Histiocitosis congénita de células de Langerhans

Barrios, Katherine; Patiño, Oscar; Muñoz, Nelson; Moneriz, Carlos

doi:10.7705/biomedica.5150

Cited by 4 publications

(4 citation statements)

References 22 publications

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“…Evaluating location and assessing for SS or MS disease is paramount in establishing treatment. 16 17 The petrous portion of the temporal bone has been described as a “current” location of LCH-associated lesions. 7 The patient in this case had SS disease, with an affection of the squamous portion of the temporal bone associated with a soft tissue mass infiltrating the temporalis muscle.…”

Section: Discussionmentioning

confidence: 99%

Langerhans Cell Histiocytosis Mimicking a Meningeal Lesion with Temporal Bone and Muscle Compromise in an Adult Patient: A Case Report

Durán-Ojeda,

Arce,

Campos-Fajardo

et al. 2024

J Neurol Surg Rep

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Introduction Langerhans cell histiocytosis (LCH) is a rare proliferative systemic disease characterized by the growth of abnormal dendritic cells and wide-ranging organ involvement. This condition can affect individuals of all ages, but most commonly children, with a peak incidence in toddlers. Symptoms may vary depending on the affected organ or system. Case Report A 43-year-old man presented with a left temporal stabbing headache unresponsive to management with therapy and nonsteroidal anti-inflammatory drugs. Initial evaluation revealed a contrast-enhanced left temporal extra-axial lesion with bone and muscle compromise. Differential diagnoses, including multiple myeloma, were explored. Initial laboratory tests and imaging studies showed no other abnormalities, except for splenomegaly and a residual granuloma in the left lung. En bloc resection of the lesion was recommended. The patient underwent surgical intervention, which included resection of the dural lesion and all borders of an infiltrating tumor within the temporalis muscle and the affected portion of the left temporal bone. Posterior pathological examination revealed LCH. Postoperative course was uneventful. Follow-up appointments were scheduled after pathology results confirmed the diagnosis. Patient has continued follow-up for the following 3 months after the surgical procedure. Further evaluations are pending. Discussion This case report corresponds to a patient with LCH. These patients are individualized and stratified based on local or systemic involvement to determine the most appropriate type of management. This is a rare case as LCH is rare in older patients and the initial presented lesion initially mimicked a meningioma; however, its atypical behavior and associated lytic compromise led to consideration of possible differential diagnoses. Conclusion LCH can present with lytic bone lesions, mimicking other conditions, including infiltrative neoplastic lesions. Early diagnosis and appropriate surgical management are essential for optimal patient outcomes. Long-term follow-up is crucial to monitor disease progression and response to treatment.

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Section: Discussionmentioning

confidence: 99%

Langerhans Cell Histiocytosis Mimicking a Meningeal Lesion with Temporal Bone and Muscle Compromise in an Adult Patient: A Case Report

Durán-Ojeda,

Arce,

Campos-Fajardo

et al. 2024

J Neurol Surg Rep

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“…7 Pancytopenia can be also observed especially when the bone marrow is affected. 7 Only two cases of prenatal diagnosis have been reported, which manifested as fetal hydrops. 6 All these findings were present.…”

Section: Discussionmentioning

confidence: 99%

“…Congenital Langerhans cell histiocytosis is an infrequent disease which can affect multiple organs, including skin, liver, spleen, lungs, and CNS, among others 7 . Involvement of the skull and facial bones is typical 7 . Pancytopenia can be also observed especially when the bone marrow is affected 7 .…”

Section: Discussionmentioning

confidence: 99%

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Congenital metastatic neuroblastoma with placental involvement as a rare cause of non‐immune fetal hydrops

Campillo‐Ajenjo,

Pena‐Burgos,

Herrero Ruiz

et al. 2024

J of Obstet and Gynaecol

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Non‐immune hydrops fetalis represents the end‐stage status of a variety of diseases, including metastatic tumors. We report a case of non‐immune hydrops fetalis associated with multiple disseminated echogenic nodular lesions detected by ultrasound and confirmed by magnetic resonance. Cordocentesis demonstrated anemia and thrombopenia. Differential diagnosis included histiocytosis X, acute leukemia or metastatic disease. A stillbirth was diagnosed at week 25 + 6. The autopsy revealed hydrops fetalis, a right adrenal gland mass, multiple disseminated nodules histologically composed of small round blue cells positive for synaptophysin, and placental involvement, concordant findings with congenital undifferentiated neuroblastoma Stage M. No chromosomal abnormalities were associated, nor amplification abnormalities in MYCN and ALK genes. Metastatic neuroblastoma should be considered in the differential diagnosis of non‐immune hydrops fetalis associated with multiple nodular lesions.

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