原发性乳腺淋巴瘤8例临床病理分析并文献复习

目的:探讨乳腺淋巴瘤的临床病理学特点及诊断要点。方法:收集2009年1月—2015年3月确诊为乳腺淋巴瘤的病例资料,进行临床资料、病理形态学及免疫组化分析,并结合文献对该病的诊断及鉴别诊断进行讨论。结果:8例均为女性,发病年龄35~82岁,6例位于左乳,1例右侧,1例双侧。多数表现为无痛性肿块,边界较清,可活动。7例为弥漫大B细胞淋巴瘤(DLBCL),1例为黏膜相关淋巴组织淋巴瘤(MALT),免疫组化染色均表达CD20、CD79a,7例MUM1阳性,4例Bcl-2阳性。治疗后4例患者获随访3~25个月,均健在。结论:乳腺淋巴瘤少见,常见类型为DLBCL,确诊依赖组织病理学和免疫组化染色。提高对乳腺淋巴瘤的认识,对避免误诊是至关重要的。     

Polypoid endometriosis: a clinicopathologic analysis of 24 cases and a review of the literature

We describe 24 cases of polypoid endometriosis, most of which were referred because of problems in differential diagnosis, particularly distinction from a low-grade müllerian neoplasm. The patients were 23 to 78 years (mean 52.5 years) of age. Seven patients were on unopposed estrogen, four on combined estrogen-progestin therapy, and one patient had a synchronous ovarian thecoma. The most common clinical presentations were a pelvic mass, vaginal polypoid masses, and large bowel obstruction. In some cases, the intraoperative findings suggested a neoplasm. Sites of involvement in order of frequency included colon, ovary, uterine serosa, cervical and/or vaginal mucosa, ureter, fallopian tube, omentum, bladder, paraurethral and paravaginal soft tissue, and retroperitoneum. Multiple sites were involved in seven cases. Five cases occurred within ovarian or extraovarian endometriotic cysts. The lesions ranged up to 14 cm in size and formed polypoid, pink, gray or tan, masses. On microscopic examination, the polypoid masses were composed of an admixture of endometriotic glands and stroma. A variety of glandular architectural patterns were observed, sometimes in combination, most commonly cystic and noncystic simple hyperplasia, but also simple or complex hyperplasia with atypia, disordered proliferative, and cystic atrophy. Various types of epithelial metaplasia (tubal, mucinous, squamous, papillary syncytial metaplasia) were common. Hemorrhage, fibrosis, prominent thick-walled blood vessels, hemosiderin-laden histiocytes, and decidual change were also present in some cases. Eighteen cases were associated with usual (nonpolypoid) endometriosis. In one case, polypoid endometriosis merged with a mucinous borderline tumor of endocervical-type. In all but two cases, polypoid endometriosis lacked periglandular stromal hypercellularity, stromal atypia, and intraglandular stromal papillae, helping distinguish it from adenosarcoma. Focal intraglandular stromal papillae were noted in two cases with focal mild periglandular stromal hypercellularity in one of them, but no stromal atypia was present in either case. Follow-up data in 17 patients indicated that 15 patients were alive without evidence of residual disease, 1 was alive with residual endometriosis, and 1 died of other causes. In conclusion, polypoid endometriosis is a rare manifestation of endometriosis that may be mistaken for a neoplasm on clinical, intraoperative, or pathologic assessment. Some cases may be attributable to exogenous hormones or hyperestrinism and, like conventional endometriosis, some may evolve into a premalignant or, rarely, a neoplastic lesion. The main lesion in the differential is a müllerian (mesodermal) adenosarcoma.     

