Bilateral Wilms' tumor: a clinicopathologic review

Bilateral Wilms' tumor occurs at a younger age than unilateral disease. While it generally has a good prognosis, it presents a therapeutic dilemma to balance curative surgical resection with preservation of renal tissue. A 15 year review of bilateral Wilms' tumors diagnosed at Princess Margaret Hospital was undertaken. Of 46 Wilms' tumor cases, eight were designated bilateral by diagnostic imaging (median age 1.1 years compared with 3.5 years for unilateral tumors). The surgical management entailed primary nephrectomy with contralateral biopsy in two patients, and bilateral biopsy and delayed resection in all remaining surviving patients (one patient died of perioperative complications). Seven patients had localized disease (stage I/II) and the six surviving patients received chemotherapy with vincristine and actinomycin; no patient received radiotherapy. All are alive and well (median follow-up 5.1 years). The remaining patient presented with pulmonary metastases and died of disease progression. Pathologic review revealed that four patients had truly bilateral disease demonstrable by histology, three had unilateral Wilms' tumor with contralateral nephrogenic rests, and in one patient the biopsies of the contralateral kidney showed neither tumor nor nephrogenic rests. In most cases pathological material was subject to external review. Follow-up demonstrates excellent renal function with compensatory hypertrophy in the remaining renal tissue. Conservative surgery and simple out-patient based, low toxicity chemotherapy is curative in most patients.     

Case report: adenocarcinoma of the kidney developing metastzsizing to both lungs, with numerous microliths radiologically imitating Microlithiasis alveolaris pulmonum (author's transl)]

A 28 years old man developed a radiological pattern like Microlithiasis alveolaris pulmonum during the following five years after exstirpation of his right kidney due to an adenocarcinoma. Tests showed, in contrast to the x-ray pulmonary function, only a slight restriction of total lung capacity over a long time up to few months before he died. Autopsy showed a spread of miliary and submiliary metastases in the lower parts of both lungs. In all metastases psammomabodylike microliths were found microscopically, especially numerous and enlarged with both lungs. A second check of the slides of the primary tumour showed rare microliths too, not mentioned at that time. A microlith producing tumour of the kidney was first published with full details by Lubarsch in 1925. The radiological aspect of the lungs of the reported case reveals serious differential diagnostical problems to Microlithiasis alveolaris pulmonum.     

Fatal outcome in Eisenmenger syndrome.

AIM: To assess correlations between fatal outcome and histologic findings of pulmonary vascular disease in different situations of Eisenmenger syndrome, either during the natural course or early-late after surgery. MATERIAL AND METHODS: The clinical follow-up and fatal outcome of 20 patients affected by Eisenmenger syndrome were investigated. In addition to the pathologic report and gross reexamination of the heart, the lung tissue was studied by histology. Patients were divided into three groups: 6 non-operated patients who died during the natural course (Group 1), 11 patients who underwent correction of the congenital defect and died in the perioperative period (Group 2), and 3 patients who died late after surgery (Group 3). RESULTS: In Group 1, five patients (83%) died of cardiac arrest a few days after the onset of hypoxic attacks; in four patients histology showed Grade IV pulmonary vascular disease with diffuse fibrinoid necrosis in the distal pulmonary arterial vasculature. In Group 2, nine patients (82%) died on the first or second postoperative day after a refractory pulmonary hypertensive crisis, with histologic evidence in three patients of fibrinoid necrosis of the distal pulmonary small arteries and arterioles. In Group 3, two patients (67%) died suddenly, 6 and 18 years after cardiac surgery, following onset of dyspnea and cardiogenic shock; autopsy showed aneurysmal dilatation of the pulmonary artery with massive thrombosis in the setting of Grades III-IV pulmonary vascular disease without fibrinoid necrosis. CONCLUSION: Fatal outcome in Eisenmenger syndrome, either in the natural course or after refractory hypertensive attacks post surgery, is frequently associated with fibrinoid necrosis of the small pulmonary arteries and arterioles.     

