Mandible lymphoma: an aggressive osteolytic lesion

Background: Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-hodgkin's lymphoma. In oral cavity represents approximately 2% of all malignancies. Case presentation: This report describes a rare mandibular involvement of DLBCL. A 56 year-old man was referred for evaluation of left mandible pain. In the anamnesis, the patient informed to be treating tooth pain in lasting 6 months. On oral evaluation, an intense mobility of the left mandibular second molar and a swelling in posterior left mandible were observed. Computed tomography showed a large osteolytic lesion affecting both mandibular body and ramus. An incisional biopsy was performed and according to histopathological and imumnohistochemical features, DLBCL was diagnosed. The treatment consisted of 8 cycles of R-CHOP and adjuvant radiotherapy. He is asymptomatic after 6 years. Conclusion: This case showed a rare bone presentation of DLBCL and such tumor should be considered as differential diagnosis of osteolytic lesion of the mandible.

A Case of Peripheral Neuropathy - A Presentation of Poems Syndrome with an Osteolytic Lesion

Introduction: POEMS syndrome is a rare paraneoplasticsyndrome that can often be mistaken for other neurologicdisorders.Case Report: A 48-year-old gentleman presented withprogressive mixed motor and sensory neuropathy involvinglower limbs, with generalized hyperpigmentation andbilateral pitting pedal edema. Neurological examinationshowed symmetrical wasting involving both proximaland distal groups of muscles of lower limbs, decreasedpower (Grade 4/5) of the lower limbs with weakness beingmore severe distally, generalized areflexia, absence ofvibration sense and proprioception over the lower limbs andgraded sensory loss over the dorsum of both feet. Workupshowed a demyelinating sensorimotor polyneuropathy onnerve conduction velocity study and albuminocytologicaldissociation in CSF. After inconclusive initial evaluation, aPET CT was done which picked up an FDG avid lytic lesionin the L1 vertebra, and lymphadenopathy, and an MRI LSspine confirmed an expansile lesion with ""Mini brain sign"",indicative of plasmacytoma. Serum free light chain ratio andserum protein electrophoresis were normal. Bone marrowshowed no evidence of marrow involvement. Consideringthe constellation of findings of demyelinating sensorimotorpolyneuropathy, lymphadenopathy, peripheral edema anda solitary plasmacytoma, the possibility of a rare variant ofPOEMS syndrome with an osteolytic lesion was thought of.The lesion was biopsied and proven to be a plasmacytoma.The patient responded well to localized radiation therapy tothe lesion, along with intravenous steroids.Conclusion: A subacute symmetric sensorimotor neuropathymay be a presentation of POEMS syndrome. There can be rareinstances of a lytic lesion on radiography in POEMS"

A Case of SAPHO Syndrome Associated with Lytic Bone Lesions Resembling Metastases / 대한피부과학회지

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome includes a variety of inflammatory bone disorders associated with dermatologic pathology. A 57-year-old female presented with pustulosis on both hands that had persisted for several months. She also had lower back pain without trauma history. On physical examination, tenderness on her lower back and left anterior chest wall pain were found, and claudication was observed. Radiological studies including computed tomography (CT), magnetic resonance imaging (MRI), and positron emission tomography (PET)-CT showed endplate lytic changes in her spine, a focal hypermetabolic lesion in a left rib, and costochondral junction. These findings raised doubt on the presence of metastatic bone lesions, and there was no indication for primary cancer after a complete medical checkup. Palmoplantar pustulosis was well controlled by treatment with acitretin. The osteitis associated with SAPHO syndrome usually presents as osteosclerosis, while reports on osteolytic lesions are rare. We report herein a rare case of SAPHO syndrome associated with bone lesions resembling bone metastasis.

Histiocitosis de células de Langerhans con compromiso esternal aislado: Caso clínico / Langerhans cell histiocytosis with isolated sternum involvement: A clinical case

La histiocitosis de células de Langerhans es una entidad poco frecuente, que se caracteriza por la proliferación clonal de células dendríticas con amplia variabilidad clínica. En algunos casos, la enfermedad tiene afectación multisistémica con compromiso de vida y, en otros casos, de compromiso exclusivamente óseo, en los que la sobrevida es del 100%. Se presenta a una niña de 8 años que consultó al Servicio de Urgencias por dolor esternal de 4 días de evolución. Mediante estudios de imágenes, se observó una imagen lítica en el esternón. Se decidió la internación para el manejo del dolor y estudio. Se realizó una punción-aspiración con aguja fina, cuya inmunohistoquímica fue positiva para el marcador CD1a, que confirmó el diagnóstico de histiocitosis. La paciente recibió 40 mg/día de metilprednisolona, con buena evolución. Se decide exponer este caso por ser una forma de presentación atípica de una enfermedad poco prevalente, pero cuyo motivo de consulta amerita la sospecha diagnóstica.

