Cystic meningioma: report of three patients.

Three patients with cystic meningioma are reported. The computed tomographic scans or magnetic resonance images of these tumors resembled those of a glial or metastatic tumor with cystic or necrotic changes. There is no definitive method for preoperatively differentiating cystic meningiomas from the more common malignant glioma. Angiographic evaluation and collaboration with a neuropathologist are important for the recognition of these potentially curable neoplasms.     

Telomerase activity is a useful marker to distinguish malignant pancreatic cystic tumors from benign neoplasms and pseudocysts

BACKGROUND: Pancreatic serous cystadenoma, mucinous cystic neoplasms, ductal adenocarcinoma with cystic change, and pseudocysts are a spectrum of pancreatic cystic lesions. Their management strategy and prognosis are extremely diverse. Imaging study, cytology, and analysis of the tumor markers of cyst fluid are not always reliable in differentiation of these disease entities. MATERIALS AND METHODS: Fifteen patients with pancreatic cystic neoplasms (including six mucinous cystadenocarcinomas, two mucinous cystic neoplasms with borderline malignancy, two mucinous cystadenomas, and five serous cystadenomas), 4 patients with pancreatic ductal adenocarcinomas with cystic change, and 10 patients with pseudocysts were studied. Echo-guided or computed tomography-guided biopsies of pancreatic cystic lesions and their normal counterparts were conducted on all patients prior to operation or other management. The specimens were assayed for telomerase activity by using TRAP (telomere repeat amplification protocol). The level of telomerase activity in each specimen was semiquantitated as strong, moderate, weak, and none. The final diagnoses were made from histopathological examination of surgically resected or biopsied specimens. The efficacy of telomerase activity as a tumor marker to predict malignancy of pancreatic cystic lesions was evaluated. RESULTS: Three of the four pancreatic ductal adenocarcinomas with cystic change had strong or moderate telomerase activity; four of the six mucinous cystadenocarcinomas had moderate or weak telomerase activity; one of the two mucinous cystadenomas with borderline malignancy had weak telomerase activity; and none of their normal counterparts had detectable telomerase activity. In contrast, none of the two mucinous cystadenomas, five serous cystadenomas, and 10 pseudocysts had detectable telomerase activity. Based on these results, the sensitivity of telomerase activity for prediction of malignancy or premalignancy of pancreatic cystic lesions was 67%, the specificity was 100%, and the positive and negative predictive values were 1.0 and 0.81, respectively. The overall accuracy was 86%. CONCLUSIONS: The differential expressions of telomerase activity have been detected specifically in malignant and premalignant pancreatic cystic tumors, but not in benign cystic neoplasms or pseudocysts. The implications of these results are that telomerase activation takes part in the malignant transformation of pancreatic cystic neoplasms and that telomerase activity is a useful marker to distinguish malignant pancreatic cystic tumors from benign neoplasms and pseudocysts.     

Human cystic kidney diseases: epithelial hyperplasia in the pathogenesis of cysts and tumors

Several examples of human renal cystic disease are associated with tubular epithelial hyperplasia. Micropapillary hyperplasia occurs in autosomal dominant polycystic kidney disease, in localized cystic disease, and in acquired cystic disease; neoplastic or severely dysplastic epithelial hyperplasia occurs in von Hippel-Lindau disease; a histopathologically distinctive epithelial hyperplasia occurs in tuberous sclerosis. In all of these conditions the epithelial hyperplasia appears to be responsible for cyst formation by causing tubular or ductal luminal obstruction, and in all of these conditions, save localized cystic disease (a rare condition with very few reported cases), epithelial hyperplasia imposes an increased risk of malignancy. The risk seems to be highest in patients under treatment with long-term hemodialysis for end-stage kidney disease. Some of these diseases may share common features, but it appears likely that the histopathological differences reflect different features converging on a common result.     