Ovarian micropapillary serous borderline tumors. Clinicopathologic features and outcome of seven surgically staged patients

We report the clinicopathologic findings for 7 patients with completely staged ovarian micropapillary serous borderline tumors (MSBTs) to further clarify tumor behavior. None of the MSBTs had microinvasion in the ovarian neoplasm. The MSBT pattern constituted 25% to almost all of the neoplasm. Four were bilateral, and 6 involved the ovarian surface. Five patients had peritoneal implants; 2 were invasive, and 3 were noninvasive MSBTs. Distribution of stages among patients was as follows: IA, 1; IC, 1; IIC, 2; IIIB, 2; and IIIC, 1. Median follow-up was 8.5 years. Four patients were alive and well at the last follow-up visit, including 1 patient with stage IIIC (lymph node metastases) disease who had noninvasive implants (12 years after surgery). One patient who was free of disease died of complications of chemotherapy and abdominal surgery. Two patients died of intra-abdominal neoplastic growth (stages IIC and IIIB) 5 and 9 years after surgery, respectively; both had invasive implants. Without invasive peritoneal implants, MSBTs seem to behave as similar staged nonmicropapillary serous borderline tumors without invasive peritoneal implants. With invasive peritoneal implants, they seem to behave as low-grade carcinomas. Pathologists should recognize MSBT as a neoplasm that can have adverse prognostic features, including invasive peritoneal implants.     

Follow-up study of long-term survivors of osteosarcoma in the prechemotherapy era

Osteosarcoma is the most common primary bone sarcoma. Several studies published in the 1960s established that approximately one fifth of patients survive when treated with surgery alone. There is no information, however, about the long-term consequences of osteosarcoma. It is especially relevant to know if these patients are at risk for a second malignancy. We reviewed all clinical records from long-term (defined as more than 10 years) osteosarcoma survivors treated at Mayo Clinic in the prechemotherapeutic era from 1900 to 1960. We re-reviewed histological sections for most cases. Patients or next of kin provided follow-up information during telephone interviews. Rates of second malignancy were compared with expected rates in the population at large. We identified 465 patients treated for osteosarcoma. Of these patients, 83 (17.8%) were long-term survivors, including 19 who were alive up to 65 years after treatment. Of the 7 patients with pulmonary metastases, 3 died. A second malignancy developed in 26 patients, 15 of whom died of the malignancy. Although long-term survivors of osteosarcoma have a higher incidence of a second malignant tumor than a normal population, this increase was not statistically significant. No demographic or histological variables predicted long-term survival.     

Renal oncocytoma and granular renal cell carcinoma. A comparative clinicopathologic and DNA flow cytometric study.

Twenty-three renal oncocytomas and 18 granular renal cell carcinomas (GRCC) were comparatively studied clinicopathologically and by DNA flow cytometry to delineate their differences. Of the patients with renal oncocytomas, 15 were men and 8 were women, and their ages ranged from 42 to 81 years (mean, 64 years). The gross appearance of renal oncocytomas was characteristically homogeneous tan-brown, with variable scarring, without areas of large hemorrhage, and with no apparent necrosis. Twenty-two renal oncocytomas were confined within the kidney (Robson stage I) and one tumor extended into the renal vein (stage IIIa). Twenty-two renal oncocytomas, including the stage IIIa tumor, manifested diploid DNA content and only one neoplasm showed a feature suggestive of near-diploid DNA aneuploidy. Of the 17 patients with renal oncocytomas who had adequate follow-up, none developed metastasis or died of disease. Of the patients with GRCC, 13 were men and 5 were women, and their ages ranged from 30 to 73 years (mean, 53 years). The gross appearance of GRCC was variegated, yellow to tan, and punctuated with geographic areas of necrosis. Eleven patients with GRCC were stage I, 4 were stage II, 2 were stage IIIa, and 1 patient had metastases at initial examination (stage IV). Seven GRCCs were DNA diploid, one was DNA tetraploid, and 10 tumors were DNA aneuploid. Twelve patients were alive with no evidence of disease (12 to 36 months; median, 26 months). All patients with DNA diploid neoplasm pursued benign clinical courses. One patient was alive with metastatic disease and two patients developed metastases and died of their disease; all three patients had DNA aneuploid tumors. Two patients died of other causes and one patient was lost to follow-up. Our data indicate that renal oncocytoma is a distinct clinicopathologic disease with characteristic gross, histologic, DNA flow cytometric, and biologic features that are different from GRCC.     

