Primary cutaneous interdigitating dendritic cell sarcoma (IDCS): Report of a new case and literature review.

Interdigitating dendritic cell sarcoma is a very rare entity in the spectrum of histiocytic and dendritic cell neoplasms that mostly occurs in lymph nodes, generally presenting as solitary lymphadenopathy, but may affect every organ. Among extra nodal sites, cutaneous interdigitating dendritic cell sarcoma is exceedingly rare; to date, only 9 cases have been described in English literature. The mean age at diagnosis was 60 years, with a male-female ratio of 1,5 to 1; clinically, two different modalities of skin presentation have been reported: solitary, represented by a single red-brownish nodular lesion, or diffuse, characterized by multiple nodular lesions in one or more body districts. The extreme rarity of this sarcoma and its morphological similarity to other poorly differentiated tumors may lead to a delay in diagnosis; in particular, cutaneous localization may be difficult to differentiate from follicular dendritic cell sarcoma, Langerhans cell sarcoma, poorly differentiated squamous cell carcinoma and more generally sarcomatoid carcinoma, atypical fibroxanthoma, malignant melanoma and several sarcomas. Immunohistochemistry plays an important role in identifying this rare entity and formulating a correct histological diagnosis, fundamental requirement for choosing the best therapeutic approach. We report herein a further case of an 81-year-old Caucasian woman who presented to the Dermatology Department to remove an asymptomatic skin papule in the left temporal region, clinically diagnosed as dermatofibroma. The overall pathological and immunohistochemical features supported the diagnosis of a malignant dendritic cell tumor, consistent of interdigitating dendritic cell sarcoma.

Primordial odontogenic tumour: A systematic review of the common but also unusual features of this novel entity.

BACKGROUND: Primordial odontogenic tumour (POT) is a novel entity that was described in 2014 and that is included in the group of benign mixed epithelial and mesenchymal odontogenic tumours. In recent years, several papers have added new cases with some clinical and histopathological aspects that slightly differ from those described in the original report. The aim of this systematic review is to update all available data on POT published in the literature and to identify those features of the neoplasm that require further investigation. MATERIALS AND METHODS: A systematic review of literature was conducted using PubMed, Embase, Web of Science and Scopus. Additional sources were also checked. Publications reporting cases with enough clinicopathological information were included, without any time or language restrictions. Histopathological or radiological studies were considered for qualitative analysis. RESULTS: A total of 30 publications were included. Seventeen papers were used for quantitative analysis while 13 papers were used only for qualitative analysis. A total of 18 cases of POT were identified. Some clinical, radiographic, histopathological and therapeutic features were common in all reported cases, while other aspects of the neoplasm were inconsistent through published cases. This inconsistency was particularly remarkable when dealing with the histopathological features of the neoplasm. DISCUSSION: Some issues about POT remain unclear and deserve to be clarified by future reports. The description of the odontogenic epithelium covering the ectomesenchyme is often contradictory, while it remains debatable whether peripheral ameloblastic epithelial islands or hard dental tissue deposition can occasionally occur within the tumour.

Pituitary apoplexy during pregnancy: a rare, but dangerous headache.

Pituitary apoplexy is a rare endocrine emergency that occurs in a small number of patients with a pituitary tumor. It is a clinical syndrome characterized by the sudden onset of headache, nausea, vomiting, visual impairment, and decreased consciousness, caused by hemorrhage and/or infarction of the pituitary gland. Pituitary apoplexy has very rarely been described during pregnancy, when it is potentially life-threatening to both the mother and the fetus, if unrecognized. Only a few cases have been published to date. The review of the existing literature underlines that pituitary apoplexy, although rare, should be borne in mind when a pregnant woman presents with severe headache and visual defects of sudden onset. After initial management, which includes intravenous glucocorticoid therapy, fluid and electrolyte replacement, the final selection of medical or surgical treatment should result from a multidisciplinary approach involving expert specialists, keeping into account both severity of clinical presentation and gestational week.

Reactive perivascular T-cell infiltrate predicts survival in primary central nervous system B-cell lymphomas.