Primary Intraosseous Adenoid Cystic Carcinoma with Widespread Skeletal Metastases Showing Features of High-Grade Transformation

Malignant salivary gland carcinomas arising primarily within the jaw bones are extremely rare. The most common salivary malignancy in these locations is mucoepidermoid carcinoma followed by adenoid cystic carcinoma and adenocarcinoma. The clinical picture and imaging studies of these malignancies may be confused with odontogenic lesions which are more common in this location. Adenoid cystic carcinomas have a prolonged clinical course, tendency for perineural invasion and distant metastasis and multiple recurrences. The diagnosis of these tumors requires thorough histopathologic examination. Immunohistochemical studies may be required in cases showing solid growth pattern. High-grade transformation, earlier termed as dedifferentiation, has been observed in several salivary gland carcinomas including adenoid cystic carcinoma. These transformed tumors are reported to have an extremely poor prognosis. Here, we report a case of primary intraosseous adenoid cystic carcinoma with extensive skeletal metastases which showed a negative staining with p63 and positive staining with CD117. The tumor had a predominant solid growth pattern with areas indicative of high-grade transformation. A negative p63 staining may indicate an incomplete or focal loss of abluminal layer and this is one of the criteria for high-grade transformation in adenoid cystic carcinoma.     

Cystic neoplasms of the pancreas: benign to malignant epithelial neoplasms.

Clinical presentation and state-of-the-art imaging permit the differentiation of most cystic pancreatic neoplasms not only from other cystic pancreatic disorders but also from one another. The differentiation of serous cystic lesions from the mucinous neoplasms (cystadenoma or carcinoma and IPMT) is crucial because of the radically different biological characteristics of these two neoplasms. Although mucinous cystic neoplasms should be resected because of their premalignant or overtly malignant tendency, most patients with serous neoplasms require no operative intervention unless they are symptomatic. IPMT is best treated by a total pancreatectomy, although lesser subtotal resections should be strongly considered depending on patient age, medical comorbidity, and psychosocial situations.     

Cutaneous Metastasis From Adenoid Cystic Carcinoma of the Parotid Gland

BACKGROUND: Cutaneous metastasis from adenoid cystic carcinoma of the salivary gland is very rare. OBJECTIVE: To present an unusual case of cutaneous metastasis from adenoid cystic carcinoma of the right parotid gland. METHODS: A 63-year-old woman with multiple subcutaneous nodules on the abdomen and a gradually enlarged mass over the right parotid area was examined. A skin biopsy was taken from one of the abdominal nodules. RESULT: Skin biopsy demonstrated the characteristic histopathologic features of metastatic adenoid cystic carcinoma. A subsequent computerized tomography of the head and neck revealed a huge soft tissue mass involving the right parotid gland. Computerized tomography of the chest revealed extensive nodular pleural thickening, and pleural biopsy also showed typical histopathologic features of metastatic adenoid cystic carcinoma. All of these results are consistent with the diagnosis of an adenoid cystic carcinoma of the right parotid gland with disseminated metastases. CONCLUSION: We report a rare case of cutaneous metastasis from adenoid cystic carcinoma of the right parotid gland. The presentation of cutaneous metastasis is often nonspecific and may mimic benign lesions. Subcutaneous nodules that are rapidly developing or eruptive, are rapidly growing and have stony hardness in nature, have pain or tenderness, and have nonhealing ulceration remind us of the possibility of cutaneous metastases. Dermatologists and dermatologic surgeons should keep the diagnosis of cutaneous metastasis in mind and always perform skin biopsy when encountering these lesions.     

The differentiation between pancreatic neoplastic cysts and pancreatic pseudocyst.

The number of small and often asymptomatic cystic lesions detected in pancreas has increased during the last decade. Historically the vast majority of the pancreatic cystic lesions were considered pseudocysts, but in recent series the incidence of various neoplastic cysts, such as intraductal papillary mucinous neoplasm, serous cystadenomas and cystic endocrine tumours, has increased. The possible malignant potential in these cystic neoplasms warrants careful diagnostic workup to choose the optimal treatment for each patient. Patient's age, symptoms and a possible history of acute or chronic pancreatitis with known aetiology together with high quality imaging studies are important in the differential diagnosis between pseudocysts and neoplastic cysts. Endoscopic ultrasound, cyst fluid analysis and positron emission tomography may be used in selected patients, but the accuracy of these methods needs further investigation.     

