Invasive squamous cell carcinoma arising from asymptomatic choristomatous cysts of the orbit. Two cases and a review of the literature

BACKGROUND: Epithelial choristomatous cysts are common orbital lesions, the most frequent of which are dermoid or epidermoid tumors. Massive enlargement or extraorbital extension of these benign lesions may occur. Malignant transformation of the epithelial lining of epidermoid cysts is rarely reported. METHODS: Two patients are presented in whom the epithelial lining of a previously asymptomatic choristomatous cyst of the orbit underwent malignant transformation to produce invasive squamous cell carcinoma. The unusual origin and clinical presentation of the lesions caused a delay in the diagnosis and therapy in both patients. Metastatic workup was negative in both patients, who underwent resection of their tumors via orbital exenteration with craniofacial resection. Reported cases of malignant transformation in analogous choristomatous cysts elsewhere in the cranium are reviewed. RESULTS: One patient is alive and well without recurrent disease 40 months postoperatively. The second patient died of a pulmonary embolus 2 months postoperatively. Autopsy showed no residual tumor. Overall, only 3 of 18 reported patients with epidermoid choristoma of the head and orbit with malignant transformation were alive when reported. CONCLUSIONS: Malignant squamous metaplasia is believed to be a rare complication of orbital dermoid or epidermoid cysts, with only two previously reported cases. However, malignant transformation is relatively frequent in analogous epidermoid cysts found elsewhere in the cranial vault, especially after incomplete excision. These reports encourage the complete removal of epithelial choristomas of the orbit.     

Testicular epidermoid cysts in prepubertal children: case report and review of the world literature

Epidermoid cysts of the testes are rare, benign lesions that account for approximately 1% of all testicular tumors. They present most often between the second and fourth decade of life, and have been reported in prepubertal children rarely. The authors report the clinical and sonographic findings in a 4 1/2-year-old child with a testicular epidermoid cyst, and they analyze the 22 prepubertal cases found in the world literature. As described below, the clinical and sonographic features of this tumor are not specific for an epidermoid cyst and do not preclude a teratomatous or malignant neoplasm. This case adds to the varied sonographic appearance of prepubertal testicular epidermoid cysts. The lack of either unique clinical or sonographic features would suggest that extreme caution be used if local excision is considered for a patient with presumed epidermoid cyst of the testicle.     

Colloid cysts of the third ventricle --report of one operated and one autopsied case (author's transl)

Colloid cysts of the third ventricle are considered as a relatively rate benign tumor, consituting approximately 0.5% of the whole brain tumors, but the surgical cases have rarely been reported in Japan. In this paper, two cases of colloid cyst are reported and the pathogenesis, the importance of the clinical diagnosis and dramatic results after complete extirpation was emphasized. In Case 1, a 29 year-old right handed male, who showed definite symptoms of increased intracranial pressure without lateralizing signs, was diagnosed as colloid cyst of the third ventricle, and operated on with dissecting microscope on April 20, 1973. The colloid cyst was totally removed and postoperatively V-A shunt was performed for adhesive arachnoiditis. Patient did well after surgery and has returned to the previous work. In Case 2, a 41 year-old male, who suffered from sudden onset of severe headache and bouts of frequent vomiting, was admitted to Tokyo Wome's Medical College Hospital on October 11, 1974. Right cerebral angiography revealed findings of increased intracranial pressure and third ventricle tumor was suspected. On October 13, progressively disturbed consciousness occurred. Immediate ventricular tap and continuous ventricular drainage could not regain consciousness and patient expired on October 30. In necropsy, colloid cyst to the third ventricle was found. As far as these are concerned, these cysts might be reasonably said as ventricular epithelial origin in view of the operative and histological findings. Some emphasis was also made in terms of dissecting microscope in removing this tumor, completely preserving the ventricular walls as well as the terminal veins under magnification.     

