Mariangela Novello

The fate of a macroporous hydroxyapatite cranioplasty four years after implantation: Macroscopical and microscopical findings in a case of recurrent atypical meningioma

Reconstruction of calvarial bone defects can be achieved using utologous bone or awide range of prostheticmaterials [1].Macroorous hydroxyapatite (MH) has histologically proven its potential steointegration in animal models [2–4]. Recent radiological and linical data in human subjects are in favour of good osteointegraion of MH cranioplasty [5,6]. However, the MH implant has weak echanic stability and is prone to fractures [7]. Furthermore, it is ot known how fast and to which extent the implant is ossified n vivo. We report a case with limited ossification of a MH prosthesis our years after implantation.

A cortical cystic epileptogenic lesion: tanycytic ependymoma

Ependymomas are neuroepithelial tumors usually found in the fourth ventricle, spinal cord and filum terminale, with higher incidence in children and young adults and two main histological types (classic cellular and myxopapillary). Tanycytic ependymoma is a rare variant with cystic appearance, occasionally found outside the ventricular system and usually presenting with seizures. Histopathological analysis is essential to make differential diagnosis with DNETs, astrocytomas and brain abscess, since treatment of choice is surgery followed by radiation for anaplastic variants, with favourable prognosis, low recurrence rate and seizures control.

Hormonal therapy for fertility and huge meningioma: a purely random association?

Sexual hormones have been related to the growth of meningiomas, also due to the almost constant expression of hormonal receptors by tumoral cells. A case of a woman with previous history of multiple treatment for infertility, harboring a huge meningioma is here described. The tumor was surgically resected and the immunohistochemical examination revealed a high expression of progesterone receptors on tumoral cells surface. A putative role of past progesterone administration in the growth of meningioma has been hypothesized. Particular caution should be paid whenever adopting sexual hormonal therapy, especially for fertility. A radiological examination (ideally MRI) could be advised before starting therapy, in order to rule out any intracranial meningioma.

Myeloid sarcoma with megakaryoblastic differentiation mimicking a sellar tumor

Myeloid sarcoma (MS) is a localized extra‐medullary tumor mass of immature myeloid cells, arising de novo or related to acute myeloid leukemia, of which it can be a forerunner, a coinciding or late event. Less commonly, MS represents an acute blastic transformation of myelodysplastic syndromes or myeloproliferative neoplasms. This rare condition commonly consists of a proliferation of more or less immature cells with a myeloid immunophenotype, very exceptional cases showing a megakaryoblastic or erythroid differentiation. The most common localization of MS is the skin, lymph node, soft tissues and bones, but CNS involvement is exceedingly rare, with no cases reported in the sellar region. We report a 54‐year‐old man, affected by myeloproliferative neoplasm, JAK2 V617F‐positive of 13 years duration, who acutely presented with a third cranial nerve palsy; neuroradiology documented a space‐occupying lesion at the level of the sellar, upper clival and right parasellar regions, that was sub‐totally removed with a trans‐sphenoidal approach. The histological examination documented a proliferation of large, blastic cells, frequently multinucleated; a diagnosis of MS with megakaryoblastic differentiation, arising in a background of chronic idiopathic myelofibrosis, was suggested by immunohistochemistry, owing to CD42b, CD45, CD61 and LAT (linker for activation of T cells) positivity. In addition, homozygous JAK2 V617F mutation was detected from the myeloid sarcoma specimen. A few weeks after surgery, an acute blastic leukemic transformation occurred and, despite chemotherapy, the patient died 2 months after surgery. To the best of our knowledge, this is the first MS case with megakaryoblastic differentiation arising within the CNS.

