Davide Patrini

Inflammatory myofibroblastic tumour of the lung: a reactive lesion or a true neoplasm?

Inflammatory myofibroblastic tumour (IMT) of the lung represents an extremely rare type of inflammatory pseudo tumor that appears most commonly in children and young individuals. There has been an ongoing controversy whether an IMT is a reactive lesion or a true neoplasm making the further management extremely challenging. Purpose of the paper is through a literature review to highlight the existence of this rare tumour along with its key features and the management options available.

Etiology and management of spontaneous haemothorax

Spontaneous haemothorax (SH) is a subcategory of haemothorax that involves the accumulation of blood within the pleural space in the abscence of trauma or other causes. The clinical presentation is variable and includes a rapid progression of symptoms of chest pain and dyspnea that can be life threatening when hemodynamic instability and hypovolemic shock occurs. Despite haemothorax, SH is much less common with data limited to case reports and case series. A literature review has been performed to identify and summarise all potentials causes leading to this clinical entity.

Hepatopulmonary Fistula: a life threatening complication of hydatid disease

Despite extensive infection control measures against parasitic diseases, hydatid disease, caused by Echinococcus granulosus, still occurs in a minor group of our population. If the infection is not treated adequately, it goes on to developing life-threatening complications, one of which is hepatopulmonary fistula. These complications usually warrant early surgical intervention, or else may lead to extensive sepsis and ultimately death. We discuss the case of an elderly female suffering from pulmonary hydatid disease, further complicated by a hepatopulmonary fistula and underwent surgical treatment. This case emphasises the importance of early recognition of pulmonary hydatid disease given its atypical nature of presentation before the disease is further exacerbated by this aggressive complication. Furthermore, it is imperative to incorporate radical surgery as the first-line treatment in established hepatopulmonary fistula, in order to prevent further clinical deterioration and curative outcome.

Hybrid multistep approach to mega-aortic syndrome: the Lupiae technique

OBJECTIVES Several techniques have been described for the treatment of thoraco-abdominal aneurysms in patients with mega-aortic syndrome (MAS), but the incidence of stroke, spinal cord injury and endoleaks remains significant. We present the mid-term results of a new hybrid, multistep technique to treat patients with MAS. METHODS From November 2005 to November 2012, 118 patients with MAS underwent surgical repair of thoracic and thoraco-abdominal aneurysms with the Lupiae technique. Fifty-five patients presented chronic aneurysms and 63 patients Type A acute dissections. Eighty-three patients underwent ascending aorta and arch replacement with a multibranched Dacron graft and epiaortic vessels rerouting (thoracic Lupiae procedure). Twenty patients had the thoracic Lupiae procedure plus partial visceral debranching (coeliac trunk and superior mesenteric artery [SMA]) through an upper mini-laparotomy. Fifteen patients had the thoracic Lupiae procedure plus a complete visceral debranching (coeliac trunk, SMA and renal arteries) using a second multibranched Dacron graft to replace the infrarenal aorta. All the patients with chronic aneurysms and 34 of 63 patients with Type A dissections underwent implant of endovascular stent grafts. RESULTS In-hospital mortality was 8.4%. No patients had stroke or spinal cord injury. The incidence of temporary renal failure was 5.2%. No patients presented endoleaks immediately and at follow-up CT scans. No death or reoperation occurred during the follow-up. CONCLUSIONS These results evidence that the Lupiae technique is a safe and effective option for the treatment of patients with MAS, achieving the complete exclusion of thoraco-abdominal aneurysms and of the residual false lumen in patients with acute aortic dissections.

Sclerosing hemangioma of the lung showing strong FDG avidity on PET scan: Case report and review of the current literature.

Sclerosing Hemangioma is a rare lung tumor with polymorphic histologic features that usually occurs in middle aged women. Based on many immunohistochemical and ultrastructural studies, it is most probably derived from undifferentiated respiratory epithelial cells. Symptoms are usually due to enlargement of the tumor and compression of the surrounding tissues. Occurrence of multiple lesions or metastasis is extremely rare although some authors consider sclerosing hemangioma as a potentially low grade malignancy tumor. It usually presents with low to moderate uptake on FDG PET imaging. We present a case of sclerosing hemangioma with strong FDG avidity on PET scan in a 41 year old lady with history of haemoptysis. A full review of the literature on this topic was performed.