Hyperthyroidism in early infancy: pathogenesis, clinical features and diagnosis with a focus on neonatal hyperthyroidism

We report here two rare cystic lesions, a lymphoepithelial cyst (LEC) and an epidermoid cyst of the accessory spleen (ECAS) occurring in the pancreas. Histologically, the LEC was lined by stratified squamous epithelium and surrounded by a layer of lymphoid tissue with germinal centers. The ECAS showed similar histologic features with scattered lymphoid tissue, but splenic pulp tissue was present in the wall. In both cysts, some small pancreatic islets and ducts were seen in the fibrous tissue surrounding the lymphoid layer or the splenic pulp, respectively. The lining epithelia of the LEC and the ECAS, as well as those of retention cysts of the pancreas and epidermoid cysts of the spleen used for comparison, were similarly positive with AE1/3, CAM5.2, CK7, CK13, and carcinoembryonic antigen. CA19-9 was also detected in the epithelial cells of the LEC, the ECAS, and the retention cyst of the pancreas, but not in those of the splenic epidermoid or branchial cleft cysts used for comparison. These findings indicate that LECs and ECASs might develop from the pancreatic ducts protruding into a lymph node or accessory spleen located in the pancreas, respectively. Some of both cysts might cause elevated levels of serum carcinoembryonic antigen and/or CA19-9 and should be distinguished from malignant cystic tumors.     

Endoscopic neurosurgery and endoscope-assisted microneurosurgery for the treatment of intracranial cysts

OBJECTIVE: Different endoscopic techniques have been introduced into neurosurgery, but accepted terminology and definitions are still missing. We propose a terminology based on whether the endoscope is used alone or in conjunction with an operating microscope and on whether the route of surgical manipulations is through or outside the endoscope. Accordingly, procedures are categorized into endoscopic neurosurgery (EN), endoscope-assisted microneurosurgery (EAM), and endoscope-controlled microneurosurgery (ECM). METHODS: We treated 36 patients with intracranial arachnoid cysts (ACs) and intraventricular cysts endoscopically. The patients ranged in age from 4 months to 69 years (mean age, 31 yr). The follow-up period ranged from 6 to 44 months (mean follow-up duration, 14 mo). The indications were hydrocephalus in 17 patients, focal neurological deficits in 4 patients, progressive nonlocalizing symptomatology in 13 patients, and space occupation in 2 asymptomatic patients. EN was used in 14 cases, EAM in 15 cases, and ECM in 7 cases. RESULTS: The overall success rate was 70%. Nine patients (25%) had unchanged symptomatology, and the condition of two patients (5%) deteriorated. The best success rates were achieved in patients with intraventricular cysts (89%) and posterior fossa ACs (78%). Symptomatic improvement was best achieved in patients with hydrocephalus or focal neurological deficits (81%). CONCLUSION: Different endoscopic techniques (i.e., EN, EAM, and ECM) provide sufficient treatment of selected intracranial cysts. Our data suggest that intraventricular cysts and suprasellar ACs should be approached using EN whereas posterior fossa and sylvian ACs may be more effectively treated using a combined technique (EAM or ECM).     

Systemic corticosteroid therapy--side effects and their management

True splenic cyst is a relatively rare disease, and the majority of the cases are classified as epidermoid cysts. Three cases of epidermoid cysts in the spleen or accessory spleen were studied using an immunohistochemical technique and staining for mucin. In case 1, serum carcinoembryonic antigen (CEA) and CA19-9, and in cases 2 and 3, serum CA19-9, before surgery were markedly elevated, and these levels decreased postoperatively. This strongly indicates the relationship between the increase of tumor marker levels and the presence of the epidermoid cyst. In addition, stratified squamous epithelium in the resected tissues of cases 1 and 2 was positive for anti-CEA antibody and anti-CA19-9 antibody, and that of case 3 was positive for anti-CA19-9 antibody. This strongly supports CEA or CA19-9 production in the squamous epithelium.     

The SOCS proteins: a new family of negative regulators of signal transduction

An asymptomatic tumor in the pelvis was incidentally found by ultrasonography in a 67-year-old woman while being examined after presenting with a common cold. Further examinations revealed a presacral cystic tumor, which measured 10 x 12cm in size. The cyst was thus removed in the normal manner for such cases. The pathological diagnosis was an epidermoid cyst. An analysis of 15 other cases previously reported in the literature indicated that large epidermoid cysts should normally be excised through an abdominal approach alone, provided that the tumor is benign.     

