Ovarian nonsmall cell neuroendocrine carcinoma: a clinicopathologic and immunohistochemical study of 11 cases

Nonsmall cell neuroendocrine carcinoma (NSCNEC) of the ovary is a rare and aggressive tumor commonly associated with other surface epithelial and germ cell neoplasms. In this study, we present the clinicopathologic and immunohistochemical features of 11 such cases seen at The University of Texas M.D. Anderson Cancer Center in a 16-year period (1990 to 2005). Patients ranged in age from 22 to 63 years (mean 46.7). The most common presentation was abdominal/pelvic pain (6 cases), followed by ascites (2 cases), pelvic mass, vaginal bleeding, and abdominal bloating (1 case each). Tumors were mostly unilateral, cystic, or solid/cystic and ranged in size from 5 to 26 cm (mean 16.2). In 8 cases, NSCNEC was associated with other epithelial neoplasms, including mucinous neoplasms of low malignant potential, mucinous carcinoma, endometrioid carcinoma, mixed endometrioid and mucinous carcinoma, and a high-grade carcinoma, not otherwise specified. In 2 cases, the tumor was associated with a mature cystic teratoma; one of them also containing an invasive moderately differentiated adenocarcinoma. A single case was associated with a benign ovarian cyst. The latter case had a dermoid cyst in the contralateral ovary. NSCNEC represented anywhere from 10% to 90% of the ovarian tumor. Microscopically, the neuroendocrine component was usually composed of large and/or intermediate oval to round cells. In 2 cases, the intermediate cells were intermixed with small cells. Three cases had also spindle cells. The neoplastic cells were mostly arranged in a solid pattern, nests, or trabeculae. All tumors had a brisk mitotic activity. Immunoperoxidase studies for keratin cocktail, cytokeratin (CK) 7, CK20, CAM 5.2, chromogranin A, synaptophysin, NSE, CD56, and c-kit were performed and the cases stained as follows: keratin cocktail 6/6, CK7 4/5, CK20 3/5, CAM 5.2 3/3, chromogranin A 8/11, synaptophysin 9/9, NSE 1/1, CD56 4/8, and c-kit 5/7. According to the International Federation of Gynecology and Obstetrics staging system, 4 cases were stage I tumors, 3 cases were stage III tumors, and 4 cases were stage IV tumors. Seven patients were treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by chemotherapy. One patient had a bilateral salpingo-oophorectomy with omentectomy and appendectomy followed by chemotherapy; 1 patient had a total abdominal hysterectomy with right salpingo-oophorectomy followed by chemotherapy; one had a bilateral salpingo-oophorectomy followed by chemotherapy, and one had a right salpingo-oophorectomy with appendectomy followed by chemotherapy. Five patients died of disease at 2, 3, 9, 20, and 36 months. One patient is alive with disease at 8 months and 5 are alive without evidence of disease at 11, 28, 37, 66, and 68 months. Four of 5 patients who died of disease had either stage III or IV tumors and 3 of 5 patients who are alive without evidence of disease have stage I tumors. In summary, ovarian NSCNEC is an aggressive tumor with a tendency to present at advanced stage and cause death within a mean of 17 months after diagnosis; however, some patients, particularly those with stage I disease and/or those who have received platinum-based therapy, may have a more favorable prognosis.     

Endometrial stromal sarcoma metastatic to the lung: a detailed analysis of 16 patients

Pulmonary metastases of endometrial stromal sarcoma (ESS) are uncommon and can pose diagnostic problems. We reviewed lung specimens from 16 patients with metastatic ESS. Patients were 31-77 years of age at the time of lung biopsy. Uterine ESSs were diagnosed an average of 9.8 years before lung biopsy in 11 patients. Uterine ESSs were originally called smooth muscle tumors in three additional patients. Thirteen patients were evaluated for new pulmonary nodules, seven of whom were asymptomatic. Nodules were multiple in 14 and solitary in four, ranging from 1.0 to 8.0 cm in greatest dimension. One patient died of metastatic disease; 14 were alive and seven of these were without disease (mean follow-up 4.1 years). Diagnostic considerations in 12 consultation cases included ESS, sclerosing hemangioma, carcinoid tumor, lymphangioleiomyomatosis, endometriosis, hemangiopericytoma, and lymphoma. Tumors were well circumscribed and usually solid, composed of plump spindle cells arranged in short fascicles. Two tumors were predominantly cystic. Sex cord-like stromal differentiation was identified in three. Neoplastic cells stained for vimentin (93%), estrogen and progesterone receptor (100%), smooth muscle actin (57%), desmin (50%), and keratin (46%). Metastatic ESS should be included in the differential diagnosis of nonepithelial neoplasms in women.     