PEComa presenting in bone: clinicopathologic analysis of 6 cases and literature review

PEComas other than angiomyolipoma, lymphangioleiomyomatosis, and clear cell sugar tumor of the lung are relatively rare, and PEComas presenting in bone are especially rare. To further characterize their clinicopathologic features, 6 cases of PEComa which first presented in bone were retrieved from the authors' consult and surgical pathology files, including both primary and metastatic lesions. Four patients were female and 2 patients were male. The age at diagnosis ranged from 35 to 71 years, with a mean of 51.5 years. As for the 3 cases known definitely to have arisen in bone, the primary sites were right tibia in 2 cases and thoracic vertebra in 1 case. In the 2 cases, presenting in scapula and femur respectively, the primary sites could not be determined with certainty. In 1 case, the lesion was first found in humerus, but the primary tumor proved to be located in the uterus. Histologically, all the tumors were composed of both epithelioid and spindle cells, showing a nested pattern with elaborate vasculature. The characteristic thin walled vessels around which tumor cells were arranged tightly, or in a radiating fashion were seen in all cases. Two or more worrisome features (tumor size >5?cm, infiltrative growth pattern, high nuclear grade, high cellularity, necrosis, and mitotic activity >1/50 HPF) were identified in 4 cases, of which 2 were primary bone tumors; these cases were classified as "malignant" histologically. We conclude that both the primary and metastatic PEComas can present in bone, although both are rare. Combining our findings with the few earlier published reports, it may be suggested that primary PEComa of bone tends to involve lower extremities (5/7 cases). Histologically, they show similar cytomorphology, nested architecture, and characteristic vessels as PEComas at other sites. In addition, a significant subset of the primary bone lesions seem to be malignant.     

Thymic mucoepidermoid carcinomas: a clinicopathologic study of 10 cases and review of the literature

BACKGROUND: Primary thymic carcinomas are rare malignant neoplasms, of which at least 10 different histologic variants have been described. Among these variants, thymic mucoepidermoid carcinomas morphologically similar to the homonymous salivary gland counterparts are particularly unusual; only 9 cases for which clinicopathologic features were available have been reported. OBJECTIVE: To study the clinicopathologic features of primary thymic mucoepidermoid carcinoma in an effort to better define their histologic features and biologic behavior. DESIGN: The clinical and pathologic features of 10 cases of thymic mucoepidermoid carcinoma were reviewed and compared with those of previously reported cases. RESULTS: The patients ranged in age from 8 to 84 years (mean 49 years), with 6 men and 4 women. The initial manifestations included respiratory symptoms and weight loss. Some patients were asymptomatic. All tumors were located adjacent to residual benign thymic tissue, and 3 cases were associated with multilocular thymic cysts. Histologically, the tumors consisted of sheets, lobules and nests of squamous, mucinous and intermediate cells in densely fibrotic stroma. There were 8 low-grade cases and 2 high-grade cases. The 2 patients with high-grade tumors died 1 year after diagnosis, whereas all but 1 patient with low-grade tumors in which follow-up was available were found to be alive and well on follow-up examination. One patient with low-grade tumor but high-stage disease died after developing a local recurrence. Among the 9 cases reported in the literature, 4 cases resulted in fatal outcomes; 2 cases were high-stage disease and 2 were high-grade tumors. CONCLUSIONS: Thymic mucoepidermoid carcinomas are predominantly low-grade tumors, and may be associated with multilocular thymic cysts. Poor prognosis is related to high-grade histology and high-stage disease.     

Ceruminous adenomas: a clinicopathologic study of 41 cases with a review of the literature

