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.     

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.     

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.     

Sarcomatoid carcinoma of the penis: a clinicopathologic study of 15 cases

Sarcomatoid carcinomas are uncommon, high-grade tumors, predominantly composed of spindle cells. Only a few cases arising in the penis have been reported. The aim of this study is to better define the clinicopathologic features of this neoplasm. A total of 400 cases of squamous cell carcinoma of the penis were reviewed from which 15 sarcomatoid carcinomas (4%) were identified. Clinical and pathologic features were evaluated in all cases. Immunohistochemical studies for expression of AE1/AE3, Cam 5.2, 34betaE12, EMA, vimentin, muscle specific actin, smooth muscle actin, desmin, S-100, p63, and p53 and in situ hybridization studies for HPV were performed in 5 cases. Information about lymph node status was available in 9 cases, and follow-up in 5 cases. The mean age was 59 years, and mean tumor size was 5 cm. Grossly, most tumors were large, polypoid, and ulcerated masses frequently affecting the glans (93%) and deeply invading corpora cavernosa (80%) and skin. Microscopically, the lesions were predominantly composed of atypical spindle cells disposed in interlacing fascicles, resembling fibrosarcoma or leiomyosarcoma, sometimes admixed with pleomorphic giant cells mimicking malignant fibrous histiocytoma. One case was predominantly composed of myxoid areas. Less frequent and focal patterns were pseudoangiomatous and epithelioid. Mitotic figures were numerous, and necrosis was prominent. Foci of heterologous differentiation toward bone (osteosarcomatous component) were present in 1 case. Four cases showed a minor mixed component of usual, papillary, verrucous, and basaloid carcinoma. Intrapenile metastasis ("satellitosis") was present in 4 tumors. One of the cases was multicentric with a separate independent focus of well-differentiated carcinoma with pseudohyperplastic features. Associated low- and high-grade squamous intraepithelial lesions were noted in 73% of the cases. Immunohistochemical studies and HPV in situ hybridization were done in 5 cases. The spindle cells were diffusely positive for vimentin and p53 and showed at least intermediate expression of 34betaE12 and p63 in all cases. EMA and AE1/AE3 were focally positive in 60% of the cases, and Cam 5.2 was focally positive in 1 case. Tumor cells failed to express muscle specific actin, smooth muscle actin, desmin, and S-100. HPV in situ hybridization was negative in all cases. Inguinal metastases were present in 89% of the cases. Two of five patients with adequate follow-up died of disease within 8 months of the diagnoses. In conclusion, penile sarcomatoid carcinomas are unusual, large, and aggressive tumors usually associated with lymph node metastasis and poor outcome. Differential diagnoses include sarcoma and melanoma. Cytokeratin 34betaE12 and p63 appear to be the more specific and sensitive markers to categorize these tumors as epithelial. Diffuse immunoreactivity for p53, compared with a more basal and focal reactivity in differentiated squamous cell carcinoma, may be indicative of a late mutation in the natural progression of the disease.     

Leiomyosarcoma of the inferior vena cava: a clinicopathologic review and report of four cases

AIM: This article describes four cases of leiomyosarcoma treated in the University Hospital of Turin and reviews current understanding of the biological behavior of the tumor, together with essential diagnostic procedures and established approaches to treatment. METHODS: Between February 2004 and December 2005, four patients (two men and two women; mean age 57.5 years) with leiomyosarcoma of the interior vena cava (IVC) were treated at the Vascular Surgery Unit. Resection and excision of the tumor were carried out (4/4 patients), with resection of the IVC above and below the mass (2/4) and reconstruction of the vein with a Dacron prosthesis with termino-lateral reimplantation of the renal vein in one and placement of a polytetrafluorethylene graft in the other. RESULTS: The postoperative course was unremarkable; the patients were discharged between postoperative days VIII and XI. Oral anticoagulant therapy with dicumarol (3/4) was given to maintain venous or prosthesis patency during the follow-up period. The mean length of follow-up was 23 months (range, 16-28). All patients recovered without local recurrence of disease. CONCLUSION: This malignant slow-growing tumor produces late clinical manifestations, making the study of its natural history clinically important. Currently, surgical excision is the only therapy that can alter disease progression and improve survival. The utility of adjuvant chemo- and radiotherapy remains controversial.     

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.     

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.     

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.     

Leiomyosarcoma of the epididymis: 2 cases and review of the literature

Paratesticular leiomyosarcomas are rare. We report two cases of leiomyosarcoma of the epididymis diagnosed and treated in a regional community hospital in Canada in 2009 and 2010. Both patients were males of ages 58 and 75, respectively. They presented with painless masses which, on ultrasonography, appeared to be solid masses demonstrating blood flow. Each patient underwent trans-scrotal resection of the affected epididymis. Pathological examination of trans-scrotal epididymectomy specimens confirmed the diagnosis of high-grade leiomyosarcoma. Leiomyosarcoma is an important differential diagnosis for paratesticular masses in the elderly male; the primary treatment is radical inguinal orchiectomy with high ligation of the spermatic cord.     

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.     

