicated by coronavirus disease 2019 (COVID-19).

Undifferentiated pleomorphic sarcoma of the chest wall: a rare diagnosis

A 31-year-old man with no predisposing factors and no history of chronic disease presented with a small painless lump over his anterior chest wall. On evaluation, it was found to be undifferentiated pleomorphic sarcoma. He underwent wide local excision of the tumour with clear margins, followed by adjuvant radiotherapy. At 6-month follow-up, the patient is clinically and radiologically disease free.

Solitary Plasmacytoma of the Sternal Stalk: A Case Report and Literature Review.

Background: It has been reported that sternal neoplasms are a rare disease, accounting for about 1% of primary bone neoplasms, of which about 60% are malignancy, mostly occurring in the manubrium sternum.Case presentation: We reviewed a 77-year-old man with a solitary plasmacytoma of the sternal stalk presenting with pain in the anterior chest wall by examining a preoperative diagnosis of sternal manubrium malignant bone tumor. We performed extensive sternal manubrium tumor resection + bilateral partial costal cartilage resection + bilateral clavicular head resection + cervical lymph node dissection + thoracic reconstruction, and the postoperative recovery was perfect.Conclusion: We report a rare case of solitary plasmacytoma of the manubrium sternum successfully treated by extensive radical surgery for the manubrium tumor. Although rare, the disease should be identified, examined, and treated early to avoid serious complications.