Malignant craniopharyngioma

Craniopharyngiomas are histologically and cytologically benign epithelial tumors of the central nervous system that may be locally aggressive and tend to recur after excision. Malignant change in craniopharyngiomas is extremely rare; we found only 4 such reports in the literature. In this report, we describe a case of squamous cell carcinoma arising in a previously benign craniopharyngioma in a 42-year-old woman. The patient was diagnosed with craniopharyngioma in 1982; during the subsequent 15 years she experienced 7 tumor recurrences, for which surgical resections and 3 courses of radiotherapy were performed. In 1998, the tumor recurred with involvement of the nasal cavity and sphenoid and ethmoid sinuses. Histologic evaluation revealed foci of typical adamantinomatous craniopharyngioma associated with a moderately differentiated squamous cell carcinoma. The transition of typical craniopharyngioma to squamous cell carcinoma was well demonstrated, suggesting that carcinoma arose from the underlying craniopharyngioma. Radiation may have been a contributing factor to carcinogenesis in this case.     

Expression of epithelial cell adhesion molecule and pituitary tumor transforming gene in adamantinomatous craniopharyngioma and its correlation with recurrence of the tumor

Craniopharyngiomas are benign tumors of the sellar region generally associated with endocrine disorders and often locally aggressive. The reliable criteria for predicting the tumor behavior are still lacking. It has been suggested that proliferative potential of the tumor cells is necessary for recurrence. The aim of this study was to evaluate the activity and correlation of epithelial cell adhesion molecule (Ep-CAM) and pituitary tumor transforming gene (PTTG-1) immunoexpression that is possibly related to relapse in 40 patients with adamantinomatous craniopharyngioma. The study involved clinical and pathologic analysis. Of the subjects, 49% were females and 51% were males. The mean age of the patients was 37 years. Relapsing rate at 5 years was 46% and for death was 22.5%. Histologically, whorl-like arrays and dense or hypercellular stellate reticulum cells were correlated with recurrence. Epithelial CAM and PTTG-1 were also higher in stellate reticulum cells and in whorl-like arrays. Both were higher in recurrence/regrowth tumors than in primary one. The PTGG-1 expression in craniopharyngioma may suggest hypophyseal metaplasia. The Ep-CAM and PTTG-1 expression in craniopharyngioma could be used as prediction markers of relapsing tumor. It has been suggested that proliferative potential of the tumor cells is necessary for recurrence.     

Mutation in exon 7 of PTCH deregulates SHH/PTCH/SMO signaling: possible linkage to WNT

The novel PTCH mutation and clinical manifestations within Gorlin syndrome family links PTCH haploinsufficiency and aberrant activation of the Wnt pathway. We report a family case with Gorlin syndrome, characterized by the usual phenotype features such as widespread basocellular tumors and craniofacial and bone malformations, but also including a less common appearance of craniopharyngioma. These clinical manifestations might be associated with a novel constitutional mutation of the PTCH gene, 1047insAGAA, which we found in exon 7. It changes the normal amino acid sequence leading to termination of the PTCH protein at exon 9. The analyzed tumors of the family show extensive loss of heterozygosity in the PTCH region, both basocellular and in particular craniopharyngioma, and in the latter a high expression of beta-catenin was detected. Our findings suggest involvement of the SHH/PTCH/SMO pathway in pathogenesis of the analyzed disorders, including its possible contribution to aberrant activation of the Wnt pathway in craniopharyngioma.     

Intracranial extra-axial ependymoma involving the petroclival region: a rare case report

Intracranial extraaxial ependymomas (IEAEs) are very rare, and extra-axial petroclival ependymoma (EAPE) has not been reported in the reviewed English-language literature by now. We present a male patient with EAPE misdiagnosed as a petroclival meningioma preoperatively, and involved recurrence at 4th months after operation though totally resection. Thereafter, we report this case and discuss the clinical characteristics of the disease by briefly review.     

