Cervical ganglioneuroma in an adult man: case report and literature review of a rare occurrence.

OBJECTIVE AND IMPORTANCE: Ganglioneuromas are rare benign tumors of the neural crest occurring in early childhood. They are occasionally diagnosed in young adults due to their mass-effect on adjacent structures. We report a case of ganglioneuroma incidentally diagnosed in an adult man. CLINICAL PRESENTATION: A 41-year-old man presented with left-sided cervical radiculopathy symptoms due to degenerative disc disease at the C5-6 and C6-7 levels. The diagnostic radiology work-up revealed a mass in the left side of the neck between the carotid artery and jugular vein. INTERVENTION: Surgical excision of the mass was performed and the histological diagnosis of ganglioneuroma was established. The patient developed left-sided Horner's syndrome post-operatively. CONCLUSION: The present case suggests that these tumors may have insidious presence in the adult population, and therefore, should be considered in the differential diagnosis of a lateral neck mass in asymptomatic adult patients.

Increased expression of p53 protein correlates with the extent of myocyte damage in cardiac allograft rejection.

Acute cardiac allograft rejection (ACAR) has been associated with a poor prognosis. The early diagnosis of ACAR necessitates the accurate detection of myocyte damage. Nuclear damage activates p53, a transcription factor that initiates apoptosis and repair. Endomyocardial biopsies (n=25) from 10 cardiac allograft recipients were stained for nuclear p53. The biopsies were divided into rejection groups based on the grading of ACAR: group 1, grade 0; group 2, grade Ia and Ib; group 3, grades II and III. While clinical indices did not correlate with myocyte damage, significantly more myocytes in group 3 stained for nuclear p53 (2.48+/-0.60/mm(2)) compared with group 1 (0.22+/-0.12/mm(2)) and group 2 (0.43+/-0.18/mm(2)). Increased expression of p53 in cardiac myocytes with grade II or grade III rejection provides an objective quantification as an aid in the diagnosis of ACAR.

Excellent survival, cancer type, and Nottingham grade after atypical lobular hyperplasia on initial breast biopsy.

BACKGROUND: Atypical lobular hyperplasia (ALH) is associated with a 10% to 20% risk of subsequent invasive carcinoma, primarily in the ipsilateral breast. Nottingham grading, special tumor types, and survival after invasive cancer diagnosis were analyzed consistently for the first time. METHODS: A longitudinal follow-up study of 252 women who underwent 261 benign surgical biopsies between 1950 and 1985 with a diagnosis of ALH was undertaken. Subsequent invasive breast cancers were graded and subtyped based on histologic features and the cohort assessed for cancer survival. RESULTS: Forty-eight (19%) women developed invasive breast cancer at an average of 15.1 years. Twenty (42%) of the tumors were special subtype tumors with good prognosis. By an average of 13 years after invasive cancer diagnosis, 2 (10%) of 20 women with special type and variant tumors had died of breast cancer, compared with 9 (32%) of 28 women with tumors of no special type (24 tumors) or an unknown type (4 tumors). Only 1 patient with a tumor of low Nottingham grade died of breast cancer. CONCLUSIONS: ALH is a nonobligate cancer precursor associated with a moderate risk of breast cancer and predicts that later cancers are associated with overall excellent survival. Nearly half of the subsequent cancers show classic or variant patterns of special types with a good prognosis and the majority are of low or intermediate combined histologic grade. Treatment of women with ALH should be influenced by their modest elevation in breast cancer risk and the good prognosis and low mortality of many of these cancers.

Neoadjuvant concurrent paclitaxel and radiation in stage II/III breast cancer.

PURPOSE: The aim of this study was to determine the safety and pathologic response rates following neoadjuvant paclitaxel and radiation in patients with stage II/III breast cancer and to evaluate the use of sequential biopsies to allow an in vivo assessment of biological markers as potential predictive markers of response to this regimen. PATIENTS AND METHODS: Patients with high-risk, operable breast cancer were treated with three cycles of paclitaxel 175 mg/m2 every 3 weeks, followed by twice-weekly paclitaxel 30 mg/m2 and concurrent radiation. Core biopsies were obtained at baseline and 24 to 72 hours after the first cycle of paclitaxel. After completing neoadjuvant treatment, patients underwent definitive surgery. The primary end point was pathologic complete response, which is defined as the absence of any invasive cancer at surgery. Potential markers of therapeutic response were evaluated including markers of proliferation, apoptosis, p21, HER2, estrogen receptor, and progesterone receptor status. RESULTS: Of the 38 patients enrolled, 13 (34%) had a pathologic complete response. There was no significant difference in baseline Ki-67 between responders (35%) and nonresponders (28%; P = 0.45). There was also no significant change in Ki-67 following paclitaxel administration. Despite this lack of immunohistologic change in proliferative activity, baseline mitotic index was higher for patients with pathologic complete response over nonresponders (27 versus 10, P = 0.003). Moreover, the increase in mitotic index following paclitaxel administration was associated with pathologic complete response. CONCLUSIONS: Neoadjuvant paclitaxel/radiation is effective and well tolerated. Tumor proliferation at baseline and response to chemotherapy as measured by mitotic activity may serve as an important indicator of pathologic response to neoadjuvant paclitaxel/radiation.

