Nodal cytotoxic lymphoma spectrum: a clinicopathologic study of 66 patients.

The expression of cytotoxic granule-associated proteins has been reported in some T-cell or natural killer (NK)-cell lymphomas of mostly extranodal origin, but rarely of nodal origin except for anaplastic large cell lymphoma (ALCL) and Hodgkin's disease (HD). This study analyzed 66 nodal lymphomas expressing T-cell intracellular antigen-1 (TIA-1) and/or granzyme B to characterize the clinicopathologic spectrum of these neoplasms. Four main groups could be delineated. The first group consisted of p80/anaplastic lymphoma kinase (ALK)-positive ALCL (n = 35). The patients were 2 to 62 years of age (median age, 16 years), and the lymphomas pursued a relatively indolent clinical course. The tumors were phenotypically of either T- or null-cell type with constant expression of CD30, epithelial membrane antigen (EMA), and p80/ALK, but not CD15 or BCL2. None harbored Epstein-Barr virus (EBV). The second group consisted of peripheral T/NK-cell lymphoma, the nodal high-grade cytotoxic type (n = 13). The patients were 29 to 72 years in age (median age, 55 years), and the tumors pursued an aggressive clinical course. The tumors often showed pleomorphic, anaplastic, or centroblastoid morphology, and were featured by either EBV association or CD56 expression. The third group consisted of peripheral T-cell lymphoma, of the nodal low-grade cytotoxic type (n = 8). The patients, three men and five women, were 31 to 75 years old (median age, 61 years). Notably, six of them exhibited lymphoepithelioid (Lennert's) lymphoma. The fourth group consisted of cytotoxic Hodgkin's-like ALCL/HD (n = 10), included seven cases of Hodgkin's-like ALCL and three cases of HD, and was characterized by the presence of Reed-Sternberg cells and often the CD15+ phenotype. The patients were all men except for one woman, and they ranged in age from 24 to 84 years (median age, 62 years). The link among these four groups was reinforced by the presence of a highly characteristic large cell with horseshoelike or reniform nuclei-the frequent expression of CD30 and EMA-and the often lack of T-cell receptor-alphabeta. In this series, the expression of p80/ALK and CD56 was also associated with favorable and poor prognoses respectively (p<0.001, log-rank test).     

Metastatic tumors of the vulva: a clinicopathologic study of 66 cases

Metastatic tumors involving the vulva are rare, with only a few series and case reports published in the English literature to date. In this study, we present the clinicopathologic features of 66 cases of metastatic tumors of the vulva seen at the University of Texas M. D. Anderson Cancer Center from 1944 to 2001. The patients' age ranged from 18 to 84 years (mean 54.8 years). The most common presentations were vulvar nodules or a mass (39 cases), pain (7 cases), and ulceration (5 cases). In 46.9% of cases, the primary tumor was of gynecologic origin, whereas in 43.9% of cases the primary tumor was of nongynecologic origin. The remainder had unknown primaries. The site most frequently involved by metastasis was the labium majus (44 cases: 18 on the right, 13 on the left, 6 bilateral, and 7 unspecified side). Thirty percent of the patients received chemotherapy as treatment for the metastasis, 27% received radiotherapy, and the rest received some combination of chemotherapy, radiotherapy, and surgery. Of the 60 patients with available follow-up, 52 died of disease within 1-81 months (median 7.5 months) from diagnosis of the metastasis. Metastatic tumors of the vulva are rare; however, the diagnosis of these tumors is facilitated by the knowledge of a preexistent malignancy and the lack of a mucocutaneous intraepithelial lesion.     