导航辅助下扩大经鼻蝶入路斜坡区的内镜解剖学研究

目的　研究导航辅助内镜下扩大经鼻蝶入路斜坡区的显露,为经鼻蝶入路切除斜坡中线区域病变提供显微解剖学资料。 方法　10例成人头颅湿性标本,导航辅助模拟内镜下扩大经鼻蝶入路,观测斜坡中线区域重要解剖结构暴露情况。 结果　打开蝶窦前下壁,磨除鞍背及斜坡骨质,剪开硬膜,可显露双侧动眼神经、三叉神经节、展神经,基底动脉及分支,小脑下后动脉及后组脑神经;导航辅助下可精确行数据测量及定位,扩大经鼻蝶入路可完全暴露斜坡,最短距离为(88.90±2.62) mm;蝶窦前下壁和下斜坡磨除范围分别以两侧翼管和破裂孔为界,各自距中线距离为(9.22±0.48) mm和(10.60±0.82) mm。 结论　斜坡中线区的病变,内镜下扩大经鼻蝶入路是一个适当的方法。导航辅助有助于对重要结构的定位,可提高安全度。     

Solitary intracranial metastasis of follicular carcinoma of the thyroid gland clinically mimicking a meningioma

We describe a rare case of solitary metastasis of follicular carcinoma of the thyroid gland into the petroclival region in a 58-year-old woman. The metastasis was the first and only manifestation of the disease. The histology of the tumor, differential diagnosis and clinical course are discussed. In a few similar cases described so far the tumor was always a well or moderately differentiated follicular carcinoma located in the petroclival region. As in this presentation, these cases also clinically mimic a meningioma. The differential diagnosis includes adenoma of the thyroid gland and thyroid gland dystopia. We demonstrated the primary focus of carcinoma within the thyroid after its detailed examination initiated by our finding. The clinical outcome of such rare cases is usually excellent, much better than in metastases of papillary carcinoma into the brain.     

Aggressive pilomatrixoma

We report a case of aggressive pilomatrixoma (PMX) in a 25‐year‐old male who presented with swelling in left supraclavicular region of 2‐month duration. A diagnosis of small round cell tumor was suggested on fine‐needle aspiration cytology. He underwent wide excision of the mass. On histomorphological examination, a diagnosis of aggressive PMX was made. The swelling recurred after 3 months of complete resection and on examination had similar morphological features. The case is presented because of the potential diagnostic difficulties on cytological examination and rare occurrence of aggressive variant of PMX. Diagn. Cytopathol. 2014;42:906–911. © 2014 Wiley Periodicals, Inc.     

Burkitt's lymphoma representing periportal infiltrating mass on CT

Imaging findings of secondary hepatic lymphoma have been reported as variable, ranging from single or multiple small nodules to diffuse infiltrative tumor patterns. We hear present a rare case report concerning aggressive B cells, secondary Burkitt's lymphoma in non-AIDS demonstrating a surprising periportal lymphoma infiltration, without upper abdominal lymphadenopathy or splenomegaly on the sonography and CT scans. Clinically, the case was characterized by atypical and highly aggressive course, with the patient presenting an abruptly developed obstructive jaundice with rapidly deteriorating hepatic function that could be indicative of cholestatic hepatitis, which differs in its clinical manifestations from hepatic lymphoma without functional deterioration in respect of its non-tissue destructive growth pattern. We suggest that hepatic lymphoma can sometimes be consistent with periportal infiltrating homogeneous mass, with no lymphadenopathy or splenomegaly on the imaging examination, with a predictable aggressive clinical course of the disease and poor prognosis.     

Combined sellar fibrosarcoma and prolactinoma with neuronal metaplasia: Report of a case unassociated with radiotherapy

We report the occurrence of a primary pituitary fibrosarcoma causally unrelated to radiotherapy, admixed in association with a prolactin cell pituitary adenoma showing neuronal metaplasia. These unique findings were associated with multiple endocrine neoplasia type 1 (MEN 1). Primary fibrosarcoma involving the sella is a very rare tumor. The majority of cases have been associated with prior irradiation of either a pituitary adenoma or a craniopharyngioma. Pituitary adenoma with neuronal metaplasia is also rare and usually occurs in the setting of acromegaly. Despite the intimate association of both elements in our lesion, no transition of adenoma to sarcoma was demonstrable by immunohistochemistry or in situ hybridization studies.     

Cytological features of a craniopharyngioma.

The fine needle aspiration cytology of a cystic suprasellar lesion, which caused bilateral hemianopia in a woman aged 63, resulted to be consistent with a craniopharyngioma and was confirmed by tissue diagnosis. The cytological pattern showed macrophages and debris with calcifications indicating the presence of a cyst. Furthermore squamous cells of basal type and ghost cells were identified. These findings together with the seat of the lesion allowed for the diagnosis of craniopharyngioma and for the differentiation from other cystic lesions of the same region. The case confirms the value of the fine needle aspiration cytology in the detection of craniopharyngioma and points out cytological peculiarities of this not rare neoplasm.     

