Langerhans cell histiocytosis (LCH) is a rare disease with limited treatment options. We present a case involving a 57-year-old woman afflicted with an isolated LCH bone osteolytic lesion. A single bisphosphonate infusion significantly alleviated pain, and follow-up scans via CT, PET-CT, and MRI revealed a substantial recalcification of the lesion. Conducting an extensive literature review, we identified 46 cases documenting the efficacy of bisphosphonates in the context of LCH. These findings have raised interest in bisphosphonate infusion as a simple therapeutic alternative in similar situations, with benefits in terms of bone recalcification and pain control for individuals with LCH.

UNASSIGNED: Aneurysmal bone cysts (ABCs) are benign, non-neoplastic cystic lesions composed of multiple blood-filled cavities, which are separated by connective tissue septa and they constitute ~6 and 1.5% of all cases of the skull and jaws lesions, respectively.
UNASSIGNED: Herein, the case of an 80-year-old male with a histologically confirmed diagnosis of ABC involving the body of the left mandible was presented. The patient underwent partial mandiblectomy after confirmation of the diagnosis using orthopantomography, a computed tomography scan, and a tissue biopsy. The patient was free from pain after 1-year of follow-up, and the control orthopantomography showed no evidence of recurrence. This was followed by reconstruction of the healed part of the bone with titanium plates and a piece of bone from the lateral two-thirds of the left femur and thereafter he was cosmetically well.
UNASSIGNED: Patients with ABCs present with expansile and radiolucent bone lesions, which may be associated with displacement and loss of teeth due to alveolar bone erosion. Complete excision of the lesions is mandatory for the prevention of recurrence and increased morbidity.
UNASSIGNED: ABCs that involve the jaws are extremely rare and are more likely to pose a diagnostic challenge as they are more likely to be confused clinically with other expansile radiolucent bone lesions such as ameloblastoma, osteoblastoma, and giant cell tumor among many others. Also, those with extensive bone matrix formation may sometimes be confused histologically with other bone forming tumors including osteosarcoma. Recurrence is common but it can be avoided or minimized by complete resection of the lesion.

BACKGROUND: ECD is a rare non-Langerhans cell histiocytosis with diverse and heterogeneous clinical manifestations, ranging from single-lesion forms to multi-system involvement, including slowly progressing unifocal forms to rapidly evolving life-threatening disease.
METHODS: A female patient presented with a 2-month history of fever. Imaging revealed multiple thromboses, bone destruction, an abnormal pituitary stalk, and clinical manifestations of diabetes insipidus. Excisional biopsy of a tibial lesion was sent for microscopic examination, and subsequent immunohistochemical testing was positive for expression of CD68 and CD163, and negative for expression of the immune markers CD1a, S100, and langerin. This confirmed the diagnosis of ECD. Treatment with methylprednisolone to inhibit the immune inflammatory response along with anti-cytokine therapy with an interleukin-6 antagonist resulted in satisfactory disease control.
CONCLUSIONS: We report a rare case of multiple thromboses, embolism, and multiple organ involvement as the main presentation of ECD, suggesting that ECD should be considered in patients presenting with multiple thromboses associated with multisystem damage. We successfully treated our patient with glucocorticoids and interleukin-6 antagonist. This patient\'s response to treatment suggests that hormone therapy and cytokine/chemokine therapy may be a potential novel treatment for patients with ECD without gene mutations.

