脑脊膜黑色素细胞瘤的临床病理分析

目的：探讨脑脊膜黑色素细胞瘤的临床病理特征、组织发生及预后。方法：报道2例罕见的椎管内软脊膜黑色素细胞瘤（0.05％），并结合国内外文献中报道的58例脑脊膜黑色素细胞瘤进行讨论。结果：2例均为妇性，分别为14岁和46岁，肿瘤均发生于脊髓软脊膜，大体上呈黑色，包膜完整。光镜下见瘤细胞由上皮样细胞和梭形细胞混合而成，细胞无异型，大部分瘤细胞质内含有多量黑色素颗粒，核分裂象无偶见（0－1个／10HPF），无出血坏死。免疫表型HMB45和S－100蛋白呈阳性表达。1例颈髓单发者全切除术后生存14个月，无肿瘤复发，另1例开始为颈髓单发性，本切除术后12年复发，为颈、胸髓多发性，再次行肿瘤部分切除术。结论：脑脊膜黑色素细胞瘤生物学行为相对良性，好发于后颅凹及脊髓的软脑脊膜，具有明显的临床和组织学特征。免疫组化和电镜检查有助于本瘤与其他黑色素性肿瘤的鉴别诊断早期完全切除有望治愈或减少复发，术后放疗对控制复发并无意义。     

A case report on intermediate-grade malignant meningeal melanocytoma with elevated cell proliferation index

Meningeal melanocytoma is a kind of extremely rare pigmented tumor of the central nervous system, which often occurs in the groove around the base of the brain and spinal pia mater. Age of onset is 40 to 50 years old, mostly presenting a benign course of disease and the prognosis is good. Case reports of partial invasion or metastasis from lesions are even rarer. This report described a case of 56-year-old meningeal melanoma patient with partial skull and muscle invasion at the lantooccipital transition zone. Intraoperative histological examination showed moderate malignancy and Ki-67 index was 10%.     

Collecting duct meningeal carcinomatosis.

Collecting duct carcinoma (CDC) is an aggressive primary renal neoplasm that represents a distinct subtype of renal cell carcinoma. Histochemical (eg, mucicarmine) and immunohistochemical (eg, Ulex europaeus) studies, taken in concert with the gross and histologic findings, allow differentiation of CDC from the conventional varieties of renal cell carcinoma in most cases. Collecting duct carcinoma generally pursues a more aggressive course than conventional renal cell carcinoma. Metastases to regional lymph nodes, bone, adrenal glands, lung, and skin have been reported in CDC. We describe the case of a 26-year-old man who presented with a clinical and radiologic impression of multifocal meningioma. Biopsies of the meninges and extracranial soft tissues revealed metastatic adenocarcinoma; subsequent studies suggested metastatic CDC. Ultrasound-guided biopsy was performed on a subsequently identified renal mass, which showed features consistent with CDC. To our knowledge, this is the first reported case of meningeal carcinomatosis due to CDC. The diagnostic features of this tumor are discussed.     

Pituitary melanocytoma mimicking an adenoma

A 62-year-old woman was referred for a pituitary tumour diagnosed because of a chronic asthenia and visual disorders. Cerebral MRI showed a pituitary tumour compressing the optic chiasm and enhanced after gadolinium injection. Biological findings showed panhypopituitarism and hyperprolactinemia. The diagnostic of pituitary macro-adenoma was performed and the patient was treated with hormone replacement therapy and dopaminergic agonist. Six months later, she presented visual disorders worsening leading to surgical excision. The diagnosis of pituitary melanocytoma was performed after anatomo-clinical confrontation. Post-operative radiation was done.     

Extracranial branches of the middle meningeal artery.

We present a case of unusual extracranial branches of the middle meningeal artery. The middle meningeal artery originated from the medial side of the maxillary artery and entered the skull through the foramen spinosum. The posterior superior alveolar artery originated from the middle meningeal artery, gave rise to the pterygoid branch, and entered the maxilla. In addition, the bifurcation of the common carotid artery was at the level of the second cervical vertebra. The embryogenesis of such rare branching pattern of the middle meningeal artery is not clear, but the anatomical consequences may have clinical implications.     

Cutaneous heterotopic meningeal nodules

The clinical, light microscopic and immunohistochemical features of six cutaneous heterotopic meningeal nodules (primary, or type I, cutaneous meningiomas) are described. These are rare lesions of the scalp and back which generally present at birth or in childhood. They appear as small subcutaneous fibrous nodules, with no specific clinical features although they can be associated with abnormalities of spinal closure. Microscopically, they show a variable architecture but have similar cytological features. Important diagnostic features include psammoma and small collagenous bodies. An intimate relationship to nerves is seen in some cases, and this may be of significance with regard to the development of these lesions. As with intracranial meningiomas, there is widespread vimentin expression, and most cases show epithelial membrane antigen expression.     

