Craniomaxillofacial Fibro-osseous Lesions in Children.

Craniofacial fibro-osseous lesions represent a diverse spectrum of pathologic conditions where fibrous tissue replaces healthy bone, resulting in the formation of irregular, woven bone. They are more commonly diagnosed in young people, with treatment strategies dependent on clinical behavior and skeletal maturity. This article discusses the examples of craniofacial fibro-osseous lesions, based on the latest classifications, along with their diagnostic criteria and management.

Melanotic neuroectodermal tumor of infancy arising in the skull: a case report.

Melanotic neuroectodermal tumor of infancy is a rare and usually benign neoplasm occurring in children of young age. This pigmented tumor typically presents in the head and neck region, but other locations may be involved. We report in this article a rare case of a 3-month-old girl presenting with a slowly growing mass localized in the anterior fontanelle. The patient's magnetic resonance imaging (MRI) showed a mass extending both extracranial and intracranial, and compressing the adjacent structures. The patient underwent subtotal resection of the mass and a histological study confirmed the diagnosis of melanotic neuroectodermal tumor of infancy. The patient presented later on with a recurrence. An early diagnosis and surgical management for these tumors remain the only guarantees to limit the progression and prevent their recurrence and metastasis.

[Primary intraosseous cavernous hemangioma of the cranium: a case report]. / Pervichnaya vnutrikostnaya kavernoznaya gemangioma kostei cherepa: obzor literatury i opisanie klinicheskogo nablyudeniya khirurgicheskogo lecheniya.

Primary intraosseous cavernous hemangioma (PICH) is a rare benign vascular tumor. This neoplasm is common in the spine and less common in skull. Toynbee J. first described this tumor in 1845. PICH of the cranium does not always have typical X-ray features and should be always differentiated with other more common skull lesions. Surgical resection is preferable since total resection is followed by favorable prognosis. We present a 65-year-old patient with asymptomatic tumor of the right parietal bone. CT revealed osteolytic lesion that required total resection and skull repair. Histopathological analysis revealed intraosseous cavernous hemangioma.

A Clinical, Radiological and Histopathological Review of 74 Ossifying Fibromas.

BACKGROUND: Ossifying fibroma (OF) is a fibro-osseous lesion of the jaws and craniofacial bones. Accurate diagnosis can be challenging due to significant overlap of clinicopathological features. This study aimed to evaluate the clinical, radiological and histological features that can aid in diagnosis and identify characteristics that allow categorisation into the three subtypes: juvenile trabecular, psammomatoid and cemento-ossifying OF. METHODS: A total of 74 cases of OF were systematically reviewed for their principle features. Of these, 46 cases were evaluated for their radiographic features including size, location and relationship to the teeth. Histological assessment and stereological point counting were performed in 69 cases to assess the pattern, type and proportion of calcification, the nature of the stroma, the border of the lesion and the presence of secondary changes. Fisher's exact test and Chi-squared tests were used to determine associations between clinicopathological parameters and maxillary, mandibular, odontogenic, non-odontogenic and psammomatoid or trabecular lesions. RESULTS: OF showed a female predilection (F: M; 2:1) and a slight bimodal age distribution with peaks in the second (23%) and fourth decades (27%) (Mean age: 32.4 years). 83% of cases presented as an intra-oral swelling, with the mandible being the most common site (73%). Histologically, a range of morphological patterns were seen, with 50% of cases showing mixed trabecular and psammomatoid features. However, there were no significant differences between the variants of OF in terms of age, gender or histological features. CONCLUSION: Histological features of OF cannot be used to differentiate between the subtypes.

Multiple myeloma complicated by skull plasmacytoma discovered after head injury.

Plasmacytoma is a malignant tumor originating from the plasma cells of the bone marrow. Those discovered after a head injury is rare. We report a case of a 48-year-old female who complained of scalp mass without other symptoms after head injury. Meningioma was considered preoperatively based on imaging findings, and surgical resection was performed. Postoperatively, multiple myeloma complicated by skull plasmacytoma was diagnosed by histopathology and systematic examinations in succession. When evaluating a head mass that appeared after a head injury, plasmacytoma should be considered at times. Osteolytic changes and biconvex form on imaging are beneficial to differentiation.

Primary yolk sac tumor of pterygopalatine fossa with loss of vision: A case report.