Cystic tumors of the pancreas: diagnosis, management and results

Pancreatic cystic tumours are rare and less frequent than other pancreatic tumours. In recent decades, these tumours are being diagnosed with increasing frequency due to the extensive availability of, and improvement in, modern imaging techniques and it is often possible not only to differentiate them preoperatively from other cystic pancreatic disorders but also from one another. Pancreatic cystic tumours comprise a variety of neoplasms with a wide range of malignant potential: serous cystic tumours are benign, whereas mucinous cystic tumours, and intraductal papillary mucinous tumours are considered premalignant, while solid pseudopapillary tumours have a non-aggressive behaviour in the vast majority of cases. Most patients have no symptoms; and when clinical signs are present, they never help us to identify the type of pathology. Serous cystic neoplasms usually do not mandate resection unless the lesion is symptomatic. Mucinous cystic neoplasms and intraductal papillary mucinous neoplasms have a premalignant or malignant tendency, and therefore need to be managed aggressively by pancreatic resection. Their prognosis is excellent in the absence of invasive disease, but the presence of invasive malignancy is associated with a poor prognosis. This review addresses the symptoms, diagnosis, management and prognosis of this group of tumours.     

Cystic pancreatic neuroendocrine tumors: Is preoperative diagnosis possible?

Pancreatic neuroendocrine tumors rarely undergo cystic degeneration leading to a radiologic appearance, which is often interpreted as a pancreatic mucinous cystadenoma or pseudocyst. We reviewed our experience with 38 neuroendocrine tumors, four of which were cystic, and 24 other cystic pancreatic tumors (mucinous cystadenoma [n = 5], cystadenocarcinoma [n = 6], serous cystadenoma [n = 3], solid/cystic papillary neoplasm [n = 3], intraductal papillary mucinous tumor [n = 6], and mucinous adenocarcinoma [n = 1]) managed operatively between 1990 and 2000. This review was undertaken to identify clinical and pathologic features useful for preoperative diagnosis of cystic neuroendocrine tumors. Two of the four patients with cystic neuroendocrine tumors presented with abdominal pain, one patient was asymptomatic, and one patient had hypoglycemia. Three of the four cystic neuroendocrine tumors were identified by CT scan, and none were biopsied preoperatively. Preoperative diagnoses included mucinous cystadenoma in two patients (n = 2), pancreatic cystic neoplasm in one patient, (n = 1) and insulinoma in one patient (n = 1). All four cystic neuroendocrine tumors were benign and were completely resected (distal pancreatectomy [n = 2], enucleation [n = 2]). Cystic neuroendocrine tumors are difficult to diagnose preoperatively because the majority of these tumors are nonfunctional, and CT does not differentiate these tumors from other cystic neoplasms. Cystic neuroendocrine tumors represent a subgroup of pancreatic cystic and neuroendocrine tumors with malignant potential. Their high resectability rate further supports the role of surgical exploration and resection in the treatment of pancreatic cystic neoplasms. Presented at the Third Americas Hepatopancreatobiliary Congress, Miami, Fla., February 22–25, 2001.     

Retroperitoneal bronchogenic cyst as a differential diagnosis of pancreatic mucinous cystic tumor

Cystic tumors of the pancreas where a pseudocyst has not been able to be excluded has been considered potentially proliferative and pre-malignant or malignant and thus aggressive surgical approach has been advocated. Retroperitoneal cystic tumors are rare and among these bronchogenic cysts are extremely infrequent. The present paper describes a case of bronchogenic cyst in association with the pancreas in which diagnostic work-up was not able to exclude a proliferative pancreatic cystic tumor.     

