Spontaneous Alteration from Rathke’s Cleft Cyst to Craniopharyngioma—Possible Involvement of Transformation Between These Pathologies

Both Rathke’s cleft cyst and craniopharyngioma are considered to arise from the remnants of Rathke’s diverticulum despite the quite different histological characteristics. These two lesions may consist of a disease spectrum extending from Rathke’s cleft cyst to craniopharyngioma. However, in spite of increasing evidence of these intermediate histologies, very few cases of the actual transformation from Rathke’s cleft cyst to craniopharyngioma have been reported in the same patient. A 47-year-old man suffered from recurrent visual dysfunction. Aspiration and partial cystectomy was performed to a suprasellar massive cystic lesion. The histological diagnosis was Rathke’s cleft cyst with a small component of squamous metaplasia. Seven months later, the cyst was re-expanded. The cyst wall was irregularly thickened. Re-operation was performed, and the thickened anterior wall was widely removed. Postoperative histological examination showed multiplication of stratified squamous epithelia forming a papillary arrangement. Ki-67 staining showed positive cells randomly distributed not only in the basal layer but also in various epithelial layers, with a labeling index of more than 20 %. The histological diagnosis was squamous papillary type of craniopharyngioma with high potential of proliferation. Subsequent immunohistochemical examinations showed positive reaction to cytokeratin 8 only in the initial epithelium and negative in the latter epithelium. The present case was thought as an actual evidence of the proposed link between Rathke’s cleft cyst and craniopharyngioma. Cytokeratin 8 could be the important examination to differentiate Rathke’s cleft cyst from craniopharyngioma.     

儿童中枢性尿崩症的病因及MRI表现

目的探讨儿童中枢性尿崩症(CDI)的常见病因及MRI表现。方法收集我院2009年1月至2019年6月经临床确诊的118例CDI患儿的实验室检查和影像学资料,回顾性分析其病因和MRI表现。结果原发性CDI患儿25例(21.19%),MRI仅表现为垂体后叶T1WI高信号消失。继发性CDI患儿93例(78.81%),常见病因包括以生殖细胞瘤和颅咽管瘤为主的肿瘤性病变(33.33%)、未定性垂体柄增粗(31.18%)、炎性病变朗格汉斯细胞组织细胞增生症(24.73%);少见病因包括原发性垂体柄阻断综合征(3.22%)、Rathke′s囊肿(2.15%)、垂体腺瘤(1.07%)、颅咽管瘤术后(2.15%)、空泡蝶鞍(1.07%)和单纯外伤(1.07%)。继发性CDI因病因不同而各具MRI表现特点,但垂体后叶T1WI高信号消失为其共有特点。此外,有4例原发性CDI在随访中转变为继发性CDI(鞍区生殖细胞瘤)。结论儿童CDI病因复杂,MRI是明确病因并进行影像诊断的重要检查方法,原发性CDI的患儿需警惕发展为继发性CDI。     

Different Keratin Profilesin Craniopharyngioma Subtypes and Ameloblastomas

Craniopharyngiomas are generally considered to arise from the remnants of Rathke's pouch or a misplaced enamel organ. We tried to refine these hypotheses, comparing the subtypes of craniopharyngioma with Rathke's cleft cyst, a known Rathke's pouch derivative, and with ameloblastoma, an enamel organ derivative. Nineteen craniopharyngiomas (14 adamantinomatous and 5 papillary type tumors) and 17 ameloblastomas were immunostained for cytokeratin (CK) 7, CK 8, CK 14, and human hair keratin (HHK). All cases of adamantinomatous craniopharyngioma were CK 7+/CK 8+/CK 14+. Two cases (40%) of papillary craniopharyngioma were CK 7+/CK 8+/CK 14+, whereas the remaining three cases (60%) were CK 7+/CK 8-/CK 14+. Fifteen cases (88%) of ameloblastoma were CK 7-/CK 8+/CK 14+. Only the shadow cells present in adamantinomatous craniopharyngiomas were positive for HHK, which may indicate their follicular differentiation. In Rathke's cleft cyst, ciliated cuboidal cells were CK 7+/CK 8+/CK 14- and metaplastic squamous cells were CK 7+/CK 8/CK 14+. These findings suggest that both subtypes of craniopharyngioma may differ from ameloblastoma in histogenesis, although cytokeratin expression patterns may change during tumor development. Adamantinomatous craniopharyngioma may be related to a heterotopic ectodermal tissue which can differentiate into hair follicles, while papillary craniopharyngioma may arise from Rathke's cleft cyst.     