Intrapelvic sciatic notch schwannoma: microsurgical excision using the infragluteal approach

Benign neurogenic tumors at the sciatic notch that are purely intrapelvic have rarely been reported. Because of this tumors particular position, a transabdominal or combined transabdominal-gluteal approach is usually used to achieve total resection. However, the transabdominal approach carries a remarkable surgical risk because of the manipulation of intraperitoneal organs. Here, the authors describe a 59-year-old woman harboring a purely intrapelvic sciatic notch schwannoma extrinsic to the sciatic nerve, which was totally removed via the infragluteal approach preserving sciatic function. The postoperative course was uneventful. The infragluteal approach can be safely used for the effective resection of intrapelvic benign neurogenic tumors at the sciatic notch that are extrinsic to the sciatic nerve, avoiding the more invasive and risky transabdominal approach.

Unusual presentation of angiomyomatous hamartoma in an eight-month-old infant: case report and literature review

BackgroundEvaluation of palpable neck masses may be a diagnostic problem in pediatric patients, with differential diagnosis including congenital, inflammatory, tumoral and traumatic lesions. Ultrasonography is usually a satisfactory method to make a correct pre-operative evaluation of neck masses, although diagnosis is often challenging for the surgeon and the radiologist and sometimes only possible after a histopathological examination of the resected lesion.Case presentationWe report an 8-month-old patient with a cervical, anterior midline mass. Ultrasonographic images showed features suggesting a partly cystic lesion, with a preoperative suspect of thyroglossal duct cyst. Histological examination, performed after surgical removal of the mass, led to a diagnosis of lymph node angiomyomatous hamartoma (AH).ConclusionsAH, a rarely occurring benign lymph node lesion, has been reported in the neck lateral region only twice. This case, presenting as a palpable neck midline mass, is the first reported case occurring in infancy. Although rare, AH should be included in the differential diagnosis of head and neck masses.

Simultaneously occurring tumours within the same cerebello-pontine angle: refining literature definitions and proposal for classification

We report on an unusual case of a patient, not affected by neurofibromatosis, harbouring two radiologically spatially contiguous tumours within the same cerebello-pontine angle. Pathological findings were consistent with the diagnosis of two spatially distinct primary tumours, namely a meningioma and a schwannoma. We proposed a classification of tumours occurring at the same location consistent with the different spatial arrangement and histological nature of these conditions. The correct classification of these nosological entities will allow further more accurate evaluations of these cases in order to clarify the pathogenesis, prognosis and best treatment of each one.

Huge cranio-cerebral rhabdomyosarcoma in HIV-positive patient

Rhabdomyosarcomas (RMS) are highly malignant tumors originating from mesenchymal cells. Typically, they affect children, and prognosis is quite poor. In adults, RMS are rare, and cranial locations are exceptional. Here, we report a case of huge cranial RMS in an immunosuppressed adult patient. To our knowledge, this is the first reported case of primitive RMS of the cranial vault in a HIV-infected patient.

Neuroendocrine Tumors of the Submandibular Gland Literature Review and Report of a Case

Primary salivary neuroendocrine neoplasms are extremely rare, with only 1 case of carcinoid tumor and 7 cases of primary large-cell neuroendocrine carcinomas having been reported. Salivary small-cell neuroendocrine carcinomas are more frequent, accounting for less than 2% of all salivary malignancies. In addition to a literature review on the subject, the authors describe a rare case of malignant intermediate-grade neuroendocrine tumor of the submandibular gland.

A rare case of life-threatening giant plexiform schwannoma

A 28-year-old woman with idiopathic scoliosis presented with a cervicalmass and progressive gait disturbance over the course of 2 years. Examination demonstrated mild spastic paraplegia, hypoesthesia with upper level at T5 and severe respiratory failure requiring assisted ventilation. Magnetic resonance imaging showed multiple thoracic masses compressing left lung, trachea, and spinal cord (Figure). Surgical resection was performed. Through C5–T1 laminectomy, the intradural part of the neoplasia was completely removed, and the extradural part was enucleated as much as possible. After surgery, the respiratory function improved. Histologic pattern and S100 protein diffuse reactivity were consistent with plexiform schwannoma, a rare benign cutaneous variation of schwannoma [1]. Although deep-seated plexiform schwannomas involving spinal cord are occasionally described [2], this case is undoubtedly impressive because of the lifethreatening tumor size (Figure).