INTRODUCTION: Primary yolk sac tumor (YST) is an infrequently-diagnosed malignant extragonadal germ cell tumors. It is likely to recur locally and may present with widespread metastases once diagnosed. Primary YST of the head is uncommon but can cause severe complications, such as loss of vision once the tumor mass invades the optic nerve. PATIENT CONCERNS: A 20-month-old boy presented to the general clinic of the local children's hospital with a complaint of swelling of left face for 1 year and proptosis of the left eye for over 2 weeks as stated by his parents. Initially, he did have some vision, as he could walk by himself, but a special ophthalmologic examination was not performed. DIAGNOSES: Cranial computed tomography and magnetic resonance imaging revealed a large tumor accompanied by peripheral bone destruction in the left pterygopalatine fossa that extended to sphenoid, ethmoid, left maxillary sinuses, left nasoethmoid, and left orbit. The optic nerve was invaded on both sides. Chest and abdominal imaging were normal. A primary diagnosis of Langerhans cell hyperplasia was made. However, blood tests on the second day of hospitalization revealed significantly elevated serum alpha-fetoprotein levels. On the third day, the boy lost his eyesight, with loss of pupillary and no light sensation during flashlight stimulation on both sides. INTERVENTIONS: Nasal endoscopy was performed on the fourth day, the vast majority of soft tissue mass was resected for biopsy. Histopathological examination revealed features of endodermal sinus tumor. A final diagnosis of primary YST of pterygopalatine fossa was made. Because the mass could not be resected completely, he received combined chemotherapy with bleomycin, etoposide, and carboplatin for 6 cycles over six months. OUTCOMES: The patient recovered with significant tumor shrinkage and without secondary metastasis after 18 months but left permanently blind. CONCLUSION: The worst complication of loss of vision after Primary YST of pterygopalatine fossa alerts us that close physical examination during the initial investigation should be performed, which is especially important in young children who cannot express complaints well. Early detection and treatment with surgical resection and chemotherapy may contribute to satisfactory outcomes and avoidance of visual impairment.

A unique case of multiple calvarial hemangiomas with one large symplastic hemangioma.

BACKGROUND: Symplastic hemangioma is a benign superficial abnormal buildup of blood vessels, with morphological features which can mimic a pseudo malignancy. A few cases have been reported in the literature. We report here, a unique case of calvarial symplastic hemangioma, which is the first case in the calvarial region. CASE PRESENTATION: A 29-year-old male patient, with a left occipital calvarial mass since childhood, that gradually increased in size with age, was associated with recurrent epileptic fits controlled by Levetiracetam (Keppra), with no history of trauma. He presented to the emergency room with a recent headache, vomiting, frequent epileptic fits and a decrease in the level of consciousness 1 day prior to admission. A CT scan showed three diploic, expansile, variable sized lytic lesions with a sunburst appearance; two that were biparietal, and one that was left occipital, which were all suggestive of calvarial hemangiomas. However, the large intracranial soft tissue content, within the hemorrhage of the occipital lesion was concerning. The patient had refused surgery over the years; however, after the last severe presentation, he finally agreed to treatment. The two adjacent, left parietal and occipital lesions were treated satisfactorily using preoperative embolization, surgical resection, and cranioplasty. Histopathology revealed cavernous hemangiomas, in addition to symplastic hemangioma (pseudo malignancy features) on top at the occipital lesion. The right parietal lesion was not within the surgical field; therefore, it was left untouched for follow-up. CONCLUSIONS: Histopathology and radiology examinations confirmed the diagnosis as symplastic hemangioma, on top of a pre-existing cavernous hemangioma. To the best of our knowledge, this is the first case of a calvarial symplastic hemangioma, which we report here.

Langerhans Cell Histiocytosis of the Skull.

This is a case of a pediatric male who presented with a postauricular mass and pain. Computed tomography and magnetic resonance imaging suggested a diagnosis of Langerhans cell histiocytosis. Characteristic imaging findings and initial workup for Langerhans cell histiocytosis are described.

Sphenoid Wing Meningioma Presenting as Sudden Sensorineural Hearing Loss: A Case Report and Literature Review.

Sphenoid wing meningiomas are tumors that typically present with vision deterioration and neurological changes due to their proximity to the sella, cavernous sinus, and other vital structures. Some unusual symptoms have also been described in the literature, such as cognitive dysfunction, parkinsonism, and intracerebral hemorrhage. In this report, we detail another unusual case of sphenoid wing meningioma in a 63-year-old female who presented with left sudden sensorineural hearing loss. A brief review of the literature is also included.

Primary Advanced Squamous Cell Carcinoma of the Temporal Bone: A Single-Center Clinical Study.