Mesonephric adenocarcinomas of the uterine cervix: a study of 11 cases with immunohistochemical findings

Mesonephric adenocarcinoma is a rare variant of cervical carcinoma with relatively few, well-documented cases reported. We describe the clinicopathologic and immunohistochemical features of 11 examples of this neoplasm, which occurred in women between the ages of 35 and 72 years (mean, 52 years). Most (64%) patients had abnormal vaginal bleeding. Eight tumors were stage IB, and one each was stage IIB and IVB; in one, the stage was unknown. Microscopically, the carcinomas showed various morphologies, most commonly a small tubular pattern or a ductal pattern resembling endometrioid adenocarcinoma; one tumor had an associated malignant spindle cell component. Ten neoplasms were adjacent to hyperplastic mesonephric remnants. Follow-up in 10 cases showed six patients to be alive without evidence of recurrence after a mean of 4.8 years. The patients with stage IIB and IVB disease had local recurrences after 2.2 and 0.7 years and died of progressive disease at 3.2 and 0.8 years, respectively. In a patient with stage IB disease, a mediastinal metastasis and a malignant pleural effusion developed 5.6 years after diagnosis, and the patient died of disease at 6.2 years. Another patient with stage IB disease and a positive vaginal cuff margin that recurred locally after 1.7 years received chemotherapy and was alive and clinically free of disease at 2.5 years. Mesonephric adenocarcinomas were immunoreactive for epithelial markers (AE1/3; CK1, CAM 5.2, cytokeratin 7, and epithelial membrane antigen) (100%), calretinin (88%), vimentin (70%), androgen receptor (33%), and inhibin (30%, focal staining). No immunostaining was detected with cytokeratin 20, estrogen receptor, progesterone receptor, and monoclonal carcinoembryonic antigen. This staining profile is similar to that of mesonephric remnants and may be useful in the distinction of mesonephric carcinoma from mullerian endometrioid adenocarcinoma, with which it may be confused.     

Endometrioid proliferative and low malignant potential tumors of the ovary. A clinicopathologic study of 46 cases

We evaluated 41 endometroid neoplasms with features intermediate between a benign endometrioid tumor and endometrioid carcinoma. Although these tumors showed various degrees of epithelial proliferation, they lacked the destructive stromal invasion of carcinoma. Intermediate endometrioid tumors were subdivided into proliferative endometrioid tumors (PET), endometrioid tumors of low malignant potential (ETLMP), and ETLMP with microscopic areas of invasion. PET were adenofibromas with solid aggregates of epithelial proliferation not exceeding 5 mm in any dimension, whereas ETLMP either had noninvasive cytologically malignant epithelium or aggregates of atypical epithelium measuring at least 5 mm in any dimension uninterrupted by fibromatous stroma. Of the seven PET, five were purely adenofibromatous, while two were mixtures of adenofibromatous and papillary components. Of the 31 ETLMP, 12 were adenofibromatous and 19 were either purely papillary or had mixtures of papillary and adenofibromatous components. An additional three ETLMP had one or more areas of microscopic invasion of the stroma in the form of an irregular or cribriform infiltration by atypical glands, often with squamous differentiation. These three neoplasms were designated "ETLMP with microinvasive carcinoma." The only neoplasm with extraovarian implantation at presentation, however, was an ETLMP with mixed adenofibromatous and papillary features, without microinvasion. None of the other patients with ETLMP had a metastasis or developed one within a follow-up period of between 0.8 and 11.2 years. Because they are very low-grade neoplasms, ETLMP should be separated from endometrioid carcinoma and not confused with PET, because PET have no malignant potential.     

Primary endometrioid adenocarcinoma of the vagina: a clinicopathologic study of 18 cases