Odontogenic classification of craniopharyngiomas: a clinicopathological study of 54 cases

Based on the striking histological similarity of craniopharyngiomas and some odontogenic tumours, we reclassified a series of 54 craniopharyngiomas (52 adamantinomatous and two papillary variants) according to the WHO classification of odontogenic tumours. Twenty-seven tumours (50%) corresponded histologically to calcifying odontogenic cyst, 13 tumours (24%) to ameloblastoma, and eight (15%) tumours showed features of both calcifying odontogenic cyst and ameloblastoma either within the same specimen or in specimens derived from different resections. Rare tumours included three cases resembling calcifying epithelial odontogenic tumour and one case resembling adenomatoid odontogenic tumour. No odontogenic counterpart could be established for papillary craniopharyngiomas. The two major subtypes, i.e. craniopharyngioma corresponding to calcifying odontogenic cyst and craniopharyngioma corresponding to ameloblastoma, did not differ in their basic clinical features. Our data confirm and extend the close histological resemblance between adamantinomatous craniopharyngioma and odontogenic tumours and cysts. Furthermore, although calcifying odontogenic cyst and ameloblastoma arising in the jaw differ in clinical presentation and outcome, our study did not reveal clinical differences for the corresponding subtypes of craniopharyngioma.     

Insulin-like growth factors and IGF binding proteins in cyst fluid from patients with craniopharyngioma prior to intracavitary irradiation with Yttrium and thereafter

Aim-To examine a series of cyst fluid samples from patients with craniopharyngioma at various stages of treatment in order to evaluate the use of insulin-like growth factors (IGFs) and IGF binding proteins as tumour markers or indicators of successful treatment, or both.Methods-Cyst fluid samples were obtained by stereotactic puncture prior to the intracavitary application of (90)Yttrium and at subsequent occasions. Analysis was performed by gel chromatography, radio-immunoassays, binding studies, and sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) with subsequent western blotting.Results-IGF-I, -II and IGF binding protein-1 concentrations were measured in three craniopharyngioma cyst fluid samples. Immunoreactive IGF-I and IGF binding protein-1 concentrations in these three samples were between 6 and 29 ng/ml, and 17 and 48 ng/ml, respectively. In contrast, the IGF-II concentrations measured in 19 cyst fluid samples from seven patients with craniopharyngioma at various stages of treatment were much higher at 25-671 ng/ml. SDS-PAGE and subsequent western blotting using [(125)I]IGF-II as the ligand gave bands with estimated molecular weights of 330, 220, 135, 96, 46, 43, 34, 29, and 13.5 kDa in one adult, and identical bands at 220, 41.5, 37.5, 32, and 19 kDa in three cyst fluid samples from three children with craniopharyngioma.Conclusions-These results suggest that IGFs and IGF binding proteins are secreted by craniopharyngiomas and that they may alter the growth characteristics of these tumours. Furthermore, the distinct pattern of IGF binding protein sizes might be used as a tool for the differential diagnosis of tumours of the central nervous system.     

Differential expression of cytokeratins 8 and 20 distinguishes craniopharyngioma from rathke cleft cyst

BACKGROUND: Craniopharyngiomas are epithelial neoplasms usually located in the sellar and suprasellar regions. Distinguishing craniopharyngioma from Rathke cleft cyst is sometimes difficult, and the distinction is clinically significant because Rathke cleft cysts have a better prognosis than craniopharyngiomas. DESIGN: We retrieved 10 cases with a primary diagnosis of craniopharyngioma and 5 cases with a diagnosis of Rathke cleft cyst for analysis. Five cases of normal pars intermedia of pituitary glands from autopsy served as controls. We evaluated the expression patterns of a broad range of low- to intermediate-molecular weight cytokeratins (CK7, CK8, CK10, CK17, CK18, CK19, and CK20) and high-molecular weight cytokeratins (K903: a combination of CK1, CK5, CK10, and CK14; and CK5/6) in these cases. RESULTS: Craniopharyngiomas had a cytokeratin expression pattern distinct from that of Rathke cleft cysts and pituitary gland pars intermedia: craniopharyngiomas did not express cytokeratins 8 and 20, whereas Rathke cleft cysts and pars intermedia of pituitary glands both expressed cytokeratins 8 and 20. CONCLUSION: The differential expression of cytokeratins distinguishes between craniopharyngioma and Rathke cleft cyst, and this difference could be useful for identifying craniopharyngioma in difficult cases in which only a small biopsy is available. The different cytokeratin profiles of craniopharyngioma and Rathke cleft cyst suggest that these lesions do not come from the same origin, or that they come from a different developmental stage of the pouch epithelium.     

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.     

Isolated Petroclival Craniopharyngioma with Aggressive Skull Base Destruction

We report a rare case of petroclival craniopharyngioma with no connection to the sellar or suprasellar region. MRI and CT images revealed a homogenously enhancing retroclival solid mass with aggressive skull base destruction, mimicking chordoma or aggressive sarcoma. However, there was no calcification or cystic change found in the mass. Here, we report the clinical features and radiographic investigation of this uncommon craniopharyngioma arising primarily in the petroclival region.     