MRI of intracranial subependymomas

OBJECTIVE: We report imaging features of two cases of subependymoma, a relatively rare benign intraventricular neoplasm. MATERIALS AND METHODS: The first case was a lateral ventricular tumor, which grew to a large size before becoming symptomatic. The second case was a fourth ventricular subependymoma with extensive cisternal spread. RESULTS: In Case 1, absence of postcontrast enhancement suggested the correct diagnosis. However, in Case 2 heterogeneous enhancement made the diagnosis uncertain. In both cases, pre- and postoperative MRI was superior to other modalities in defining tumor extent and delineating the surrounding anatomy. CONCLUSIONS: Magnetic resonance imaging is the modality of choice for studying subependymomas. The diagnosis should be placed among differential considerations for intraventricular tumors.     

Cystic meningiomas resembling glial tumors

BACKGROUND: Meningiomas can be associated with peripheral or intratumoral cysts. Meningiomas with intratumoral cysts, also called "true cystic" meningiomas, are rare and can frequently be confused with glial or metastatic tumors. METHODS: We report three cases of "true cystic" meningiomas and discuss the preoperative evaluation, etiology, and surgical treatment of these cystic lesions with reference to the literature. RESULTS: Magnetic resonance imaging (MRI) and computed tomography (CT) studies usually display a cystic lesion close to the dura with or without enhancement of the cyst wall. Multiplanar MRI scans are very useful to show the presence of a solid tumor or some dural enhancement. Some cases of "true cystic" meningiomas however, are still erroneously preoperatively diagnosed. Complete surgical removal of the tumors and of the entire cyst wall was performed in our cases. Despite the absence of a typical ring enhancement of the cystic lesion in two cases, histopathologic studies displayed the presence of tumor cells on the cyst wall of both cases. CONCLUSIONS: Tumor cells can be present on the cyst wall and therefore can be one of the causes of tumor recurrence if not totally removed. Accurate preoperative radiologic diagnosis (multiplanar MR images) and intraoperative histopathologic studies are fundamental in that they will definitely influence the surgical strategy and outcome.     

Human papillomavirus 57 identified in a plantar epidermoid cyst

We report a 23-year-old Japanese man who had plantar warts on the right sole, beneath one of which an epidermoid cyst developed. On microscopic examination, an acanthotic epidermis markedly invaginated into the underlying dermis, resulting in an open epidermoid cyst. Not only the polymerase chain reaction but also an in situ hybridization detected HPV 57 DNA in the cyst. HPV 60 is the only type of HPV that has been identified in epidermoid cysts. To our knowledge, this is the first case report of an epidermoid cyst, in which a different type of virus from HPV 60 was identified. Histological features of the cyst were also different those of HPV 60-associated epidermoid cysts.     

Apico-aortic shunt: a support technique during surgery on the descending thoracic aorta

Although colloid cysts of the third ventricle are unusual in children, we have recently encountered six examples. Histologically they were lined by cuboidal, pseudostratified or columnar ciliated and mucous-secreting epithelial cells. Two cases showed small microcysts within the fibrovascular stroma surrounding the main cyst. The outermost layer consisted of a glial-ependymal envelope, in keeping with the postulated supraventricular origin of colloid cysts. Scanning electron microscopy showed 10-40% ciliated cells, and no ballooning of non-ciliated cells. Aspiration of cyst contents was performed in three patients, two of whom subsequently required surgical resection 4 months and 8 years after drainage, respectively. In adults colloid cysts may be asymptomatic, whereas in children they have not been documented as incidental findings at autopsy. Two of our six cases died, both before a diagnosis was established. A colloid cyst of the third ventricle must be included in the evaluation of acute neurological deterioration in children, in whom they are more frequently lethal.     

Cystic dilation of extrahepatic bile ducts in adulthood: diagnosis, surgical treatment and long-term results

To evaluate the long-term results of surgery for choledohal cyst in adulthood, a series of 13 patients over the age of 16 operated on for choledochal cyst during a period of six years and followed-up for a minimum of 3 years was analyzed. Patients with type I and IVa cysts underwent extrahepatic cyst resection and Roux-en-Y hepatico-jejunostomy. Choledochoceles (type III) were managed endoscopically. No operative mortality or morbidity occurred. Type I and III cysts showed almost ideal follow-up with no sign of stricture on HIDA scan. One type IVa cyst patients developed recurrent cholangitis due to anastomotic stricture, managed percutaneously. Whenever possible, complete cyst resection and Roux-en-Y reconstruction is the treatment of choice for all extrahepatic biliary cysts. Intra- and extrahepatic dilatations are adequately treated by extrahepatic resection and careful endoscopic or radiologic surveillance. Small choledochoceles can be safely managed by endoscopic sphincterotomy.     