Cavernosography in diagnosis of metastatic tumors of the penis: 5 new cases and a review of the literature

Secondary tumors of the penis are rare, with 266 cases having been reported. We describe 5 new cases studied by cavernosography. The primary tumors were bladder cancer in 3 patients and prostatic cancer in 2. Cavernosography suggested the diagnosis, which was confirmed by a guided biopsy or fine needle aspiration biopsy. The prognosis has been poor, with the average survival being less than 9 months.     

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.     

Pulmonary and mediastinal glomus tumors--report of five cases including a pulmonary glomangiosarcoma: a clinicopathologic study with literature review

Pulmonary and mediastinal glomus tumors are rare lesions, with four previously reported primary pulmonary cases and three mediastinal cases. The authors report one mediastinal glomus tumor, a locally infiltrative type, and four pulmonary glomus tumors, including the first case of primary pulmonary glomangiosarcoma. These tumors show a variety of clinical and pathologic differences from the more common cutaneous variety, including later age at presentation, larger size, and more frequent atypical/malignant features. Mediastinal and pulmonary glomus tumors both have an average patient age at presentation of 45 years. However, compared with their pulmonary counterparts, mediastinal glomus tumors are less common, more often symptomatic, and are larger (average size, 5.4 cm). Additionally, mediastinal glomus tumors more often demonstrate malignant or atypical features. Pulmonary glomus tumors average 3.3 cm in greatest dimension, with the majority measuring less than 2.5 cm. The pulmonary glomangiosarcoma presented was large, measuring 9.5 cm, and showed increased mitotic count (9 mitoses/10 high-power fields), necrosis, cytologic atypia, and was associated with disseminated disease. Regardless of clinical symptoms, histologic features, and even metastases, the vast majority of all benign and malignant glomus tumors are indolent and cured surgically, with adjuvant therapy needed only for occasional patients with more advanced disease. The four patients with glomus tumors reported are currently alive and free of disease as of last follow up. The patient with the glomangiosarcoma developed widespread metastases and died of disease 68 weeks after initial therapy.     

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.     

Basaloid squamous cell carcinoma of the penis with papillary features: a clinicopathologic study of 12 cases

There are 3 distinct variants of penile squamous cell carcinoma frequently associated with human papillomavirus (HPV): basaloid, warty-basaloid, and warty carcinomas. Considering the high incidence rates of penile cancer in some countries, a large international study was designed to evaluate the presence of HPV, its genotype distribution, and its association with histologic types of penile cancer. In this international review of >900 cases, we found a group of highly distinct papillary neoplasms composed of basophilic cells resembling urothelial tumors but frequently associated with HPV. Macroscopically, tumors were exophytic or exoendophytic. Microscopically, there was a papillomatous pattern of growth with a central fibrovascular core and small basophilic cells lining the papillae. Positivity for HPV was present in 11 of 12 tumors (92%). Single genotypes found were HPV-16 in 9 tumors and HPV-51 in 1 tumor. Multiple genotypes (HPV-16 and HPV-45) were present in another case. Overexpression of p16 was observed in all cases. Uroplakin-III was negative in all cases. The differential diagnosis was with basaloid, warty-basaloid, warty, and papillary squamous cell carcinoma and with urothelial carcinomas. Local excision (4 cases), circumcision (3 cases), or partial penectomy (5 cases) were preferred treatment choices. Tumor thickness ranged from 1 to 15 mm (average, 7 mm). Two patients with tumors invading 11 and 15 mm into the corpus spongiosum developed inguinal nodal metastasis. Of 11 patients followed up (median 48 mo), 7 were alive with no evidence of metastatic disease, 3 died from causes other than penile cancer, and another died postoperatively. This morphologically distinct tumor probably represents a papillary variant of basaloid carcinomas (papillary-basaloid carcinomas). Unlike typical basaloid carcinomas, the overall prognosis was excellent. However, deeply invasive tumors were associated with regional nodal metastasis indicating a potential for tumor-related death.     

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.     

膀胱平滑肌肉瘤3例报告并文献复习

目的 探讨膀胱平滑肌肉瘤的临床病理和诊治方法.方法 患者3例,肉眼血尿,膀胱镜检见肿瘤分别发生于膀胱左侧壁、左前壁和右后壁,直径2.0～3.5 cm,以菜花样和乳头样为主,基底较宽,瘤体表面光滑.结果 3例均行全膀胱切除术,病理表现为上皮下间质富于黏液,黏液中可见散在或束状分布的细长梭形瘤细胞,其胞质淡染,核呈长梭形,有轻度异型,核分裂象易见.免疫组化SMA(+)3例、MSA(+)3例、Des(+)2例、S-100(-)2例、CD34(-)3例,均诊断为膀胱低级别平滑肌肉瘤.随访6个月～8年,1例术后2.5年死亡,2例存活.结论 膀胱平滑肌肉瘤临床罕见,确诊依赖病理及免疫组化检查,应与平滑肌瘤、横纹肌肉瘤、癌肉瘤、炎性肌纤维母细胞瘤等鉴别,治疗以手术为主,需根据肿瘤分级、分期决定手术方案及术后是否需行辅助治疗.