Vaginal adenocarcinoma is the second most common primary cancer of the vagina, yet there has been very little study of most subtypes other than clear cell carcinoma. We reviewed 18 cases of primary vaginal endometrioid adenocarcinoma, in our experience the second most common subtype. The patients ranged from 45 to 81 years of age (mean 60). Most presented with vaginal bleeding, and had had a prior hysterectomy. Five had a history of unopposed estrogen therapy but none had a history of intrauterine diethylstilbestrol exposure. The tumors were at the vaginal apex in 10 cases, in the posterior wall in 3, the lateral wall in 3, and the anterior wall in 1. On microscopic examination, each of the tumors had a pure or predominant component of typical endometrioid adenocarcinoma. There was squamous metaplasia in 4 cases, mucinous metaplasia in 4, and prominent nonvillous papillae in 2. The tumors were grade 1 of 3 in 4 cases, grade 2 in 13, and grade 3 in 1. Eleven cases were FIGO stage I, 5 stage II, and 2 stage IV. Vaginal endometriosis was identified in 14 cases, and is important in indicating a primary vaginal tumor, rather than secondary spread from the endometrium. Other subtypes of adenocarcinoma (such as serous when the tumor has a papillary pattern) and atypical forms of endometriosis, including polypoid endometriosis, are the most common other differential diagnostic considerations. The prognosis seems to be good in low-stage patients, with 11 patients alive and well and 2 alive with recurrent disease.     

Endometrioid neoplasms with clear cells: a report of 21 cases in which the alteration is not of typical secretory type

The presence of clear cells in genital tract neoplasms often reflexly prompts the diagnosis of a clear cell tumor but clear cells may be seen in many other neoplasms. In one such, those of endometrioid type, they are well known and generally readily characterized when they have the secretory pattern that recapitulates the well-known morphology of early secretory endometrium. In this report, we describe various clear cell morphologies, some possibly related to the secretory variant, but others not, and all potentially the source of significant diagnostic difficulty. We reviewed 21 endometrioid tumors that occurred in patients from 27 to 88 (median 64) years. Most had an adnexal mass (13) or abdominal swelling (4), but 4 presented with vaginal bleeding. One tumor formed a cystic mass in the pelvis, 1 tumor involved the right fallopian tube, 1 the endometrium, and 18 the ovary. One tumor was a cystadenofibroma, 1 a borderline tumor, and 19 were adenocarcinomas. Twelve patients had stage I, 4 stage III, 1 stage IV, and 4 were unstaged. One patient who had a previous hysterectomy and salpingo-oophorectomy underwent resection of a cystic pelvic mass; others all were treated with abdominal hysterectomy and salpingo-oophorectomy. All the neoplasms had the typical architecture of endometrioid tumors but differed markedly in their cytoplasmic features. In 18 of the tumors at least one-third of the cells had clear cytoplasm and 3 had only clear cells. The clear cytoplasm varied from foamy to empty and the nuclei had a variable location, basilar, central, and apical. The clear cells were associated with squamous differentiation in only 1 case in which the change appeared hydropic. By immunohistocytochemistry, the clear cells were focally positive for epithelial markers in most cases but in some cases one or more of these immunostains were negative. Tubulocystic, solid, and papillary patterns of clear cell carcinoma were absent. Periodic acid-Schiff was focally positive for glycogen in the cytoplasm in 5 cases. Although a few cases had a focal slight resemblance to secretory endometrioid carcinoma most did not and the orderly morphology of classic secretory carcinoma was noteworthy for its absence. The nature of the cytoplasmic clarity is usually uncertain but is likely variably owing to lipid, mucin or glycogen accumulation, or is a hydropic change. The distinction from clear cell carcinoma depends on awareness of this unusual variant of endometrioid neoplasia and a lack of the distinctive patterns of clear cell carcinoma; at this time, special studies, including immunohistochemistry, do not aid significantly although certainly negative reactions, such as for thyroglobulin protein (arguing against clear cell struma ovarii) may play a role in some differential diagnostic considerations. There are prognostic and therapeutic implications in the distinction with clear cell carcinoma.     

The surgical management of renovascular hypertension in children and young adults.

OBJECTIVES: To assess the outcome and durability of operative revascularisation in young patients with renovascular hypertension. DESIGN: Retrospective study. METHOD: The records of all young patients (under 25 years) operated on for renovascular hypertension at St Mary's Hospital 1988-1998 were reviewed. We assessed the aetiology of hypertension, operations performed, effect of treatment on blood pressure, renal function and requirement for antihypertensive medication during follow-up. RESULTS: Ten patients were identified who had been considered for surgery, of median age 16 years (22 months to 22 years). Fibromuscular dysplasia was present in five patients, mid-aortic syndrome (MAS) in four and neurofibromatosis in one. Operations performed were aortorenal bypass (three), aorto-aortic bypass+/-renal bypass (three), splenorenal bypass (one) and autotransplantation (one). Of the three patients treated by balloon angioplasty, only one had a successful result. One patient with MAS is currently awaiting surgery. Over a median follow-up of 24 months (8-144), seven patients are normotensive off all antihypertensive medication. Of two patients on reduced doses of medication, one (splenorenal bypass) required surgical repair of a late (9 years) coeliac stenosis. CONCLUSIONS: The surgical treatment of renovascular hypertension in carefully selected young patients gives durable results. Blood pressure is well controlled long-term, and the need for antihypertensive medication is removed altogether in the majority of patients.     

