Langerhans cell histiocytosis of the suprasellar region: diagnosis based on thyroid cytology

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Abstract

Langerhans cell histiocytosis (LCH) may present as unifocal disease of the suprasellar region, with symptoms and signs of hypopituitarism, arginine vasopressin deficiency (AVP-D), and weight gain. Transcranial biopsy is necessary to define diagnosis and guide treatment decisions, but it is associated with significant morbidity. We describe a patient with Hashimoto thyroiditis and a single hypothalamic mass in whom LCH diagnosis was made by thyroid fine-needle aspiration cytology (FNAC) performed despite nonspecific findings in thyroid imaging, on the basis of a slightly elevated [ ¹⁸F]-fluorodeoxyglucose (FDG) avidity on PET/CT and volume increase during follow-up.

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Most cited references 24

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Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages.

Jean-François Emile, Oussama Abla, Sylvie Fraitag … (2016)

The histiocytoses are rare disorders characterized by the accumulation of macrophage, dendritic cell, or monocyte-derived cells in various tissues and organs of children and adults. More than 100 different subtypes have been described, with a wide range of clinical manifestations, presentations, and histologies. Since the first classification in 1987, a number of new findings regarding the cellular origins, molecular pathology, and clinical features of histiocytic disorders have been identified. We propose herein a revision of the classification of histiocytoses based on histology, phenotype, molecular alterations, and clinical and imaging characteristics. This revised classification system consists of 5 groups of diseases: (1) Langerhans-related, (2) cutaneous and mucocutaneous, and (3) malignant histiocytoses as well as (4) Rosai-Dorfman disease and (5) hemophagocytic lymphohistiocytosis and macrophage activation syndrome. Herein, we provide guidelines and recommendations for diagnoses of these disorders.

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Langerhans cell histiocytosis in adults. Report from the International Registry of the Histiocyte Society.

M Aricò, M Girschikofsky, T Généreau … (2003)

Langerhans cell histiocytosis (LCH), characterised by the infiltration of one or more organs by large mononuclear cells, can develop in persons of any age. Although the features of this disease are well described in children, they remain poorly defined in adults. From January 2000 to June 2001, 274 adults from 13 countries, with biopsy-proven adult LCH, were registered with the International Histiocyte Society Registry. Information was collected about clinical presentation, family history, associated conditions, cigarette smoking and treatment, to assist in future management decisions in patients aged 18 years and older. There were slightly more males than females (143:126), and the mean ages at the onset and diagnosis of disease were 33 years (standard deviation (S.D.) 15 years) and 35 years (S.D. 14 years), respectively. 2 patients had consanguineous parents, and 1 had a family history of LCH; 129 reported smoking (47.1%); 17 (6.2%) had been diagnosed with different types of cancer. Single-system LCH, found in 86 patients (31.4%), included isolated pulmonary involvement in 44 cases; 188 patients (68.6%) had multisystem disease; 81 (29.6%) had diabetes insipidus. Initial treatment consisted of vinblastine administered with or without steroids, to 82 patients (29.9%), including 9 who had received it with etoposide, which was the sole agent given to 19 patients. 236 patients were considered evaluable for survival. At a median follow-up of 28 months from diagnosis, 15 patients (6.4%) had died (death rate, 1.5/100 person years, 95% Confidence Interval (95% CI) 0.9-2.4). The probability of survival at 5 years postdiagnosis was 92.3% (95% CI 85.6-95.9) overall, 100% for patients with single-system disease (n=37), 87.8% (95% CI 54.9-97.2) for isolated pulmonary disease (n=34), and 91.7% (95% CI 83.6-95.9) for multisystem disease (n=163). Survival did not differ significantly among patients with multisystem disease, with or without liver or lung involvement) 5-year survival 93.6% (95% CI 84.7-97.4) versus 87.5% (95% CI 65.5-95.9), respectively; P value 0.1). LCH in adults is most often a multisystem disease with the highest mortality seen in patients with isolated pulmonary involvement. It should be included in the differential diagnosis of disseminated or localised disease of the bone, skin and mucosa, as well as the lung and the endocrine and central nervous system, regardless of the age of the patient. A prospective international therapeutic study is warranted.