Primary meningeal mesenchymal chondrosarcoma

A primary meningeal mesenchymal chondrosarcoma initially resembled an angioblastic meningioma because the typical chondroid islands were not demonstrable. Cartilage was seen only in an intracerebral recurrence and in subsequent extracranial metastases. Ultrastructural examination of noncartilaginous regions of the tumor demonstrated mesenchymal cells with features suggestive of cartilaginous differentiation, viz, scalloped cell membranes, sac-like distension of abundant rough endoplasmic reticulum, and a matrix containing fibrillary and finely granular material. Features of meningeal or pericytic cells were not seen.     

Prostaglandin D synthase (beta-trace) in meningeal hemangiopericytoma.

The level of prostaglandin D synthase (PGDS), a major protein constituent of cerebrospinal fluid (CSF), is altered in various brain diseases, including meningitis. However, its role in the brain remains unclear. PGDS is mainly synthesized in the arachnoid cells, the choroid plexus and oligodendrocytes in the central nervous system. Among brain tumors, meningiomas showed intense immunoreactivity to PGDS in the perinuclear region. Thus, PGDS has been considered a specific cell marker of meningioma. In this study, we examined 25 meningeal hemangiopericytomas (HPCs) and found that 16 of the tumors (64%) showed immunoreactivity for PGDS in the perinuclear region. For comparison, 15 meningiomas, 14 soft-tissue HPCs, 1 mesenchymal chondrosarcoma, 3 choroid plexus papillomas, and 7 oligodendrogliomas were also examined. Meningiomas showed positive immunoreactivity for PGDS in 13 cases (80%). Except for one case located at the sacrum, none of the other soft-tissue HPCs showed immunostaining for PGDS. Mesenchymal chondrosarcoma arises in the bones of the skull, and its histological pattern resembles that of HPC; however, it showed no immunoreactivity for PGDS. Neither choroid plexus papillomas nor oligodendrogliomas were immunopositive for PGDS. These findings suggest that meningeal HPCs may have a unique molecular phenotype that is distinct from that of the soft-tissue HPCs. The origin of meningeal HPCs may be more closely related to the arachnoid cells.     

脑膜血管周细胞瘤临床病理分析

目的:探讨脑膜血管周细胞瘤(hemangiopericytoma,HPC)的临床病理特点及鉴别诊断.方法:对9例脑膜HPC患临床病理资料进行回顾性分析和随访15~95个月,并复习相关文献.结果:脑膜HPC常见症状为头痛头昏、肿瘤压迫和颅内高压;CT/MRI检查见肿瘤与脑膜相连,增强扫描明显强化.光镜下肿瘤细胞丰富,核圆形或卵圆形,核仁不明显;间质富含"鹿角状"血管,网状纤维包绕单个瘤细胞.免疫标记瘤细胞CD34局灶阳性,PR阴性,1例EMA局灶弱阳性.7例随访15~95个月,4例术后复发,复发病例中1例术后35月腰椎转移.结论:脑膜HPC是一种少见肿瘤,确诊需依靠病理学检查.脑膜HPC影像学上与脑膜瘤相似,但其生物学行为、病理组织学及预后不同于脑膜瘤,易复发和远处转移,术后需长期随访.     

Multifocal sporotrichosis with meningeal involvement in a patient with AIDS.

This report describes a 29-year-old man with AIDS and disseminated lymphocutaneous sporotrichosis diagnosis presenting a poor therapeutic adhesion to itraconazole therapy that later evolved to neurological impairment and death. Necropsy showed granulomatous reaction and yeast-like forms similar to Sporothrix schenckii in meninges, lymph nodes, marrow bone, skin, testicles, epididymides and pancreas. Meningeal sporotrichosis cases associated to AIDS are reviewed in brief.     

Spinal hydatidosis. Failure of albendazole

A recession of eighteen months is not enough to evaluate efficacy of albendazole therapy on spine hydatic cyst in spite of a negative specific serology by HAI and electrophoresis. Specific IgE and precipitates system number 5 seem not good tests for the following of evolution. Inoculation of mouse with bone biopsy is an interesting test to evaluate the viability of the cyst.     

Meningeal melanocytoma: case report and review of the literature

We report a case of meningeal melanocytoma in the thoracic spinal cord of a 44-year-old woman and review previously documented cases. Our patient experienced numbness and tingling in her left leg for 8 years, and low back pains with intermittent claudication for the previous 2 months. A histologically benign 20-mm tumour was totally resected. Radiation therapy was not given. The tumour showed the histological, immunohistochemical and ultrastructural features of a meningeal melanocytoma. The patient is alive without recurrence 4.5 years after surgery.