Histiocitosis de células de Langerhans ósea a forma poliostótica.A propósito de un caso / Histiocytosis of Langerhans cells bone to polyostotic form. A case report

RESUMEN Se presenta el caso de un paciente masculino de 39 años de edad, con antecedentes de buena salud aparente que fue ingresado en la sala de clínica oncológica, por presentar dolores articulares en región costal, cervical y lumbo sacra que le dificultaban la marcha, además de notar un aumento de volumen en la región lateral derecha de la frente. En inicio se admite con diagnóstico de mieloma múltiple y compresión medular para recibir tratamiento con radioterapia. Los estudios imagenlógicos, histopatológicos e inmunohistoquìmicos confirmaron la histiocitosis de langerhans ósea a forma poliostótica.

ABSTRACT A case of a 39-year-old male patient is presented with a history of good apparent health who was admitted to the oncology clinic, due to articular pain in the costal, cervical and lumbar sacral regions that made walking difficult. In addition, It was noticed an increase in volume in the right lateral region of the forehead. Initially, he was admitted with a diagnosis of multiple myeloma and spinal cord compression to receive treatment with radiotherapy. Imaging, histo-pathological and immune-histo-chemical studies confirmed the histio-cytosis of bone langerhans to a polyostotic form.

Erdheim-Chester disease: description of two illustrative cases involving the lung.

AIM: Erdheim-Chester disease represents a clonal systemic proliferation of histiocytes. Bone is the most common site of involvement, although almost any organ, including the lungs, can be affected. METHODS AND RESULTS: The diagnosis of Erdheim-Chester disease can be difficult, owing to its rarity and protean presentation. Correlation between clinical, radiological and histological findings is mandatory for identification of the disease. Foamy histiocytes, lacking Langerhans cell markers, represent the typical histological findings, although their absence does not rule out Erdheim-Chester disease. Identification of BRAF mutation can be helpful in making the diagnosis, and allows for the development and application of targeted therapies in this setting. CONCLUSIONS: Herein, we describe two cases presenting with lung involvement and vertebral lesions, lacking the more typical long-bone involvement. One case histologically mimicked Rosai-Dorfman disease. However, both cases harboured the pathognomonic BRAFV600E mutation.

Torcicolo agudo como manifestação clínica isolada de histiocitose / Acute torticollis as a single clinical manifestation of histiocitosis

Menino, com 9 anos de idade, teve episódio agudo prolongado de torcicolo (laterocólis à esquerda), com duração de quinze dias. Radiografia de coluna evidenciou colapso da vértebra C5 e o diagnóstico final foi granuloma eosinofílico. O torcicolo pode ser agudo, recorrente ou prolongado. No adulto, a maioria dos casos de torcicolo recorrente é postural, mas na infância, torcicolos recorrentes ou prolongados tiveram possibilidade significativamente aumentada de haver uma causa, sendo asmais comuns lesões estruturais de coluna e lesões de neurônio motor primário.

A 9-year old boy presented an acute episode of torticollis (left deviation) that lasted for fifteen days. Conventional radiology of the cervical spine showed collapse of the C5 vertebra. His conclusive diagnosis was thatof eosinophilic granuloma. Torticollis can be acute recurrent or prolonged. In adulthood, most cases of recurrent torticollis are caused by postural problems; however, in childhood, recurrent or prolonged torticollis is much more likely to have a cause, and the most common are spine structural lesions or lesions in the primary motor neuron.

Association between Pituitary Langerhans Cell Histiocytosis and Papillary Thyroid Carcinoma.

Here we report a case of panhypopituitarism caused by pituitary Langerhans cell hystocitosis (LCH) in a 22-year-old woman affected by papillary thyroid carcinoma (PTC). Although several cases of the coexistence of PTC and LCH within thyroid tissue have been described in relative literature, in this case, the patient presented a unique suprasellar retrochiasmatic histocytosis localization which, to the best of our knowledge, had never been described before in association with PTC. Even if this aspect is not addressed in the present case report, it is worth noting that about 50% of the patients affected either by LCH or PTC are characterized by activating mutations of the proto-oncogene BRAF. This, along with other clinical studies, may warrant further biomolecular large-scale case study investigations in order to evaluate a possible connection between the 2 conditions and shed light on the etiology of these diseases, which are still largely unknown.