We report a case of multisystem Langerhans cell histiocytosis in a pediatric patient with central nervous system involvement, highlighting F-18(FDG) uptake characteristics of dural sites of disease. We also highlight the advantages of functional data offered by FDG-PET as a useful follow-up tool to assess viability and, therefore, treatment response of previously known central nervous system lesions. The utility of recognizing characteristic patterns of FDG uptake in dural disease is also applicable in cases of diagnostic uncertainty, such as when evaluating isolated dural lesions or when distinguishing between Langerhans cell histiocytosis and similar appearing lesions such as meningiomas.

A 72-year-old woman with no significant past medical history was admitted to the hospital for new-onset of leukocytosis with neutropenia, anemia, and thrombocytopenia, as well as a pruritic skin eruption. She was found to have acute myeloid leukemia (AML) with myelomonocytic differentiation. Her skin eruption consisted of widespread hemorrhagic crusted papules on the scalp and trunk. A skin biopsy was performed, which revealed a proliferation of mononuclear cells in the dermis with prominent epidermotropism and positive expression of CD1a and langerin (CD207), supporting a diagnosis of Langerhans cell histiocytosis (LCH). LCH is an uncommon proliferative disorder of activated Langerhans cells, which generally presents in children. In adults, it is exceptionally infrequent. Associated malignancies and rare reports of AML developing in subsequent years after an initial presentation of LCH have been described. Here we present an unusual concurrent presentation of LCH and AML in an adult.

UNASSIGNED: Lobular capillary hemangioma (LCH) is a benign vascular lesion typically affecting the head and neck region, especially the nasal cavity. However, its occurrence in the nasal cavity of the pediatric population is rare, making its diagnosis and management crucial in such cases.
METHODS: A 7-year-old female presented with left-sided nasal obstruction and recurrent epistaxis for 4 months. Examination revealed a dark purplish-red nasal mass, which bled easily upon probing. CT scans showed a soft tissue lesion with post-contrast enhancement. Histopathological examination confirmed lobular capillary hemangioma. The patient successfully underwent surgical excision without pre-operative embolization.
UNASSIGNED: LCH affects both the skin and mucous membranes. Although its exact etiology remains debated, trauma is a leading risk factor for its development. Biopsy and radiological investigations confirm its diagnosis. Differentiating LCH from other vascular disorders with similar presentations is essential for a thorough understanding and better management. Endoscopic surgical excision combined with electrodesiccation is the treatment of choice.
CONCLUSIONS: Intranasal LCH in children is infrequent. An accurate diagnosis is essential for a comprehensive understanding. Surgical excision with electrodesiccation is the preferred treatment, but the role of pre-operative embolization is still under discussion.

Carcinosarcoma ex pleomorphic adenoma is a rare malignant neoplasm, with most cases reported in the parotid gland. We herein report a case of a 75-year-old male referred to our hospital with a long-standing right parotid lesion that was treated in an outside hospital by a superficial parotidectomy. The patient reported a painful, rapidly enlarging mass following the excision. Histopathological examination showed the proliferation of malignant epithelial and mesenchymal elements with a solid sheet of Langerhans cells admixed with eosinophils. The Langerhans cells were reactive to CD1a and BRAF; hence, a diagnosis of carcinosarcoma ex pleomorphic adenoma with Langerhans cell histiocytosis was given. Complete clinical and radiographic workup showed no other organ involvement. The patient underwent total parotidectomy with adjuvant chemoradiation; however, the tumor progressed and showed lung metastasis. We herein report the first case of a concurrent Langerhans cell histiocytosis with associated carcinosarcoma ex pleomorphic adenoma.

Pyogenic granuloma (PG) is a reactive connective tissue disorder with female predilection, which exhibits a tumor-like mass with occasional bleeding and superficial ulceration. It most commonly occurs in the maxillary gingiva followed by the mandibular gingiva. It can also occur in extra gingival sites like buccal mucosa, labial mucosa, and palate. There are two histopathological types of PG, namely, a lobular capillary hemangioma (LCH) variant and a non-LCH variant. The various management methods include surgical resection or laser excision along with deep curettage, and there are various nonsurgical methods like local steroid injection, topical administration of various drugs, and sclerotherapy. During the surgical excision, there is a risk of bleeding, and the surgeon should be equipped for the same. The PG (both LCH and non-LCH variant) has an increased chance of recurrence because of which complete excision is mandatory along with the removal of the local irritants. In this case report, a 28-year-old female patient reported recurrent painless swelling in the lower front gums for the past nine months. The surgical excision was done in-toto along with the removal of local irritants (calculus). The swelling was sent for histopathological examination. The patient was kept on regular follow-ups. The patient was followed up continuously for nine months. The swelling did not recur after the excision. Hence, it was concluded that complete excision and removal of local irritants are extremely crucial to prevent a recurrence.

Langerhans cell histiocytosis (LCH) is a rare disorder most commonly involving skin, bone and lung. The gastrointestinal tract (GIT) is an uncommon site of disease and only a handful of case reports exist. We present a case of a 15-year old boy with treated LCH involving the skin, bones, central nervous system (CNS) and pituitary gland. He presented with rectal bleeding and on investigation was found to have a single rectal polyp which was confirmed histologically and immunologically to be LCH. Further investigation revealed no other foci of disease.

Flexible bronchoscopes are increasingly being used in diagnostic and therapeutic medicine and have obviated the need for and risks associated with general anesthesia. Here the authors present the case of a 2-y-old girl who was diagnosed with isolated pulmonary Langerhans cell histiocytosis by histopathology; a lung biopsy sample was obtained using a cryoprobe via a flexible bronchoscope. The girl was brought in with complaints of loss of weight, appetite loss, and rapid breathing for the past 2 mo. Examination revealed hypoxia, tachypnea, clubbing with failure to thrive, and bilateral crepitations on auscultation. Imaging studies showed ground-glass opacities with multiple cystic lesions in both lungs. A preliminary diagnosis of LCH was made, and transbronchial cryobiopsy was done via a flexible bronchoscope. Histopathology confirmed the diagnosis with Cluster of differentiation 1a (CD1a) staining. A flexible bronchoscope can be a useful tool for obtaining lung biopsy samples using a cryoprobe in children.

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.
METHODS: 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.
CONCLUSIONS: 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.

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Langerhans cell histiocytosis (LCH) is a childhood pathology with a peak of incidence ranging from 1 to 4 years of age, though diagnosis is often made in adult age. LCH is clinically classified into three types: eosinophilic granuloma, Hand-Schuller-Christian disease and Abt-Letterer-Siwe disease. We report a case of Hand-Schüller-Christian disease with diabetes insipidus, skull and maxillary involvement in a 16-year-old boy referred to our observation for gradual increase in mobility of the teeth and subsequent gradual loss of the second premolars and the first molars of the upper jaw. Due to the extension of the lesion and the age of the patient, surgery, and chemotherapy was chosen as the more fit treatment according to the current protocol. The clinical and radiological evaluation at the end of the therapy and after 5 years showed complete remission. The absence of relapse has allowed to initiate a fixed orthodontic dental alignment treatment with a good response to orthodontic treatment despite the underlying disease. The present case exemplifies the importance of close multidisciplinary dental and medical collaboration including general dentistry, periodontology, oral medicine, oral and maxillofacial pathology, oral radiology, orthodontics and hematology-oncology for diagnosis, management, treatment monitoring, and decision-making.