OBJECTIVES/HYPOTHESIS: The extreme rarity of temporal bone squamous cell carcinoma (TB-SCC) has delayed the accumulation of high-quality clinical evidence. For the purposes of retrospective meta-analysis in the future, a large dataset with information from various institutions would be ideal. Our objective here was to retrospectively review cases of TB-SCC encountered at a single tertiary referral center and explore survival outcomes and prognostic factors. STUDY DESIGN: Retrospective chart review. METHODS: The medical records of all TB-SCC cases were retrospectively reviewed. The resulting dataset contained 71 cases of primary cancer eligible for initial definitive (curative) treatment. RESULTS: T4 status was associated with lower disease-specific 5-year survival than T1 to T3 staging (T1: 100%, T2: 92%, T3: 86%, T4: 51%). Survival was significantly higher in operable than in inoperable cases, even when restricted to advanced (T3/T4) cancers. The tumor extension to the middle ear cavity was observed in 13/17 of T3 cases, but it was not associated with poor survival. In addition, among operable cases, negative surgical margins were associated with significantly higher survival than positive margins. CONCLUSIONS: Definitive treatments can offer disease-specific 5-year survival of over 85% in T1 to T3 cases of TB-SCC. The tumor extension to the middle ear cavity is not associated with poor survival. T4 status, inoperability, nodal invasion, and positive surgical margin are identified as a predictor of poor prognosis. Still, the matter of how to deal with unresectable tumors remains an outstanding issue in the treatment of TB-SCC. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E583-E589, 2021.

Benign purely intraosseous meningioma of the skull: Diagnosis and surgical outcomes.

Primary intraosseous meningioma (PIM) is a rare subtype of extradural meningiomas that originates within bone. We aimed to characterize the clinical, radiographic, and pathologic features of PIM and the resulting outcomes following resection. Herein we examined a retrospective case series of all patients with a pathologically confirmed WHO grade I PIM that were managed at one of three tertiary care centers. Patients with tumors that demonstrated extraosseous extension or involvement of the dura mater were excluded. The main outcomes included surgical safety and duration of local tumor control. Nine patients were identified with benign PIMs, presenting with headaches or painless enlarging subcutaneous masses if involving the calvarium or with neurologic deficits if involving the skull base, or otherwise incidentally identified. Surgery was pursued for symptomatic relief and/or tissue diagnosis. Lesions were evaluated by radiographic imaging - including sensitive detection by plain X-ray films - and definitive diagnosis ascertained by histopathological examination. Maximal resection of both calvarial and skull base lesions was safely tolerated. PIM represents a rare benign skull lesion, whose identification depends on the integration of radiographic findings with intraoperative findings and histopathological confirmation; it should be considered in the differential for slow-growing expansile intraosseous lesions of the skull.

[Osteoblastoma of the parietal bone of the cranial vault: about a case]. / Ostéoblastome de l'os pariétal de la voûte du crâne: à propos d'un cas.

Osteoblastoma is an uncommon primary bone tumor. Its occurrence in the cranial vault is extremely rare. We here report our first case of right parietal bone osteoblastoma in a 46-year old woman with a history of benign cranial traumas. She reported progressive painful, non-inflammatory right parietal bone swelling. Craniocerebral CT scan showed hyperdense bone lesion with sparing of the internal table of the right parietal bone. The patient first underwent biopsy, then complete resection of the bone lesion with methyl-methacrylic cement cranioplasty. The postoperative course was uneventful. Anatomopathological examination showed osteoblastoma with no sign of malignancy. This study and literature review highlight the clinical manifestation, the radiological and anatomopathological features as well as the management of osteoblastoma of the parietal bone of the cranial vault.

Calvarial metastasis from adenocarcinoma of lung: An uncommon initial presentation diagnosed by cytology.

Bone metastasis from lung primary is not uncommon and about one-third of bone metastases originate from lung. However, skull bone metastasis is uncommon from lung carcinoma. Metastasis to skull bone and scalp as an initial presentation of lung carcinoma is a very rare phenomenon. We have diagnosed a case of calvarial metastasis with scalp swelling as an initial presentation of adenocarcinoma of lung by fine-needle aspiration cytology in an aged female. Radiologically, it was suggested as tuberculous lesion but cytology gave the correct diagnosis. Here, we present a rare case of calvarial metastasis as a presentation of adenocarcinoma of lung in an elderly female.

[Burkitt's lymphoma of the skull calotte: a case report]. / Linfoma de Burkitt de la calota craneana: a propósito de un caso.

Introduction: Burkitt's lymphoma is a non- Hodgkin B-cell lymphoma whose cranial location is extremely rare. Clinical case: The case of a 35-year-old Peruvian man with a progressive parietal left cranial tumor is described. A biopsy with immunohistochemistry was performed for the diagnostic confirmation of Burkitt's lymphoma. First-line outpatient treatment with CODOX-M/IVAC was initiated, however, he stopped attending his treatment for 2 months. He returns, he is shown progression of the disease and is treated with rituximab, ifosfamide carboplatin and etoposide. Does not respond satisfactorily and dies. Conclusions: Burkitt's lymphoma is a very aggressive tumor that, if not treated in a timely manner, has high mortality, as happened with the patient in the case.

