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.

Female gender and exogenous progesterone exposition as risk factors for spheno-orbital meningiomas.

OBJECTIVE: The great heterogeneity of meningiomas is challenging and we need to distinguish relevant subgroups. Spheno-orbital osteomeningiomas (SOOM) constitute a clinically specific entity, with slow-growing benign osteo-meningiomatous tumors, which recur after surgery in one fourth of cases. Neurosurgical daily practice, supported by the literature, shows that the vast majority of patients with SOOM are women, and we explored whether their epidemiological and hormonal profiles suggest a progesterone influence. METHODS: We retrospectively documented all radiologically and histologically confirmed cases of SOOM operated in 2005-2019 in our institution. We completed the clinical and hormone history by systematic telephone interviews. RESULTS: In the literature, SOOM occur significantly more often in women than other meningiomas (749/847, 86.4% versus 73.8%, p = 0.002). Among 175 cases, we included 124 patients, 93.5% were women, younger than men (51 ± 5 versus 63 ± 8, p = 0.02). Women' meningiomas showed more progesterone receptors (96.4% versus 50%, p < 0.001). Exogenous hormonal intake, reliable in 82 cases, concerned 83.3% (64/78) of women, with frequent progesterone intake: 13 oestroprogestogenic treatment only, with old-generation progesterone analogs, 41 progesterone analogs (cyproterone acetate, nomegestrol acetate, chlormadinone, promegestone, etonogestrel, levonogestrel), 7 substitutive hormonal therapy for menopause, 3 others. Duration of treatment was 2-40 years, median 10 years. CONCLUSIONS: SOOM develop preferentially in women in their fifties, who often received progesterone analogs, and show progesterone receptors. Progesterone analogs are incriminated in skull base meningiomas, and this is the first report on the prevalence of exogenous hormone therapy specifically in SOOM. Whether SOOM reduce after treatment discontinuation, in particular the osteoma part, needs to be explored. Anti-progesterone treatments may represent an avenue for future research in soom.

Treatment-related calvarial lesions in pediatric brain tumor survivors.

BACKGROUND: Despite improved survival, many pediatric brain tumor survivors receiving radiation therapy (RT) experience late effects. PROCEDURE: To study calvarial lesions in this population, we retrospectively reviewed records of patients undergoing neurosurgical evaluation for calvarial bone lesions detected in posttreatment follow-up imaging at St. Jude Children's Research Hospital. Primary tumor diagnosis, treatment, imaging, surgical intervention, and histopathology from patients with radiographic evidence of lesions followed for ≥2 years post-RT were studied. RESULTS: For 17 patients with 18 index lesions, median time to lesion manifestation was 2.34 years. Medulloblastoma patients developed lesions at a shorter interval from RT than ependymoma patients (P = .05). Twelve of 14 lesions requiring surgery were benign fibro-osseous or sclerotic. Two malignant lesions distinct from the primary tumor had genetic predisposition to malignancy. CONCLUSION: Most calvarial lesions arising post-RT are benign and fibro-osseous. Serial imaging is recommended, and high index of suspicion for malignant lesions is warranted for patients genetically predisposed to cancer.

Radiation-induced osteosarcoma of the skull mimicking cutaneous tumor after treatment for frontal glioma.

Radiation-induced sarcomas are recognized complications of radiation therapy and are associated with poor prognosis. Radiation-induced osteosarcoma is one of the rare types of radiation-induced sarcomas, with the risk of radiation-induced osteosarcomas being only 0.01%-0.03% among all patients treated with radiotherapy. There have been only four reported cases of radiation-induced osteosarcomas after radiotherapy for gliomas. Here, we report a unique case of radiation-induced osteosarcomas arising on the skull and extending to the skin, with a short latent period. We also review the clinical features of the previously reported cases.

Head trauma complicated with primary cranial vault lymphoma: A case report.

RATIONALE: Primary cranial vault lymphoma (PCVL) is an extremely rare extranodal lymphoma in the skull. This case study investigates the clinical features, so as to improve the understanding of the diagnosis and therapy. PATIENT CONCERNS: A 31-year-old male presented painful scalp mass at the site of 1-month-old head trauma. DIAGNOSIS: The final diagnosis was plasma cell lymphoma, which is a rare subtype of diffuse large B-cell lymphoma based on biopsy and immunohistochemistry findings. INTERVENTIONS: The patient received total tumor resection in combination with chemotherapy OUTCOMES:: The patient survived without signs of systemic dissemination for 12 months after surgery at the time of last follow-up. LESSONS: Trauma may be one of the factors that induce PCVL. The final diagnosis of PCVL depends on pathology and immunohistochemistry findings. A combined treatment of surgery, chemotherapy, and radiotherapy can achieve favorable outcomes.

Langerhans Cell Histiocytosis of the Skull in a Burr Hole Site Covered with Hydroxyapatite Material.

