Langerhans Cell Histiocytosis in Sphenoid Sinus: Uncommon Bone Involvement.

BACKGROUND Langerhans cell histiocytosis (LCH) is a rare and uncontrolled proliferation of dendritic cells of myeloid origin. The incidence of LHC was estimated at 5 cases per million children ages 0-15 years old. The most common places for this tumor are the jaw, vertebra, pelvis, and the extremities. The disease with multisystem involvement can present a mortality rate of 20% and one-third of children have multisystem involvement. We present a case with unusual bone involvement of the anterior cranial base with a challenging diagnosis and a complex surgical approach. CASE REPORT We report the case of a 6-year-old boy who manifested the disease with daily holocranial headache, worse in the frontal region and refractory to analgesia for 10 days, strabismus homonymous, diplopia, and right palpebral ptosis. The tumor affected the sphenoid sinus, internal carotid artery, and sella turcica, and made contact with the pituitary gland. A joint surgery with Otorhinolaryngology and Neurosurgery was performed by nasal endoscopic access to the skull base by means of the right medial turbinectomy (for the access) and right sphenoid opening, septectomy and opening of the left sphenoid to work with 4 hands and, after resection of lesion, inside the sphenoid. CONCLUSIONS This patient had rare bone involvement from LCH and atypical clinical presentation next to the important and delicate structures of the anterior skull base, but had a satisfactory outcome.

Langerhans cell histiocytosis with aneurysmal bone cyst-like changes: a case-based literature review.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a neoplastic transformation of myeloid precursors that commonly presents as an osteolytic lesion of the long or flat bones in children. Aneurysmal bone cysts (ABC) are benign neoplasms that frequently affect the metaphysis of long bones and the spine, often revealing a rapidly expansile lesion with fluid-fluid levels. LCH with secondary ABC-like changes is a rare condition that has only been reported five times, with two presentations in the cranium. The aim of this paper is to review the etiology, clinical and radiographic presentations, and treatment of this condition, as well as to present a novel case on the topic. CASE DESCRIPTION: We describe a 5-year-old boy with a rapidly growing head mass and eye pain resulting in a diagnosis of LCH with secondary ABC-like changes. Radiography demonstrated an expansile, lytic lesion of the left parietal bone with fluid-fluid levels. A confirmatory diagnosis was made through histopathology, demonstrating an inflammatory, histiocytic infiltrate staining positive for CD1a, CD68, CD207 (Langerin), and S-100. The lesion was surgically excised, and the patient recovered without any complications. CONCLUSION: We present a novel case of LCH with secondary ABC-like changes managed with surgical excision. While a radiographic workup with multiple imaging modalities is helpful for diagnosis, a thorough immunohistochemical analysis is essential as imaging characteristics are variable and nonspecific. Furthermore, surgical excision should be considered first-line treatment for lesions involving the skull in surgically accessible areas as it is curative, alleviates symptoms, and allows for histopathological diagnosis.

Langerhans Cell Histiocytosis in the Pediatric Spine Requiring Stabilization: A Literature Review and Report of 3 Cases.

CASE: This report describes 3 cases of Langerhans cell histiocytosis (LCH) of the cervical and thoracic spine in patients aged 4 to 10 years. Each patient had painful lytic spinal lesions with vertebral body collapse and posterior involvement suggesting instability requiring corpectomy, grafting, and fusion. All 3 patients were doing well at their most recent follow-up without pain or recurrence. CONCLUSION: Although LCH of the pediatric spine is usually successfully treated non-operatively, we recommend corpectomy and fusion when there is instability of the spinal column and/or severe stenosis. Posterior element involvement occurred in all 3 cases and may lead to instability.

Eosinophilic granuloma of the calvarium: is conservative management a valid option? Illustrative case and systematic review of the literature.

