Histiocitosis pulmonar: más allá de la histiocitosis de células de Langerhans relacionada con el tabaco / Pulmonary histiocytosis: beyond Langerhans cell histiocytosis related to smoking

Objetivo: Revisar los hallazgos de imagen de las diferentes histiocitosis pulmonares. En concreto, además de la conocida histiocitosis de células de Langerhans relacionada con el tabaco y su posible aparición sin antecedentes de este, la enfermedad de Rosai-Dorfman y la enfermedad de Erdheim-Chester. También se revisa su etiopatogenia, histología, clínica y tratamiento. Conclusión: La histiocitosis de células de Langerhans, la enfermedad de Rosai-Dorfman y la enfermedad de Erdheim-Chester son un conjunto de patologías de causa idiopática en las que la proliferación e infiltración de histiocitos es el hallazgo anatomopatológico diagnóstico. La histiocitosis de células de Langerhans se manifiesta en forma de nódulos y quistes que respetan los ángulos costofrénicos, característicamente en pacientes fumadores. Aunque es poco frecuente, debe pensarse en esta entidad en pacientes no fumadores, en tratamiento quimio y radioterapéutico, con nódulos cavitados de nueva aparición e incluirse en el diagnóstico diferencial junto con la enfermedad metastásica y la infección oportunista. La enfermedad de Rosai-Dorfman y la enfermedad de Erdheim-Chester se presentan con hallazgos torácicos más inespecíficos, como adenopatías, engrosamiento intersticial y derrame pleural. En la enfermedad de Erdheim-Chester, las características manifestaciones extratorácicas suelen ser claves en el diagnóstico

Objective: To review the imaging findings for the different types of pulmonary histiocytosis. In particular, in addition to the well-known pulmonary Langerhans cell histiocytosis related to smoking and its possible appearance in nonsmokers, we focus on non-Langerhans cell histiocytosis in Rosai-Dorfman disease and Erdheim-Chester disease. We also review the etiopathogenesis, histology, clinical presentation, and treatment of pulmonary histiocytosis. Conclusion: Langerhans cell histiocytosis, Rosai-Dorfman disease, and Erdheim-Chester disease are idiopathic diseases in which the proliferation and infiltration of histiocytes is the histologic finding that confirms the diagnosis. Langerhans cell histiocytosis manifests as nodules and cysts that spare the costophrenic angles; it typically appears in smokers. Although it is uncommon in nonsmokers, Langerhans cell histiocytosis should also be considered in nonsmokers treated with chemotherapy and radiotherapy in whom cavitated nodules appear and should be included in the differential diagnosis together with metastatic disease and opportunistic infections. Rosai-Dorfman disease and Erdheim-Chester disease present with less specific thoracic findings such as adenopathies, interstitial thickening, and pleural effusion. In Erdheim-Chester disease, the characteristic extrathoracic manifestations are usually key for the diagnosis

Pulmonary histiocytosis: beyond Langerhans cell histiocytosis related to smoking. / Histiocitosis pulmonar: más allá de la histiocitosis de células de Langerhans relacionada con el tabaco.

OBJECTIVE: To review the imaging findings for the different types of pulmonary histiocytosis. In particular, in addition to the well-known pulmonary Langerhans cell histiocytosis related to smoking and its possible appearance in nonsmokers, we focus on non-Langerhans cell histiocytosis in Rosai-Dorfman disease and Erdheim-Chester disease. We also review the etiopathogenesis, histology, clinical presentation, and treatment of pulmonary histiocytosis. CONCLUSION: Langerhans cell histiocytosis, Rosai-Dorfman disease, and Erdheim-Chester disease are idiopathic diseases in which the proliferation and infiltration of histiocytes is the histologic finding that confirms the diagnosis. Langerhans cell histiocytosis manifests as nodules and cysts that spare the costophrenic angles; it typically appears in smokers. Although it is uncommon in nonsmokers, Langerhans cell histiocytosis should also be considered in nonsmokers treated with chemotherapy and radiotherapy in whom cavitated nodules appear and should be included in the differential diagnosis together with metastatic disease and opportunistic infections. Rosai-Dorfman disease and Erdheim-Chester disease present with less specific thoracic findings such as adenopathies, interstitial thickening, and pleural effusion. In Erdheim-Chester disease, the characteristic extrathoracic manifestations are usually key for the diagnosis.

Multimodality management of a locally advanced primary oesophageal melanoma with radical surgery and adjuvant chemoimmunoradiotherapy.

We present the case of a 46-year-old woman diagnosed with a primary oesophageal melanoma (PEM), who was treated with radical surgery followed by combined chemoimmunotherapy (interferon, carboplatin, dacarbazine and external radiotherapy) and who achieved a complete response after this treatment. PEMs are rare malignancies, with less than 300 cases described in the literature. The main differential diagnosis is with metastases of skin or ocular malignant melanomas. They are usually diagnosed at advanced stages and prognosis is typically poor. The main treatment modality should be radical surgery. The role of adjuvant treatment is uncertain, although some long responses have been seen with the use of chemotherapy or immunotherapy alongside surgery.

Multimodality management of a locally advanced primary oesophageal melanoma with radical surgery and adjuvant chemoimmunoradiotherapy

We present the case of a 46-year-old woman diagnosed with a primary oesophageal melanoma (PEM), who was treated with radical surgery followed by combined chemoimmunotherapy (interferon, carboplatin, dacarbazine and external radiotherapy) and who achieved a complete response after this treatment. PEMs are rare malignancies, with less than 300 cases described in the literature. The main differential diagnosis is with metastases of skin or ocular malignant melanomas. They are usually diagnosed at advanced stages and prognosis is typically poor. The main treatment modality should be radical surgery. The role of adjuvant treatment is uncertain, although some long responses have been seen with the use of chemotherapy or immunotherapy alongside surgery (AU)

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