Well-established histopathological prognostic factors are lacking in primary central nervous system (CNS) lymphomas (PCNSL). The present study investigated the presence and prognostic role of tumour necrosis (TN) and reactive perivascular T-cell infiltrate (RPVI), defined as a rim of small reactive T-lymphocytes occurring alone or located between the vascular wall and large neoplastic cells, in tumour samples from 100 immunocompetent patients with PCNSL. World Health Organization histotypes of the patients were: 96 diffuse large B-cell lymphomas, two Burkitt-like lymphomas, one anaplastic large T-cell lymphoma and one unclassified B-cell lymphoma. TN was observed in 24 (24%) cases and RPVI in 26 (36%) of 73 assessable cases. Patients with RPVI-positive lesions exhibited a significantly better overall survival (OS) than patients with RPVI-negative lymphoma, particularly among patients treated with high-dose methotrexate-based chemotherapy (3-year OS: 59 +/- 14% vs. 42 +/- 9%, P = 0.02). By contrast, the presence of TN did not demonstrate prognostic significance. Multivariate analysis confirmed an independent association between RPVI and survival. In conclusion, the presence of RPVI is independently associated with survival in PCNSL. This parameter can be easily and routinely assessed at diagnosis on histopathological specimens.

Third ventricle cavernoma associated with venous angioma. Case report and review of the literature.

A case of third ventricle cavernous angioma associated with venous angioma is reported. By a transventricular approach, the cavernoma was totally removed with successful preservation of the venous malformation. After review of the literature, the clinical characteristics and the surgical approach to third ventricle cavernous angioma are discussed; the importance of preservation of associated venous angioma is also underlined.

Subependymoma of the spinal cord: case report and review of the literature.

Spinal cord subependymoma is a rare tumour with only 39 reported cases in the literature. The authors report a further case of this neoplasm in a 53 year old man with a progressive paraparesis, paraesthesias of the lower limbs and sphincter disturbance. The tumour was partly removed, without progression 5 years after surgery. After a careful review of the literature, the optimal treatment of this spinal tumour is debated.

Cytogenetic and ultrastructural study of a pineocytoma case report.

The authors report a case of pineocytoma in a 44-year-old woman suffering from headache, vomiting and Parinaud syndrome. At histopathological examination the neoplasm showed a ill-defined lobulate pattern with some small pineocytomatous rosettes. The electron-microscopy revealed cells of moderate size and oval nuclei with smooth nuclear envelopes; well-developed organelles were found in the, abundant cytoplasm. The chromosome analysis revealed this kariotype: 58-59, XXX, -4, -5, -13, - 14, -15, + 19. This is the first report of a pineocytoma with ultrastructural and cytogenetic study; it confirms the literature findings of the electron-microscopy, whereas there is partial accordance with the previous cytogenetic studies.

A tumor suppressor locus in familial and sporadic chordoma maps to 1p36.

Previous cytogenetic/FISH data have demonstrated 1p36 deletions in a relapsing familial clivus chordoma developed by a patient who has 2 daughters, respectively affected with childhood astrocytoma and clivus chordoma. Using an approach that combined the LOH (loss of heterozygosity) study of the father chordoma and the daughter astrocytoma and a segregation analysis from parents to sibs using 17 CA-repeats spanning 1p36.32-1p36.11, we mapped the cancer susceptibility locus in this family to the 1p36 region. The LOH and haplotype information was elaborated using a pairwise linkage analysis that gave a maximum lod score of 1.2. Additional LOH data relating to 6 sporadic chordomas allowed us to define an SRO (the smallest region of overlapping loss) of about 25 cM from D1S2845 (1p36.31) to D1S2728 (1p36.13). Our overall findings converge on mapping to 1p36 a tumor-suppressor gene involved in familial and sporadic chordoma.

Malignant optic glioma of adulthood. Case report and review of the literature.

INTRODUCTION: The malignant optic glioma in adulthood is a rare tumour of middle-age which causes an early loss of vision and always leads to death within a year. CASE REPORT: The authors report a case of this disease in a 68-year-old woman with a history of rapidly deteriorating vision and death 6 months after surgery. CONCLUSION: A review of the previous cases showed the accordance of these with the syndrome defined first by Hoyt et al. in 1973; a statistical analysis reveals that the radiotherapy improves the survival whereas the role of chemotherapy is still not definite.

Iatrogenic intracranial aneurysm. Case report and review of the literature.

A case of a 56-year-old woman that developed subarachnoid haemorrhage from iatrogenic post-traumatic aneurysm of the cortical middle cerebral artery due to a ventricular tap for hydrocephalus is presented. Previous reported cases of postsurgical aneurysms of the cerebral vessels are discussed and the pathogenesis, the clinical course, the outcome and the advisable treatment of this condition are debated.

Oncocytic meningioma.