INTRODUCTION: Eosinophilic granuloma (EG) is the most common form of Langerhans cell histiocytosis, presenting as a single osteolytic lesion of the calvarium. Its diagnosis is based on typical clinical and radiological features. While surgical resection has been the standard treatment for EG, growing evidence favors watchful waiting, as unifocal calvarial lesions appear to frequently undergo spontaneous remission. However, histopathological confirmations of this hypothesis are still very limited. METHODS: Methods. Here, we report a case of EG with typical clinical and radiological features which, due to intervening circumstances, was resected in a delayed fashion. Moreover, we perform a systematic review of the literature on conservative management of EG. RESULTS: In our case, histological examination showed ongoing bone regeneration with no traces of the disease. Through our literature review, we found 47 cases of calvarial EG managed with watchful waiting. No active intervention was required in 43 cases (91%). Four patients (9%) received surgery or chemotherapy due to the persistence/progression of symptoms or family request. Three reports other than ours documented spontaneous disease remission in surgically resected EG upon histopathological examination. CONCLUSION: Our report provides further evidence that watchful waiting can be a reasonable option in the management of single calvarial EG.

Cholesteatoma Masquerading as Recurrent Langerhans Cell Histiocytosis.

Langerhans cell histiocytosis is a rare condition affecting the temporal bone in up to 60% of cases. Symptoms are non-specific and the differential diagnosis includes infection, benign lesions such as cholesteatoma, and malignant lesions of the skull base. Here, we report the case of a 14-yearold child referred with chronic ear discharge, and background of multifocal Langerhans cell histiocytosis 9 years prior. Recurrence of Langerhans cell histiocytosis was initially suspected and systemic treatment was considered. Further imaging workup and surgical exploration of the mastoid showed a secondary acquired cholesteatoma arising from a dehiscent posterior ear canal wall. Surgical removal of the cholesteatoma was performed with a canal wall down procedure. We review the presentation and management of temporal bone Langerhans cell histiocytosis. We recommend that cholesteatoma should be considered in case of recurrence of otological symptoms in patients with a background of Langerhans cell histiocytosis.

Stereotactic Radiosurgery for Localized Cranial Langerhans Cell Histiocytosis: A Single Institution Experience and Review of Literature.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare idiopathic disease characterized by the clonal proliferation of Langerhans histiocytes in various parts of the body and capable of leading to organ damage and tumor formation. Reports of cranial LCH in the adult population are extremely rare. Although surgery remains the preferred option for localized LCH lesions, the role of stereotactic radiosurgery (SRS) is emerging. OBJECTIVE: To retrospectively review a rare case series to determine the safety and effectiveness of SRS for patients with localized cranial LCH. METHODS: We retrospectively reviewed histopathologically confirmed cases of localized cranial LCH treated with SRS at our institute in the adult population between January 2005 and September 2022. Five patients were identified with a median age of 34 years (19-54 years). The tumor location was in the pituitary stalk in 3 patients, the orbit in one patient, and the parietal skull in one patient. The median target volume was 2.8 cc (range: 0.37-6.11). Treatment was delivered in a single fraction in 4 patients (median margin dose of 8 Gy, range: 7-10 Gy) and in 3 fractions (22.5 Gy) in 1 patient. The median follow-up was 12 years (range: 4-17). None of the patients required craniotomy for tumor debulking before or after SRS. RESULTS: The local tumor control rate for the lesions was 100%. All 3 patients with LCH in the pituitary stalk had diabetes insipidus at the initial presentation and developed panhypopituitarism after SRS. Diabetes insipidus was not improved after SRS. The other 2 patients presented no adverse radiation effects. Based on the literature review, our case series was the largest retrospective series on SRS for localized cranial LCH, with the longest median follow-up. CONCLUSIONS: SRS for patients with localized cranial LCH was a safe and effective treatment modality in this case series. Larger studies are encouraged to validate the role of SRS in the treatment of localized cranial LCH.

Case Report: Langerhans cell histiocytosis involving the cervical spine in an adult patient.