Failure in self care and heart failure, thiamine deficiency in geriatric patients

The ultrasonographic findings of 25 lesions in 23 patients with surgically proven ovarian masses were reviewed. There were 10 cystic teratomas, two simple cysts, two follicular cysts, two mucinous cystadenomas, two NHL, one corpus luteum cyst, one hydrosalpinx, one serous cystadenoma, one yolk sac carcinoma, one dysgerminoma, one embryonal carcinoma, and one mixed form (yolk sac carcinoma, choriocarcinoma). All patients were less than 15 years old. We classified all cases into four patterns: cystic, cystic with mural nodule, mixed, and solid. Eight lesions of the cystic pattern included two simple cysts, two follicular, cysts and one corpus luteum cyst. The other lesions were benign, too. Nine lesions with the cystic with mural nodule pattern consisted of eight cystic teratomas and one mucinous cystadenoma. All lesions were benign. The mixed pattern was seen in four lesions, half of which were malignant, i. e., one embryonal carcinoma and one yolk sac carcinoma. Four lesions with the solid pattern were all malignant masses: one dysgerminoma, two NHL and one mixed form. In this classification, the cystic and cystic with mural nodule patterns are benign, while mixed and solid patterns are highly suggestive of malignancy.     

Our experience in treating obstructive lesions of the main airway by Nd-Yag laser resection

We performed 164 laser resections with a neodymiumyttrium-aluminum-garnet (Nd-Yag) laser in 116 patients between January 1992 and December 1997. Seventy-eight patients had malignant neoplasms, 5 had neoplasms of intermediate malignancy and 33 had inflammatory tracheal lesions. Eighteen resections were emergency procedures. All resections were performed with the patient under general anesthesia and preferably breathing spontaneously. Immediate results varied according to the nature and location of the lesion. Treatment was palliative for tumors showing intraluminal proliferation, providing successful reopening of the airway as shown endoscopically in 70% of patients. Mean survival of the 44 patients with malignant lesions who could be followed was 29 weeks, with a median of 15.19 (range, 1-120). The tracheas of patients with inflammatory stenosis were reopened rapidly and emergency tracheostomy was avoided in all cases. One patient with malignant tracheal tumors died during the procedure due to asphyxia related to tracheal hemorrhage.     

Diverticula of the female urethra. Comparison of imaging techniques

Splenic epidermoid cysts are rare lesions traditionally treated by splenectomy. Concerns about overwhelming postsplenectomy sepsis have led to the development of splenic preservation procedures in the treatment of cystic diseases of the spleen. We present the first case report of successful laparoscopic complete excision of a splenic epidermoid cyst.     

Epithelial cystic lesions of the sellar and parasellar region: a continuum of ectodermal derivatives?

Cystic epithelial lesions of the sellar and parasellar region are classified on the basis of histology and location into Rathke's cleft cysts, epithelial cysts, epidermoid cysts, dermoid cysts, and craniopharyngiomas. A retrospective review of the clinical presentation, radiological findings, and histology was performed on 19 such lesions, and a survey of the literature pertinent to the classification, clinical presentation, and embryology of these lesions was conducted. Presentation was nonspecific and not predictive of histology. Imaging studies were generally useful in distinguishing these tumors, with the exception of Rathke's cleft cysts, suprasellar epidermoid cysts, and craniopharyngiomas, which frequently could not be differentiated. On microscopic examination, most lesions fit into distinct categories; however, overlap was common among all and some could not be definitively categorized by histological criteria. Evidence supportive of an ectodermal ancestry for sellar and parasellar epithelial-lined cystic lesions is presented. Based on the current findings and a review of the literature, it is suggested that these lesions represent a continuum of ectodermally derived cystic epithelial lesions.     