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare dendritic histiocytic disorder that affects the bones, especially the skull. Langerhans cell histiocytosis (LCH) developing in a burr hole site for chronic subdural hematoma is extremely rare. CASE DESCRIPTION: A 53-year-old man underwent a burr hole irrigation for chronic subdural hematoma, and the burr hole was covered with a burr hole button made of hydroxyapatite. Seven months after the first surgery, the connective tissue rapidly proliferated around the burr hole button, and the pathologic diagnosis was LCH. LCH recurred at 13 and 19 months after the first operation, with curettage performed each time. At 3 months after the final operation, no recurrence was identified on magnetic resonance imaging. CONCLUSIONS: If there is rapid proliferation of connective tissue at an operative site where artificial material has been used, LCH should be considered.

Bone and dental abnormalities as first signs of familial Gardner's syndrome in a Chinese family: a literature review and a case report.

Gardner's syndrome (GS) is an autosomal dominant disease characterized by the presence of familial adenomatous polyposis (FAP) as well as extraintestinal manifestations such as osteomas, dental anomalies, epidermoid cysts and ocular abnormalities. These intestinal polyps carry a 100% risk of malignant change, so early diagnosis is crucial. As craniofacial osteomas and dental anomalies of GS usually precede gastrointestinal symptoms, otolaryngologists, oral surgeons and dentists play an important role in the diagnosis of GS. GS is extensively reported in literature in the Caucasian race but not in the Mongoloid race. We report a case of a 22-year-old patient with a manifestation of three features of GS - multiple osteomas, soft tissue tumors and dental anomalies in the craniofacial region, with no intestinal polyps at the time of reporting. A family pedigree with our patient as the proband was constructed and revealed 3 consecutive generations in his lineage with GS.

Chondroblastoma-like tumor of the skull in a patient with cardio-facio-cutaneous syndrome.

Cardio-facio-cutaneous syndrome (CFCS) is a rare genetic disorder characterized by craniofacial deformities and heterogeneous cardiac and cutaneous manifestations. The condition is caused by de novo activating mutations in one of four genes encoding proteins involved in the RAS-MAPK signaling pathway; specifically BRAF, MEK1, MEK2, or KRAS. Variable malignancies have been reported in patients with CFCS. Herein we report a chondroblastoma-like lesion of the skull in a 20-year-old man with a clinical diagnosis of CFCS and a long-standing history of medically intractable epilepsy. Patients with CFCS have previously been noted to have poorly-defined giant cell lesions and this may be one such example.

A case of radiation-induced osteosarcoma of the skull presenting as a cutaneous epidermotropic tumor with a short latent period.

Radiation-induced sarcoma (RIS) is an unusual but well documented tumor. The frequency of RIS of the head and neck region has been reported as 0.143%. In the literature the median interval between irradiation and development of sarcoma is 11 years. Cases of RIS with a short latent period, that is, less than 4 years are rare. We report a case of a 34-year-old female who developed an osteosarcoma of the scalp, over a previous craniotomy scar, 3 years after excision of a frontal anaplastic oligodendroglioma which had been followed by a course of 6 weeks radiotherapy (58 Gy) and 6 cycles of temozolomide. The histological features were those of a high-grade osteosarcoma with epidermotropism of tumor cells. Lymph nodes were partially replaced by high-grade metastatic osteosarcoma, with extra-nodal lymphatic tumor thrombi. To our knowledge the only other case report of post-radiation osteosarcoma with a short latency period was a case of osteosarcoma in the craniofacial bone 3 years after radiotherapy for maxillary squamous cell carcinoma. The histological finding of prominent replacement of the epidermis by osteosarcoma has not been reported before.

More than just tissue diagnosis in a patient with maxillofacial bony lesions and hypercalcemia.

Brown tumors are a definitive feature of hyperparathyroidism. They are well-demarcated osteolytic lesions commonly in the appendicular skeleton. Primary hyperparathyroidism is typically suggested by hypercalcemia and hypophosphatemia on routine labs. Much more rarely do these cases present with a craniofacial mass. Here we investigate a unique presentation of terminal stage primary hyperparathyroidism with a growing maxillary mass emphasizing the importance of a broad differential diagnosis and key diagnostic studies. Hyperparathyroidism can present in very unique ways. As otolaryngologists in the frontline, we must think beyond just tissue diagnoses so that appropriate and expedited care may be implemented. Laryngoscope, 127:1318-1321, 2017.

Osteolytic clear cell meningioma of the petrous bone occurring 36 years after posterior cranial fossa irradiation: Case report.

OBJECTIVE AND IMPORTANCE: While bone invasion and hyperostosis are frequent phenomena in meningiomas, primary intraosseous meningiomas are rare and their occurrence in the skull base is an extraordinary exception. Moreover, radiation-induced meningiomas represent a unique clinical dilemma given the fact that patients with these tumors had often received a prior full course of radiotherapy. CLINICAL PRESENTATION: A 42-year-old man presented with a 3-month history of progressively worsening facial asymmetry. His medical history was consistent for a posterior cranial fossa irradiation at the age of 6 years for a non-confirmed brain stem tumor. On admission his Karnofsky performance status was graded as 50% and his neurological examination showed a complete right facial nerve paralysis and hearing impairment. Computed tomography and magnetic resonance imaging demonstrated an osteolytic tumor invading the whole right petrous bone without intracranial involvement. INTERVENTION: As the tumor reached the external auditory canal, a tissue sample was obtained locally. Pathological examination of the lesion identified a grade II clear cell meningioma and the patient was consequently addressed for an intensity modulated radiation therapy. His condition remained unchanged till the most recent follow-up examination, 8 months later. CONCLUSIONS: To the best of our knowledge, a radiation induced osteolytic clear cell meningioma of the petrous bone has not been previously reported. As little literature exists regarding the use of adjuvant therapies for these tumors, intensity modulated radiation therapy remains an attractive treatment option in case of pervious irradiation and general status alteration.