Pleomorphic lung cancer; a clinicopathologic study

Pulmonary pleomorphic carcinoma is a comparatively rare histologic type of lung carcinoma, and the incidence among all lung carcinomas has been reported to be 0.4%. We reported our experience with 8 patients who had been diagnosed as pulmonary pleomorphic carcinoma, and discussed clinicopathologically the preoperative diagnosis and treatment. In 2 of 8 patients, preoperative transbronchial lung biopsy revealed spindle cell component, highly suggesting pulmonary pleomorphic carcinoma. All patients underwent surgical treatment and 2 of then had incomplete resections because of intrathoracic disseminations or carcinomatous pericarditis. Pathological findings showed invasions into the surrounding thoracic organs such as the chest wall, pericardium, adjacent pulmonary lobe or mediastinal pleura in 5 cases, intrapulmonary metastasis of the same lobe in 3 and lymph node involvement in 3. Recurrence occurred in 6 patients immediately after the operation. Although the preoperative diagnosis of biphasic tumor such as pulmonary pleomorphic carcinoma is difficult, it is possible to suspect the diagnosis when sarcomatous components were detected by preoperative biopsy. The efficacy of chemotherapy and radiotherapy have not been established yet, and thus we would like to emphasize that surgery might be the treatment of choice.     

Sclerosing hemangioma of the lung: a clinicopathologic study of 51 cases

The clinical and pathologic features of 51 sclerosing hemangiomas of the lung are reviewed. This tumor is benign and occurs predominantly in women. It has a variegated histologic appearance characterized by an admixture of four major histologic patterns: solid, hemorrhagic, papillary, and sclerotic. Characteristic uniform round cells are found within the stroma in all patterns and are unique to this tumor. Sclerosing hemangioma is a distinct clinicopathologic entity and should be distinguished from other benign neoplasms or inflammatory lesions of the lung.     

Intraosseous lipomas. A clinicopathologic study of 66 cases

Sixty-one cases of histologically confirmed solitary intraosseous lipomas were analyzed with respect to clinical, roentgenographic, gross, and histologic features. Two additional cases with multiple intraosseous sites and three additional cases not treated with surgery are also described. Intraosseous lipomas may be subdivided into three groups depending on the degree of involution: I, solid tumors of viable lipocytes; II, transitional cases with partial fat necrosis and focal calcification but also regions of viable lipocytes; and III, late cases in which the fat cells have died with variable degree of cyst formation, calcification, and reactive new bone formation of a characteristic morphology. The tumor is a well-defined entity that may present with varying features due to its stage of evolution. Thus, lipomas have been confused with other benign tumors, cysts, and cases of bone infarction. Intraosseous lipoma is not as rare as the literature suggests, but has been rarely diagnosed. The lesion appears to undergo spontaneous involution, so that surgical excision may not be necessary in some cases.     

Spindle-cell carcinoid tumors of the lung: a clinicopathologic study of 35 cases

Thirty-five cases of spindle-cell carcinoid tumors of the lung were studied. Fifteen patients were male and 20 female, and they ranged in age from 33 to 78 years, with a mean of 57.6 years. Eleven neoplasms were located in the left lung and 23 in the right lung; a disproportionately large number of neoplasms were present in the right middle lobe. The tumors were most commonly encountered as an incidental finding on chest roentgenogram. None of the patients had unequivocal evidence of the carcinoid or any other endocrine syndrome. All but two of the lesions were located n the periphery of the lung and most were subpleural. They ranged in size from 0.7 to 4 cm, with 82% of the neoplasms having a maximum dimension of 2 cm or less. Microscopically, the neoplasms were composed predominantly or entirely of spindle cells which were fairly uniform in length in any single lesion, but showed moderate variation from case to case. The shorter spindle cells were often arranged in an "organoid" pattern, while the neoplasms composed of larger spindle cells were more "mesenchymal" in appearance. Silver stains were performed on 12 cases and all were argyrophil-positive. Cytoplasmic dense-core granules were identified in all four neoplasms examined ultrastructurally. Twelve cases were treated by wedge resection or segmental resection, and 18 cases by lobectomy. Hilar lymph nodes were removed from seven patients, and in two there were microscopic lymph node metastases. One other patient had a single microscopic bony metastasis. Follow-up information was available for 22 patients and ranged from 1 to 13 years with a mean of 5.4 years. None of the patients have had metastases other than those described above and none of the patients had died of their disease.     