Concomitant choroid plexus papillomas involving the third and fourth ventricles: A case report and review of the literature.

Choroid plexus papillomas (CPP) are histopathologically benign and rare central nervous system (CNS) neoplasms arising from the epithelium of the choroid plexus. The most common site of presentation of these lesions is in the fourth ventricle in adults and lateral ventricles in children. Third ventricular CPP are uncommon. In this study, we present a case of a 66-year-old woman with complaints of progressive confusion, lethargy, and weakness who was found to have concomitant third and fourth ventricular masses on imaging studies. The patient underwent a biopsy of the third ventricular mass. The biopsy was followed by staged resections of the fourth and third ventricular masses, respectively. Pathology from the biopsy and both resections was benign CPP. Multifocal concomitant CPP is rare. Concomitant CPPs may be secondary to mere coincidental tumor occurrence or to biologic seeding of cerebrospinal fluid (CSF) from a primary CPP despite otherwise benign histopathology. The primary treatment for CPP is surgical resection. Post-operative chemotherapy or radiation for CPP is of controversial benefit.

Adenomyoepithelioma: clinical, histologic, and immunohistologic evaluation of a series of related lesions.

Adenomyoepithelioma, strictly defined, is a proliferation of both epithelial and myoepithelial elements. The broad range of lesions that may fall under this umbrella, however, may be quite diverse. The diagnostic confusion surrounding this entity and its prognostic implications have led to a diagnosis by default as malignant and to overtreatment of some patients. We evaluated available material from a series of 35 women whose slides were seen in consultation and who were diagnosed with adenomyoepithelioma or a closely related lesion. This comprehensive review of the varied histology of adenomyoepithelioma and similar lesions and their immunohistochemical properties will assist general pathologists in evaluating these sometimes difficult lesions. Follow-up and treatment information demonstrates their benignity. Architecture and histologic features should be combined with immunohistochemistry when determining categorization.

Papillary carcinomas of the gallbladder: analysis of noninvasive and invasive types.

CONTEXT: Although papillary carcinomas have been recognized as distinct morphologic variants of gallbladder neoplasms, they have been lumped together in a single group despite the recognition of noninvasive and invasive types. As a result, the biologic behavior of each type remains undescribed. OBJECTIVE: To compare the biologic behavior of noninvasive and invasive papillary carcinomas of the gallbladder. DESIGN: The clinical and morphologic features of 16 noninvasive papillary carcinomas (>1 cm) of the gallbladder were analyzed, and their clinical behavior was compared with that of 370 invasive papillary carcinomas recorded in the Survey Epidemiology and End Results (SEER) Program of the National Cancer Institute from 1973 through 2001. The biologic behavior of invasive papillary carcinomas was compared with that of invasive nonpapillary carcinomas of the gallbladder recorded in SEER. Hematoxylin-eosin-stained sections were available for review in the 16 noninvasive papillary carcinomas. The number of slides examined per case varied from 3 to 16, with an average of 7. RESULTS: The 16 patients with noninvasive papillary carcinomas included 11 women and 5 men, aged 34 to 83 years (mean age, 61 years). Thirteen patients had cholelithiasis. Laparoscopic cholecystectomy was performed on 12 patients and open cholecystectomy on 4. The tumors measured from 1.3 to 8.6 cm and were well to moderately differentiated. Fourteen noninvasive papillary carcinomas showed biliary phenotype, and 2 showed intestinal phenotype. Follow-up was obtained in 11 patients; 6 were asymptomatic 5 to 11 years after surgery, 2 were symptom free 9 months to 4 years following cholecystectomy, and 3 died of unrelated causes 2 to 3 years after surgery. Three hundred seventy cases of invasive papillary carcinomas were recorded in SEER. The 10-year relative survival rate for 225 patients with invasive papillary carcinomas confined to the gallbladder wall was 52%, while the 10-year relative survival rate for 83 patients with papillary carcinomas that had spread to the lymph nodes was less than 10%. Of the remaining 62 invasive papillary carcinomas, 58 had distant metastases and 4 were not staged. The 10-year relative survival rate for invasive nonpapillary carcinomas confined to the gallbladder wall was 30%. CONCLUSION: Noninvasive papillary carcinomas of the gallbladder-regardless of size, cell phenotype, and degree of differentiation-do not metastasize, and a simple cholecystectomy appears to be a curative procedure. In contrast, invasive papillary carcinomas do metastasize and are associated with a poor prognosis (10-year relative survival rate for tumors confined to the gallbladder wall was 52%, while the 10-year relative survival rate for tumors with lymph node metastasis was <10%). The separation of papillary carcinomas into noninvasive and invasive types is clinically relevant and therefore fully justified.