Langerhans Cell Histiocytosis (LCH) is a typically benign disorder that affects infants predominately, with adult occurrence being uncommon. We discuss the case of a 22-year-old guy who visited our clinic complaining of three months of acute nape pain and upper limb radiculopathy. Notably, the patient had no history of trauma, fall injuries, or tuberculosis. Radiological tests identified a single osteolytic lesion within the C3 vertebral body. The lesion was removed, an anterior C3 corpectomy and discectomy were performed, and the patient's spine was reconstructed with a titanium cage and plating. The patient's nape discomfort and radiculopathy vanished almost instantly after surgery. A definitive diagnosis of LCH was confirmed through histological examination. This case report illustrates the unusual and uncommon occurrence of LCH at the C3 vertebral body, for which fusion surgery was the only viable therapeutic option. The patient's recovery from radiating pain following the surgical procedure demonstrates the effectiveness of the intervention. LCH in the cervical spine is rather rare, but it is nevertheless important to be aware of the possibility of developing it.

Current perspectives on the role of liver transplantation for Langerhans cell histiocytosis: A narrative review.

Langerhans cell histiocytosis (LCH) is a malignant disease of the histiocytes involving various organ systems. The spectrum of liver involvement in LCH ranges from mild transaminitis to end-stage liver disease. The hallmark of hepatic LCH is secondary sclerosing cholangitis, which manifests due to a progressive destruction of the biliary tree by malignant histiocytes. Chemotherapy remains the mainstay of treatment for active LCH. Early recognition, diagnosis and a systematic approach to the management of LCH can ameliorate the disease process. Nonetheless, the liver involvement in these patients may progress despite the LCH being in remission. Liver transplantation (LT) remains central in the management of such patients. Various facets of the management of LCH, especially those with liver involvement remain unclear. Furthermore, aspects of LT in LCH with regards to the indication, timing and post-LT management, including immunosuppression and adjuvant therapy, remain undefined. This review summarises the current evidence and discusses the practical aspects of the role of LT in the management of LCH.

Aggressive unifocal bone Langerhans cell histiocytosis with soft tissue extension both responsive to radiotherapy: a case report.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare haematological neoplasm characterized by the accumulation of CD1a+, CD207/Langerin+ histiocytes within inflammatory lesions. LCH can involve any organ, but osteolytic bone lesions are most often encountered. Unifocal bone lesions may regress spontaneously after a thick needle biopsy has been taken. CASE PRESENTATION: In this case report, we describe the initial presentation of a single BRAFV600E mutated osteolytic LCH lesion in the left proximal humerus of a 46-year-old previously healthy woman. Despite multiple surgical interventions, she unexpectedly experienced progressive disease manifestation with significant soft tissue extension to the surrounding musculature, subcutis and epidermis. Because the disease manifestation remained loco-regional, radiotherapy (RT) (total dose of 20 Gy in 10 fractions) was initiated. CONCLUSION: The patient achieved a complete remission without any side effects. This case highlights that RT is a rational and relative mild local treatment option for patients with aggressive LCH affecting the bone and surrounding soft tissue.

Liver transplantation in a child with liver cirrhosis caused by langerhans cell histiocytosis: a case report.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare condition that has a variety of clinical manifestations. But LCH in children localized only in the hepatobiliary system is unusual. CASE PRESENTATION: Here we reported a rare case of a 2-year-old boy who was serendipitously found to have elevated liver enzymes while undergoing treatment of a perianal abscess. After a period of earlier conservative treatment in another hospital, the perianal abscess had resolved but the levels of liver enzymes were still rising slowly. The child was then referred to our institution for a definitive diagnosis. After laboratory tests, imaging and pathological examinations, a diagnosis of liver cirrhosis and sclerosing cholangitis was established, although the cause was unclear. Subsequently, living-donor liver transplantation was performed due to deterioration in liver function. Following successful liver transplantation, a diagnosis of LCH localized only within the hepatobiliary system was finally confirmed, based on additional pathological and imaging investigation. Additionally, the BRAF V600E mutation in this patient was also confirmed. The child has now recovered without evidence of LCH recurrence. CONCLUSIONS: LCH localized only within the hepatobiliary system is unusual. The presence of unexplainable sclerosing cholangitis and liver cirrhosis in any child should raise the suspicion of LCH.

