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PubMed2001

Intracranial Ewing sarcoma/'peripheral' primitive neuroectodermal tumor of dural origin with molecular genetic confirmation.

Ewing sarcoma/'peripheral' primitive neuroectodermal tumor (ES/pPNET) is the designation given to a family of small cell neoplasms that typically arise in bone or soft tissue and are unified by their common expression of the MIC2 antigen and specific translocations involving a gene on chromosome 22q12 [the most common being t(11;22)(q24;q12)]. ES/pPNET of intracranial origin is extraordinary. We report the case of a 6-year-old boy with a large left frontal region mass that adhered to dura and was extracerebral at surgery. Histologic study revealed a high-grade, undifferentiated-appearing neoplasm of small cell type that was negative on immunostudy for glial fibrillary acidic protein, synaptophysin, desmin, leukocyte common antigen, smooth muscle actin and epithelial membrane antigen, but positive for vimentin and neuron-specific enolase and diffusely labeled by antibody O13 (which recognizes the MIC2 gene product). RNA-based polymerase chain reaction assay confirmed the diagnosis of ES/pPNET by demonstrating fusion transcripts indicative of t(11;22) translocation. Bone scan, computerized tomography of the chest and bone marrow examination revealed no systemic tumor. The limited observations published to date suggest that primary intracranial ES/pPNET is most likely to present in childhood as a circumscribed, contrast-enhancing and dural-based extracerebral mass. It must be distinguished from a variety of small cell neoplasms, particularly PNETs of central neuroepithelial origin.

Brain Neoplasms↗

PubMed2006

The malignant potential of freshly developed colorectal polyps according to age.

BACKGROUND: Although malignant colorectal neoplasms are found more frequently in older population, polyps found at one-time colonoscopy may be a mixture of lesions that developed at various earlier ages. Newly developed adenomas found at the follow-up colonoscopies will reflect the exact relation between malignant potential and the age of development of colorectal polyps. METHODS: The results of 44,065 follow-up colonoscopies on 11,912 subjects were analyzed. The proportion of invasive cancer or high-grade dysplasia among all neoplasms, "proportion of malignancy," was evaluated in relation to age groups (young: <50 years old; middle: 50-59 years old; and old: >or=60 years old). RESULTS: At the follow-up colonoscopies, a total of 8,271 newly developed neoplasms were found, of which 41 (0.50%) lesions were malignant. The proportion of malignancy was 0.35%, 0.31%, and 1.07% in the young, middle, and old age groups, respectively (P(trend) = 0.002). This trend remained significant when stratified by the size of polyps. The proportion of malignancy was higher on the left-side colon than on the right-side colon, except in the old age group, where it was similar on either side. The proportion of malignancy at the follow-ups was not associated with the lesions found at the initial colonoscopies. CONCLUSIONS: The development of malignant lesions in small sizes increased on the colon or rectum at older ages.

Adenocarcinoma↗

PubMed1982

The role of bronchoplastic procedures in the surgical management of benign and malignant pulmonary lesions.

Conservative pulmonary resection is appropriate for the majority of patients with endobronchial neoplasms of low-grade malignant potential in the proximal airways and for a small but significant number of patients with carcinoma. A portion of the bronchus is removed, with or without lobectomy, as a sleeve resection and a primary bronchial reanastomosis is performed to preserve ventilatory function. Bronchoplastic techniques can also be used to repair traumatic airway injuries and benign strictures. The present series of bronchoplastic procedures consists of 28 patients undergoing operation with pathological diagnoses of carcinoma in 20, adenoma in six, hamartoma in one, and a post-traumatic laceration in one. There were minimal morbidity and no deaths. From 1947 to 1981 a total of 565 bronchoplastic procedures have been reported in the literature. Of these, 504 were sleeve resections for carcinoma. The remaining 61 bronchoplastic procedures were for either excision of endobronchial adenomas or repair of airway injuries of strictures. It appears that bronchoplastic procedures are the ideal surgical therapy for benign endobronchial lesions, tumors of low-grade malignant potential, such as adenomas, and for repair of airway injuries. This approach is also applicable to a select group of patients with carcinoma of th lung, and long-term survival periods comparable to those achieved by pneumonectomy can be demonstrated.