Cylindroma (dermal analog tumor) of the breast: a comparison with cylindroma of the skin and adenoid cystic carcinoma of the breast.

We compared 4 breast cylindromas with 50 dermal cylindromas and 8 adenoid cystic breast carcinomas. Except for a modest increase in the number of eccrine ducts and reactive Langerhans cells in dermal cylindromas, breast and dermal cylindromas showed identical histologic and immunohistochemical features. Both were characterized by epithelial islands containing central basaloid cells and peripheral myoepithelial cells surrounded by a thickened, continuous, periodic acid-Schiff-positive basement membrane that was immunoreactive for collagen IV. Clusters of sebaceous cells and a few eccrine ducts are described in breast cylindromas. Cytokeratin 7 labeled predominantly the central basaloid cells, and smooth muscle actin stained peripheral myoepithelial cells in breast and dermal cylindromas. Eccrine ducts were highlighted by epithelial membrane antigen and carcinoembryonic antigen. S-100 protein and CD1a showed a variable number of dendritic Langerhans cells. Cylindromas of the breast and skin did not express cytokeratin 20, gross cystic disease fluid protein 15, or estrogen or progesterone receptor. Breast cylindroma might be confused with the solid variant of adenoid cystic carcinoma, especially in needle core biopsy specimens, because they share nodular and trabecular patterns, basaloid cells, myoepithelial cells, eccrine ducts, and hyaline globules of basement membrane material. However, adenoid cystic carcinoma displays an infiltrative growth pattern, cytologic atypia, and mitotic figures and lacks the continuous, thickened basement membrane.

Immunohistochemical expression of estrogen receptor in enlarged lobular units with columnar alteration in benign breast biopsies: a nested case-control study.

The prognostic and therapeutic implications of estrogen receptor (ER) status in breast cancer are well known. Whether ER status plays a role in benign breast lesions and the progression to malignancy has not been proven. Enlarged lobular units with columnar alteration (ELUCA), also known as unfolded lobular units, have been associated with mild elevations in subsequent breast cancer risk. We examined the association of ERalpha expression in ELUCA with invasive breast cancer risk. A nested case-control study was performed of women with ELUCA who had undergone benign breast surgery. Eighty-two women who developed invasive breast cancer on follow-up were matched by age and year of biopsy with 166 women who did not develop invasive breast cancer. Entry biopsies were stained for ERalpha (clone 6F11) without knowledge of subsequent cancer outcome. ELUCA lesions were scored as positive if greater than 10% of epithelial nuclei stained with ERalpha and at least one ELUCA contained >50% of cells staining for ERalpha. Relative risks of breast cancer were estimated by odds ratios derived from conditional logistic regression analyses. Thirty-nine percent of cases and 56% of controls had positive ERalpha staining in ELUCA. The relative risk of invasive breast cancer in women with ERalpha-negative ELUCA was 1.85 times that of women with ERalpha-positive lesions (95% confidence interval, 1.0-3.4, P=0.04). The presence of ERalpha staining in women with ELUCA is associated with a lower risk of developing subsequent invasive carcinoma. These findings have implications for risk assessment in benign breast biopsies and are of particular interest given the controversy currently surrounding hormone replacement therapy.

P53 protein is a reliable marker in identification of renal tubular injury.

Acute tubular necrosis (ATN) ranges from minimal histologic changes to overt necrotic tubules. Although histologic changes on routine stained sections can be seen in many ATN cases, they may be subtle in some cases. In some cases, electron microscopy may reveal more reliable findings to support a diagnosis of ATN. Thus, a molecular marker to confirm acute tubular damage and to differentiate mild from moderate tubular injury could provide more reliable detection of ATN at the light microscopic level. In this study, sections from native and transplant renal biopsies with the diagnosis of ATN were stained immunohistochemically for p53, an upstream marker for DNA damage, and compared with donor baseline biopsies as controls. The transplant and native ATN kidney groups had significantly higher numbers of p53 nuclear staining in renal tubular epithelium (transplant ATN: 4.58 +/- 1.51/mm2, n = 18, and native ATN: 6.12 +/- 1.99/mm2, n = 13) than the donor baseline group (1.09 +/- 0.51/mm2, n = 16) or controls-normal renal parenchyma away from tumors (0.029 +/- 0.017). Cases with moderate ATN changes showed significantly increased p53 tubular staining (transplant ATN: 9.20 +/- 2.59/mm2, n = 8, and native ATN: 14.3 +/- 1.88/mm2, n = 5) when compared with the mild ATN cases (transplant ATN: 0.87 +/- 0.30/mm2, n = 10, and native ATN: 1.01 +/- 0.39/mm2; n = 8). In summary, there was direct correlation between nuclear p53 staining and morphologic changes seen microscopically and ultrastructurally, suggesting that p53 can be used as a reliable marker of cellular damage to aid in the diagnosis of ATN.