Nodular lymphoid hyperplasia of the lung: a clinicopathologic study of 14 cases

Nodular lymphoid hyperplasia is a controversial entity in which its existence in the lung has been doubted. The current opinion is that most, if not all, such cases represent extranodal marginal zone B-cell lymphomas masquerading as reactive lesions. We found 14 cases of nodular lymphoid hyperplasia in the files of the Pulmonary Department at the Armed Forces Institute of Pathology from 1974 through 1998. All had clinical histories and hematoxylin-eosin slides. In 12 of 14 with paraffin blocks, we applied immunohistochemical antibodies for CD20, CD3, CD43, CD5, bcl-2, bcl-1, CD45RA, and kappa and lambda immunoglobulin light chains. Molecular genetic analysis was performed on paraffin sections in 10 of 14 by the polymerase chain reaction for rearrangements of the immunoglobulin heavy chain gene and the minor and major break-point regions of the chromosomal translocation t (14;18). There were eight women and six men ranging in age from 19 to 80 years (median, 65 yrs). Most lesions (71%) were incidental findings on routine chest x-rays. Most patients (64%) had a single lesion by chest x-ray whereas the remainder had two to three lesions, except for one patient who had "multiple" lesions. There was associated regional lymphadenopathy in five of 14 cases (36%) which, on biopsy, proved to be reactive follicular hyperplasia. The only treatment was surgical excision. Of the seven patients with follow-up information from 8 months to 6 years (mean, 30 mos), none had clinical recurrence and no patient died of disease. The histology and immunophenotype of the lesions were strikingly similar, including abundant reactive germinal centers, intense interfollicular polyclonal plasmacytosis, and a variable degree of interfollicular fibrosis. No case showed a molecular rearrangement of the immunoglobulin heavy chain gene or the minor or major break-point region of the t (14;18). We conclude that nodular lymphoid hyperplasia of the lung, although rare, does exist and deserves its place in the spectrum of reactive pulmonary lesions that ranges from follicular hyperplasia to diffuse hyperplasia of the bronchus-associated lymphoid tissue (lymphoid interstitial pneumonitis).     

Intramuscular myxoma: a clinicopathologic study of 17 patients

The office and hospital records of 17 patients treated for intramuscular myxomas between 1979 and the present were reviewed. Thirteen women and four men were diagnosed with an intramuscular myxoma at an average age of 55 years (range, 31-76 years). Each patient presented with a noticeable mass, and six patients had symptoms of pain or aching related to the mass. The masses were located primarily in the thigh muscles with eight in the quadriceps muscles, two in the gluteal muscles, and two in the adductor muscles. The majority (nine of 11) of the masses were relatively hypointense on T1-weighted images, hyperintense on T2-weighted images, homogeneous and well-circumscribed, and showed peripheral enhancement with gadolinium contrast. All patients were treated by marginal excision of the tumor. Fine needle aspiration biopsy correlated with final surgical diagnosis in only three of eight masses biopsied. The size of the excised tumors ranged from 3.5 to 9.5 cm. No tumors recurred during an average followup of 7 years after excision (range, 1-20 years). None of the intramuscular myxomas in the series was associated with either Mazabraud's syndrome or Albright's syndrome.     

Large cell neuroendocrine carcinoma of the lung: a clinicopathologic study of eighty-seven cases