Adenoma↗

PubMed2006

A single-nucleotide polymorphism in the XPG gene, and tumour stage, grade, and clinical course in patients with nonmuscle-invasive neoplasms of the urinary bladder.

OBJECTIVE: To evaluate whether the single nucleotide polymorphism (SNP), Asp1104His (G3507C), in the XPG gene affects malignant phenotypes of nonmuscle-invasive urinary bladder neoplasms (NIBN), by investigating associations between the SNP and clinicopathological variables in patients with NIBN. PATIENTS AND METHODS: The 233 patients constituted newly diagnosed cases of primary NIBN in the Stockholm area. The Asp1104His polymorphism in the XPG gene was genotyped using a polymerase chain reaction-restriction fragment length polymorphism technique. RESULTS: The GC + CC genotypes were more frequent in stage pT1 tumours at initial diagnosis than pTa (odds ratio 1.9, 95% confidence interval 1.0-3.5, P = 0.048). The difference was larger in the young group (4.6, 1.9-11.8, P = 0.001). In the young group, the GC + CC genotypes were significantly more frequent in high-grade than in low-grade tumours (3.1, 1.5-6.8, P = 0.004) whereas in the older group the genotypes were less frequent in high-grade tumours (0.3, 0.1-0.7, P = 0.007). The XPG genotypes were not associated with tumour recurrence, stage progression or survival. CONCLUSION: These results suggest that the SNP in the XPG gene might be related to tumour invasiveness in NIBN.

Adult↗

PubMed1990

[The membrane sialoglycolipids of tumor cells and the metastasis of bone tumors].

Level and profile of gangliosides were studied in osteogenic and chondrosarcoma cells. Level of lipid-binding sialic acids in bone- and cartilage-producing tumors proved different. Most osteogenic sarcoma samples showed higher level of lipid-binding sialic acids as compared to chondrosarcoma. In the latter tumor, level of lipid-binding sialic acids was related to grade of tumor cell differentiation, peak levels being observed in undifferentiated neoplasms as compared to those showing grade I-II cell anaplasia. Chondro- and osteogenic sarcoma revealed different profiles of sialoglycolipids, particularly, due to markedly reduced set of gangliosides and nearly complete loss of polysialogangliosides in the latter tumor.

Adolescent↗

PubMed2003

Human DNA topoisomerase-IIalpha expression as a prognostic factor for transitional cell carcinoma of the urinary bladder.

OBJECTIVE: To investigate the immunohistochemical expression of topoisomerase II-alpha (TII-alpha, a nuclear enzyme, the expression of which increases rapidly at the end of the S to G2/M phase and declines when mitosis ends) in bladder urothelial neoplasms (transitional cell carcinoma), and its correlation with grade, stage and survival. PATIENTS AND METHODS: Histological sections from 57 urothelial neoplasms were stained immunohistochemically for TII-alpha expression; the percentage of positive cells in the area of greatest staining was recorded as the TII-alpha index. RESULTS: TII-alpha nuclear staining was positive in all the samples except one. The mean (sd) TII-alpha index was 10.7 (5.9) in urothelial neoplasms of low malignant potential (grade 1), 15.5 (5.0) in low-grade (grade 2) and 42.1 (13.4) in high-grade urothelial carcinoma (grade 3). The mean TII-alpha index was 10.7 (5.9), 26.3 (14.8) and 44 (19) in stages pTa, pT1 and pT2, respectively. The TII-alpha index was significant for predicting death from cancer, independently of the stage or grade of the disease (P = 0.019, hazard ratio 1.1). CONCLUSIONS: A higher TII-alpha index indicates a greater probability of recurrence of disease and lower overall survival. Therefore TII-alpha expression has prognostic value in bladder carcinoma.

Adult↗

PubMed2002

Laparoscopic hepatobiliary and pancreatic surgery: an overview.