Paratesticular liposarcoma: a clinicopathologic study

Paratesticular liposarcomas are rare and typically reported as isolated cases or as components of larger studies of liposarcomas. We studied a series of these tumors. All cases of paratesticular liposarcomas were retrieved from the archives of the Royal Marsden Hospital and the Johns Hopkins Hospital. Slides were reviewed and clinical information obtained. There were 30 paratesticular liposarcomas from men aged 41-87 years (mean 63 years; median 65 years) that involved the spermatic cord (23, 76%), testicular tunics (6, 20%), and epididymis (1, 4%). Tumors ranged from 3 to 30 cm (mean 11.7 cm; median 10 cm). Nineteen were well-differentiated liposarcomas (WDLs) and 10 were dedifferentiated liposarcomas (DDLs, five with high-grade and five with low-grade dedifferentiation). One was a myxoid/round cell liposarcoma with 70% round cell areas. All patients were treated by radical orchiectomy. One patient with WDL received radiation after his second recurrence and the myxoid/round cell liposarcoma received radiation and chemotherapy. Follow-up information was available for 16 of the patients, including 10 WDLs (range 24-216 months, mean 106 months), 5 DDLs (14-30 months, median 24 months), and for the myxoid/round cell liposarcoma (14 months) (range for all cases 14 months to 22 years; mean 87 months, median 36 months). Six of the WDLs (60%) recurred at 2, 4, 6, 10, 18, and 21 years (median 8 years). The lesion that recurred at 18 years (case no. 6) displayed foci of high-grade dedifferentiation in the recurrence, although the patient was disease free at 19 years. One patient with WDL had two recurrences at 4 and 7 years, and another had six recurrences over a 17-year period. Only one example of DDL recurred, at 30 months; another patient, who refused therapy for 15 years, had a primary tumor 30 cm in diameter, displayed pulmonary metastases 1 month after excision, and died after 14 months. The patient with MRCL had abdominal metastases after 1 year and was alive at 14 months. In summary, paratesticular WDL had a prolonged course with recurrences in more than half the cases, sometimes late. There were no metastases and the overall prognosis was good. One DDL recurred and only one of five (20%) developed metastases, but the mean follow-up for DDL was only 24 months.     

Ovarian endometrioid tumors of low malignant potential: a clinicopathologic study of 30 cases with comparison to well-differentiated endometrioid adenocarcinoma

Thirty cases of ovarian endometrioid tumor of low malignant potential (ETLMP) were studied and compared with 32 cases of well-differentiated endometrioid adenocarcinoma. ETLMP was distinguished from well-differentiated endometrioid adenocarcinoma by the absence of destructive stromal invasion, glandular confluence, or stromal disappearance. Intraepithelial carcinoma in a low malignant potential tumor was defined as areas showing grade 3 nuclei, sometimes associated with an intracystic villoglandular or cribriform pattern. Microinvasion in an ETLMP was defined as one or more areas of invasion with an area of < or =10 mm2. Because a cribriform pattern may be seen in purely intraglandular proliferations, the latter was not taken as evidence of invasion. The patients with ETLMP ranged from 28 to 86 years of age (mean 54.9 years), and only one patient (3%) had other than stage I disease at presentation. The patients with well-differentiated endometrioid carcinoma ranged from 26 to 87 years of age (mean 51.1 years), and three patients (9%) had stage II disease at presentation. An adenofibromatous pattern was present in 47% of cases of ETLMP and squamous differentiation in 47%; intraepithelial carcinoma occurred in 7% of cases and stromal microinvasion in 7%. None of these findings appeared to influence the prognosis because all patients with ETLMP were free of recurrent disease or metastasis on follow-up, whereas 20% of patients with well-differentiated endometrioid adenocarcinoma followed for >6 months developed recurrent disease. Thus, the prognosis of ETLMP, when defined by the above criteria, is favorable and is superior to that of well-differentiated endometrioid adenocarcinoma.     

Peritoneal serous micropapillomatosis of low malignant potential (serous borderline tumors of the peritoneum). A clinicopathologic study of 17 cases.