OBJECTIVE: Large cell neuroendocrine carcinoma of the lung is a newly recognized clinicopathologic entity. The clinical characteristics and optimal treatment of patients with large cell carcinomas are not yet established. The aim of this study was to define the clinicopathologic characteristics of large cell neuroendocrine carcinoma. METHODS: The histologic characteristics of the patients receiving an initial diagnosis of poorly differentiated non-small cell lung carcinoma (n = 484), small cell carcinoma (n = 55), carcinoid (n = 31), and large cell neuroendocrine carcinoma (n = 12) were retrospectively reviewed according to World Health Organization criteria. Immunohistochemistry was performed to confirm the neuroendocrine phenotype. The outcomes and other clinical characteristics of those patients with large cell neuroendocrine carcinoma were retrospectively analyzed and compared with those of patients with poorly differentiated carcinoma of other histologic types. RESULTS: A total of 87 patients were given a diagnosis of large cell neuroendocrine carcinoma after the histologic review. These patients comprised 3.1% of all patients undergoing resection for primary lung cancer during the same period. The overall 5-year survival was 57%. The 5-year survivals of patients with stage I, II, III, and IV disease were 67%, 75%, 45%, and 0%, respectively. There was no statistically significant difference between the overall survival of patients with large cell neuroendocrine carcinoma and those with other non-small cell lung cancers. There was a significant difference between the survival of patients with stage I large cell neuroendocrine carcinoma and that of patients with the same stage of other non-small cell lung carcinomas. The site of the first documented recurrence was locoregional in 12 patients (34%), distant metastases in 20 patients (57%), and both simultaneously in 3 patients. Locoregional lymph node recurrences were observed frequently. More than 80% of recurrences were found within 1 year after the operation. CONCLUSION: In terms of prognosis, large cell neuroendocrine carcinoma is distinctly different from other non-small cell lung cancers. The prognosis of large cell neuroendocrine carcinoma was poor, even for early stage disease; the prognosis of the stage I disease of large cell neuroendocrine carcinoma was poorer than that of the same stage of other non-small cell lung cancers. Because of its aggressive clinical behavior and poor prognosis, large cell neuroendocrine carcinoma should be recognized as one of the poorest prognostic subgroups among primary lung cancers, and therefore novel therapeutic approaches should be established.     

Large cell neuroendocrine carcinoma of the lung: a 10-year clinicopathologic retrospective study

BACKGROUND: Large cell neuroendocrine carcinoma is a recently recognized histologic entity whose clinical features and optimal treatment have not yet been well defined and are still being assessed. We report our retrospective assessment of cases of large cell neuroendocrine carcinoma observed from 1989 to 1999 in terms of survival. METHODS: Cases of large cell neuroendocrine carcinoma diagnosed between 1989 and 1999 were reassessed retrospectively according to the World Health Organization classification. The clinical outcome and pathologic features of all cases are described. Survival rates of patients with large cell neuroendocrine carcinoma are compared with those patients with small cell lung cancer treated in the same period. RESULTS: Patients were 41 men and 7 women with an average age of 63.7 years. Twenty-nine patients (60.4%) had pathologic stage I disease, 11 patients (22.9%) had pathologic stage II disease, and 7 patients (14.6%) had pathologic stage IIIA disease. One patient (2.1%) had pathologic stage IIIB disease. No patient underwent induction chemotherapy. Two patients underwent adjuvant chemotherapy and 2 underwent mediastinal radiotherapy for N2. No death was reported in the perioperative period. The median follow-up was 5 years. The actuarial survival for the entire group was 60.4% at 1 year, 27.5% at 3 years, and 21.2% at 5 years. The actuarial survival of accurately staged, stage I patients at 5 years was 27%. CONCLUSIONS: The findings suggest that treating large cell neuroendocrine carcinoma by means of applying treatment for nonsmall cell lung cancer leads to a prognosis that is worse than that for nonsmall cell lung cancer, even in terms of low pathologic stages.     

Primary adenoid cystic carcinoma of the lung: A clinicopathologic study

Background  Primary adenoid cystic carcinoma (ACC) of lung is a rare but distinctive salivary gland-type malignant epithelial neoplasm. Methods  The clinical and pathological features in seven cases of primary ACC of the lung were reviewed. The age of patients ranged from 48 years to 65 years. All were males and smokers. The tumours arose from submucosal bronchial glands as endobronchial growth measuring 2.6 cm to 3.8 cm in greatest dimension. The presenting symptoms were cough, wheezing, dyspnoea and hemoptysis all related to obstruction to airway passage. The tumours were diagnosed by computed tomography (CT) scan and bronchial biopsy. Histologically three main growth patterns were identified admixed in various proportions: cribriform, tubular and solid. Lobectomy or pneumopnectomy was carried out in all the patients. In one patient chemotherapy was given apart from surgery due to detection of tumour infiltration in adjoining lung at the time of surgery. Follow up studies showed all the patients were well except two died of wide spread metastases. Results  Of the two patients who had died one had a predominantly solid pattern of growth limited to bronchus while the other had a predominant cribriform pattern with local infiltration in the adjoining lung parenchyma. Conclusion  Prognosis of ACC of lung depends on the histological type (predominantly tubular has best prognosis while predominantly solid pattern has worst prognosis) and clinical stage at the time of diagnosis (size of tumour, extent of spread and metastases). Despite their generally slow and indolent growth in other locations, ACC arising in lung may in certain cases be more aggressive.     