Mediastinal hemangioendothelioma: Case report and review of the literature

Background Epithelioid haemangioendothelioma (EHE) is a rare low-grade vascular neoplasm that can arise in the lung, liver, soft tissues or, less commonly, bone. Due to its low prevalence of less than one in a million and its non-specific clinical features, EHE is often misdiagnosed and managed inappropriately. Here we discuss the case of a 58 year-old gentleman with mediastinal EHE and review existing literature on pulmonary EHE (PEH). Case history A 58 year-old gentleman presented to our outpatient Clinic with chest discomfort and palpitations. A whole-body FDG-CT-PET showed an FDG-avid single 6.3cm nodule in the superior anterior mediastinum which was fully excised by robotic approach. Histology showed a nodular structure with clusters of epithelioid and spindled cells with a low proliferative index and mitotic count, suspended in a sclerotic stroma. Immunohistochemistry staining was positive for CD3 and CD34, confirming endothelial lineage, and SMA, identifying smooth muscle clusters. Discussion PEH typically presents in young Caucasian women, either incidentally as multiple small pulmonary nodules on CT or with respiratory symptoms that include cough, dyspnoea, chest pain and occasionally pleural effusions. Aetiology and prognosis remain unclear, although indicators of poor prognosis include the presence of respiratory symptoms, male gender, older age and multi-organ disease. Diagnosis is difficult and PEH is often misidentified as chronic granulomatous disease, amyloidosis or other malignancy of the lung. Histological features suggestive of PEH include nodules of hypocellular sclerotic stroma containing spindle-shaped tumour cells with abundant eosinophilic cytoplasm, vacuoles containing erythrocytes and low mitotic counts. CD31, CD34 and Fli-1 positive immunohistochemistry is strongly indicative of epithelioid lineage. There is no standard treatment for PEH but curative resection is the preferred treatment option where possible, with chemotherapy being used as adjuvant treatment or in widespread inoperable disease. Conclusion This case report outlines the clinicopathological features that are characteristic of EHE with the hope of facilitating correct and early diagnosis in the future.

IgG4 related lung disease extending to the thoracic vertebrae

IgG4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect practically every organ. Although it was first identified in pancreas and salivary glands, major organs like liver, biliary tree, kidney, thyroid glands and lungs are commonly involved, sometimes resulting in organ failure. We describe a case of an 41-year-old man presented with back pain after a rotator cuff injury. A Computed Tomography (CT) revealed incidentally a right lower lobe paravertebral lesion extending across the T5 and T6 vertebral levels and invading into the adjacent pleural surface. The laboratory findings and the CT guided biopsy were inconclusive. Morphological and immunohistochemical findings after a lung biopsy by video-assisted thoracic surgery (VATS) were suggestive to IgG4-related lung disease (IgG4-RLD), which was confirmed with high serum levels of IgG4. This represents the first case of a IgG4-RLD lesion located in the mediastinum and extending to the adjacent pleural surface and vertebrae and should be included in the differential diagnosis of posterior mediastinal masses.

Key Features in the Management of Pulmonary Carcinosarcoma

Pulmonary carcinosarcoma represents a category of extremely rare tumours accounting for 0.1% of all lung malignancies. It is defined as a poorly differentiated non-small-cell carcinoma that contains a component of sarcoma or sarcoma-like elements. These biphasic tumours typically have a poor prognosis due to late diagnosis and early metastases. Preoperative tissue diagnosis is usually difficult due to the heterogeneity of the tumour, with biopsies often just reflecting one element of the tumour. By means of a case illustration and review of the literature, we discuss the optimal management of patients with pulmonary carcinosarcoma.

Non-intubated awake uniportal VATS: how to start?

The history of thoracoscopic surgery started more than 100 years ago when Hans Christian Jacobaeus, a Swedish internist, performed and described in 1910 a procedure to treat pleural adhesions and tuberculosis. After the 1950s with the success of the medical treatment against tuberculosis, the “Jacobaeus operation” was gradually abandoned until the 1970s when some pulmonologists became the reference persons for the thoracoscopy, especially in Continental Europe.

Pros-cons debate about the role and evolution of uniportal video-assisted thoracic surgery (VATS)

Uniportal video-assisted thoracic surgery (VATS) is the latest advancement in minimally invasive surgery, combining the advantages of triportal thoracoscopic surgery, such as less pain or paresthesia and faster recovery, with optimal cosmetic outcomes. The uniportal access was first described by Marcello Migliore and Gaetano Rocco for minor thoracic procedures like wedge resection (1,2) and nowadays, thanks to the gained experience in the past years, more complex cases are performed hence expanding the indications for single-incision thoracoscopic surgery.