BACKGROUND: Ceruminous gland neoplasms are rare neoplasms. To date, a large clinicopathologic study of benign ceruminous gland neoplasms has not been reported. DESIGN: Forty-one cases of ceruminous gland adenomas diagnosed between 1970 and 2000 were retrieved from the files of the Armed Forces Institute of Pathology. Histologic features were reviewed, immunohistochemical analysis was performed (n = 21), and patient follow-up was obtained (n = 40). RESULTS: The patients included 22 men and 19 women, 24 to 85 years of age (mean, 54.2 years). Patients presented clinically with a painless mass of the outer half of the external auditory canal (n = 33) or with hearing changes (n = 11). Symptoms were present for an average of 16.3 months. The polypoid masses affected the external auditory canal only and ranged in size from 0.4 to 2 cm in greatest dimension (mean, 1.1 cm). Histologically, the tumors demonstrated glands and small cysts lined by a tubuloglandular proliferation of inner ceruminous cells (cerumen-secreting epithelium with decapitation secretion) subtended by a spindled to cuboidal myoepithelial layer. A hyalinized stroma created an infiltrative pattern of growth; surface involvement (n = 8) was seen. Tumors were divided into ceruminous adenoma (n = 36), ceruminous pleomorphic adenoma (n = 4), and syringocystadenoma papilliferum (n = 1) types. The luminal cells were strongly and diffusely immunoreactive with CK7, while the basal cells were highlighted with CK5/6, S-100 protein, and p63. CD117 highlighted the luminal cells preferentially. The proliferation markers revealed a low index. Adenocarcinoma and middle ear adenoma are the principal differential consideration. Surgical excision was used in all patients. Four patients developed a recurrence due to incomplete excision. All patients were without evidence of disease at the last follow-up: alive (n = 28, mean 16.3 years) or dead (n = 12, mean 11.8 years). CONCLUSION: Ceruminous gland adenomas are the most common external auditory canal tumors. They demonstrate a dual cell population of basal myoepithelial-type cells and luminal ceruminous (ceruminal) cells. Cerumen pigment, CK7, and p63 can help to distinguish this tumor from other neoplasms that occur in the region. Complete surgical excision results in an excellent long-term clinical outcome.     

Osteoblastoma of the mandible: clinicopathologic study of four cases and literature review

BACKGROUND: Osteoblastoma is a benign bone tumor accounting for 1% of all bone tumors; it commonly involves the spine and the sacrum of young individuals, with less than 5% being localized to the posterior mandible. In view of its rarity in the maxilla and mandible, osteoblastoma is rarely diagnosed as such in the absence of interdisciplinary cooperation. METHODS: A retrospective study of four benign osteoblastomas was performed based on a review of the clinical, radiographic, and histopathologic features of all cases. RESULTS: The tumors involved the posterior mandible of young patients (age range, 10-21 years; two male and two female patients) and appeared as painful bone expansions. Radiologically, they were poorly defined, radiolucent/radiopaque lesions containing calcifications and not showing sclerotic borders or periosteal reactions. Histologically, they were composed of osteoid and woven bone surrounded by plump osteoblast-like cells with interposed fibroblasts, inflammatory cells, and red blood cells. All patients were disease free after prolonged follow-up. CONCLUSIONS: Osteoblastomas may be distinguished from other bone tumors, fibro-osseous lesions, and odontogenic neoplasms on the basis of integrated clinical, radiologic, and histologic features and usually manifest an indolent clinical course.     

Primary laryngeal T-cell lymphoma: A case report and review of the literature

IntroductionPrimary lymphoma of the larynx is extremely rare, representing less than 1% of all primary laryngeal neoplasms. It consists mainly of non-Hodgkin lymphomas (NHLs), represented particularly by diffuse large B-cell. Extranodal natural killer/T-cell lymphoma, presented in larynx is a rare condition that accounts for less than 11% of all lymphomas without distinctive clinicopathologic features, as well as challenging pathologic diagnosis.Case reportWe report here a case of a 64-years-old man who presented with primary lymphoma type T of the subglottic larynx. A histopathological examination of the biopsy confirmed non-Hodgkin T cell lymphoma. Given his age, he underwent chemotherapy and radiation therapy. The patient was disease-free after 18 months follow up.ConclusionThe clinicopathological characteristics and rational treatment of primary laryngeal lymphoma are still unclear and need to be further defined due to the paucity of this pathology.     