Retreatment of recurrent cystic craniopharyngioma with chromic phosphorus P 32

A cystic craniopharyngioma in a two-year-old boy recurred six months after surgery and postoperative external-beam radiotherapy. Successful retreatment was accomplished with radioisotope injection of 0.5 mCi of chromic phosphorus P 32 into the intracranial cyst, which delivered approximately 300.00 Gy to the cyst wall. The patient''s symptoms were relieved, and he is without evidence of disease or cystic fluid accumulation four years after intracavitary ³²P irradiation.     

Insulin-like growth factors and IGF binding proteins in cyst fluid from patients with craniopharyngioma prior to intracavitary irradiation with 90Yttrium and thereafter

Aim—To examine a series of cyst fluid samples from patients with craniopharyngioma at various stages of treatment in order to evaluate the use of insulin-like growth factors (IGFs) and IGF binding proteins as tumour markers or indicators of successful treatment, or both.     

Pituitary Adenoma with Cholesterol Clefts

Histologically, cholesterol clefts are often observed in craniopharyngioma, Rathke’s cleft cyst, and various granulomas. However, pituitary adenomas with cholesterol clefts are rare. A 46-year-old woman developed visual field disturbance. She had no history of severe headache that would suggest pituitary apoplexy. She presented with homonymous bitemporal hemianopsia and galactorrhea. Blood prolactin level was 63.1 ng/mL. Other hypophysial hormone levels were within normal range. Magnetic resonance imaging revealed a pituitary tumor with intratumoral cyst. The cyst showed high intensity on T1- and T2-weighted images. The tumor was demonstrated with iso intensity on T1-weighted image and with high intensity on T2-weighted image. She underwent trans-sphenoidal surgery. The tumor was soft, with yellowish, oily fluid, probably the cyst content. By light microscopy with hematoxylin and eosin staining, a typical chromophobic adenoma of the pituitary was identified. Immunostaining revealed immunoreactivity for ACTH in several cells. Many cholesterol clefts and several hemosiderin pigment containing macrophages were observed. Electron microscopy demonstrated a pituitary adenoma with sparse and small secretory granules and numerous lysosomes. The cyst was most likely caused by focal hemorrhagic infarction, followed by the formation of cholesterol crystals, the appearance of hemosiderin containing macrophages, foreign body product cells, and accumulation of lysosomes.     

颈内动脉－眼动脉区域常见病变的CT、MR影像表现

目的 观察CT和MRI获取颈内动脉-眼动脉（ICA-OA）区域常见病变的影像特征,分析病变与ICA-OA的关系,为临床进一步治疗提供帮助.方法 回顾性收集我院2011年6月—2013年12月确诊的194例与ICA-OA区域相关的常见病变患者的影像资料,采用数字影像工作站对病变进行分析.采用五分区法进行研究,即以ICA-OA为轴,将周围分为眶区、鞍区、颞区、鼻窦区和眶颅交界区5个分区,对各区病变相关数据进行统计并分析病变与ICA-OA的关系.结果 194例患者影像资料分析结果显示,ICA-OA区域病变发生率大于5％的常见病变有垂体瘤73例（37.63％）、动脉瘤21例（10.82％）、脑膜瘤18例（9.28％）、Rathke囊肿15例（7.73％）、海绵状血管瘤13例（6.70％）、视神经胶质瘤10例（5.15％）和颅咽管瘤10例（5.15％）,分析病变对ICA-OA产生影响的情况显示,ICA-OA动脉瘤最容易对ICA-OA造成影响.结论 ICA-OA周围结构复杂,常见病变种类较多,通过CT和MRI可以很好地显示病变及其与ICA-OA的关系,为临床诊治提供可靠资料.     

Knee synovial cyst presenting as iliotibial band friction syndrome

We present the case of a 28-year-old competitive runner with iliotibial band (ITB) friction syndrome associated with a synovial cyst. Magnetic resonance imaging (MRI) did not demonstrate a fluid collection. However, open exploration revealed a large cyst beneath the ITB arising from the capsule of the knee proximal to the lateral meniscus. The cyst disappeared on extension. The pre-operative MRI scan may have revealed the cyst, if it had been taken with the knee flexed.     