Short-Term Resorption of Grafted Bone After Split Calvarial Bone Grafting for an Occipital Skull Defect in a Patient With Multisystem Langerhans Cell Histiocytosis.

ABSTRACT: Langerhans cell histiocytosis (LCH) is a rare disease in which LCH cells derived from myeloid dendritic cells proliferate and invade single or multiple systems. We have encountered a case of short-term resorption of the grafted bone after split calvarial bone grafting for an occipital skull defect in a 6-year-old girl with multisystem LCH in remission who was referred to our department for reconstructive surgery. We performed cranioplasty using a split calvarial bone graft. Two months after surgery, computed tomography scans revealed that most of the grafted bone had been resorbed. To our knowledge, there have been no such reports previously. There are 2 possible explanations for the resorption of an autologous bone graft in this patient. The first possibility is that the recipient site contained an active lesion and the second is that the bone resorption occurred because the bone graft was placed on the periosteum.

Solitary Langerhans Cell Histiocytosis of Skull: Case Report with One-year Follow-up.

Langerhans cell histiocytosis (LCH) is a rare disorder characterised by increased production of Langerhans-type histiocytes. It is more common in the pediatric age group with a predilection for osseous involvement, though any organ may be involved. A 10-year male child was brought to the neurosurgical clinic with a slow growing painful tender mass on the head. Initial attempt to biopsy the lesion failed due to excessive bleeding. It was later imaged and removed with a frontal craniotomy. Histopathological evaluation along with immunohistochemistry revealed the true nature of the lesion. Follow-up revealed complete excision of the lesion and no recurrence at one-year after surgery. Key Words: Langerhans cell histiocytosis, Pediatric age, Skull.

Spontaneous Thyroid Hemorrhage Caused by Langerhans Cell Histiocytosis: A Case Report and Literature Review.

Purpose: Langerhans cell histiocytosis (LCH) is a rare clonal disorder of Langerhans antigen-presenting cells. However, thyroid LCH involvement is relatively rare. We present the first case of spontaneous thyroid hemorrhage due to LCH progression and discuss the clinical features, diagnosis, and treatments of thyroid LCH in a literature review. Methods: Clinical data were collected. Previously published articles on thyroid LCH involvement were reviewed to assess the clinical features, diagnosis, and treatments for thyroid LCH. Results: A 54-year-old female presented with a multi-system LCH, affecting the uterus, liver, pituitary gland, and thyroid gland. Clinical stability was achieved after systemic chemotherapy. After 7 years of regular follow up, the patient complained of a sudden painful neck swelling and progressive dyspnea. Computed Tomography revealed bilateral goiter with hematoma, and the patient was diagnosed with spontaneous thyroid bleeding based on her clinical symptoms and radiological findings. The patient was incubated to relieve airway compromise and partial thyroidectomy was performed for definitive treatment. Pathological evaluation further confirmed the diagnosis of thyroid LCH. The patient recovered well after surgery. Conclusion: Spontaneous thyroid bleeding due to thyroid LCH progression is extremely rare. Treatments for LCH vary depending on the severity of the disease. We suggest that, for patients with multi-system LCH with thyroid lesion, long-term active surveillance of thyroid hormone concentrations, and thyroid gland volume is required. Physicians should be alert of the potentially life-threatening spontaneous thyroid hemorrhage when aggravated diffuse goiter and hypothyroidism appear. Further investigation is required to establish the guidelines for thyroid LCH treatment.