Carcinoma ex microcystic adenoma of the pancreas: a report of a novel form of malignancy in serous neoplasms

Serous cystic neoplasms of the pancreas are generally considered benign lesions. Malignant counterparts have been occasionally described, and the diagnosis of malignancy is based solely on the presence of synchronous or metachronous metastases to the lymph nodes or liver, direct tumor invasion into adjacent organs, or vascular invasion. However, these malignant serous cystic tumors are lined by benign-appearing glycogen-rich cuboidal cells, which have been morphologically indistinguishable from benign microcystic serous cystadenoma in all the cases reported so far. We report a unique case of microcystic serous cystadenoma giving rise to carcinoma with distinctive histologic features including signet ring-like cells and solid nests. We believe that this case represents the first case of a cytologically malignant neoplasm arising from a benign serous cystadenoma (carcinoma ex microcystic serous cystadenoma).     

Presacral schwannoma with purely cystic form

STUDY DESIGN: Case report. OBJECTIVE: To point out that presacral schwannoma can display markedly cystic degeneration. SUMMARY OF BACKGROUND DATA: Benign schwannoma sometimes displays degenerative changes, such as cyst formation, calcification, hemorrhage, and hyalinization. Usually these degenerations are partially seen in the tumors. METHODS: Medical history, physical findings, imaging features, and histologic findings were reviewed in a case with a large presacral schwannoma. RESULTS: A 62-year-old woman presented with an 18-month history of right sciatic pain. Magnetic resonance images detected a large cystic, presacral tumor. Initially the tumor was misdiagnosed as a cystic ovarian tumor. Preoperative diagnostic workup led to the diagnosis of cystic schwannoma. Intralesional curettage achieved good pain relief. CONCLUSIONS: This tumor was unusual in its totally cystic appearance and resemblance to a cystic ovarian tumor or anterior sacral meningocele. Preoperative diagnosis is important to prevent major neurologic deficit at surgery.     

The changing spectrum of cystic pulmonary lesions requiring surgical resection in infants

Congenital labor emphysema and cystic adenomatoid malformation have been the most common surgically treatable cystic lesions of the lung. With the successful treatment of newborn respiratory distress syndrome, an increased frequency of an acquired form of cystic disease, interstitial pulmonary emphysema, has been observed. Some degree of interstitial pulmonary emphysema is relatively common, and during the years 1980 to 1983 this disease was diagnosed in 372 infants. Seven of these infants, all premature, underwent resection of relatively localized areas of persistent cystic interstitial pulmonary emphysema. Lobectomy or wedge resection was performed in five patients because of their inability to be weaned from the ventilator. A sixth patient with this disease underwent lobectomy for recurrent pneumothoraces. A seventh patient underwent lobectomy because the cystic interstitial pulmonary emphysema produced atelectasis and recurrent infections. All seven patients were extubated by the fourth postoperative day, have been discharged, and are showing respiratory improvement. Within the same period, four infants had congenital lobar emphysema and two had congenital cystic adenomatoid malformation. They were gestational age 36 weeks or older and, although respiratory distress was present to some degree in all six, only one was ventilator dependent at operation. All underwent lobectomy and one infant had a left upper lobe resection and right middle lobectomy on separate occasions for bilateral congenital cystic adenomatoid malformation. All patients with congenital labor emphysema and congenital cystic adenomatoid malformation have been discharged and are doing well. Our results suggest the following conclusions: Persistent interstitial pulmonary emphysema is now the most common indication for pulmonary resection in the newborn period. The anatomic distribution of resected interstitial pulmonary emphysema is similar to that of congenital lobar emphysema. Although only a small fraction (less than 2%) of patients with interstitial pulmonary emphysema require operation, the development of relatively large discrete cystic areas that (1) significantly decrease effective lung volume and produce respirator dependence, (2) produce atelectasis and recurrent infections, or (3) lead to pneumothoraces may make pulmonary resection beneficial. Finally, despite the presence of generalized lung disease in patients with interstitial pulmonary emphysema, these patients can be expected to improve significantly after resection, and the long-term outcome is generally good.     