Foramen magnum arachnoid cyst induces compression of the spinal cord and syringomyelia: case report and literature review

It is very rare that a foramen magnum arachnoid cyst induces compression of the spinal cord and syringomyelia, and currently there are few treatment experiences available. Here we reported the case of a 43-year-old male patient who admitted to the hospital due to weakness and numbness of all 4 limbs, with difficulty in urination and bowel movement. MRI revealed a foramen magnum arachnoid cyst with associated syringomyelia. Posterior fossa decompression and arachnoid cyst excision were performed. Decompression was fully undertaken during surgery; however, only the posterior wall of the arachnoid cyst was excised, because it was almost impossible to remove the whole arachnoid cyst due to toughness of the cyst and tight adhesion to the spinal cord. Three months after the surgery, MRI showed a reduction in the size of the arachnoid cyst but syrinx still remained. Despite this, the symptoms of the patient were obviously improved compared to before surgery. Thus, for the treatment of foramen magnum arachnoid cyst with compression of the spinal cord and syringomyelia, if the arachnoid cyst could not be completely excised, excision should be performed as much as possible with complete decompression of the posterior fossa, which could result in a satisfying outcome.     

Magnetic resonance imaging of the ovarian cyst: its diagnostic value of endometrial cyst

The role of magnetic resonance imaging (MRI) in diagnosing the ovarian cyst was reviewed with emphasis on its diagnostic value of endometrial cyst. 94 cystic ovarian masses in 77 patients were evaluated using 0.15 Tesla MRI just before laparotomy at Okayama University Hospital from 1984 to 1987 and these images were followed up by histopathological data. From these obtained images, 6 parameters; that is (1) laterality and (2) delineation of the cyst, (3) presence or absence of septal image, and (4) homogeneity, (5) signal intensity and (6) T1 value of the cyst contents, were extracted and analyzed respectively. In 95.5% of endometrial cysts, homogenous internal pattern was observed in all 3 images (short spin echo (SE), inversion recovery (IR) and long SE) and its signal intensity was at the same level or higher than that of myometrium. On the other hand, in all cases of follicular cyst, para-ovarian cyst, corpus luteum cyst and in 95.0% of serous cystadenoma and in 90.9% of mucinous cystadenoma, cyst contents showed either lower signal intensity than the myometrium or similar to it in T1 contrast images (short SE and IR). 93.1% of dermoid cyst showed heterogenous signal intensity in all 3 images and their signal intensity levels were widely ranging. High signal intensity of cyst contents in all 3 images could be observed in 72.7% of endometrial cyst, 5.0% of serous cystadenoma with internal hemorrhage, 9.1% of mucinous cystadenoma with internal hemorrhage and 3.4% of dermoid cyst consisted of fatty tissue only. Endometrial cyst showed markedly shortened T1 value (209.6 +/- 102.7 ms) and the other hemorrhagic cyst showed comparatively short value (360-970 ms). On the other hand, T1 value of non hemorrhagic cyst was as high as the level of urine. All of endometrial cysts were clearly defined from the other pelvic structures. In result, endometrial cysts were found to exhibit the characteristic findings in MRI images such as homogenous high signal intensity of internal fluid and clear delineation of cyst contour. The diagnostic accuracy of MRI in endometrial cyst were considered 96.8% in our cases.     

小脑血管母细胞瘤的MR诊断

目的探讨血管母细胞瘤的MRI诊断。方法回顾性分析经手术病理证实的24例小脑血管母细胞瘤，探讨其MRI表现。结果18例血管母细胞瘤位于小脑半球，6例位于小脑蚓部。15例表现为大囊小结节型，结节强化明显；9例表现为实质型；24例瘤内或瘤周均可见血管流空信号。本组MRI正确诊断22例，准确率为91．67％。结论MRI是诊断小脑血管母细胞瘤的有效检查方法，但不典型者需与胶质瘤、转移瘤、动静脉畸形等鉴别。     

脉络膜裂蛛网膜囊肿的解剖学基础及MRI表现

目的:探讨脑部脉络膜裂蛛网膜囊肿的解剖学基础和MR表现,提高对它的认识.方法:同顾性分析32例脉络膜裂蛛网膜囊肿病人的MR资料(其中5例做了增强扫描),并观察12例尸体脑部断层解剖标本.结果:32例均表现为脉络膜裂内类圆形、卵圆形或纺锤形脑脊液信号灶(增强扫描无强化),6例解剖标本脉络膜裂区可见多发小间隙,其中1例可见脉络膜裂囊肿.结论:(1)脉络膜裂蛛网膜囊肿是一种良性的先天性发育异常,脉络膜裂区组织结构较疏松,可能与脉络膜裂囊肿形成有关.(2)利用MR的多种序列及多方位扫描,可做出明确诊断.     

Gartner duct cyst in pregnancy presenting as a prolapsing pelvic mass

Gartner duct cysts are the remnants of the Wolffian duct and they are rarely seen in adulthood. We present a case of a pregnant patient with a prolapsing vaginal mass. A diagnosis of Gartner duct cyst was made after MRI was performed. The Gartner duct cyst was drained when the patient went into labour allowing vaginal delivery to be performed.     

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.