Search

Pulmonary adenoid cystic carcinoma (PACC) is an exceedingly rare tumor of low-grade malignancy. Diagnosis is often late, so the tumoral process may be huge at presentation. Surgical resection could be challenging, and the recurrence rate would be greater. We report, hereby, a case of proximal PACC with involvement of the carina in a young male adult, without respiratory distress. Surgical resection was performed through a left pneumonectomy followed by a complex trachea-bronchoplastic procedure. During the operative time, the assisted ventilatory mode was carefully chosen. No adjuvant treatment was needed. Our patient is still under clinicoradiological surveillance and remains disease-free.

Background: Fractures of the clavicle are common injuries, which often require reduction and internal fixation. Although Kirschner pins have been commonly used to treat these fractures with good results, migration of these devices may result in severe internal lesions., Case Presentation: We report herein the case of 61-year-old man, who presented for intrapulmonary migration of a Kirschner pin, 25 years after closed reduction and fixation of a clavicle fracture., Conclusion: Migration of an osteosynthesis pin can be lethal. Patients with osteosynthesis pins, should have a regular follow, until the removal of the wires., (© 2024. The Author(s).)

Cervical teratomas are extremely rare germ cell tumours and it is much more common in newborn than adults, and in contrast to the paediatric cases adult teratomas have been highly malignant. Cervical teratoma incorporates lesions arising in the anterior and posterior triangles of the neck. This tumor can reach enormous size and cause airway obstruction and patients should be quickly treated. Surgery is the primary modality of treatment as malignant transformation can occur. Hereby, we present a case of benign teratoma of neck in adult which was completely misdiagnosed preoperatively due to its rare occurrence in adults.Even though cervical teratoma of adult is extremely rare, it should be considered as an important differential diagnosis in patient of midline cystic neck swelling. Preoperative radiological investigations requires high index of suspicion. Complete surgical resection is recommended. We believe that upper cervicotomy approach is a safe and effective method for the treatment of mature cervical teratoma with a few protruding into the superior mediastinum., Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03748-8., Competing Interests: Competing InterestNo., (© Association of Otolaryngologists of India 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.)

Background: Young Patients with lung cancer represent a distinct subset of patients with this neoplasm. Young International studies show increased lung cancer rates in females, while the incidence in males continues to decline. There is evidence to suggest that this trend recurs in younger patients. We studied the effects of gender differences on the incidence of surgical stages of lung cancer in young adults and its mortality rate., Methods: This study is a retrospective review (2010-2020) of young adults (aged under 45 years) with surgical-stage of lung cancer. We calculated female-to-male differences in incidence rate ratios, tumor characteristics, surgical management, and survival. Cumulative survival curves were generated by the Kaplan-Meier method., Results: We examined 46 men and 24 women, under 45 years. Female patients were diagnosed at earlier stages. The proportion of stage IA disease was significantly higher in women than in men (46% versus 13%, respectively) (p=0.03). Women were more likely never smokers (42% versus 83%, p=0.02). A histologic subtype, females were more likely to have typical carcinoid tumors (13.54% versus 10.21% for males) (p>0.05). The largest histological type in men was adenocarcinoma (25.53% versus 4.16%, p>0.05). All the patients were operated. Three men had neoadjuvant chemotherapy and one was operated on for cerebral oligometastatic before his chest surgery. Adjuvant chemotherapy was given to 7 women and 21 men. Despite the small number of postoperative complications in our study (n= 8, 11.2%), the male sex was significant in predicting this complication (p<0.05). The mortality rate was 1.4%. The 5-year overall survival rates were 84% in men and 87% in women., Conclusion: Our study identified sex differences in the incidence and mortality rates for surgical lung cancers in young adults, but the biological and endocrine mechanisms implicated in these disparities have not yet been determined., Competing Interests: The authors declare that they have no competing interests, (© 2023 Abdennadher et al.)

Mediastinal paraganglioma presented as a large dumbbell tumor is a rare entity. We report a case of a 47 year old woman who suffered from spinal pain and sporadic lower limb paresis. The imaging studies showed a voluminous mass occupying the posterior mediastinum with right foraminal extension. For excellent results, a combined effort was necessary including thoracic and neurosurgeons teams. Complete resection was successfully performed without laminectomy. The operative course was uneventful., (Copyright © 2022. Published by Elsevier Ltd.)

Introduction and Importance: Primary adenoid cystic carcinoma (ACC) of the lung is extremely rare. This tumor can be asymptomatic or have non characteristics symptoms, and the diagnosis is often late. The treatment of choice is surgery when it's possible., Case Presentation: We herein report the case of a young patient with ACC of the left main bronchus. He had dyspnea and chest pain for 6 months. Complete atelectasis of the left lung was found on the chest x-ray. Bronchoscopy showed a tumor obstructing the LMB and invading the carina. The CT scan revealed a 5 cm tumor obstructing the left main bronchus (LMB) with extension to the carina and thoracic trachea. The extension assessment was without abnormalities. The treatment was surgical. A left carinal pneumonectomy by double lateral thoracotomy was performed. The postoperative results were satisfactory. There was no recurrence with a follow-up of 2 years., Clinical Discussion: The therapeutic management of ACC is essentially based on surgical resection, which should be as radical as possible. However, complete resection is often difficult given the infiltrating nature of the tumor., Conclusion: Sleeve pneumonectomy with carinal resection is a curative option for patients with ACC of the main bronchi and carina that require expertise of the surgeons., (Copyright © 2021. Published by Elsevier Ltd.)