Primary peritoneal serous micropapillomatosis of low malignant potential, or serous borderline tumor of the peritoneum, is a relatively rare lesion that is histologically indistinguishable from peritoneal "implants" associated with ovarian papillary serous tumors of low malignant potential. We analyzed 17 cases to further define the pathologic features and prognosis of this entity. The ages of the patients ranged from 16 to 67 years (mean, 33 years). Eight patients were symptomatic with chronic pelvic or abdominal pain (five patients), adnexal mass (one patient), small-bowel obstruction (one patient), and possible endometriosis (one patient). In nine cases (53%), peritoneal serous micropapillomatosis of low malignant potential was an incidental finding discovered during evaluation or treatment of other conditions. Grossly, the peritoneal lesions were focal or diffuse. They commonly appeared as miliary granules and often were believed to be peritoneal carcinomatosis. Microscopically, peritoneal serous micropapillomatosis of low malignant potential had all of the patterns seen in superficial ("noninvasive") peritoneal implants of ovarian serous borderline tumors. Psammoma bodies were a prominent feature of all cases. Twelve patients also had typical endosalpingiosis. Most patients were treated by hysterectomy and bilateral salpingo-oophorectomy. Surgical treatment in seven patients consisted only of biopsy. Ten patients had residual unresected disease at the time of their initial operation. Several patients received adjuvant chemotherapy. Follow-up was available for 14 of the 17 patients. One patient died of metastatic breast carcinoma at 3.8 years; another patient died 7 weeks after operation, possibly as a complication of therapy. The other 12 patients were alive at last known contact after follow-up intervals of 8 months to 16.2 years (mean, 7.5 years). Two of these 12 patients developed multiple episodes of small-bowel obstruction due to persistent peritoneal serous micropapillomatosis of low malignant potential; neither received adjuvant chemo- or radiotherapy. Both were alive without progressive disease 10.9 and 16.2 years after initial diagnosis, respectively. This excellent prognosis supports a regimen of conservative therapy for these patients.     

Leiomyosarcoma of the paratesticular region: a clinicopathologic study.

The behavior of leiomyosarcoma (LMS) is site related, but there are limited data on such tumors presenting in the paratesticular region. Cases diagnosed as LMS of the paratesticular region from the files of three institutions were reviewed. Immunohistochemistry was performed in cases with available blocks, and follow-up information was obtained. From 31 cases originally diagnosed as LMS, 24 were retained after review. These were from men aged 34-86 years (mean 62 years; median 64 years) and involved the testicular tunica (10), spermatic cord (10), scrotal subcutis and dartos muscles (1 each), and the epididymis (1). Tumors ranged in size from 2-9 cm (mean 5 cm; median 4 cm). On immunohistochemical staining they expressed muscle-specific actin (13 of 14), smooth muscle actin (10 of 10), desmin (16 of 17), and CD34 (3 of 9); all of the latter three were strongly desmin-positive. Focal reactivity for cytokeratin (3 of 8) and S-100 protein (1 of 8) was seen. Follow-up information was available in 14 patients. Four (29%) had recurrences, in one case four times. Metastases to lymph nodes, lungs, or liver were seen in four patients (29%), of whom two had prior recurrences. Ten were alive with no evidence of disease (ANED), and four were dead of disease (DOD). Comparing outcome with tumor grade, all seven patients with grade 1 tumors (of whom two had recurrences) and all three with grade 2 tumors were ANED, whereas all four patients with grade 3 tumors were DOD. In summary, paratesticular LMSs are rare neoplasms. The majority in this site are low-grade, although high-grade lesions behave aggressively.     

A clinicopathologic analysis of atypical proliferative (borderline) tumors and well-differentiated endometrioid adenocarcinomas of the ovary