Although they are not widely employed, advanced laparoscopic hepatobiliary pancreatic (HBP) procedures can be performed. Laparoscopic common bile duct (CBD) exploration has gained wide acceptance, and endoscopic retro-grade cholangiopancreatography/endoscopic sphincterotomy (ERCP/ES) may become less important in the treatment of CBD stones. Choledochal cyst is another example that is suitable for laparoscopic treatment. It can be removed, and bilioenteric flow is reestablished laparoscopically. Simple cyst of the liver is an excellent indication for laparoscopic surgery. Cysts are unroofed, and recurrence is rare. Hydatid disease can also be treated laparoscopically. In liver resection, the use of laparoscopy is limited to wedge resection and left lateral segmentectomy at most. Laparoscopic staging for pancreatic cancer can demonstrate respectability in 90% of cases. This staging may obviate unnecessary laparotomy. Although laparoscopic Whipple is feasible, laparoscoic distal pancreatectomy is a realistic indication for pancreatic resection. Laparoscopic distal pancreatectomy may be indicated for cystic neoplasms of low-grade malignancy, and for islet cell tumors. When internal drainage is indicated, pseudocysts can be treated laparoscopically. If the cyst is located close to the posterior gastric wall, cystgastrostomy can also be achieved with an endoluminal surgical technique.

Biliary Tract Surgical Procedures↗

PubMed2006

Pentostatin, cyclophosphamide, and rituximab is an active, well-tolerated regimen for patients with previously treated chronic lymphocytic leukemia.

PURPOSE: Purine analogs and alkylators are important agents for treating chronic lymphocytic leukemia (CLL). Early studies combining fludarabine and chlorambucil were abandoned owing to increased toxicity from overlapping myelosuppression and immunosuppression. Of the purine analogs active in CLL, pentostatin appears to be the least myelosuppressive. We previously reported that pentostatin and cyclophosphamide (PC) is active and well-tolerated in patients with relapsed or refractory CLL. Subsequently, we added rituximab, and now report on this three-drug combination. PATIENTS AND METHODS: We treated 46 patients with either previously treated CLL (32 patients) or other low-grade B-cell neoplasms (14 patients). Patients received pentostatin 4 mg/m2, cyclophosphamide 600 mg/m2, and rituximab 375 mg/m2 (PCR). All drugs were administered on the same day (rituximab omitted from cycle 1), and patients received six cycles at 3-week intervals. Filgrastim, sulfamethoxazole/trimethoprim, and acyclovir were administered prophylactically. RESULTS: The median age was 62 years (range, 30 to 80 years). The median number of prior regimens was two (range, one to seven). For CLL patients, there were 24 responses (75%), including eight complete responses (25%). In fludarabine-refractory patients, 75% responded. Toxicity was acceptable, with grade 3/4 infections (including fever of unknown origin) in 28%. The regimen was well tolerated, with 72% of patients receiving the planned treatment at full dose. CONCLUSION: PCR is safe and effective in previously treated patients with CLL. In comparison with our prior two-drug regimen, we find that rituximab did not seem to add significantly to the toxicity, but did appear to confer a survival advantage. Based on these results, we are currently studying PCR as initial therapy for patients with CLL.

Adult↗

PubMed2004

Endolymphatic sac tumor in a 4-year-old boy.

INTRODUCTION: Endolymphatic sac tumors (ELST) are rare, low-grade, locally aggressive papillary neoplasms. We present a case of a 4-year-old boy with an ELST, the youngest described in the literature. CASE: A boy presented with a right-sided serous otitis media and sudden-onset right facial nerve palsy. An audiogram revealed right-sided profound sensorineural hearing loss. Radiographic imaging demonstrated a 3-cm expansile lytic lesion along the posterior face of the petrous bone. INTERVENTION/RESULTS: The patient initially underwent a right transmastoid-infralabyrinthine biopsy. Pathologic examination revealed a papillary lesion suspicious for an ELST. Subsequently, a transtemporal-transcochlear approach with intra-and extradural resection of the tumor was performed. The facial nerve was dissected and transposed anteriorly and preserved. Histopathologic and immunohistochemical studies confirmed the ELST. At his 6-month follow up, there is no evidence of recurrence and the facial nerve function has returned to Grade II palsy. CONCLUSION: ELST are rare tumors of the temporal bone. This is the youngest case of ELST reported. Presentation, evaluation, and management of ELST is discussed.

Adenocarcinoma, Papillary↗

PubMed2001

Pilomatrix carcinoma: a clinicopathologic study of six cases and review of the literature.