Unicystic ameloblastoma: a clinicopathologic study of 33 Chinese patients

The term unicystic ameloblastoma refers to those cystic lesions that show clinical, radiographic, or gross features of a jaw cyst, but on histologic examination show a typical ameloblastomatous epithelium lining part of the cyst cavity, with or without luminal and/or mural tumor growth. To ascertain the clinicomorphologic spectrum and biologic behavior of this tumor group, the clinicopathologic features of 33 unicystic ameloblastomas from Chinese patients were studied. This series represents approximately 19% of all cases of ameloblastoma accessioned in the authors' hospital during a 15-year period. Twenty-one patients were male and 12 were female, for a total of 33 patients. The age at diagnosis ranged from 8 to 60 years (mean, 25.3 yrs) and peaked at the second and third decades (70%), Thirty tumors (91%) occurred in the mandible and three in the maxilla. Of the 29 patients with a radiographic record, an expansive unilocular radiolucency was seen in 22 cases, and was multilocular in seven cases. Microscopically, all tumors demonstrated a generally monocystic growth pattern. Eight tumors were simple cystic, 10 comprised intraluminal tumor nodules, and the remaining 15 had a conspicuous component of infiltrative tumor islands in the cyst capsule. The cystic tumor linings invariably showed, at least in part, a typical ameloblastomatous pattern that was often accompanied by epithelial areas of various histologic appearance. Follow up of 29 patients revealed no recurrence in less than 4 years of follow up, but did reveal a 35% recurrence rate at more than 4 years of follow up. The average interval to recurrence was approximately 7 years. Recurrence also appeared to relate to histologic subtypes of unicystic ameloblastoma, with those invading the fibrous wall having a rate of 35.7%, but other types having a rate of 6.7%. Despite the fact that unicystic ameloblastoma may, in general, compare favorably with its solid or multicystic counterpart in terms of clinical behavior and response to treatment, the subsets of the maxillary lesions or tumors containing invading islands in the fibrous wall could have a high risk of recurrence. Furthermore, recurrence of unicystic ameloblastoma may be long delayed, and a long-term postoperative follow up is essential to the proper management of these patients.     

Myofibrosarcoma: a clinicopathologic study

The concept of soft tissue sarcomas composed predominantly of myofibroblasts has been controversial. We examined a series of such lesions of low- and intermediate-grade malignancy to further define their clinical and pathologic features. Histologic appearances of four cases diagnosed as myofibrosarcoma by electron microscopy were reviewed. Eleven additional cases with similar morphology were then identified from 249 tumors originally indexed as fibrosarcoma. Electron microscopy was performed on five of these, and immunohistochemistry was carried out on all cases for which material was available. There were 11 men and 4 women aged 33 to 73 years (median, 54 yrs; mean, 53 yrs). Lesions mainly involved the head and neck, extremities, and trunk and ranged in size from 1.5 to 12 cm. The tumors were composed of bland or pleomorphic stellate to spindled cells with eosinophilic cytoplasm and tapered nuclei in a collagenous stroma; 10 were grade 1 and five were grade 2. All sarcomas displayed fascicular or storiform patterns, and some of the grade 1 lesions superficially mimicked nodular fasciitis. Electron microscopy of nine cases showed myofibroblastic differentiation, and immunohistochemistry showed smooth muscle actin in 13 of 15 cases, muscle-specific actin in 7 of 9, desmin in 6 of 14, and cytokeratin in 0 of 11. Four of nine grade 1 and three of four grade 2 tumors recurred (one twice), and one grade 2 tumor metastasized to the lungs. Myofibrosarcomas are indolent low-grade or occasionally aggressive intermediate-grade sarcomas which can be recognized by light microscopy. Their clinical importance lies in the resemblance, particularly of low-grade examples, to reactive or pseudosarcomatous conditions.     