Endoscopic management of colloid cysts

OBJECTIVE: Several surgical approaches have been proposed for the treatment of colloid cysts, which still remains controversial. The most recent technique used is endoscopy. By its nature, endoscopy cannot offer complete removal, as compared to microsurgical techniques, but can do more than puncture. To evaluate the usefulness of endoscopy for colloid cyst surgery, a series of 15 patients who were operated on for colloid cysts under endoscopic control since 1994 was reviewed. METHODS: The presenting symptoms of our patients (10 men and 5 women) were intermittent headache (10 patients), nausea (3 patients), short-term memory loss (4 patients), coma (2 patients), gait disturbance (3 patients), blurred vision (2 patients), and mental status changes (3 patients). The sizes of the cysts ranged from 4 to 50 mm (median, 22.93 mm). Depending on the radiological appearance, the procedure was performed via a right (10 patients) or left (5 patients) precoronal burr hole. A rigid neuroendoscope was used. Initial stereotactic placement of the neuroendoscope was used in two patients who had moderate hydrocephalus. In the other patients, hand-guided endoscopy was performed using an articulated arm. The cysts were perforated with a needle. The opening was enlarged with microscissors. The cyst material was aspirated, and the remaining capsule was coagulated. RESULTS: The average follow-up was 15.26 months (range, 1-28 mo). Total aspiration of the cysts was achieved in 12 patients, as revealed by normal postoperative magnetic resonance imaging. Control magnetic resonance imaging revealed residual cysts in three patients. One patient presented with an asymptomatic recurrence at 1 year. Resolution of the symptoms was obtained in all patients except for two of the four patients with preoperative memory deficit (improvement without complete recovery). There was no mortality or morbidity. CONCLUSION: These results show that endoscopy is a safe and promising percutaneous technique for the treatment of colloid cysts of the third ventricle. Longer follow-up is, however, still required.     

Intrathyroidal lymphoepithelial cyst. A report of two cases not associated with Hashimoto's thyroiditis

Two cases of intrathyroidal lymphoepithelial cyst are described. Both of them were solitary, one being found incidentally in a patient operated on for a multinodular goiter, the other being clinically obvious as a cold nodule. They exhibited features of cysts of branchial cleft origin, i.e. squamous cell lining epithelium and abundant lymphoid tissue with reactive germinal centers. The thyroid gland parenchyma showed a discrete lymphoid infiltration consistent with the diagnosis of focal lymphocytic thyroiditis. In the first case a single epidermoid solid cell nest was found. The histogenesis of intrathyroidal lymphoepithelial cysts remains unclear, but their origin from cystically degenerated ultimobranchial body remnants (solid cell nests) seems to be most probable. This assumption is supported by a similar immunohistochemical profile of solid cell nests and epithelial cells lining the cysts and also by the presence of one solid cell nest in the proximity to the cyst in one of our cases.     

The contrast enhancement of brain and spinal cord tumors using Gd-DTPA in magnetic resonance tomography with an extra-low magnetic-field intensity

The contrasting agent Gd-DTPA (Magnevist, Schering AG, Germany) was tested when used in magnetic resonance tomography using extra-low (0.04 T) magnetic field intensity in 68 patients with various brain and spine tumors. The agent was injected intravenously in a dose of 0.1 mmol/kg. No complications were found. The tests showed its high efficiency in the diagnosis of brain neoplasms (particularly in malignant intracerebral disorders, meningiomas and neurinomas). Benign astrocytomas induced no noticeable signal intensification on post-contrasting T1-weighed tomograms. In all cases of spinal tumors, the application of Gd-DTPA could specify the extent of tumor invasions, the presence of cysts and identify accessory tumor nodes in one case.     

Hemangiopericytoma of the third ventricle. Case report

The authors present the first reported case of a hemangiopericytoma (HPC) occurring in the third ventricle. Most of these lesions are based in the meninges. There is only one other reported case of an intraventricular HPC; in that case the lesion was found in the lateral ventricle. A 40-year-old right-handed man presented with a 3-month history of headaches. Clinical evaluation, including computerized tomography and magnetic resonance imaging studies, revealed a 1-cm enhancing lesion in the third ventricle. Given the findings on the preoperative imaging studies, the lesion was not consistent with some of the more commonly occurring tumors of the third ventricle, namely colloid cysts. A transcortical approach and resection of the lesion was performed without complication. The final pathological findings were consistent with those of an HPC. Hemangiopericytomas rarely occur in the ventricles and may pose a difficult diagnostic dilemma based on their radiographic and gross appearances, as shown in this case. Because of this difficulty, histological confirmation is required to make a definitive diagnosis. These lesions have a propensity to recur and metastasize in the central nervous system and periphery, thus making the goal of treatment a complete surgical resection followed by postoperative radiation therapy in most cases.