The Langerhans Cell Histiocytosis (LCH) is a rare condition, characterized by the proliferation of dendritic cells. Its clinical presentation is variable and ranges from an isolated skin or bone disease, mainly the skull, to a life-threatening multisystemic disease. This case is about a healthy 8-year-old girl with a history of four days of severe sternum pain and no other symptomatology. At the initial evaluation at the emergency department a chest X-ray (Figure 1) and thoracic computed tomography scan with 3-D reconstruction were performed and they showed the sternal osteolytic lesion (Figure 2). She was admitted to the hospital for further evaluation. The immunohistochemistry evaluation of the fine needle aspiration sample was positive for CD1a, confirming the diagnosis of histiocytosis. The patient received methylprednisolone 40 mg/day with clinical improvement.

Clinical analysis of aneurysmal bone cyst in jaw bone / 口腔疾病防治

Objective @#To investigate aneurysmal bone cyst (ABC) in oral and maxillofacial of clinical appearance, clinical treatment and prognosis.@* Methods @#From July 2003 to December 2015, 8 patients with aneurysmal bone cyst in jaw bone was treated and recorded with the general condition, clinical symptoms, imaging examinations, surgical treatment and prognosis.@*Results @#Among the 8 patients, there were 5 females and 3 males with the age from 5 to 48 years old. 5 cases happened in mandibular bone and 3 cases originated from the maxillary bone. All the patients were primary lesions and diagnosed by pathology. CT scan showed multilocular cystic expansive space occupying lesions, septa, residual bone crest and liquid-liquid form; enhanced imaging showed the tortuous and dilated small blood vessels. Cystic wall composed of spindle shaped fiber cells, tissues and cells, broken bone like multinucleated giant cells and chronic inflammatory cells form and a ribbon like structure, cysts separated by fibrous tissue containing bone and bone fragments, hemorrhage and hemosiderin deposition was observed which was confirmed under the light microscope. The main treatment of aneurysmal bone cyst was radical operation. After 1~6 years of follow-up, no patient was found recurrence. @* Conclusion@# The imaging findings of aneurysmal bone cyst in jaw bone was distintive, with diagnosis of the disease depended on pathology; the main treatment was radical operation with good prognosis.

Idiopathic Hypertrophic Spinal Pachymeningitis with an Osteolytic Lesion

Idiopathic hypertrophic spinal pachymeningitis (IHSP) is a chronic, progressive, inflammatory disorder characterized by marked fibrosis of the spinal dura mater with unknown etiology. According to the location of the lesion, it might induce neurologic deficits by compression of spinal cord and nerve root. A 58-year old female with a 3-year history of progressive weakness in both lower extremities was referred to our institute. Spinal computed tomography (CT) scan showed an osteolytic lesion involving base of the C6 spinous process with adjacent epidural mass. Magnetic resonance imaging (MRI) revealed an epidural mass involving dorsal aspect of cervical spinal canal from C5 to C7 level, with low signal intensity on T1 and T2 weighted images and non-enhancement on T1 weighted-enhanced images. We decided to undertake surgical exploration. At the operation field, there was yellow colored, thickened fibrous tissue over the dura mater. The lesion was removed totally, and decompression of spinal cord was achieved. Symptoms improved partially after the operation. Histopathologically, fibrotic pachymeninges with scanty inflammatory cells was revealed, which was compatible with diagnosis of idiopathic hypertrophic pachymeningitis. Six months after operation, motor power grade of both lower extremities was normal on physical examination. However, the patient still complained of mild weakness in the right lower extremity. Although the nature of IHSP is generally indolent, decompressive surgery should be considered for the patient with definite or progressive neurologic symptoms in order to prevent further deterioration. In addition, IHSP can present as an osteolytic lesion. Differential diagnosis with neoplastic disease, including giant cell tumor, is important.

