Perineurioma of the Adrenal Gland

ABSTRACT

The authors report the first case of perineurioma of the adrenal gland. The tumor was composed of elongated wavy spindle cells focally arranged in a fascicular pattern. It was positive for epithelial membrane antigen (EMA) and claudin-1, and was negative for S-100 protein and glial fibrillary acidic protein (GFAP). Electron microscopy showed long, slender cytoplasmic processes coated by discontinuos basal lamina and presence of many pinocytotic vesicles.     

腰椎管内混杂性神经鞘瘤/神经束膜瘤1例

患者女,58岁,无明显诱因出现右侧腰骶部及右下肢疼痛麻木3年余,伴右下肢上抬无力和大小便感觉异常,近半年来症状加重;既往无腰部外伤史及其他疾病史。查体:右下肢肌力4级,实体觉、图形觉、两点分辨异常,巴宾斯基征及霍夫曼征阴性。实验室检查未见明显异常。腰椎CT:L3~L4椎体层面椎管内不规则稍高密度影,内见斑点状钙化(图1A),邻近骨质无破坏。     

Intraneural perineurioma in a child with Beckwith-Wiedemann syndrome

To the authors' knowledge, this is the first report of an intraneural perineurioma in a child with Beckwith-Wiedemann syndrome. Intraneural perineurioma, previously known as localized hypertrophic neuropathy, is a rare benign peripheral nerve sheath tumor arising from perineurium. This report adds a new entity in the spectrum of tumor formation in Beckwith-Wiedemann syndrome.     

Congenital melanocytic nevus with features of hybrid schwannoma/perineurioma

Neural differentiation by melanocytic nevi represents a well‐recognized phenomenon, and melanocytic nevi with perineurial differentiation have been reported recently. We reported a case of a congenital melanocytic nevus with histopathologic features of hybrid schwannoma/perineurioma. The patient was a 36‐year‐old male who presented with a black tumor on his arm since birth. Histopathology showed a congenital melanocytic nevus in the superficial dermis, but more strikingly, in continuity with the melanocytic nevus, there was a well‐circumscribed but unencapsulated nodule in the deep dermis. The nodule was composed of cellular and myxoid areas with storiform, laminated or whorled growth patterns. The cellular area was mainly composed of proliferation of plump spindle, oval or epithelioid cells. The myxoid area was mainly composed of proliferation of slender spindle cells with mucin deposition. Immunohistochemical stains showed that the cellular area was positive for S100 and CD34, weakly positive for EMA, negative for Glut‐1 and collagen IV, the myxoid area was positive for S100, negative for CD34, strongly positive for EMA and focally positive for Glut‐1 and collagen IV. Our results show that congenital melanocytic nevi may show neural differentiation with histopathologic features of hybrid schwannoma/perineurioma.     

Ultrastructural Study of a Perineurioma with Ribosome-Lamella Complexes

The participation of the perineurial cell in peripheral nerve tumors is the subject of much debate. The case of a 75-year-old female with a soft tissue tumor on her left shoulder is presented. The tumor had histological, ultrastructural, and immunohistochemical characteristics of a pure perineurial cell neoplasm. Ultrastructurally, distinctive ribosome-lamella complexes were found in the cytoplasm of the perineurial cells. This may be the first time that these structures have been described in perineurioma.     

Spindle cell proliferations of the sigmoid colon,rectum and anus: a review with emphasis on perineurioma

A wide range of spindle cell proliferations are found uncommonly in the sigmoid colon, rectum and anus. They usually present as polyps, and include reactive lesions and benign and malignant neoplasms which may be primary or metastatic. They are less frequently described in the literature compared to those in the upper gastrointestinal tract, and may be underdiagnosed. The widespread use of sigmoidoscopy in symptomatic patients and bowel cancer screening programmes means that histopathologists must be aware of, and adopt a logical approach to, diagnosing spindle cell proliferations in biopsy and polypectomy specimens. This is particularly relevant given the strong association of some mesenchymal polyps with hereditary cancer syndromes. This review article will focus on perineurioma and the recent debate in relation to its overlap with fibroblastic polyp. The clinical, endoscopic, histological and immunohistochemical features of spindle cell proliferations which should be considered in the differential diagnosis of perineurioma will be discussed. There is also a brief reference to malignant spindle cell tumours of diagnostic importance.     

Intraneural perineuriomas: diagnostic value of magnetic resonance neurography

Intraneural perineurioma (IP) is an under‐recognized hypertrophic peripheral nerve tumor. It affects young patients involving frequently the sciatic nerve and its branches and presents with a progressive, painless and predominantly motor deficit. Magnetic resonance neurography (MRN) is a useful tool to localize the lesion, evaluate its extension, and discriminate between different etiologies. We reviewed the clinical records of 11 patients with pathologically confirm IP. Eight patients were males with mean age 19 years. Initial complains were unilateral steppage (seven patients), bilateral steppage (one patient), unilateral gastrocnemius wasting (one patient), unilateral thigh atrophy (one patient), and unilateral hand weakness (one patient). Nine patients had mild painless sensory loss. Examinations revealed involvement of sciatic nerve extending into the peroneal nerve (eight patients), posterior tibial nerve (one patient), radial nerve (one patient), and femoral nerve (one patient). MRN revealed enlargement of the affected nerve isointense on T1‐weighted, hyperintense on T2 fat‐saturated images, and with avid enhancement on post‐contrast imaging. In all patients, a nerve biopsy confirmed the diagnosis. MRN allows early and non‐invasive identification of this tumor and is a key tool providing localization and differential diagnosis in patients with slowly progressive focal neuropathies.     

Pediatric sciatic neuropathy associated with neoplasms

Seven children with sciatic neuropathy associated with an underlying neoplasm are reported. Clinical presentation, electrophysiological data, imaging, pathology, and/or autopsy results are described. Pain and weakness, primarily foot drop, were the most common presenting symptoms. The mechanism of sciatic neuropathy was varied and included: nerve infiltration by the adjacent neoplasm (neuroblastoma, rhabdomyosarcoma, and leukemic or lymphomatous infiltration); an expanding, intrinsic neurogenic tumor (perineurioma); or intraoperative stretch injury (osteosarcoma resection). The prognosis for sciatic nerve recovery was good among children who survived their associated cancer. Three children died from the cancer or complications of treatment. One child with perineurioma remained clinically stable, and two children improved after treatment of their neoplasm.     

Intraoral Perineurioma,Soft Tissue Type: Report of Five Cases,Including 3 Intraosseous Examples,and Review of the Literature

Soft tissue type perineuriomas (STP) are uncommon tumors, oral examples being very rare. They have been described in the mandible, gingiva, lips, retrotonsillar mucosa and maxillary vestibule. Herein, we report the clinicopathologic features of five STP, two affecting the buccal mucosa and three the mandible. Three patients were women and two men. All tumors were characterized by a proliferation of cytologically bland, mitotically inactive spindled cells with round, ovoid or spindled nuclei, embedded in a variably collagenous and myxoid matrix. Interestingly, two mandibular tumors featured psammoma bodies and one, in addition, contained irregular calcifications. Tumor cells showed the immunohistochemical profile of perineurial cells including epithelial membrane antigen, Glut-1, claudin-1 and collagen type IV. S100 and neurofilament proteins were not expressed by the tumor cells. A few minute, peripherally situated, entrapped nerves were identified. All tumors were reported gross-totally excised and no recurrences have taken place. The clinical characteristics of STP are summarized and its differential diagnosis relative to other spindle cells tumors and meningioma is discussed.