Six cases of meningioma showing oncocytic changes are described. The lesions were composed mostly of sheets, nests, and cords of large polygonal cells with finely granular eosinophilic cytoplasm rich in mitochondria. Neoplastic cells showed nuclear pleomorphism with prominent nucleoli. Necrosis and high mitotic rate were present in the majority of cases. Oncocytic differentiation was demonstrated by conventional histology, immunocytochemistry, electron microscopy, and Western-blot analysis. Oncocytic meningiomas showed an aggressive behavior; recurrences were observed in three cases, and invasion of brain cortex was evident in other two cases.

[Solitary fibrous tumor of the nasopharynx. Apropos of a case]. / Tumeur fibreuse solitaire du nasopharynx. A propos d'un cas.

Solitary fibrous tumor (SFT) is a rare neoplasm, arising in the adult and more commonly in the pleura. For many years it has been referred to the serous surfaces of the body, namely pleura, peritoneum and pericardium; recently cases arising in mesenchymal organs such as lung, mediastinum, liver and paranasal sinuses were reported. We present a SFT of the nasopharynx, observed in a 41 year-old patient complaining a 6 months history of aural fullness at the right ear. Clinical examination revealed the presence of secretory otitis media and swelling of the lateral wall of the nasopharynx. CT scan and MR showed the presence of a solid mass, with scarce vascularization, extending from the right side of the nasopharynx to the infratemporal and pterigoid fossae. The surgical approach consisted in a facial translocation by the rotation of a maxillary-check flap through different osteotomies; a firm whitish mass not invading the surrounding tissues was identified and enucleated. A definitive diagnosis cannot be made at frozen sections, requiring more accurate processing and immunohistochemical staining. Literature reports fourteen cases of SFT of the upper aero-digestive tracts (nasopharynx, paranasal sinuses, larynx) and some 8 more cases in the head and neck area (thyroid, salivary glands and parapharyngeal spaces). This case report adds a further contribution to support the mesenchymal origin of the SFT.

Associated olfactory groove meningioma and acoustic neurinoma.

The authors describe the case of a 71-year-old man without signs of phacomatosis with a history of progressive psychomotory lowering and two simultaneous tumors of the olfactory groove and of the cerebellopontine angle, as demonstrated by CT scans. Histologic examinations showed the lesions to be a meningioma and a neurinoma. The rarity and the possible pathogenesis of this association are discussed.

[Anatomical pathology of cutaneous head and neck carcinomas]. / Anatomia patologica dei carcinomi cutanei della testa e del collo.

The author after a short summary about the morphological classification of head and neck cutaneous carcinomas, stresses the necessity of a strict interface of cooperation between surgeon and pathologist. For a better management of the patient with a cutaneous lesions suspected for malignancy is mandatory to take a biopsy and to indicate in the histological report the exact diagnosis of histotype associated with the morphological features related to prognosis.

Intracranial meningioma and astrocytoma in the same patient. Case report and review of the literature.

The authors describe the case of a 58-year-old man with an history of seizures because of right parietal parasagittal meningioma. Twenty-two months after complete removal of this neoplasm, the patient showed progressive mental deterioration and at CT-scans revealed a right temporal anaplastic astrocytoma which was operated. In literature the association between intracranial meningioma and astrocytoma is reported in 41 cases. After a brief review of the previous cases, the authors debate the clinical presentation, the diagnostic problems, the aetiopathogenetic hypotheses and the best surgical treatment of this association.

Intraethmoidal encephalomeningocele presenting with spontaneous cerebrospinal fluid rhinorrhea in an elderly man. Case report.

The case of a 63-year-old patient with spontaneous cerebrospinal fluid rhinorrhea from a intraethmoidal encephalomeningocele is presented. The patient was asymptomatic until the old age. The preoperative coronal CT-scan and the preoperative and postoperative MRI are shown. The rarity of this onset in an elderly man and the surgical indications of this disease are debated.

Solid supratentorial haemangioblastoma. Case report.

A rare case of solid supratentorial haemangioblastoma presenting in a 67-year-old female with psyco-motory lowering, dysphasia and mild haemiparesis is reported and the pertinent literature is reviewed. The distinction between angioblastic meningioma and haemangioblastoma with the non specific radiological findings of this supratentorial uncommon tumour in are also debated.

Isolated aspergillosis of the sphenoid sinus. Case report.

The Authors report a rare case of isolated sphenoid sinus aspergillosis in a 69-year-old man. The patient had a complete recovery. The preoperative CT-scans and the preoperative and postoperative MRI are shown. The incidence, the pathology, the various clinical presentations, the prognosis and the management of this disease are debated.