IgG4-related disease diagnosed in a paratesticular pseudotumor simulating malignancy, in a patient with retroperitoneal fibrosis.

IgG4 related disease is a term used to describe a fibroinflammatory condition characterized by storiform fibrosis, inflammation with a dense lymphoplasmocytic infiltrate rich in plasma cells expressing IgG4, and often, if not always, raised serum levels of IgG4. We report a case of a patient with a past history of retroperitoneal fibrosis presenting with a swollen left testicle, who underwent an orchidectomy due to suspicion of malignancy. The surgical specimen revealed a paratesticular pseudotumor with histopathological and immunohistochemical characteristics of IgG4 related disease. To the best of our knowledge, just nine such cases have previously been reported, of which only three were manifestations of systemic disease, as in the present case. Whilst it is important to recognize the clinical and radiological features of this entity, histopathology is often essential in order to confirm the diagnosis.

Inflammatory pseudotumor and myofibroblastic inflammatory tumor. Diagnostic criteria and prognostic differences.

INTRODUCTION: Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) are two very rare entities that were formerly included in the same category; however, today they are considered two different diseases due to the neoplastic origin of the IMT. Our objective is to share our experience in the management of these two types of tumors that we must take into account in the differential diagnosis of pulmonary masses or nodules. METHODS: Thirteen patients with a pathological diagnosis of IPT and IMT who underwent surgery between 2008 and 2019 were retrospectively studied. We recorded the pre and postoperative information of each one, as well as the survival analysis. RESULTS: Of the 13 patients, 8 were men and 5 women. The mean age of presentation was 53,5 years. An atypical segmentectomy was performed in 6 patients; a lobectomy was necessary in 6 and a pneumonectomy in 1 case. In all cases a complete resection was achieved. Diagnosis was possible thanks to histology, immunohistochemical (IHQ) and fluorescent in situ hybridization (FISH) techniques determining the expression of IgG4 and the rearrangement of ALK, respectively. After a median follow up of 49 months, we didn't find any loco-regional or distant recurrence in the patients studied. CONCLUSION: IPT and IMT are rare tumors with a very good prognostic. The diagnosis of both entities is based mainly on specific anatomopathological techniques. Surgery has, in most cases, both a diagnostic and therapeutic role.

Inflammatory pseudotumor and myofibroblastic inflammatory tumor. Diagnostic criteria and prognostic differences. / Seudotumor inflamatorio y tumor miofibroblástico inflamatorio. Criterios diagnósticos y diferencias pronósticas.

INTRODUCTION: Inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) are two very rare entities that were formerly included in the same category; however, today they are considered two different diseases due to the neoplastic origin of the IMT. Our objective is to share our experience in the management of these two types of tumors that we must take into account in the differential diagnosis of pulmonary masses or nodules. METHODS: Thirteen patients with a pathological diagnosis of IPT and IMT who underwent surgery between 2008 and 2019 were retrospectively studied. We recorded the pre and postoperative information of each one, as well as the survival analysis. RESULTS: Of the 13 patients, 8 were men and 5 women. The mean age of presentation was 53,5 years. An atypical segmentectomy was performed in 6 patients; a lobectomy was necessary in 6 and a pneumonectomy in 1 case. In all cases a complete resection was achieved. Diagnosis was possible thanks to histology, immunohistochemical (IHQ) and fluorescent in situ hybridization (FISH) techniques determining the expression of IgG4 and the rearrangement of ALK, respectively. After a median follow up of 49 months, we didnt find any loco-regional or distant recurrence in the patients studied. CONCLUSION: IPT and IMT are rare tumors with a very good prognostic. The diagnosis of both entities is based mainly on specific anatomopathological techniques. Surgery has, in most cases, both a diagnostic and therapeutic role.

Seudotumor inflamatorio pulmonar: presentación de 2 casos y revisión de la literatura / Pulmonary inflammatory pseudotumour: two case report and review of theliterature

Primary lung tumours in children are rare, the most common lesions seen in clinical practice are metastatic disease. The majority of children who present with a primary or secondary pulmonary malignancy will present coincidentally while seeking attention for another medical problem, or with non-specific abnormalities such as cough with collapse or consolidation on the chest x-ray. Primary malignant tumours of the lung are the most common, this group is made up of carcinoid tumours, bronchogenic carcinoma and pleuropulmonary blastoma. Benign primary pulmonary tumours are inflammatory pseudotumour or plasma cell granuloma and hamartoma. Often, the possibility of a primary or secundary pulmonary tumour is considered only when radiographic abnormalities or symptoms persist or fail to respond to therapy, many children are asymptomatic until they have advanced disease, which delays diagnosis even further. The aim of this article is show our experience of two patients with inflammatory pseudotumour and literature review.

Los tumores pulmonares primarios en niños son muy poco frecuentes, siendo más reportadas las lesiones metátasicas. La mayoría de los pacientes que presentan un tumor pulmonar ya sea primario o secundario, son hallazgos de algún examen generalmente imagenológico, como parte del estudio de otras causas o en presencia de síntomas inespecíficos como tos asociada a atelectasia o condensación. Los tumores pulmonares primarios malignos son los más frecuentes, comprenden el tumor carcinoide, carcinoma broncogénico y blastoma pleuropulmonar. Los tumores pulmonares primarios benignos son el seudotumor inflamatorio (sTi) o granuloma de células plasmáticas y el hamartoma. Se debe tener un alto índice de sospecha en el diagnóstico de tumor pulmonar primario o secundario, frente a la persistencia de síntomas o si presenta una lesión en la radiografía de tórax que no se modifica o invade localmente a otros tejidos adyacentes a pesar del tratamiento. El objetivo de este artículo es mostrar nuestra experiencia de 2 pacientes portadores de sTi y hacer una revisión de la literatura.