Craniopharyngioma: A clinicopathological study of 141 cases

Craniopharyngioma is a tumor of the suprasellar region that histologically has two distinct variants with some differences in clinical behavior. The papillary type is almost always seen in adults and has a more indolent course compared with the adamantinomatous type, which is more common in childhood. In the present study, surgical specimens of craniopharyngiomas from 141 patients were reviewed. Their histomorphologic types were determined and the clinical features and prognosis of each group were assessed. The sizes of papillary type tumors were smaller and during the follow-up period there was no recurrence in the squamous papillary group. Aside from surgical resection (total vs subtotal), the recurrence rate for papillary type craniopharyngioma was lower than for adamantinomatous type. Histologic typing of craniopharyngioma especially in adults is useful for decision making with regard to treatment and follow-up.     

Maxillary aggressive angiomyxoma showing ineffective to radiotherapy: a rare case report and review of literature

Aggressive angiomyxoma, mostly originating in the female pelvis and peritoneum or in the male analogous sites, is a rare mesenchymal neoplasm characterized with infiltrative growth to adjacent tissue and local recurrence after primary excision. Herein, we report a case of aggressive angiomyxoma of maxilla in a 60-year-old male patient for its rarity. The patient presented with a one-year history of progressively enlarging maxillary mass on left side. Before referred to our hospital, he was given a biopsy and diagnosed as aggressive angiomyxoma by immunohistochemical staining. After that, he underwent 60Gy radiotherapy. Unfortunately, CT scan showed bigger mass infiltrated to adjacent facial soft tissues and bones compared with that of before radiotherapy. Besides that, he began to suffer with ingravescent headache. The mass was surgically removed and the diagnosis was confirmed by immunohistology in our hospital. As a case of aggressive angiomyxoma occurred in a rare site and experienced an ongoing growth in spite of radiotherapy, its characteristics was discussed with a brief literature review, which may aid further understanding of aggressive angiomyoma.     

Collision Tumors of the Sella: Craniopharyngioma and Silent Pituitary Adenoma Subtype 3: Case Report

Collision tumors of the sella turcica are rare and consist mainly of more than one type of pituitary adenoma, usually a corticotropin- and a prolactin-producing adenoma. The association of a craniopharyngioma and a pituitary adenoma is rare. Herein, we report the first case of an association between craniopharyngioma and silent pituitary adenoma subtype 3. It involved a 12-year-old boy who underwent a frontal craniotomy with surgical removal of a calcified sellar tumor. Histology revealed an adamantinomatous craniopharyngioma; however, by electron microscopy, there was conclusive evidence of adenoma cells showing the ultrastructural features of silent pituitary adenoma subtype 3. Endocrine and neuroimaging as well as detailed immunohistochemical and ultrastructural studies were undertaken. The literature is also reviewed.     

December 1998--16 year old female with headaches, lethargy and a sellar/suprasellar mass

A 16 year female with a history of developmental delay and shunted hydrocephalus presented with two months of progressive headaches, lethargy and visual disturbances. An MRI of the brain revealed a sellar and suprasellar cystic mass which was absent on a previous MRI six years earlier. The pre-operative clinical diagnosis was pituitary adenoma vs. craniopharyngioma. Histologically, the fibrous wall of the ciliated epithelial-lined cyst was thickened by non-caseating granulomatous inflammation, hemorrhage, hemosiderin, and cholesterol clefts, consistent with cyst rupture. Rathke's cleft cysts are uncommon symptomatic lesions in young people, and must be distinguished from craniopharyngioma.     

Periosteal chondroma of the ischium; an unusual location

Periosteal chondroma is a rare benign tumor of hyaline cartilage. Periosteal chondroma arising in the ischium is an extremely rare event. By presenting this case report, we aim to remind that differential diagnosis of these lesions should be carefully performed especially for unusual locations. A 51-year-old man visited the orthopedics clinic with the complaints of left pelvic mass and pain lasting about one year. Computed tomography showed a solid mass with abundant specks of calcification that was 4x5 cm in diameter and associated with the ischium of the left pelvic bone. There was no evidence of penetration into the medullary cavity and cortical sclerosis. No radiological aggressive appearance was observed. The lesion was removed with the covering periosteum. Microscopically, there were lobules of hyaline cartilage composed of chondrocytes with foci of endochondral ossification and calcification. The case was reported as 'periosteal chondroma'. In conclusion, this case report presents a case of periosteal chondroma, a rare tumor, which occurred in an unusual location and age range. The differential diagnosis of periosteal chondroma from malignant lesions is necessary for avoiding aggressive and inappropriate treatment.     