Pilomatrix carcinoma, the malignant variant of pilomatrixoma, is a rare entity. The authors report on six patients with pilomatrix carcinoma and review the pertinent literature. The lesions showed a predilection for elderly individuals (mean age, 61 years) with a male:female ratio of 5:1, and they presented as dermal or subcutaneous tumors located on the head and neck (5 neoplasms) and chest (1 neoplasm). Tumors varied in size from 0.6 cm to 2.5 cm (mean, 1.78 cm). None of the lesions recurred after wide local excision. On scanning magnification, all tumors showed the architectural features of a malignant neoplasm (asymmetry and poor circumscription, presence of several markedly sized and variably shaped basaloid aggregations, and ulceration). The tumors were composed of pleomorphic basaloid cells with prominent nucleoli and frequent atypical mitoses accompanied by central areas with keratotic material, shadow cells, and foci of necrosis. The tumor nests were surrounded by a desmoplastic stroma and infiltrated the adjacent tissues. Vascular or perineural infiltration was not observed. In one case, the basaloid cells contained abundant melanin pigment in their cytoplasms. Pilomatrix carcinoma is a neoplasm of low-grade malignancy that should be distinguished from the conventional pilomatrixoma and its variants (aggressive pilomatrixoma and proliferating pilomatrixoma), matricoma, and basal cell carcinoma with matrical differentiation. Clinicians and pathologists should be aware of the occurrence of pilomatrix carcinoma because of its potential for distant metastases.

Adult↗

PubMed1992

Non-Hodgkin's lymphomas arising in patients successfully treated for Hodgkin's disease. A clinical, histologic, and immunophenotypic study of 14 cases.

We report on 14 patients who developed Hodgkin's disease (HD), were successfully treated, and subsequently developed non-Hodgkin's lymphoma (NHL). The median interval between the diagnosis of HD and the diagnosis of NHL was 136 months (range 11-336). The clinical features of the patients with HD were similar to other patients with HD. Results of biopsies showed 12 nodular sclerosis and one mixed cellularity; one was not further classified. Immunophenotypic studies in nine cases showed that the Reed-Sternberg (RS) and Hodgkin's (H) cells were LeuM1+ LCA-. The patients were treated for HD in a nonuniform manner: two received radiation therapy, four received chemotherapy, and eight received both modalities. The NHLs were usually extranodal (79%) with frequent presentation as an abdominal mass. According to the Working Formulation, six lymphomas were small noncleaved cell (four non-Burkitt's, two Burkitt's), three were diffuse large cell, and two were follicular and diffuse large cell. Three neoplasms were not classified: two lymphomas with plasmacytoid differentiation were placed in the intermediate and low-grade categories, respectively, and one neoplasm was a plasmacytoma. All 14 neoplasms had an immunophenotype typical of NHL of B-cell lineage and were LeuM1-. Seven of the 12 patients treated with combination chemotherapy experienced a complete remission of their NHL. We conclude that the clinical, histologic, and immunophenotypic findings of the NHLs in these patients are analogous to those of NHLs that occur in immunosuppressed patients, suggesting that immunodeficiency plays a role in the pathogenesis of NHLs arising after HD.

Adolescent↗

PubMedDate not supplied

Organ preservation in the treatment of brain tumors.

Within the many histological forms, the preservation of function in the central nervous system has always been predominant. However, the limited or null therapeutic interval for high grade gliomas enables organ preservation in small neoplasms only. In case of favorable histology (e.g. dysgerminoma, low grade small glioma), organ preservation is feasible with adequate techniques. When local control is predominant (e.g. neoplasms of eye) the techniques are long known but applied in very few Centers.

Astrocytoma↗

PubMed1992

Stratified diagnostic approach to fine needle aspiration of the breast.