Tyrosinemia type II, also designated as oculocutaneous tyrosinemia or Richner-Hanhart syndrome (RHS), is a very rare autosomal recessive disorder. In the present study, we report clinical features and molecular genetic investigation of the tyrosine aminotransferase (TAT) gene in two young patients, both born to consanguineous unions between first-degree cousins. These two unrelated families originated from Northern and Southern Tunisia. The clinical diagnosis was based on the observation of several complications related to Richner-Hanhart syndrome: recurrent eye redness, tearing and burning pain, photophobia, bilateral pseudodendritic keratitis, an erythematous and painful focal palmo-plantar hyperkeratosis and a mild delay of mental development. The diagnosis was confirmed by biochemical analysis. Sequencing of the TAT gene revealed the presence of a previously reported missense mutation (c.452G>A, p.Cys151Tyr) in a Tunisian family, and a novel G duplication (c.869dupG, p.Trp291Leufs 6). Early diagnosis of RHS and protein-restricted diet are crucial to reduce the risk and the severity of long-term complications of hypertyrosinemia such as intellectual disability., (© 2013 Elsevier B.V. All rights reserved.)

Objective: A bronchopleural fistula (BPF) is a serious complication after pulmonary resection and carries a high mortality rate. It remains a therapeutic challenge. The lack of a consensus suggests that no optimal therapy is available; however, endoscopic closure of a fistula may avoid extensive and potentially risky surgery., Methods: Seventeen patients (15 men and 2 women) with a BPF after a pneumonectomy (n = 2) or a lobectomy (n = 15), seen between 1995 and 2010, were reviewed. Their median age was 50 years (range, 14-75 years). Underlying diseases were malignant (n = 4) and nonmalignant (n = 13)., Results: The mean interval between surgery and fistula development was 20 days (range, 5-270 days). Clinical symptoms leading to a diagnosis of BPF were a persistent air leak (n = 2), a persistent air leak associated with pleural empyema (n = 3), pleural empyema alone (n = 11), and dyspnea (n = 1). Mean fistula size was 3.3 mm (range, 2-9 mm). Treatment consisted of oriented pleural drainage, adequate antibiotic therapy, and endoscopic closure of the fistula with local application of silver nitrate through a flexible bronchoscope (3-15 sessions, 3 times per week). Fistula closure was successful in 16 patients, but failed in 1 patient, who died from acute respiratory distress., Conclusions: BPF is a severe complication in thoracic surgery. The combination of pleural drainage, adequate antibiotic treatment, and mucosal application of silver nitrate, through a flexible bronchoscope, is an efficient alternative and avoids extensive surgical intervention., (Copyright © 2013 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Dermatomyositis (DM) is a rare inflammatory autoimmune disease for which an iatrogenic origin has been described in a few cases. The authors report a case of DM occurring after simvastatin intake. A 50-year-old male sought medical attention for a photodistributed rash and considerable muscular weakness present for 3 months. One year earlier, simvastatin had been introduced. Serum creatine kinase levels were elevated. Histological examination of a muscle biopsy was consistent with a diagnosis of DM. Investigation for neoplasia and associated autoimmune disease proved negative. All clinical and laboratory abnormalities diminished corticosteroid therapy (1 mg/kg/day). Case reports have suggested that lipid-lowering drugs, especially statins, could induce or reveal chronic muscle diseases. In statins myopathy, reduction of coenzyme Q has been discussed as a key mechanism. Our case of DM in a patient receiving simvastatin adds to the previous reported cases in the literature and highlights the potential role of statins as triggers of immune systemic diseases.

Transgressive palmoplantar keratoderma (PPK) is the phenotypic hallmark of Mal de Meleda (MDM, MIM 24300). It is characterized by erythema and hyperkeratosis that extend to the dorsal face of the hands and feet. The disease is distributed worldwide and includes the Mediterranean population. The gene responsible for MDM, ARS (component B) mapped on chromosome 8qter, encodes for the SLURP-1 protein (Ly-6/uPAR related protein-1). A variety of mutations within the ARS gene have been shown to underlie MDM in different populations. Genetic heterogeneity of MDM is suspected. We have recently shown that three different homozygous mutations (82delT, C77R, C99Y) were responsible for MDM in 17 patients from Northern Tunisia belonging to eight unrelated consanguineous families. We report here a Tunisian family with three siblings presenting with recessive transgressive PPK closely resembling the MDM phenotype that excludes linkage to the ARS gene.