Ependymomas: a clinicopathologic study

Since 1924, when ependymomas were first classified as a distinctive glial neoplasm by Bailey, much has been published concerning these tumors, but there are important points of interest that are still not clear. In order to study more fully the clinical and pathologic characteristics of the ependymoma, we identified 62 patients with histologically proven neoplasms. Twenty-two were supratentorial, 21 were infratentorial, and 19 were intramedullary spinal cord tumors. These groups had mean ages of 17, 7, and 41 years, respectively, at the time of first symptoms. The presenting and accompanying symptoms were related to location and included headaches, nausea, visual changes, hemiparesis, and neck, back, and radicular pain. Neurological signs included papilledema, nystagmus, gait disturbance, cranial nerve palsies, altered mental status, paraparesis, and sensory dysfunction. Radiologic modalities of particular importance included computed tomography and myelography. Surgery and radiation therapy were the primary treatment modalities with median survival times from first symptoms being 92, 36, and 117 months for the above groups, respectively. Based on computer-generated survival curves, several characteristics significantly affected survival. These included tumor site, age, and neuraxis metastases. In patients with supratentorial tumors, cranial nerve palsies, microcystic changes, and mitotic figures were important, while in patients with infratentorial tumors, widened sutures, increased head circumference, age, epithelial features, and subependymal features significantly affected survival. Patients who had complete gross resection of a spinal cord tumor had no recurrences or mortality.     

Myoepithelial carcinoma of the salivary glands: a clinicopathologic study of 25 patients

Salivary gland carcinomas displaying exclusively myoepithelial differentiation (myoepithelial carcinoma) are considered rare. Their histopathologic features, immunohistochemical profile, and clinical behavior are not well characterized. The authors reviewed the clinicopathologic features of 25 salivary gland tumors fulfilling two fundamental histologic criteria: unequivocally malignant and exclusively myoepithelial. For most of these, the original diagnosis was malignant mixed tumor. Thirteen men and 12 women aged 24 to 77 years (mean age, 55 yrs) participated in the study, and most presented with a painless mass. The parotid gland was the most common site (n = 15). Tumors ranged from 2.1 to 5.5 cm, arising either in association with a benign mixed tumor (n = 15) or de novo (n = 10). Histologically, all the tumors displayed infiltrative growth and most had a characteristic multinodular architecture with a cellular periphery and central necrotic/myxoid zones. Epithelioid, hyaline, spindle, clear, or mixed cell types were noted with accompanying myxoid and/or hyalinized extracellular matrix. Ten tumors were high grade cytologically and 15 were low grade. The mitotic rate ranged from three to 51 mitoses per 10 high-power fields. Necrosis was present in 15 tumors and perineural and vascular invasion were identified in 11 and four neoplasms respectively. Immunoreactivities included CAM5.2 (89%), AE1:AE3 (100%), 34betaE12 (92%), cytokeratin 7 (21%), cytokeratin 14 (53%), vimentin (100%), S-100 protein (100%), smooth muscle actin (50%), calponin (75%), muscle-specific actin (31%), glial fibrillary acidic protein (31%), carcinoembryonic antigen (0%), and epithelial membrane antigen (21%). Ultrastructural examination of three tumors showed myoepithelial features. Ten patients developed recurrences, mostly multiple. Follow up of 17 patients showed that eight patients (47%) developed metastases (six high grade, two low grade) and five patients (29%) died of disease (four high grade, one low grade) after a mean of 32 months. Two patients were alive with disease (19 and 49 mos). Ten patients (59%) were without any evidence of disease after a mean of 42.2 months. Myoepithelial carcinomas exhibit a wide spectrum of cytomorphologic features and diverse clinical outcomes. As a result of their morphologic heterogeneity, they can be confused easily with many tumors. Myoepithelial carcinomas have been underrecognized in the past, primarily by being lumped under a broader category of "malignant mixed tumor." Awareness of their unique cytoarchitectural patterns and immunohistochemical profile is crucial for accurate identification.     