The clinical value of fine needle aspiration (FNA) of the breast is enhanced by incorporating into the cytologic diagnosis explicit comments on the level of diagnostic certainty. This stratification of diagnostic certainty is based predominantly on the cytologic features but occasionally also takes into consideration the clinical situation. Strong clinical and mammographic suspicion of mammary carcinoma associated with FNA, diagnostic of typical, intermediate to high-grade mammary carcinoma, warrants proceeding to definitive therapy without further diagnostic studies. False-positive results are virtually eliminated by placing cases with any uncertainty into a "probable" category, which does not support definitive therapy. In addition, oversimplified "benign versus malignant" approaches to FNA diagnoses ignore the heterogeneity of breast masses, with in situ and low-grade carcinomas warranting special clinical management and usually being placed in the "probable" category. Thus, malignant diagnoses are stratified into "definite" and "probable," with only the former supporting definitive therapy. Within our recent series of 1,005 FNAs of the breast, we were able to confirm the diagnosis in all 62 patients with a "definite" carcinoma diagnosis, and only 3 of 25 "probable" cancer diagnoses were benign at tissue biopsy. Thus, false-positive results were successfully avoided in the "definite" category. Furthermore, a much greater incidence of unusual and good prognosis tumor types were identified by the "probable" category. If the clinical setting is relatively suspicious only, a definitive diagnosis of cancer by FNA is rare and not necessary because the clinical question to be addressed is only whether to biopsy. This approach to FNA diagnosis, unlike the oversimplified "benign versus malignant" scheme, provides an approach that is more likely to result in optimal therapy for breast neoplasms, with low-grade or in situ carcinomas requiring special clinical management since these types of cancers are found predominantly in the "probably malignant" category. It also provides additional security against false-positive diagnoses by incorporating clinical level of certainty statements into FNA diagnostic categories, which more closely reflect the diversity and inherent complexity in the appropriate diagnosis and therapy of mammary carcinomas.

Biopsy, Needle↗

PubMed2001

AgNOR/P53 expression compared with different grades in bladder carcinoma.

OBJECTIVE: The aim of this study is to assess the relation between silver-strained nucleolar organizer regions (AgNOR)/P53 expression and urothelial neoplasm of the bladder which graded according to the WHO/ISUP tumor grading system. METHODS: Transurethral resection materials (TUR) and cystectomy specimens from 52 patients with bladder urothelial carcinomas were stained by AgNORs and analyzed immunohistochemically using an avidinbiotin peroxidase method for p53 staining and also silver for AgNORs. RESULTS: Nuclear p53 accumulation was determined in two of 5 LMP cases (%40), in 10 of 22 LGC (%45.4), and in 8 of 25 HGC (%32). Positivity of p53 accumulation was observed in 20 (%57.7) of all cases. There was no statistically significant relationship between AgNOR score of p53(+) and p53(-) cases. There was no correlation between LGC and LMP, also between LGC and HGC (p > 0.05), but a meaningful relationship was found between LMP and HGC (p = 0.03). Association between P53 and grade, AgNOR value and grade, p53 and AgNOR was found statistically insignificant. CONCLUSIONS: Although we found a statistically significant correlation between AgNOR count of LMP and HGC, this study did not show a satisfying correlation between AgNOR, p53 and any grade of urothelial carcinoma.

Carcinoma, Transitional Cell↗

PubMed2002

Bronchial carcinoid tumors: clinical and radiological correlation.

PURPOSE: To evaluate the incidence of bronchial carcinoid tumor (BCT) in population affected by various lung tumors, retrospectively reviewed between 1986-2001, and to emphasize the radiographic patterns if they are characteristic. MATERIALS AND METHODS: In the above mentioned period, chest x-ray and CT were performed in 1110 patients, both male and female, affected by lung neoplasms. RESULTS: 20 patients were affected by BCT (16 males, and 13 females), aged between 26 and 75 years (mean age 57.5), with histopathologic diagnosis of typical (9 cases, 31%) and atypical (20 cases, 69%) bronchial carcinoid tumors. Localized right lung lesion were as follows: 7 cases in superior, 9 in middle and 4 in inferior lobes; in the left lung, 5 cases in superior, and 4 in inferior lobes. Size of the lesions was as average 2.4 cm in diameter (with a range of 0.5-12 cm). On x-ray and CT images, BCT appeared as a well marginated nodule, of which 9 were peripheral and 20 central. DISCUSSION AND CONCLUSION: BCT are classified as neuroendocrine carcinomas,and are divided in typical and atypical forms, with variable grade of malignancy. Central neoplasms are symptomatic due to bronchial obstruction (i.e., pneumonia, atelectasis, bronchiectasis, emphysema and/or lung abscess); if airway obstruction is partial, then cough, wheezing and recurrent pulmonary infections occur. Peripheral tumors are generally asymptomatic and they are discovered occasionally, when chest x-ray is made for other reasons. Radiographic features are similar in typical and atypical BCT. In central tumors a rounded well circumscribed hilar mass is noted, with lobulated or bumpy margins. Central cavitation is not referred to. Peripheral BCT appear as a solitary nodule, inferioer then 3 cm in size, marginated, surrounded by normal pulmonary tissue. Signs and symptoms of BCT are evasive and vague. No current clinical or laboratory procedures are useful in confirming the diagnosis; particularly, no imaging modalities are able to differentiate between BCT and other pulmonary tumors. For this reason, a clinical radiologic endoscopic and histopathologic approach is necessary. CT is more sensiticve then conventional radiography, especially in detecting small lesions, calcification and enlarged lymph nodes. MRI may be useful in those patients, who cannot tolerate IV contrast media. Scintigraphy may be employed in discovering relapses and long standing metastases.