Introducción: El linfoma de Burkitt es un linfoma no Hodgkin de células B cuya ubicación craneal es extremadamente rara. Caso clínico: Se describe el caso de un hombre peruano de 35 años con una tumoración craneal parietal izquierda de crecimiento progresivo. Para la confirmación diagnóstica de linfoma de Burkitt se realizó una biopsia con inmunohistoquímica. Se inició tratamiento ambulatorio de primera línea con CODOX-M/IVAC, sin embargo, deja de acudir a su tratamiento por 2 meses. Retorna, se le evidencia progresión de la enfermedad y se le trata con rituximab, ifosfamida carboplatino y etopósido. No responde de forma satisfactoria y fallece. Conclusiones: El Linfoma de Burkitt es un tumor muy agresivo que si no es tratado oportunamente presenta alta mortalidad, tal como ocurrió con el paciente del caso.

Multiple Occipital Bone Lytic Lesions Containing Ectopic Cerebellar Parenchyma Mimicking Neoplasia.

BACKGROUND: Nonlethal neural tube defects are developmental malformations with complex pathogenesis usually manifested at birth or in childhood. CASE DESCRIPTION: We report the case of a 61-year-old woman without significant previous clinical history presenting for neck pain and stiffness. An extensive workup detected multiple lytic lesions within the occipital bone and cervical vertebrae, suspicious for multiple myeloma or metastatic disease. Surgical resection of the occipital bone lesions revealed ectopic cerebellar tissue, some containing folia with mature cortical lamination, and no evidence of malignancy. CONCLUSIONS: To our knowledge, this study describes the oldest individual presenting with ectopic cerebellar tissue and the only instance in which oncologic workup for malignancy was carried out prior to resection. It also proposes surgical resection as a diagnostic and curative approach for this complex basicranium and neural developmental defect, and discusses retinoic acid toxicity as a possible cause of its occurrence.

Congenital dermoid inclusion cyst over the anterior fontanel in Chinese children.

Congenital dermoid inclusion cyst (CDIC) over the anterior fontanel is a rare and benign tumor. This study reports nine Chinese cases (three females and six males) with CDIC over the anterior fontanel. The clinical manifestations and imaging were analyzed retrospectively. Surgical resection was undertaken in all cases. The diagnosis of CDIC over the anterior fontanel was confirmed by histological examination. The cysts were all noticed soon after birth and enlarged gradually. They were soft, nontender with a sessile base without inflammatory signs and breaking. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed soft tissue mass over the anterior fontanel without intracranial extensions. The histopathological examination displayed stratified squamous epithelium with skin appendages. There were no complications or recurrence after operation during a follow-up for one year. CDIC over the anterior fontanel is a benign tumor. Imaging is recommended preoperatively to aid differential diagnosis. The main management is total excision with good prognosis.

Glomus Tumor of Sella Turcica With Synaptophysin Expression Mimicking Pituitary Adenoma.

Glomus tumor can rarely arise in the central nervous system as a sella turcica mass. In this article, we report a case of sellar glomus tumor in a female patient who presented at the age of 8 years with visual impairment. The tumor recurred at 4 years and 26 years after initial excision and gamma knife therapy. Histologic examination showed a monotonous population of oval cells accompanied by delicate blood vessels, features mimicking pituitary adenoma. The tumor showed histologic progression at the second recurrence. Synaptophysin staining was positive, but chromogranin and CD56 were negative. The tumor cells were negative for epithelial markers but expressed actin and SMA. Awareness of the rare occurrence of glomus tumor at this region, careful analysis of morphology, and appropriate immunohistochemical workup are essential to solve this diagnostic challenge. The clinicopathologic features of all previously reported cases are reviewed.

Intraosseous cavernous malformations of the skull: clinical characteristics and long-term surgical outcomes.

Intraosseous cavernous malformations (ICMs) of the skull are relatively rare, benign, and slow-growing tumors. Knowledge of these lesions is poor. The goals of this study were to describe the clinical manifestations, radiological features, and long-term surgical outcomes of this disease. We performed a retrospective analysis of a series of 16 cranial ICM patients who underwent surgical treatment in our hospital between 2003 and 2016. The incidence of cranial ICM was 1.15% among the entire series of intracranial and intraspinal CMs. Our cohort included 6 male and 10 female patients; their mean age at operation was 38.7 years (range, 1.9 to 63 years). Slowly growing swelling was the commonest clinical manifestation of this disease. Complete lesion resection was achieved in all but one patient, and cranioplasty was performed using titanium mesh in 11 cases. Postoperative complication was found in one patient who developed diplopia, and this symptom resolved spontaneously before discharge. The mean follow-up period after operation was 76.2 months (range, 19 to 119 months). Only the patient with petroclival ICM had some occasional headaches (mRS = 1); others were all in stable neurological status (mRS = 0). No lesion recurrence was found during the follow-up period. Although cranial ICM is rare, it should always be considered as a differential diagnosis in the case of firm, gradually enlarging skull lesions. Surgical resection should be the treatment of choice for the symptomatic patients and their long-term outcomes were excellent after gross total removal.