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International expert consensus recommendations for the diagnosis and treatment of Langerhans cell histiocytosis in adults

Gaurav Goyal, Abdellatif Tazi, Ronald S. Go … (2022)

Abstract Langerhans cell histiocytosis (LCH) can affect children and adults with a wide variety of clinical manifestations, including unifocal, single-system multifocal, single-system pulmonary (smoking-associated), or multisystem disease. The existing paradigms in the management of LCH in adults are mostly derived from the pediatric literature. Over the last decade, the discovery of clonality and MAPK-ERK pathway mutations in most cases led to the recognition of LCH as a hematopoietic neoplasm, opening the doors for treatment with targeted therapies. These advances have necessitated an update of the existing recommendations for the diagnosis and treatment of LCH in adults. This document presents consensus recommendations that resulted from the discussions at the annual Histiocyte Society meeting in 2019, encompassing clinical features, classification, diagnostic criteria, treatment algorithm, and response assessment for adults with LCH. The recommendations favor the use of 18F-Fluorodeoxyglucose positron emission tomography-based imaging for staging and response assessment in the majority of cases. Most adults with unifocal disease may be cured by local therapies, while the first-line treatment for single-system pulmonary LCH remains smoking cessation. Among patients not amenable or unresponsive to these treatments and/or have multifocal and multisystem disease, systemic treatments are recommended. Preferred systemic treatments in adults with LCH include cladribine or cytarabine, with the emerging role of targeted (BRAF and MEK inhibitor) therapies. Despite documented responses to treatments, many patients struggle with a high symptom burden from pain, fatigue, and mood disorders that should be acknowledged and managed appropriately.

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Author and article information

Journal

Journal ID (nlm-ta): Eur Thyroid J

Journal ID (iso-abbrev): Eur Thyroid J

Journal ID (publisher-id): ETJ

Title: European Thyroid Journal

Publisher: Bioscientifica Ltd (Bristol )

ISSN (Print): 2235-0640

ISSN (Electronic): 2235-0802

Publication date (Electronic): 30 May 2024

Publication date (Electronic preprint): 07 May 2024

Publication date Collection: 01 June 2024

Volume: 13

Issue: 3

Electronic Location Identifier: e240011

Affiliations

[1 ]Department of Endocrinology , Diabetology, Nutrition and Therapeutic Education, Geneva University Hospitals, Rue Gabrielle-Perret-Gentil, Geneva, Switzerland

[2 ]University of Geneva , Faculty of Medicine, Rue Michel Servet, Geneva, Switzerland

[3 ]Department of Internal Medicine , Lugano Regional Hospital, Ente Ospedaliero Cantonale, Via Tesserete Lugano, Switzerland

[4 ]Department of Pathology , Geneva University Hospitals, University of Geneva, Rue Gabrielle-Perret-Gentil, Geneva, Switzerland

[5 ]Department of Neuroradiology , Geneva University Hospitals, University of Geneva, Rue Gabrielle-Perret-Gentil, Geneva, Switzerland

[6 ]Department of Neurosurgery , Geneva University Hospitals, University of Geneva, Rue Gabrielle-Perret-Gentil, Geneva, Switzerland

[7 ]Department of Internal Medicine , Geneva University Hospitals, University of Geneva, Rue Gabrielle-Perret-Gentil, Geneva, Switzerland

[8 ]Department of Thoracic and Endocrine Surgery , Geneva University Hospitals, Rue Gabrielle-Perret-Gentil, Geneva, Switzerland

[9 ]Department of Nuclear Medicine , Geneva University Hospitals, Rue Gabrielle-Perret-Gentil, Geneva, Switzerland

[10 ]Department of Hematology , Geneva University Hospitals, Rue Gabrielle-Perret-Gentil, Geneva, Switzerland

[11 ]Service and Central Laboratory of Hematology , Lausanne University Hospital, rue du Bugnon Lausanne, Switzerland.

[12 ]Service of Hematology and Laboratory of Hematology , Institut Central des Hôpitaux, Hôpital du Valais, Av. du Grand-Champsec, Sion, Switzerland

Author notes

Correspondence should be addressed to Maria Mavromati: maria.mavromati@ 123456hcuge.ch

^*(M Mavromati and V Caironi contributed equally to the work and are joint first authors)

Author information

Maria Mavromati http://orcid.org/0000-0003-3085-2928

Article

Publisher ID: ETJ-24-0011

DOI: 10.1530/ETJ-24-0011

PMC ID: 11227055

PubMed ID: 38718824

SO-VID: 0c7870ec-375d-4718-bb37-bbaf3b7eb75c

License:

This work is licensed under a Creative Commons Attribution 4.0 International License.

History

Date received : 17 January 2024

Date accepted : 07 May 2024

Custom metadata

ifp:collection ETJ-cancer-clinical

Keywords: histiocytosis,thyroid fna,hypothalamic lesion

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Keywords: histiocytosis, thyroid fna, hypothalamic lesion

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Langerhans cell histiocytosis of the suprasellar region: diagnosis based on thyroid cytology

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