Leiomyosarcoma of the kidney: a clinicopathologic study

Leiomyosarcoma of kidney is a rare lesion for which limited data are available. Cases coded as leiomyosarcoma of the kidney from three institutions were reviewed. These cases comprised 3 men and 7 women, 40 to 75 years of age. Immunohistochemistry was performed where possible and clinical follow-up information was obtained. On immunohistochemical staining, 6 of 6 tumors expressed smooth muscle actin, desmin, calponin, and h-caldesmon, and epithelial membrane antigen was positive in 1 of 5. Tumors were negative for cytokeratin and S-100 protein. Follow-up information was available for 9 patients. Two patients had metastases at diagnosis, four developed metastases, and two had recurrent disease. Five of 9 died of disease. Two patients were alive with no evidence of disease after 19 and 60 months, and 2 patients were alive with disease after 48 months and 56 months. Comparing outcome with tumor grade, the one patient with grade 1 tumor was alive with no evidence of disease; of 5 patients with grade 2 tumor, 2 died of disease, 1 was alive with no evidence of disease, 1 was alive with disease, and 1 was alive with extensive disease; all 3 grade 3 patients died of disease. In summary, the majority of renal leiomyosarcomas are intermediate or high grade with correspondingly poor prognosis.     

Thymic neuroendocrine carcinoma: a clinicopathologic study in four patients.

BACKGROUND: Thymic neuroendocrine carcinoma (carcinoid) is rare. Here we present four cases of this unusual neoplasm to provide more clinical, radiologic, and prognostic data. MATERIALS AND METHODS: Four male patients with an average age of 44 years (range 27-63) were identified as having thymic neuroendocrine carcinoma and were reviewed retrospectively. RESULTS: One patient had Cushing's syndrome with elevated serum ACTH. Three others were asymptomatic with normal laboratory findings, one case was associated with MEN type 1. All underwent complete resection along with invaded adjacent structures. Local recurrence developed in two patients at 45 and 98 months after the initial excision. Both patients died at 90 and 105 months, respectively. The other two patients are alive and have been disease-free for 27 and 120 months, respectively. CONCLUSIONS: Thymic neuroendocrine carcinomas have a rather poor prognosis based on their tendency to recur and metastasize many years after the initial operation. Therefore, prolonged follow-up is essential for these tumors.     

Class V lupus nephritis: a clinicopathologic study in 152 patients

BACKGROUND: Class V lupus nephritis (LN) can be divided into two subgroups according to the 1995 WHO modified classification, but the difference in clinical characteristics between these subgroups is not well known. METHODS: We classified 152 patients with Class V LN, confirmed by renal biopsy, into two subgroups (61 Class Va, 91 Class Vb), and enrolled 488 patients with Class IV as controls. The clinical manifestations, serologic results and prognosis were compared for Classes Va and Vb. RESULTS: The incidence of hypertension and anemia in Class Vb patients was significantly higher than in Class Va (38.5% vs 21.3%, 72.5% vs 52.5%, p<0.05). The incidence of hematuria and renal insufficiency in Class Vb was 64.8%and 15.4%, which was higher than Class Va (44.2% and 3.3%), but lower than Class IV (89.1% and 35%), p<0.05. The percentage of patients with positive anti-dsDNA antibody and hypocomplementemia in Class Vb tended to be higher than Class Va (35.2% vs 26.2%, 50.6% vs 31.2%). Repeated renal biopsies in 24 patients (11 Class Va, 13 Class Vb) showed that eight Class Vb patients had "transformed" to Class IV LN, while only two Class Va patients did (p<0.05). In three Class Va patients serum creatinine doubled during follow-up, but none of them progressed to end-stage renal disease (ESRD). In Class Vb serum creatinine doubled in ten patients, and three progressed to ESRD. CONCLUSIONS: The renal injury and extrarenal manifestations of Class Vb patients were severer than Class Va. Class Vb patients were more likely to shift to Class IV LN, and the prognosis was poorer than for Class Va.     

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.