原发性脾脏淋巴瘤五例报告并文献复习

目的探讨原发性脾脏淋巴瘤临床特点,为临床早期诊断和治疗提供依据,降低临床误诊率。方法收集2005年1月至2016年4月收治原发性脾脏淋巴瘤5例病例资料,复习相关原发性脾脏淋巴瘤文献并进行分析。结果 5例均行脾切除术,术后病理检查为脾非霍奇金淋巴瘤,其中3例为弥漫性大B细胞淋巴瘤,2例为脾边缘区B细胞淋巴瘤。结论原发性脾脏淋巴瘤早期症状和体征无特异性,临床易误诊,而通过提高对该病的认识,并辅以相关检查,可早期诊断,降低临床误诊率。     

Lipoblastoma and liposarcoma in children: an analysis of 9 cases and a review of the literature

Objectives

To review the experience at a children’s hospital of lipoblastoma and liposarcoma and to identify any factors that would differentiate one type of tumour from the other.

Design

A retrospective case series.

Setting

British Columbia’s Children’s Hospital a tertiary-care pediatric centre.

Patients

All patients with a pathological diagnosis of lipoblastoma and liposarcoma recorded over 12 years.

Main outcome measures

The frequency of lipoblastoma and liposarcoma, identified from biopsy specimens of pediatric adipose tumours. The clinical, pathological and cytogenetic variables between lipoblastoma and liposarcoma.

Results

One hundred and forty-nine adipose tumours were recorded. Seven (4.7%) were lipoblastomas and 2 (1.3%) were liposarcomas. All tumours presented as asymptomatic, slow-growing, soft-tissue masses. The children with lipoblastoma tended to be younger, but 29% were over 3 years of age. The liposarcoma patients were aged 9 and 14 years. One liposarcoma was of myxoid type and the other was a round cell variant. Karyotypes were reported for 1 lipoblastoma and 1 liposarcoma. The myxoid liposarcoma karyotype was 46,XY,t(12;16)(q13;p11), and the lipoblastoma was reported as 46,XY,der(8)?t(8q;?),+mar.

Conclusions

Lipoblastoma is an unusual childhood neoplasm and liposarcoma is very rare in children. Both tumours may present in a similar fashion, and differentiating them histologically can be difficult. Age cannot be relied upon to accurately predict their behaviour. The tumour karyotype is very helpful in differentiating these neoplasms.     

Colonic mucosubmucosal elongated polyp: a clinicopathologic study of 13 cases and review of the literature

Colonic mucosubmucosal elongated polyp (CMSEP) is a distinctive non-neoplastic colorectal polyp characterized by pedunculated, elongated shape and is composed mainly of expanded submucosa with a normal mucosal lining. Only a small number of these polyps have been reported, exclusively from Japan. We report the clinicopathologic characteristics of 13 CMSEPs occurring in 11 patients, mostly from European ancestry. Ten of these polyps were resected during colonoscopy, and 3 were diagnosed in a patient who underwent sigmoid resection for diverticular disease. Among patients who had undergone a colonoscopy, 4 had altered bowel habit, and 1 suffered from abdominal discomfort; the other 5 patients had routine screening colonoscopy. Eight polyps were located in the sigmoid colon, 3 in the right colon, 1 in the rectosigmoid junction, and 1 in the descending colon. Polyp size ranged from 10 to 150 mm. Histologically, CMSEPs were characterized by unremarkable large bowel mucosa and submucosal stalk containing dilated thick-walled veins running parallel to the long axis of the polyp. Mucosal inflammation or fibromuscular proliferation characteristic of mucosal prolapse was absent. The pathogenesis of CMSEP may involve mechanical traction of the mucosa and the superficial submucosa during peristalsis in a fragile area of the colon. Despite the occasional large size, CMSEP is a benign lesion seldom leading to clinical complications.     