Atypical proliferative (borderline) endometrioid tumors (APTs) and well-differentiated endometrioid carcinomas of the ovary constitute a spectrum of morphologically diverse proliferative tumors. There is currently no agreement on the criteria for distinguishing them. We report the clinicopathologic features of 56 proliferative endometrioid tumors focusing on the criteria for invasion, the clinical significance of microinvasion and cytologic atypia, and prognosis. Endometriomas, adenofibromas, adenosarcomas and moderately to poorly differentiated carcinomas were excluded, as were patients with concurrent endometrioid carcinoma of the endometrium. The tumors were classified as atypical proliferative tumor (APT) (33 tumors), APT with intraepithelial carcinoma (high-grade cytology in a tumor lacking stromal invasion) (three tumors), APT with microinvasion (invasion <5 mm) (five tumors), and invasive carcinoma (invasion > or = 5 mm) ( 15 tumors). All tumors were confined to the ovary (stage I). In 50 patients, the tumor involved one ovary, and in three patients, the tumors were bilateral. The predominant growth pattern was adenofibromatous in 29 tumors and glandular or papillary in 27 tumors. In 8 (24%) of 41 APTs, areas of benign adenofibroma were identified, and in 13 (87%) of 15 carcinomas, areas of associated APT were identified. Stromal invasion was manifested by confluent glandular growth in all 15 invasive carcinomas and all tumors with microinvasion. Destructive infiltrative growth was also present in 2 (13%) of 15 carcinomas. Confluent glandular growth was the most common manifestation of stromal invasion and therefore served as the best criterion for the diagnosis of carcinoma. Squamous differentiation was observed in 24 tumors, and mucinous differentiation was seen in 20 tumors and was most often seen in APTs. Endometriosis was present in 14 patients with APTs and one patient with carcinoma. Four patients had hyperplasia or atypical hyperplasia of the endometrium. One patient with an APT had a concurrent peritoneal serous neoplasm. Twenty-one patients had available clinical follow-up. Twenty (95%) of 21 patients, including six with invasive carcinoma, two with microinvasion, one with intraepithelial carcinoma, and 11 with APT were alive with no evidence of disease with a mean follow-up of 47 months. One patient with carcinoma had recurrent tumor after 46 months and was alive 40 months after resection of the recurrent tumor. In this large series of proliferative endometrioid tumors, all were stage I and only one patient had a recurrence. Most carcinomas contained evidence of a precursor APT, and in some APTs, an associated benign adenofibroma was identified. Microinvasion or intraepithelial carcinoma occurred in 19% of APTs. This finding likely reflects the various stages of endometrioid carcinogenesis in the ovary. For clinical management, we suggest that these tumors be divided into two categories-APTs and well-differentiated carcinoma-because based on the available data, cytologic atypia and microinvasion appear not to affect the prognosis.     

Islet cell carcinoma of the pancreas

To define the course of malignant pancreatic islet cell tumors, 20 patients seen over 14 years with these neoplasms were reviewed. The 12 men and 8 women ranged in age from 22 to 76 years, with a mean of 52. Seven functional tumors included three insulinomas, two glucagonomas, one gastrinoma, and one somatostatinoma. One insulinoma was associated with a multiple endocrine neoplasia type I (MEN-I) syndrome. The 13 patients with nonfunctioning tumors had abdominal pain (3), jaundice (2), and steatorrhea (2). Seven had a palpable abdominal mass. Diagnosis of malignancy was based on local invasion (4), distant metastases (15), or both (1). One patient had percutaneous biopsy of a hepatic metastasis. All others had laparotomy for diagnosis and/or treatment. Each patient had a single tumor except the patient with MEN-I syndromes, who had multiple tumors throughout the pancreas. The head was involved in seven patients, the body in seven, and the tail in five. Operations included six curative and three palliative resections, five biliary diversions (two with concomitant enteric bypass), and five biopsies. Palliative resections were done for hormonal or local symptoms such as gastrointestinal (GI) bleeding and pain. Multiple chemotherapeutic agents were used, but the best results were obtained with DTIC (50% response). Four patients had radiation for liver, brain, or bone metastasis, with some improvement. Of five patients who had curative resections, four are alive 15 to 144 months, with a mean of 75 months. One died six years after diagnosis. Of the remaining 15 patients who had liver metastasis, seven patients are alive 8 to 168 months later, with a mean of 87 months.(ABSTRACT TRUNCATED AT 250 WORDS)     

Gangliogliomas in cerebral hemisphere

Gangliogliomas are rare central nervous system neoplasms. The clinical features, radiological findings and surgical results in 6 histologically proven cases of gangliogliomas in the cerebral hemisphere are reviewed. The ages varied between 8 and 58 years old at the time of diagnosis. Five patients had seizure disorders without focal neurological deficit or clinical signs of increased intracranial pressure. These 5 patients had been treated as epilepsy for 6 months to 12 years. The other one patient had right sided motor weakness and left homonymous hemianopsia without seizure disorder. Plain roentgenograms of the skull revealed abnormal calcification in 3 out of 6 patients. Angiography showed an avascular mass in all 6 patients. CT scan were examined in 5 patients. In 3 patients the tumor were isodense, 2 of which demonstrated contrast enhancement. The other 2 showed low density lesion. One showed a single large cyst and another ill-defined low density containing high density calcification. In all 5 patients mass effect was slight. Three tumors were located in the temporal lobe and another 3 in the parietal lobe. At surgery total removal was accomplished in 3 patients. One patient had subtotal removal and 3 patients partial removal. Histological study revealed figures characteristic to the ganglioglioma. The predominant glial cell was astrocytic in 4 cases and oligodendroglial component was found in the other 2 cases. All patients either completely or nearly asymptomatic at the follow up for 1 to 12 years. The clinical study in 6 cases, along with a review of the literature suggest that these epileptogenic neoplasms should be aimed at surgical removal or total extirpation if possible.     