Cystic angiomatosis (hamartous haemolymphagiomatosis) of bone. A clinicopathological study of three cases

The clinical and radiological features in three cases of cystic angiomatosis of bone are reported. Although these features are generally diagnostic except from histiocytosis X, the definitive diagnosis must be established by a pathological study, preferably of a segment of an involved rib or fibula. The prognosis varies according to the type of clinical presentation-in particular upon whether the lesions are solely skeletal or whether there is extraskeletal visceral involvement. Whereas these last cases may often prove fatal, those with only skeletal involvement have a favourable prognosis: indeed, the cystic bone lesions may regress without any treatment, as occurred in some cases reported in the literature and in two of our three cases.     

Renal cystic diseases: diverse phenotypes converge on the cilium/centrosome complex

Inherited renal cystic diseases constitute an important set of single-gene disorders that frequently progress to end stage renal disease (ESRD). Transmitted as autosomal dominant, autosomal recessive, or X-linked traits, renal cystic diseases are phenotypically diverse with respect to age at onset, rate of disease progression, and associated extra-renal manifestations. These disorders involve defects in a set of gene products commonly referred to as cystoproteins that, while functionally distinct, appear to co-localize, at least in part, with the cilia/centrosome complex. Therefore, investigations are increasingly focused on the role of this complex in the pathogenesis of renal cystic disease. Sorting out the functional relationship between these cystoproteins and the cilia/centrosome complex will undoubtedly provide a better understanding of renal cystic disease pathogenesis and, potentially, identify new targets for therapeutic intervention.     

Cystic nephroma: morphologic spectrum and implications

Among 216 primary renal tumors collected from a pediatric population in metropolitan Chicago, 6 instances corresponded in multiloculated cystic masses conforming to the diagnostic criteria of so-called cystic nephroma. Histopathologically, the component tissues were entirely mature in 2 cases, but in the remaining 4 the septa between cysts contained variable amounts of primitive or embryonic tissues; in one of these, a grossly visible mass formed a nodule of typical Wilms tumor. Our experience agrees with previous reports that favor closer structural similarity of these lesions to nephroblastoma than to any known form of cystic malformation. This concept has important consequences on the therapeutic approach to preoperatively diagnosed multilocular cystic masses of the kidney, and supports the idea that nephrectomy is the treatment of choice. Metastases or local recurrence have not been reported in cases of cystic nephroma.     

Renal cell carcinoma- diagnosis by computed tomography

In approximately 10% of cases, renal cell carcinoma (RCC) could present as a fluid- filled cystic mass. There are three mechanisms by which RCC may become cystic: extensive cystic necrosis, intrinsic cystic growth and origin from the epithelium lining a simple renal cyst. Simple renal cysts are very common. Uncommonly these cysts are complicated by hemorrhage, infection and possibly ischemia. The goal of the radiologist in evaluating these cystic lesions is to distinguish malignant neoplastic cystic masses from non-neoplastic complicated cysts so that appropriate management can be undertaken: RCC is best treated by surgical excision while non-neoplastic complicated cysts do not require surgery. The radiologic findings in these cystic masses which must be carefully evaluated include calcification, abnormal density, septations, nodularity, wall thickening and enhancement.     

Non-neoplastic cystic and cystic-like lesions of the pancreas: may mimic pancreatic cystic neoplasms