Diagnosis of a growing radiation-induced skull lesion in a patient: an unusual scar.

New lesions arising from within an area of previous irradiation often present a diagnostic dilemma, with new malignancy or metastasis of particular concern. The authors report a case of reactive fibroblast proliferation emerging from a previous radiation field and presenting as a growing lesion of the frontal and parietal skull. Following complete gross resection of the skull lesion and histopathological analysis, it was discovered that this lesion consisted of dense fibroblast proliferation with areas of osteonecrosis. This unusual reactive phenomenon offers a novel differential diagnosis for a new contrast-enhancing lesion in a region of previous radiation.

Gardner's syndrome, a rare combination in surgical practice.

A 45-year-old man presented to the emergency ward with features of intestinal obstruction of 2 days duration. On admission, there was abdominal distension and multiple sessile polyps found on digital rectal examination. In addition, a soft tissue swelling near the elbow and a bony swelling over scalp were noted. Abdominal radiography revealed gaseous distension of the small and large bowel, and ultrasound revealed diffuse, gas-filled bowel with sluggish peristalsis. The obstruction failed to resolve with conservative measures and at emergency laparotomy an irregular hard recto-sigmoid junction mass was identified. A defunctioning transverse loop colostomy was undertaken and the abdomen closed. During recovery, a colonoscopy was performed and a malignant appearing lesion was identified 15 cm proximal to the anal verge. Further per-stomal colonoscopy revealed multiple sessile polyps from the ileo-caecal valve to the descending colon. The cutaneous and abdominal findings were consistent with a rare acute presentation of Gardner's syndrome.

Radiation-induced dedifferentiated chondrosarcoma with orbital invasion.

PURPOSE: To report 2 interventional cases of dedifferentiated chondrosarcoma with orbital involvement after radiotherapy performed in childhood and to review the literature on chondrosarcoma in the orbit following radiation treatment. METHODS: Retrospective analysis of medical records of 2 patients with chondrosarcoma of the orbits with review of the literature. RESULTS: The first patient developed chondrosarcoma of the orbital and maxillary sinus 36 years after external beam radiation therapy to the OS to treat retinoblastoma. The second patient developed a large orbital chondrosarcoma 35 years after external beam radiation therapy in the treatment of craniofacial fibrous dysplasia. CONCLUSIONS: These cases highlight the risk of secondary chondrosarcoma in patients following radiotherapy and the importance of lifetime monitoring.

Posttraumatic cavernous hemangioma of the skull.

A 59-year-old man presented with a slowly enlarging mass in the lateral orbital rim of the left eye for 1 year. He also reported a history of crania defect in the left front bone (6 years earlier) which was confirmed on the former computed tomographic (CT) scan. On examination, the solid mass lesion located in the left frontal extends to the lateral orbital rim of the left eye. CT scan showed a mass with poorly defined margins, which invaded the neighboring tissue and nearly damaged the bone, and located on the exact site of the preceding crania defect. Complete resection was performed, and the clinical diagnosis of cavernous hemangioma was confirmed on histopathologic examination. Skull cavernous hemangiomas are rare tumors for which the origin is not yet clear. We report a case in which the intraosseous hemangiomas developed at the same site of the preceding crania defect.

Osteolipoma in the glabella: pathogenesis associated with mesenchymal lipoma-derived stem cells.

Lipoma is a benign tumor that often arises in the craniomaxillofacial region. Osteolipoma containing bone tissue is very rare and the developmental mechanism is unclear. Mesenchymal stem cells in adipose tissue that have potential to differentiate into fat, bone, cartilage, and vascular components may be involved in the development of osteolipoma, in which adipose and bone tissues coexist. We encountered a patient with osteolipoma that arose in the glabella. We describe the case and the results of an investigation of the presence in lipomas of mesenchymal stem cells with differentiation potential similar to that of normal adipose cells. The patient was a 66-year-old woman. Histopathologically, bone tissue surrounded by fibrous connective tissue was present in the nodular adipose tissue and was diagnosed as osteolipoma. Mesenchymal stem cells were collected by collagenase treatment of lipoma tissue, and their potential to differentiate into fat, bone, and cartilage was shown. On the basis of this study, we suggest that lipoma-derived mesenchymal stem cells are the basis of the pathogenesis of osteolipoma. The conditions that induce differentiation of mesenchymal stem cells into bone remain to be investigated.