Unilateral hydronephrosis resulting from intraluminal obstruction of the ureter by adenosquamous endometrioid carcinoma arising from disseminated endometriosis

A case of adenosquamous carcinoma arising in the background of disseminated pelvic endometriosis presented as unilateral hydronephrosis and a polypoid intraluminal ureteral mass. This is the first case of a malignancy arising in endometriosis presenting as an obstructive ureteral mass. The patient had a history of total hysterectomy and bilateral salpingo-oophorectomy 5 years earlier because of an endometriotic cyst, and had since been under unopposed estrogen replacement therapy. An analysis of the case and related literature is presented. Possible pathogenic mechanisms are discussed.     

The Budd-Chiari syndrome. Treatment by mesenteric-systemic venous shunts.

Twelve patients with the Budd-Chiari syndrome have been managed surgically. Ten of the patients were female, two were male, with a mean age of 40 years. Three of the patients had polycythemia vera, two had pre-existing cirrhosis, one had ingested estrogens, one had an occult tumor, and in four there were no associated factors. Ten patients presented with ascites and two with bleeding esophageal varices. The diagnosis was confirmed in all 12 patients by liver biopsy and hepatic vein catheterization. Inferior vena cavography revealed the abdominal vena cava to be thrombosed in six patients. The superior mesenteric vein was used to decompress the congested liver in all 12 patients. In five patients, a mesocaval shunt (MCS) was performed and in seven patients, a mesoatrial shunt (MAS) was carried out. There were four hospital deaths (two MCS, two MAS). One late death (MAS) occurred from liver failure following shunt thrombosis. Two additional patients (one MCS, one MAS) re-developed ascites immediately following surgery and angiography revealed a thrombosed shunt. Ascites has been controlled with a LeVeen shunt in these two patients, but liver biopsies showed progression to cirrhosis. The remaining five patients (three MAS, two MCS) did well, and angiography revealed patent shunts. Two of these patients, however, re-developed ascites at 4 and 10 months following MAS and required a second MAS. Follow-up ranges from 6 to 68 months. In three of the patients (two MCS, one MAS) with patent shunts, liver biopsy shows a remarkable return toward normal liver architecture and histology.     

Peritoneal keratin granulomas with carcinomas of endometrium and ovary and atypical polypoid adenomyoma of endometrium. A clinicopathological analysis of 22 cases.

Twenty-two cases of keratin granulomas of the peritoneum associated with endometrioid adenocarcinoma with squamous differentiation of the endometrium, the ovary, or both, and with an atypical polypoid adenomyoma of the endometrium were reviewed. Follow-up data were available in 18 cases. Twelve patients were well and disease free 13 months to 15.2 years postoperatively; one patient died of unrelated disease 21 years postoperatively; three patients were tumor free with a short duration of follow-up; one patient, who had a stage Ic ovarian tumor, died of pulmonary embolism during the treatment of recurrent tumor 1 year after operation; and a final patient, who had been followed for 3 months after operation for stage IV disease, was alive with residual tumor. At least six patients with stage I carcinomas were treated with postoperative irradiation because the granulomas had raised a suspicion of advanced disease. Follow-up data on the patients in this series suggest that peritoneal keratin granulomas have no prognostic significance and should be distinguished from viable tumor implants on microscopic examination.     