Adult↗

PubMed1993

Immediate free tissue transfer for distal musculoskeletal neoplasms.

Sarcomas and other aggressive neoplasms of the extremities present particular challenges for limb salvage when the tumors are located distally in the arms or legs. These tumors require a large area of excision, yet there are no suitable options for local tissue coverage. In the past, amputation was often necessary due to the inability to adequately reconstruct the extremity. We performed immediate free tissue transfers on 10 patients with aggressive tumors in the forearm and lower leg. Eight patients had sarcomas, 1 had a melanoma, and 1 had a recurrent fibromatosis. Seven patients had rectus abdominis flaps, 1 had a latissimus dorsi flap, 1 had a groin flap, and 1 had an osteocutaneous flap from the iliac crest. Three patients had tendon transfers at the time of surgery. Four patients required radiation therapy after resection due to the high-grade nature of their neoplasms. The flaps tolerated the radiation well, with only 1 patient sustaining flap necrosis that required skin grafting. Average follow-up was 28 months. All patients are alive and none had local recurrences of their tumors. All free flaps have survived and remained durable. There have been no late breakdowns of skin grafts, and all tendon transfers have functioned well. Immediate free tissue transfer provides an excellent method of reconstruction for distal tumors of the extremities.

Adult↗

PubMed1997

Amplification and overexpression of MDM2 in primary (de novo) glioblastomas.

Glioblastoma multiforme (WHO Grade IV), the most malignant neoplasm of the human nervous system, develops rapidly de novo (primary glioblastoma) or through progression from low-grade or anaplastic astrocytoma (secondary glioblastoma). We recently reported that mutations of the p53 gene are present in more than two-thirds of secondary glioblastomas but rarely occur in primary glioblastomas, suggesting the presence of different genetic pathways (Watanabe et al, Brain Pathol 1996:6:217-24). In the present study, primary and secondary glioblastomas were screened by immunohistochemistry for MDM2 overexpression and by differential PCR for gene amplification. Tumor cells immunoreactive to MDM2 were found in 15 of 29 primary glioblastomas (52%), but in only 3 of 27 secondary glioblastomas (11%; P=0.0015). MDM2 amplification occurred in 2 primary (7%) glioblastomas but in none of the secondary glioblastomas. Only one out of 15 primary glioblastomas overexpressing MDM2 contained a p53 mutation. These results suggest that MDM2 overexpression with or without gene amplification constitutes a molecular mechanism of escape from p53-regulated growth control, operative in the evolution of primary glioblastomas that typically lack p53 mutations.

Adult↗

PubMed1980

Müllerian adenosarcoma of uterine cervix. Report of a case with rapidly fatal outcome.

Müllerian adenosarcoma of the uterus has been regarded as a neoplasm of low-grade malignancy; notwithstanding frequent local recurrences, sometimes after long intervals, the overall prognosis of the three dozen or so tumors reported in the literature has been good. This report documents an unusual clinical course of an adenosarcoma of uterine cervix occurring in an 80-year-old patient. Following clinically disease-free period of 25 months after hysterectomy, the tumor recurred in vaginal vault, grew with devastating rapidity, and proved fatal within 4 months. At autopsy, massive sarcomatosis peritonei and discrete tumor nodules involving the stomach and bowel were found.

Aged↗