Chondrosarcoma of the larynx: a clinicopathologic study of 111 cases with a review of the literature

Chondrosarcomas of the larynx are rare tumors accounting for about 0.5% of all laryngeal primary tumors. A total of 111 laryngeal chondrosarcoma cases, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology. There was a 3.6:1 male/female ratio of patients 25-91 years of age (mean, 64.4 years). Patients presented most frequently with hoarseness (n = 72 patients) present for a mean of 28.2 months. The majority of tumors involved the cricoid cartilage (n = 77) with a mean size of 3.5 cm. All tumors were invasive and malignant by radiology and/or histology (into bone within the ossified laryngeal cartilages in 52 tumors). Most tumors were low-grade lesions: grade 1 (n = 51), grade 2 (n = 54); there were six grade 3 tumors. An associated benign chondroma with (n = 41 tumors) or without ischemia (n = 24 tumors) was noted. All patients had surgery and five had radiation therapy. Wide excision or voice-sparing surgery was used in 73 patients, whereas 37 patients had a laryngectomy. Recurrences occurred in 20 (18%) patients, 10 of whom underwent salvage laryngectomy. At the last follow-up, 102 patients had no evidence of disease (alive or dead, mean 11.2 years) and five patients had evidence of disease (alive, one patient, 6.5 years; dead, four patients, mean 6.4 years). The six patients with high-grade chondrosarcoma were all without disease at the last follow-up (mean, 15.1 years). There was no difference in clinical outcome based on grade (p = 0.210), location (p = 0.078), or treatment (p = 0.607) but was worse for patients with a myxoid-type chondrosarcoma (p = 0.044). Primary laryngeal chondrosarcomas are typically low- to moderate-grade lesions involving the cricoid cartilage, frequently associated with a chondroma. They usually portend an excellent overall long-term prognosis with initial conservative voice-sparing surgery.     

Primary carcinosarcoma of the liver: clinicopathologic features of 5 cases and a review of the literature

Carcinosarcoma of the liver is very rare worldwide. The terminology and pathogenesis of hepatic carcinosarcoma remain controversial issues. In this article, we studied the clinicopathologic features of 5 cases of hepatic carcinosarcomas (matching the World Health Organization definition), analyzed the clinical data, histologic and immunohistochemical (IHC) results, and discussed the terminology, pathologic differential diagnoses, pathogenesis, and prognosis. The patients were 40 to 68 years old, and included 4 males and 1 female. All patients were Hepatitis B surface antigen positive with para-tumorous cirrhosis. The largest dimensions of the neoplasms ranged from 6.0 to 14.0 cm. Satellite nodules, portal vein tumor thrombi, direct invasion into local tissues (right diaphragm, right adrenal gland, and gastric wall) as well as metastatic foci in lungs and abdominal lymph nodes were identified. Pathologically, the neoplasms consisted of carcinomatous and sarcomatous components. The carcinomatous components were exclusively conventional hepatocellular carcinomas in all 5 cases, whereas the sarcomatous components exhibited complex features. Confirmed by IHC studies, the sarcomatous elements in different cases included rhabdomyosarcomas, malignant fibrous histiocytomas, fibrosarcoma, and poorly differentiated spindle cells without distinctive differentiation. Furthermore, the sarcomatous elements in these 5 neoplasms stained negative for all the epithelial markers we applied for IHC staining, which support the pathologic diagnosis of carcinosarcoma rather than sarcomatoid carcinoma. The presence of transitional zones between carcinomatous and sarcomatous components may support the transformation theory. Four patients with palliative hepatectomy died within 6 months, whereas 1 patient is still alive 21 months after radical resection. The poor prognosis of hepatic carcinosarcoma may be due to their highly invasive and metastatic features. Radical resection of early stage hepatic carcinosarcoma may contribute to a relatively optimistic prognosis.     

Extracranial sinonasal tract meningiomas: a clinicopathologic study of 30 cases with a review of the literature