Laparoscopic colorectal resection for endometriosis

Background The rectosigmoid colon is affected by deep pelvic endometriosis in 3–37% of cases. In the past, treatment of the affected gastrointestinal tract generally required conversion to conventional surgery. We describe our experience with complete laparoscopic management of deep pelvic endometriosis with bowel involvement.Methods From March 1995 to March 2003, 29 consecutive patients with endometriosis requiring laparoscopic intervention were evaluated. In seven patients (24%) colorectal involvement was identified prior to the operation. A low anterior resection was performed in four patients (57%) and a sigmoid resection in three (43%). In all cases, colonoscopy showed a normal mucosa. In all cases, treatment consisted of resection of the bowel involved together with the excision of all other implants. Data analysis included age, previous abdominal operations, previous history of endometriosis, operative time, conversion rate, complications, length of stay, and pain relief.Results There were seven patients with colorectal involvement whose median age was 32.8 years (range, 28–40), with a history of previous abdominal operation in two (28%). Preoperative symptoms were as follow: dysmenorrea in four patients (57%), dyspareunia in four (57%), pelvic pain in seven (100%), rectal bleeding in one (14%), and tenesmus in five (71%). Mean operative time was 190 min (range, 165–230). Length of stay was 8.3 days (range, 7–11). There were no anastomotic leak and no major postoperative complication. One patient had temporary urinary retention. At a median follow-up of 38.7 months (range, 1–84), complete relief of pelvic symptoms was achieved in five patients (71%), and there was improvement in one patient. In one patient complaining of persistent pain, a new colonic implant was diagnosed two years after the surgery requiring reoperation.Conclusions The results show that provided that the surgeon is highly skilled in laparoscopy, laparoscopic resection of deep pelvic endometriosis with rectosigmoid involvement is feasible and effective in nearly all patients.     

Urothelial neoplasms in patients 20 years or younger: a clinicopathological analysis using the world health organization 2004 bladder consensus classification

PURPOSE: Urothelial neoplasms in patients younger than 20 years are rare, with conflicting data regarding clinical outcomes. MATERIALS AND METHODS: We identified 23 patients 4 to 20 years old with urothelial neoplasms, reclassified the microscopic diagnoses using the 2004 WHO/International Society of Urologic Pathology grading classification and collected data on presentation, risk factors and outcomes. RESULTS: Pathological grading revealed 2 urothelial papillomas, 10 papillary urothelial neoplasms of low malignant potential (PUNLMPs), and 8 low grade and 3 high grade papillary urothelial cancers, all without invasion. Mean patient age was 13.2 years (range 4 to 20), 19 patients were male and 19 presented with gross hematuria. All lesions were solitary and measured 0.1 to 6 cm. One patient had a history of smoking and 1 had parents who smoked. Three patients (13%) had recurrences classified as either urothelial papilloma (1) or PUNLMP (2). All patients were alive with no evidence of disease after a mean followup of 4.5 years (range 6 months to 13 years). CONCLUSIONS: Urothelial neoplasms in individuals younger than 20 years more commonly occur in males and are predominantly low grade with a favorable clinical outcome. Before the current classification system the 10 patients with a diagnosis of PUNLMP would have been classified as having papillary carcinoma. Thus, the diagnostic category of PUNLMP allowed 43.5% of patients in this series to avoid being labeled with "cancer" at a young age.     

原发性肾上腺非霍奇金淋巴瘤七例报告

目的 探讨原发性肾七腺非霍奇金淋巴瘤的临床特点和诊治方法.方法 以肾上腺肿瘤为首发表现的淋巴瘤患者7例.男5例,女2例.年龄33～62岁,平均48岁.单侧2例、双侧5例.体检发现2例;低热、乏力伴体质量下降3～4个月2例;睾丸肿大2个月1例;单侧腰痛伴脾肿大2例.7例血清乳酸脱氢酶367～568 U/L(正常值100～245 U/L),β2微球蛋白5.9～6.3 mg/L(正常值<2.4 mg/L).CT检查示肾上腺肿瘤直径6.3～13.0 cm,呈不规则肿块,境界不清、密度不均、增强后肿瘤轻度强化,未见出血和钙化.结果 术前误诊4例,分别误诊为皮质腺痛2例,肾上腺转移瘤、嗜铬细胞瘤各1例.行肾上腺肿瘤切除4例,其中2例行同侧肾切除,穿刺活检汪实2例,睾丸活检证实1例.病理诊断为非霍奇金淋巴瘤,T细胞型1例、B细胞型6例.免疫组化T细胞型CD3、CD45-RO阳性、B细胞型L26、CD79a阳性.术后4例接受化疗,1例随访2年健在,余3例分别生存2、6、20个月后死亡;穿刺活检和睾丸活检3例接受放化疗,分别生存1 9、32、38个月后死亡.结论以肾上腺肿瘤为首发表现的淋巴瘤临床表现缺乏特异性,术前诊断困难,肿瘤局部病变广泛,影像学表现形态不规则.原发性肾上腺淋巴瘤恶性度较高,预后差,治疗上主要采用手术联合放化疗.