Desmoplastic small round-cell tumour of the peritoneal cavity: case report and literature review.

Desmoplastic small round-cell tumour is a very rare neoplasm, which usually arises from the abdominal or pelvic peritoneum of adolescents and young adults. Early diagnosis is difficult, because most tumours present with non-specific gastrointestinal symptoms after a long asymptomatic period. It is generally a very aggressive tumour, which grows rapidly with poor prognosis and an overall five-year survival rate of 15% despite multimodal treatment. Despite multiple treatment strategies, the management of desmoplastic small round-cell tumour still remains a clinical challenge and no consensus about a therapeutic protocol has been established. A 35-year-old man presented with mild abdominal pain, constipation and weight gain, and was eventually diagnosed with desmoplastic small round-cell tumour, which was shown to be limited to the abdomen. After incomplete debulking surgery, radiotherapy and chemotherapy, he developed multiple metastatic nodular foci in chest and the pleura and, unfortunately, he died due to disease progression.

Large dermoid cyst of the spermatic cord presenting as an incarcerated hernia: a rare presentation and literature review.

Dermoid cyst of the spermatic cord is a very rare clinical entity with only a few cases reported in the literature so far. We herein describe an extremely rare case of a large dermoid cyst of the spermatic cord measuring 8.5 x 5 x 5 cm in a young patient who presented with clinical manifestations of an incarcerated inguinal hernia. After the cyst excision, a diffuse direct hernia became apparent and a Lichtenstein polypropylene mesh repair was performed. Direct hernia was likely the result of chronic pressure on the inguinal floor maintained by the large cyst. We conclude that although very rare, dermoid cyst of the spermatic cord should be considered as a part of the differential diagnosis in patients presenting with an irreducible inguinal mass of a long course.

Prognostic impact of HER2/neu protein in urothelial bladder cancer. Survival analysis of 80 cases and an overview of almost 20 years' research.

PURPOSE: This study was conducted to evaluate the quantitative assessment of HER2/neu immunohistochemical expression in urothelial bladder cancer in order to determine its prognostic significance. MATERIALS AND METHODS: Archival tumor tissue from 80 patients with primary urothelial carcinoma were analysed for HER2/neu immunohistochemical expression. A highly reproducible standardized procedure on a Bond-X automated slide stainer was used. RESULTS: HER2 protein was overexpressed in 41 of 80 patients (51.25%), demonstrating an increase in the expression rate corresponding to progressively advanced tumor stage (p=0.032) and tumor grade (p=0.0001). Kaplan-Meier analyses showed that positive membranous expression of HER2/neu was not associated with an increased probability of tumor recurrence (p=0.362). In contrast, HER2 scores correlated strongly with specific survival probability (p=0.002) and overall survival (p=0.025). Multivariate analysis revealed that only stage was an independent predictor of specific survival (p=0.016). HER2 expression was an independent predictor of specific survival with borderline statistical significance (p=0.08). CONCLUSION: HER2 overexpression represents a prognostic factor for adverse disease outcome.

Primary pulmonary melanoma diagnosis: the role of immunohistochemistry and immunocytochemistry.

We report a rare case of a primary melanoma of the lung initially presenting with haemoptysis, which was diagnosed using 2 new immunohistochemistry markers and cytology with immunocytochemistry. A 67-year-old male underwent repeated major lung resections and died because of early recurrences of a primary pulmonary melanoma as detailed study excluded other primary foci. The diagnosis of the patient's last recurrence was achieved on a fine needle aspiration (FNA) specimen with immunocytochemistry. To the best of our knowledge this is the first case of primary pulmonary melanoma diagnosed by immunocytochemistry on FNA material.

Pseudomalignant spindle cell proliferation of the urinary bladder: an unusual cause of voiding symptoms in a young patient.

A case of an unusually large pseudomalignant spindle-cell proliferation (or inflammatory pseudotumor) of the urinary bladder in a young man is presented. The only symptom was urinary frequency. The clinical and radiologic evaluation (ultrasonography, computed tomography, and magnetic resonance imaging) revealed a massive tumor (11 x 7.5 x 5.5 cm) arising from the bladder wall that was eventually resected without compromising bladder function. Although the histologic findings initially suggested a sarcoma, the immunohistochemical findings were consistent with that of an inflammatory pseudotumor. The patient remained asymptomatic, without evidence of recurrence, after 12 months of follow-up. Awareness of this inflammatory pseudotumor and its immunophenotypic characteristics is important to avoid an erroneous diagnosis and unnecessarily aggressive therapy.

Intrasellar paraganglioma presenting as nonfunctioning pituitary adenoma.

Paragangliomas arising in the sellar region are rare. We report a case of intrasellar paraganglioma of a 54-year-old man who presented with gradually decreasing visual acuity. Physical examination revealed bitemporal hemianopsia with no apparent signs of endocrinologic dysfunction. Magnetic resonance imaging revealed a large sellar mass believed to be a nonfunctioning pituitary adenoma. The tumor was removed transsphenoidally and submitted for histologic examination. The morphologic features, based on histologic, immunocytochemical, and electron microscopic analyses, were consistent with the diagnosis of paraganglioma. The diagnostic morphologic features and the immunocytochemical profile of the tumor are reported.