Extracranial meningiomas of the sinonasal tract are rare tumors. These tumors are frequently misclassified, resulting in inappropriate clinical management. To date, there has been no comprehensive study to evaluate the clinicopathologic aspects of meningioma in these anatomic sites. Thirty cases of sinonasal tract meningiomas diagnosed between 1970 and 1992 were retrieved from the files of the Otorhinolaryngic Registry of the AFIP. Histologic features were reviewed, immunohistochemical studies were performed, patient follow up was obtained, and the results were statistically analyzed. The patients included 15 females and 15 males, aged 13 to 88 years (mean, 47.6 yrs). Patients presented clinically with a mass, epistaxis, sinusitis, pain, visual changes, or nasal obstruction, dependent on the anatomic site of involvement. Symptoms were present for an average of 31.1 months. The tumors affected the nasal cavity (n = 14), nasopharynx (n = 3), frontal sinus (n = 2), sphenoid sinus (n = 2). or a combination of the nasal cavity and ethmoid, frontal, sphenoid, and/or maxillary sinuses (n = 9). The tumors ranged in size from 1.0 to 8.0 cm in greatest dimension (mean, 3.5 cm). Radiographic studies demonstrated a central nervous system connection in six cases. The tumors often eroded the bones of the sinuses (n = 18) and involved the surrounding soft tissues, the orbit, and occasionally the base of the skull. Histologically, the tumors demonstrated features similar to intracranial meningiomas. The majority were of the meningothelial type (n = 23), although there were three atypical meningiomas. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (EMA) and vimentin (all tested). The differential diagnosis includes paraganglioma, carcinoma, melanoma, psammomatoid ossifying fibroma, and angiofibroma. Surgical excision was used in all patients. Three patients died with recurrent disease (mean, 1.2 yrs), one was alive with recurrent disease (25.6 years), and the remaining 24 patients were alive or had died of unrelated causes (mean, 13.9 yrs) at the time of last follow up (two patients were lost to follow up). Extracranial sinonasal tract meningiomas are rare tumors which need to be considered in the differential diagnosis of sinonasal tumors. A whorled growth pattern and psammoma bodies, combined with positive EMA and vimentin immunohistochemical reactions, can confirm the diagnosis of meningioma. The overall prognosis is good, without a difference in outcome between benign and atypical meningiomas.     

Sinonasal lymphoma: a clinicopathologic analysis of 58 cases from the Massachusetts General Hospital.

Few large series compare lymphomas of the nasal cavity with those of the paranasal sinuses. We studied the cases of 58 patients, 34 males and 24 females, aged 7 to 92 years (mean, 57 years), who had lymphoma involving the nasal cavity or paranasal sinuses. Thirty-three patients had diffuse large B-cell lymphoma (DLBCL). Twenty-three were male and 10 were female, with an age range of 7 to 91 years (mean, 63 years); two were HIV-positive. Only 2 of 11 cases tested (one in an HIV-positive patient and one of lymphomatoid granulomatosis type) were Epstein-Barr virus (EBV)-positive. Thirty (91%) involved paranasal sinuses, 10 with nasal involvement, whereas three cases had nasal, but not sinus, involvement. At last follow-up, 16 (67%) were free of disease 7 to 169 months later (mean, 65 months), and 8 (33%) had died of disease 2 to 166 months later (mean, 45 months). Seventeen patients had nasal-type natural killer (NK)/T-cell lymphoma. There were 10 women and 7 men, aged 27 to 78 years (mean, 48 years). Thirteen of 14 were EBV-positive. Sixteen patients had nasal involvement, eight with sinus involvement. Eleven (73%) of 15 were alive and well 6 to 321 months later (mean, 139 months), three (20%) died of lymphoma 1, 11, and 12 months later, and one (7%) is alive with disease. There was one case each of marginal zone B-cell lymphoma, Burkitt's lymphoma, Burkitt-like lymphoma, peripheral T-cell lymphoma of unspecified type, and adult T-cell lymphoma/leukemia. In an additional three cases, the lymphomas were composed predominantly of large cells, but no immunophenotyping could be performed for subclassification. In 19 cases (17 DLBCLs, 1 Burkitt-like lymphoma, and 1 lymphoma of uncertain lineage), presenting symptoms included complaints related to the eyes. In 16 cases (13 DLBCLs, 1 Burkitt-like lymphoma, 1 nasal NK/T-cell lymphoma, and 1 lymphoma of uncertain lineage), the orbit was invaded by lymphoma. In our series, the most common lymphoma to arise in the sinonasal area is DLBCL, followed by nasal NK/T-cell lymphoma. Comparison of these two types of lymphoma showed that lymphomas involving sinuses without nasal involvement were predominantly DLBCLs (20 of 21), whereas nasal cavity lymphomas without sinus involvement were usually NK/T-cell type (8 of 11) (p = 0.000125). Compared with patients with DLBCL, patients with nasal NK/T-cell lymphoma were overall younger, with a lower male-to-female ratio. Lymphomas of B-cell lineage were more likely to be associated with symptoms related to the eyes (p < 0.0005) and to have extension to the orbit (p < 0.01) than were lymphomas of T- or NK-cell lineage. In contrast to results of Asian studies in which nasal NK/T-cell lymphoma has a very poor prognosis, our nasal NK/T-cell lymphomas had an outcome similar to that of DLBCL.     