内镜下远外侧髁后锁孔手术入路至岩下斜区的应用解剖学

目的　为内镜下远外侧枕髁后锁孔入路治疗岩下斜区及周围疾患提供解剖学基础。 方法　10具尸体头标本于乳突后作纵向“S"型、约7 cm长头皮切口,上缘起自乳突中点向后2 cm处,下界至C2水平。做直径约2 cm的骨窗,牵开小脑半球,内镜下观察所显露的解剖结构。 结果　内镜下可以观察到后组脑神经的出颅部位,包括颈静脉孔和舌下神经管内口,此外,还可以显露脑干腹侧面的结构及椎动脉的末端。神经与血管间复杂毗邻关系亦可得以显示;骨窗外缘至颈静脉内口的距离为(1.75±0.29) cm。 结论　内镜的使用,可缩小远外侧枕髁后锁孔入路的骨窗;副神经的脊髓根及颈静脉孔内口均可以作为经远外侧枕髁后锁孔入路内镜下岩下斜区结构的定位标识。     

Pleuro-pulmonary blastoma in a 45 year-old woman after Hodgkin’s lymphoma: a case report

Pleuro-pulmonary blastoma is a rare and aggressive intrathoracic mesenchymal tumor occurring in childhood. We report the exceptional case of a forty-five year-old woman, presenting with respiratory distress. She had previously been treated with chemotherapy and radiotherapy for Hodgkin lymphoma. Chest X rays and computed tomography scan showed a left lung lower lobe mass and pleural effusion. Biopsy cores revealed a pleura-pulmonary blastoma tumor with mixed components. Neoadjuvant chemotherapy was decided in multidisciplinary round. Because of the rarity of these tumors, no consensus for the treatment exists.     

颅咽管瘤患儿血清IL-6、IL-8、MIP-1α含量变化及其意义探讨

目的:研究颅咽管瘤患儿血清IL-6、IL-8、MIP-1α含量变化及临床意义.方法:应用Luminex 200多功能液相芯片分析仪检测39例颅咽管瘤患儿和54例健康对照儿童血清IL-6、IL-8、MIP-1α浓度.结果:颅咽管瘤组血清IL-6、IL-8、MIP-1α浓度均显著高于健康对照组(均P＜0.01),其中MIP-1α浓度变化最为显著.颅咽管瘤复发组血清MIP-1α浓度显著高于初发组(P＜0.05),而IL-6、IL-8浓度两组间比较差异无统计学意义(P＞0.05).根据Spearman等级相关分析,肿瘤组血清IL-6、IL-8、MIP-1α浓度两两之间呈正相关(均P＜0.01).结论:血清IL-6、IL-8和MIP-1α浓度变化与颅咽管瘤发生、发展密切相关,三者可能相互关联,共同调节肿瘤进展.     

Review of xanthomatous lesions of the sella

Xanthomatous lesions of the sellar region have traditionally been divided into two separate categories, xanthomatous hypophysitis (XH) and xanthogranuloma (XG) of the sellar region. The seminal article on XH, a condition typified by foamy histiocytes and lymphoplasmacytic infiltrates in the pituitary gland/sellar region, but usually little or no hemosiderin pigment, detailed three patients. However, most reports since that time have been single cases, making understanding of the entity difficult. In contrast, the seminal report on XG, characterized by sellar region cholesterol clefts, lymphoplasmacytic infiltrates, marked hemosiderin deposits, fibrosis, multinucleated giant cells around cholesterol clefts, eosinophilic granular necrotic debris, and accumulation of macrophages, included 37 patients, allowing more insights into etiology. Few examples could be linked to adamantinomatous craniopharyngioma, and although ciliated epithelium similar to that of Rathke cleft cyst (RCC) was identified up to 35% of the 37 cases, it could not be proven that XG was related to hemorrhage into RCC. Case reports since that time, however, occasionally linked XG to RCC when an etiology could be identified at all, and a few recognized that a spectrum exists in xanthomatous lesions of the sella. They review literature, adding 23 cases from our own experience, to confirm that overlap occurs between XH and XG, and that the majority—but not all—can be linked to RCC leakage/rupture/hemorrhage. It was suggested that progressive accumulation of hemosiderin pigment in the lesion, possibly caused by the multiple episodes of bleeding, could account for the transition of at least some cases of XH to XG.