Langerhans cell histiocytosis (LCH) is a rare disease that originates from the uncontrolled proliferation and accumulation of bone marrow-derived immature myeloid dendritic cells. Dendritic cells are a type of histiocyte that play an important role in the human immune system and are found in the bone, skin, stomach, eyes, intestines, and lungs.
This systematic review aimed to collect and report published case reports of rare bone disease caused by LCH to avoid misdiagnoses or delays in diagnosis.
We systematically searched Scopus, PubMed, Embase, and Web of Sciences from August 1, 2000 to December 31, 2019. Studies reporting cases of LCH with rare bone involvement were included.
We identified 60 articles including 64 cases. Of the identified cases, 31 (48.4%) involved children, and 33 (51.6%) involved adults. Additionally, 46.9% (30 individuals) were from Asian countries. The mean age of the children was 7.6 ± 4.3 years and that of the adults was 36 ± 12 years. The findings indicated that unifocal bone involvements were the most prevalent form of the disease (68.7%), and, overall, the skull and chest wall were the most commonly affected bones in both adults and children. The spine and long bones were the second most commonly affected bones in children, and the spine and jaw were the second most commonly affected bones in adults. Pain and swelling were the most frequent presenting signs among the investigated cases, and loss of consciousness, myelopathy, nerve palsy, visual loss, torticollis and clicking sounds were rare signs. Osteolytic lesions were the most frequent radiologic feature (62.5%), and intracranial hemorrhage, fluid-fluid level, dura and intracranial extension and pathologic fractures were rare radiological features. Total excision, curettage and observation in the unifocal group of patients and systemic chemotherapy in the other groups (i.e., multifocal and multisystem) were the most frequent management approaches. The recovery rates of the unifocal and multifocal groups were 77.3% and 81.8%, respectively, while that of the multisystem group was 55.5%. The rates of recurrence and mortality in the multisystem group were 11% and were higher than those in the other groups.
LCH is a rare disease that can affect any organ in the human body. However, bone is the most commonly involved organ, and rare bone involvements may be the first or only symptom of the disease due to the rarity of such lesions; a lack of familiarity with them may result in misdiagnosis or delayed diagnosis.

The objective was to integrate the available published data on chondrosarcoma (CHS) of the jaw bones into a comprehensive analysis of its clinical and histological features, treatment, and prognostic factors. An electronic search was undertaken in October 2017. To be eligible, the publication had to provide sufficient clinical/histological data to confirm the diagnosis. One hundred and ten publications (224 cases of CHS) were identified and included. There was a slightly higher prevalence of CHS in males than in females. Most subjects with CHS were in the second to fifth decades of life. The most common symptom was swelling and the most commonly observed location was the maxilla. Histologically, most tumours were of the conventional type and were low grade tumours. The treatment of choice was tumour resection. Histological grade, treatment with chemotherapy alone, and the presentation of recurrence or metastasis were found to be significant independent prognostic factors: patients who presented high-grade tumours, who received chemotherapy alone as the treatment of choice, and those who presented recurrence or metastasis were more likely to have a worse prognosis. In addition, radical surgery associated with radiotherapy as the treatment protocol showed a better prognosis.

OBJECTIVE: To integrate the available data published on central mucoepidermoid carcinoma (CMC) into a comprehensive analysis of its clinical aspects, histology, treatment, and prognostic factors.
METHODS: An electronic search was undertaken in July 2017. Eligibility criteria for publications included having clinical, histological, treatment, and time of follow-up data to confirm the diagnosis.
RESULTS: In addition, gender, histological grade, conservative treatment, and lymph node metastasis were significant independent prognostic factors.
CONCLUSIONS: Male patients with histological grade III CMC of the jaw bone, who used conservative treatment as the main treatment, and presented with lymph node metastasis, were more likely to have a worse prognosis.

OBJECTIVE: The present study reviewed all cases reported of isolated Langerhans cell histiocytosis bone lesions in the head and neck of pediatric patients. The objective was to create a treatment algorithm to facilitate the diagnosis and management of these lesions.
METHODS: Eligible articles were identified through a comprehensive search of the following electronic databases: PubMed, Ovid Medline, Embase, and Cochrane Library.
METHODS: Two authors independently reviewed the titles and abstracts retrieved by the electronic search concordant with the criteria for study eligibility. The lists of articles from each author were jointly reviewed and a common list created. All relevant articles were reviewed in hard copies and as full texts to justify inclusion.
RESULTS: A total of 201 patients (67% male, mean age: 8.1 ± 4.3 years) were included from 45 studies. Clinical presentations included swelling (64%), pain (9%), or both (18%). The skull (61%) and the orbit (24%) were the most common location for isolated bone lesions in the head and neck. The most frequently documented management option was resection, followed by observation, chemotherapy, and intralesional steroid injection.
CONCLUSIONS: Due to its rarity and variability in presentation and severity, treatment of these lesions has yet to be standardized. Based on the review, a diagnosis and treatment algorithm was created for head and neck surgeons when encountering these types of lesions.