Carcinoid tumor of the thymus: a clinicopathologic report of two cases with a review of the literature

Thymic carcinoid, a tumor with neuroendocrine-derived cells, is extremely rare. Its clinical and pathological features are distinct from other thymic tumors or carcinoids in other organs. The prognosis is poorer compared with other thymic tumors. Herein, we describe two cases with thymic carcinoids that were completely resected and underwent radiotherapy after surgery. The postoperative follow-up periods were 7 years and 7 months. These rare cases are discussed in light of the clinico-pathological picture of thymic carcinoid reported in the literature.     

Adenomatoid tumor of the adrenal gland: a clinicopathologic study of five cases and review of the literature

We report the clinicopathologic, immunophenotypic, DNA ploidy, and MIB-1 proliferative findings of five adenomatoid tumors of the adrenal gland. All patients were male, and tumors were incidental radiologic, surgical, or autopsy findings. Mean patient age at diagnosis was 41 years (range 31-64 years). The tumors ranged from 1.2 to 3.5 cm (mean 2.8 cm; median 3.2 cm) in greatest dimension, and all originated within the adrenal gland. The tumors were composed of anastomosing variably sized tubules lined by epithelioid as well as flattened cells. Signet-ring-like cells were present in all cases. The previously described histologic patterns of adenomatoid tumor, adenoid, angiomatoid, cystic, and solid, were observed, and each tumor contained multiple histologic patterns. In three of five cases, there was extra-adrenal extension of tumor into periadrenal adipose tissue. All adenomatoid tumors infiltrated the adrenal cortex, and in four cases the adrenal medulla was involved. All tumors exhibited strong immunoreactivity for calretinin, cytokeratins AE1/AE3, and CAM 5.2, cytokeratin 7, and vimentin. Tumors showed weak and focal immunoreactivity for cytokeratin 5/cytokeratin 6 and were negative for CD15, CD31, CD34, cytokeratin 20, MOC31, and polyclonal carcinoembryonic antigen. Ploidy analysis using Feulgen-stained sections and image analysis showed that three tumors were diploid and two were tetraploid. Tumors exhibited low MIB-1 proliferative activity, ranging from 0.2% to 2.7% (mean 1.6%). In three cases with clinical follow-up, no recurrence or metastases occurred. Adrenal gland adenomatoid tumors are morphologically and immunophenotypically identical to adenomatoid tumors of the genital tract and appear benign.     

原发性肾脏淋巴瘤2例报道并文献复习

目的探讨原发性肾脏淋巴瘤的临床特点。方法总结2例原发性肾脏淋巴瘤患者的临床资料,结合文献讨论其发病特点、影像学特征、治疗及预后。结果 2例患者均实施手术加化疗,病理诊断均为非何杰金淋巴瘤,1例死于术后2月,另1例已存活1年,仍在随访中。结论原发性肾脏淋巴瘤是一种罕见的恶性淋巴瘤,影像学征象与肾细胞癌相似,以成人发病为主,易误诊为肾癌,病理类型多为B细胞来源的非何杰金淋巴瘤,综合治疗是延长生存的较好方式。