Granuloma eosinófilo craneal solitario: caso clínico / Solitary eosinophilic granuloma of the skull: a clinic case

La histiocitosis de las células de Langerhan (HCL), es una enfermedad Granulomatosa de etiología no bien precisada todavía, la cual puede tener diferentes manifestaciones y localizaciones,1 aunque algunos la consideran una proliferación neoplásica de las células de Langerhan que aparecen en el escenario de sitios nodulares o no.2 La Histiocytosis X incluye tres componentes: El Granuloma Eosinófilo, la enfermedad de Hand-Schüller-Christian y el síndrome de Letterer-Siwe.5 El Granuloma Eosinófilo es un tumor óseo benigno muy raro, que se presenta en más del 90 por ciento de los casos en niños menores de 10 años, 6 teniendo además cierta predilección por el sexo masculino. Esta lesión es usualmente encontrada en los huesos largos y planos. Se trata de una paciente femenina de 50 años de edad con antecedentes de procesos alérgicos y asma bronquial grado II y fumadora inveterada, que hacía 8 meses, previo a su ingreso, había comenzado a presentar cefalea holocraneal, fue notando irregularidades en su cuero cabelludo y fue hospitalizada porque al realizarle una radiografía simple de cráneo se evidenció una imagen radiotransparente de bordes mal definidos, lo que fue constatado por tomografía simple de cráneo, la paciente fue operada, realizándole una craniectomía y excéresis de un tejido Granulomatoso sobre la duramadre con colocación de injerto de cemento quirúrgico en el área de la craniectomía. Se le diagnosticó un Granuloma Eosinófilo. Posterior a su recuperación fue tratada con oncología donde se le aplicó radioterapia. La evolución de la paciente fue satisfactoria, ahora se encuentra asintomática.

The Langerhans cells hystiocitosis is a Granulomatous ill, it has unknown etiology today. This disease can be many places and clinical manifestations, although some authors consider it a neoplasic proliferation of the Langerhans cells which can be on nodular region scenery or not. X Hystiocitosis include three diseases: Eosinophilic Granuloma, Hand-Schuller- Chritian disease and Letterer-Siwe syndrome. Eosinophilic Granuloma is a very uncommon benign bone tumor, which may be present in more than 90 percent in childhood under 10 years old. They have predominance on male patients. Usually it is found on long and flat bones. The case report is about 50 years old, female patient, with allergic history and bronchial asthma II degree, she come to the hospital because she don’t filled well since 8 months ago and she complained mild headache, furthermore she noticed irregularity over her skull, on vertex region, at her admission she was screening with cranial X ray, where was found a radio lucid images over the painful area, moreover it had irregular edge whit more than 10 cm long. After that she was screening on CT-scan, where was found a very large osteolytic lesion without intracranial grow. She was approached with craniectomy and graft of surgical cement. She was diagnosed Eosinophilic Granuloma on pathologic department. After her discharge she was treated by oncology, here she received holocraneal radiotherapy. Her outcome has been satisfactory because she is asymptomatic and her CT-scan showed not evidences of recidivism.

Isolated Metacarpal Bone Metastasis from Advanced Rectosigmoid Carcinoma

Distant metastasis is a common sequelae of stage III colorectal adenocarcinoma. Liver and lungs are the most common sites for distant metastases, but, metastases to extremities are very rare. We report a case of rectosigmoid adenocarcinoma, which metastasizes to left first metacarpal bone, which is the first occurrence in our experience. The investigations and management of the patient are discussed.

Hypercalcemia and Extensive Osteolytic Lesion with Increased Plasma Prostaglandin E2 Level in a Child with Acute Lymphoblastic Leukemia / 대한혈액학회지

In this report, we present a rare case of childhood ALL with hypercalcemia and extensive osteolytic lesions. The case was a 7-year-old girl presenting with vomiting and aggravating bone pain. Radiologic examinations showed severe osteolytic lesions of the skull and extremities. Laboratory findings revealed low hemoglobin, normal WBC count with absent circulating blasts, and an increased serum calcium level. Serum intact PTH and 1,25-(OH)2 vitamin D3 levels were below the normal ranges and parathyroid hormone-related peptide (PTHrP) was not detected, whereas serum levels of prostaglandin E2 were elevated. The hypercalcemia resolved with specific antileukemic chemotherapy along with supportive care. The elevated plasma prostaglandin E2 levels decreased slightly after complete remission with induction chemotherapy. These findings suggest that increased plasma prostaglandin E2 levels may be one of the pathogenetic mechanisms responsible for the occurrence of hypercalcemia in this patient.

Histiocitosis: reporte de un caso / Histiocytosis: case report

La histiocitosis de células de Langerhans se describió desde el siglo XIX. Su origen y patogénesis se basan actualmente en tres eventos: clonalidad comprobada, un proceso reactivo a una infección viral, una proliferación y daño mediados por citocinas. El diagnóstico es histopatológico con la demostración de gránulos de Birbeck o la positividad para CD1a. Afecta a pacientes de cualquier edad y se manifiesta como una lesión osteolítica única o hasta como una enfermedad sistémica. El tratamiento es la resección quirúrgica, la radioterapia o la quimioterapia. El pronóstico varía de acuerdo a la presentación clínica.