Immunocytochemical accumulation of p53 in corticotroph adenomas: relationship with heat shock proteins and apoptosis.

The pathogenesis of corticotroph adenomas is unknown. In a recent study accumulation of p53 protein was detected by immunohistochemistry in a substantial proportion of pituitary corticotroph adenomas, and it has been suggested that it may be causally related to their development. However, other immunohistochemical studies have not confirmed the high incidence of p53 accumulation in this tumor type. Therefore, in the present study, p53 protein accumulation was re-examined in a series of 31 cases of corticotroph adenomas, using different sets of well validated anti-p53 antibodies. Furthermore, in view of the known association of p53 protein with apoptosis, and the known property of p53 to form complexes with heat shock proteins (HSPs), the relationship of p53 accumulation in corticotroph adenomas with apoptosis and HSP-70 was also investigated. Tumor samples from 31 patients with Cushing's disease or Nelson's syndrome were studied. Accumulation of p53 protein was tested by the standard ABC method using two different sets of clone Pab1801 and DO-7 monoclonal antibodies, applied after incubation of sections in a microwave oven. Using the DO-7 antibody, nuclear accumulation of p53 protein was detected in a total of 15 cases, with cytoplasmic staining observed in only 3 tumors. In contrast, using the Pab1801 antibody nuclear staining was observed in only 5 adenomas, with 11 adenomas demonstrating focal cytoplasmic immunoreactivity. Parallel sections of all corticotroph tumors demonstrating cytoplasmic accumulation of p53 protein were tested for the immunohistochemical presence of heat shock protein HSP-70. A striking similar distribution pattern of these two proteins was observed. Apoptosis, identified by the in situ end labeling technique, was detected in a total of 15 out of 28 corticotroph adenomas tested. Calculation of the apoptotic labeling index (ALI) by image analysis showed a significantly lower ALI in those corticotroph adenomas demonstrating nuclear p53 accumulation compared to those with no nuclear p53 immunostaining (p < 0.05). There was no significant difference in the ALI between cytoplasmic p53 positive and negative tumors. It is concluded that depending on the antibody used there is a significant variation of p53 protein detection in corticotroph adenomas. Overall, a significant proportion of corticotroph adenomas studied expressed the p53 protein, which depending on the antibody used, was located either in the nucleus and/or the cytoplasm of tumorous corticotroph cells. Cytoplasmic accumulation of p53, as shown by our colocalization studies with HSP-70, may be due to p53/HSP-70 complex formation. Although such a complex-mediated cytoplasmic exclusion of p53 has no significant effect on apoptosis, nuclear accumulation of p53 protein is associated with a significantly lower apoptotic index indicating a failure of p53 protein to exert its apoptotic action in at least a subset of this tumor type.

Huge non-functioning pancreatic cystic neuroendocrine tumour: a case report.

A case of an 18-cm large cystic, non-functioning endocrine (islet cell) tumour located at the tail of the pancreas in a 29-year-old Caucasian female is reported here. Pre-operative radioimmunoassays for pancreatic peptides were negative, while immunohistochemistry confirmed the neuroendocrine nature of the tumour. This is the largest cystic non-functioning islet-cell tumour so far described.

Apoptosis in human pituitary adenomas: a morphologic and in situ end-labeling study.

Apoptosis seems to be an important process in normal tissues and in neoplastic lesions. Although electron microscopic features of apoptosis are characteristic, it is difficult to detect apoptotic cells with accuracy by light microscopy. Labeling of intranucleosomal DNA fragmentation can provide information on the apoptotic status of tumors. We studied apoptosis by the in situ end-labeling technique in 85 pituitary adenomas (63 functioning, 22 nonfunctioning). The functioning tumors included 19 somatotroph, 17 lactotroph, 9 mixed growth hormone/prolactin-producing, 2 thyrotroph, and 16 corticotroph adenomas. A few scattered cells displaying characteristic apoptotic changes were observed by histologic examination and electron microscopy. We estimated the apoptotic labeling index (ALI) of the adenomas by quantitating the percentages of positive nuclei. Overall, functioning adenomas showed a significantly higher ALI (5.64%) than did nonfunctioning tumors (1.84%). The ALI was higher in thyrotroph adenomas (10.26%) and lower in corticotroph (5.94%), somatotroph (5.51%), lactotroph (5.25%), and mixed growth hormone/prolactin-producing adenomas (5.11%). In conclusion, in situ end-labeling showed that apoptosis mostly occurs in functioning pituitary adenomas. These data suggest that assessment of apoptosis can be used to evaluate drug effects and to define which adenoma subtypes are more susceptible to drug therapy.