BACKGROUND: Cystic lesions of the pancreas consist of a broad range of pathological entities. With the exception of the pancreatic pseudocyst, these are usually caused by pancreatic cystic neoplasms. Non-neoplastic pancreatic cystic and cystic-like lesions are extremely rare. In the present article, the surgical experience with these unusual entities over a 14-year period is reported. METHODS: Between 1991 and 2004, all patients who underwent surgical exploration for a cystic lesion of the pancreas were retrospectively reviewed. Patients with a pancreatic pseudocyst were excluded. There were 106 patients of whom 8 (7.5%) had a final pathological diagnosis consistent with a non-neoplastic pancreatic cystic or cystic-like lesion, including 3 patients with a benign epithelial cyst, 2 with a pancreatic abscess (one tuberculous and one foreign body), 2 with mucous retention cysts and 1 with a mucinous non-neoplastic cyst. These eight patients are the focus of this study. RESULTS: There were six female and two male patients with a median age of 61.5 years (range, 41-71 years). All the patients were of Asian origin including seven Chinese and one Indian. Four of the patients were asymptomatic and their pancreatic cysts were discovered incidentally on radiological imaging for other indications. All the patients underwent preoperative radiological investigations, including ultrasonography, computed tomography or magnetic resonance imaging, which showed a cystic lesion of the pancreas. Three patients, all of whom were symptomatic, were diagnosed preoperatively with a malignant cystic neoplasm on the basis of radiological imaging. Two patients were eventually found to have a pancreatic abscess, one tuberculous and the other, secondary to foreign body perforation. The third patient was found on final histology to have chronic pancreatitis with retention cysts. The remaining five patients had a preoperative diagnosis of an indeterminate cyst; on pathological examination, they were found to have a benign epithelial (congenital) cyst (n = 3), retention cyst (n = 1) and mucinous non-neoplastic cyst (n = 1). At a median follow up of 20 months (range, 3-34 months), none of the patients had any evidence of recurrent disease. CONCLUSION: Non-neoplastic cystic and cystic-like lesions of the pancreas are rare causes of pancreatic cystic lesions that are generally benign and do not require surgery when asymptomatic. However, despite advances in diagnostic investigations such as endoscopic ultrasound with fluid aspirate and magnetic resonance imaging, the preoperative diagnosis remains unreliable. Hence, the challenge for all clinicians is to recognize these lesions preoperatively and to avoid 'unnecessary' surgery.     

Bilateral Subclavian Artery Aneurysm Associated with Idiopathic Cystic Medial Necrosis

Although cystic medial necrosis, either idiopathic or associated with Marian's syndrome, usually becomes manifest as an ascending aortic aneurysm, aortic insufficiency, aortic dissection, or a combination of these disorders, a rare case of bilateral subclavian artery aneurysm secondary to idiopathic cystic medial necrosis has occurred. Subclavian artery aneurysms most commonly represent poststenotic dilatation from anterior scalene or cervical rib compression, occasionally are associated with generalized arteriosclerotic peripheral vascular disease, and rarely are secondary to syphilitic or mycotic infections.Subclavian artery aneurysms have a major risk of rupture, embolus, or thrombosis, and therefore should be repaired. A reverse saphenous vein or prosthetic bypass graft from the carotid to the axillary artery provides adequate flow to the upper extremity. The aneurysm should be completely excised if possible, since reexpansion through small collaterals or through insufficient closure by ligation can occur and compress the brachial plexus after successful bypass. The clinical presentation, angiographic findings, and operative repair of a subclavian artery aneurysm secondary to cystic medial necrosis are described.     

Clinical Management of Large Adrenal Cystic Lesions

The widespread use of ultrasonography and computed tomography has resulted in an increased diagnosis of large sized adrenal cysts with diameters of more than 5 cm. Most of these adrenal cystic lesions are clinically silent and are therefore often diagnosed incidentally. Since up to 7% of adrenal cysts are malignant, a careful hormonal, morpho-functional and instrumental evaluation is mandatory. In particular, functioning adrenal carcinomas or pheochromocytomas have to be ruled out. Fine needle aspiration cytology as well as examination of a punch biopsy specimen of the cystic wall are of limited value, as there is considerable overlap in cytologic and histologic features of benign and malignant adrenal cystic lesions. Immediate surgical excision is indicated in the presence of symptoms, suspicion of malignancy, increase in the size or detection of a functioning adrenal cyst. En bloc adrenalectomy, preferably by a laparoscopic approach, has become the treatment of choice.