Langerhans’s cell histiocytosis was first described in the XIX century. Its origin and pathogenesis are based mainly on 3 events: clonal proliferation, a cytokines – mediated reactive process secondary to a viral infection. Diagnosis is based on histopathologic studies by the presence of Birbeck granules or CD1a positivity. Langerhans’ cell histiocytosis affects patients of any age; it may present as a single osteolitic lesion or as a systemic disease. Treatment is based on surgical resection, radiotherapy or chemotherapy. Outcome varies on the clinical presentation of the disease.

Case of Tuberculosis of the Skull, Presenting as Osteolytic Lesion: Case Report

Tuberculosis of the skull bone tuberculosis is a very rare disease. We report a case occurring in a 52 year-old male was referred to us because of headache and painful swelling in the right frontal area. Plain chest x-ray revealed fibrous, streaky, nodular, patchy opacities in the LUL together with pleural effusion. Plain skull x-ray and CT scan demonstrated osteolytic lesion in the right frontal bone; scanning showed hot uptake by the right frontal area, and this corresponding to skll findings revealed by plain film. After frontal craniotomy the involved bone flap and epidural abscess were removed. Pafthologically, typical granulomatous inflammation was seens; this was composed of caseation necrosis, epithelioid cells and surrounding lymphocytes and plasma cells.

A Case of Waldenstrom's Macroglobulinemia with Multiple Punched-out, Osteolytic Lesions / 대한혈액학회지

Waldenstr m's macroglobulinemia (WM) is a chronic lymphoproliferative disorder characterized by the association of serum monoclonal IgM and marrow infiltration by lymphoid population. A 69-year-old man was first seen for evaluation of anemia. He had complained of generalized weakness and exertional dyspnea. On physical examination there was generalized lymphadenopathy, palpable liver and spleen. He did not show signs and symptoms of hyperviscosity. Skull X-ray demonstrated multiple punched-out, osteolytic lesions. The total serum protein concentration was 10.6g/dL, with a fast gamma peak accounting for 48.3% of the total. The peak was identified as an IgM of the kappa chain type by immunoelectrophoresis. Bence-Jones protein (kappa type) was found in the urine. A Sia test gave positive result. The patient's serum contained a pyroglobulin. Serum viscosity was 5.3. The bone marrow aspirate and biopsy showed infiltration by atypical lymphocytes (about 90% of all nucleated cells), plasmacytic lymphocytes, and plasma cells. By flow cytometric analysis, these cells expressed CD19 (54%), CD20 (80.5%), and CD38 (92%), but did not express CD3, CD5, and CD10. Biopsy specimen from cervical lymph node demonstrated proliferation of abnormal lymphoid cells, composed of a similar population of cells seen in the bone marrow. He was treated with cyclophosphamide, vincristine, and prednisolone and now he improves slightly in clinical and laboratory aspects.

Extracranial vertebral arteriovenous fistula presenting as an osteolytic lesion of the axis: Case report

Arteriovenous fistulas of the extracranial vertebral artery are rare. The authors report a case of a spontaneous arteriovenous fistula of the extracranial vertebral artery presenting as an osteolytic lesion at the body of the axis. The patient presented with headache and posterior neck pain. The fistula was obliterated by an endovascular trapping.

Malignant Fibrous Histiocytoma Presenting as Osteolytic Lesion on the Left Temporal Bone: A Case Report

Malignant fibrous histiocytoma(MFH) is well known as a soft tissue tumor of the extremities and retroperitoneum, but MFH of the skull is very rare. We report a case of MFH arising from the temporal bone in a 27-year-old male. This tumor presented as an osteolytic lesion and soft tissue mass on the left temporal bone without obvious invasion of the underlying brain parenchyma. The patient underwent tumor and bone removal, follwed by radiation therapy. Hostologic examination disclosed pleomorphic spindle cells in a storiform pattern and tumor cells showed positive reaction for vimentin, lysozyme, alpha 1-antitrypsin and (1-antichymotrypsin in immunohistochemical stain.

A Case of Skull Bone Tuberculosis: Case Report

Tuberculosis originating in the skull bone is rare. We report a case of skull bone tuberculosis. A 72 year-old female was referred to us because of headache, swelling and tenderness in the left parietal area. She has been treated interstitial pulmonary fibrosis and pleural effusion at the department of internal medicine. Plain skull X-ray and CT scan revealed a 1.5x1.5 cm sized osteolytic lesion in the left parietal bone. Bone scan demonstrated a lesion of hot uptake in the left parietal region. After a left parietal craniotomy was performed, the involved bone flap and granulation tissue that attached to the dura mater were removed. Pathologic findings showed granulomatous inflammation with central caseous necrosis and peripheral collar of epithelioid cell interspersed with lymphocytes. A brief review of the literature was given.