Immediate adjuvant chemotherapy versus observation with treatment at relapse in pathological stage II testicular cancer

Between 1979 and 1984, 195 evaluable patients were entered in an international multicenter study comparing two regimens for patients with completely resected pathological Stage II testicular cancer (that is, with positive retroperitoneal lymph nodes). All patients had undergone orchiectomy and dissection of the retroperitoneal lymph nodes. They were randomly assigned to be treated with two cycles of immediate adjuvant cisplatin-based chemotherapy or to be observed monthly with treatment at relapse. The median follow-up period was four years. Of the 97 patients assigned to adjuvant chemotherapy, 6 (6 percent) had a recurrence; however, only 1 had received adjuvant chemotherapy before the recurrence. Three died (one of testicular cancer), and 94 of the 97 survived. Of the 98 patients who were observed, 48 (49 percent) had a relapse. However, almost all patients with relapses were effectively treated, and 93 of the 98 are alive and disease-free; 3 have died of testicular cancer. No identifiable factors were strongly associated with the risk of relapse. We conclude that two courses of cisplatin-based adjuvant chemotherapy will almost always prevent relapse in pathological Stage II testicular cancer treated with orchiectomy and retroperitoneal-lymph-node dissection. However, when surgery, follow-up, and chemotherapy are optimal, observation with chemotherapy only for relapse will lead to equivalent cure rates.     

High-grade carcinoma component in epithelial-myoepithelial carcinoma of salivary glands clinicopathological, immunohistochemical and flow-cytometric study of three cases

 Three cases of epithelial-myoepithelial carcinoma (EMC) with coexisting areas of high grade carcinoma are reported. In two of the cases there was a previous recurrence, and in all three patients there had been a sudden increase in size before final surgery. The typical ductal and myoepithelial components of EMC showed the usual biphasic pattern and the expected immunophenotypes, with expression of wide spectrum cytokeratins, Cam 5.2 and EMA in the ductal part, and muscle-specific actin, smooth muscle actin, S-100 protein, vimentin and cytokeratins in the myoepithelial component. These areas also had a low mitotic count and low proliferation rate as measured by immunohistochemistry and by flow cytometry. Conversely, areas of high-grade tumour had the features of a large cell carcinoma, with focal mucin secretion in two cases. This high-grade component showed an epithelial immunophenotype in two cases, and was negative for all tested markers in the third one. The mitotic counts and the proliferation rates were much higher in these anaplastic areas. One of the patients died 3 months after treatment; another developed lymph node metastases 1 year later and was alive after 6 years of follow-up. The third patient was alive without evidence of disease 7 months after wide surgical resection of the tumour. The possibility of anaplastic transformation in EMC makes thorough sampling mandatory in this type of neoplasm. Received: 14 Octobre 1998 / Accepted: 7 December 1998     

Adenoid Cystic Carcinoma of the Breast

Twelve cases of pure adenoid cystic carcinoma of the breast were reviewed. Patients ranged in age from 34 to 69 years. Seven carcinomas were in the right breast, and five in the left; five of the 12 were located in the central region of the breast, five in the upper outer quadrant, and the two in the upper inner and lower inner quadrants, respectively. Average diameter of the primary tumors was 2.5 cm (range, 0.7 to 6.0). We graded the tumors according to a system used for adenoid cystic carcinoma of the salivary gland: five tumors were grade I, six were grade II, and one was grade III. An average of 5 years after diagnosis, all patients with grade I tumors were either alive without evidence of disease or had died of unrelated causes. Among the six patients with grade II tumors, one developed a local recurrence 5 years after diagnosis and subsequent pulmonary metastasis, and one died of metastatic adenoid cystic carcinoma 13 years after diagnosis. The one patient with grade III tumor had shown metastases in axillary lymph nodes at mastectomy, and she died of disease 2 years later. These findings suggest that the grading of adenoid cystic carcinoma of the breast may be important in prognosis and treatment selection.     

Vertebral metastases from pineoblastoma.

Two male patients aged 26 and 18 years presented with vertebral metastases originating from pineoblastomas on which surgery had been performed 8 years and 5 months earlier, respectively. In the first case in which the metastasis developed in the T8 corpus, the disease is presently under control after high-dose chemotherapy and autologous blood stem cell transplantation. The second patient (sacral metastasis), despite aggressive adjuvant therapy, died 2 years after the last operation because of spinal seeding. These uncommon cases are discussed with reference to the literature on extraneural metastases that originate from neuroepithelial tumors of the central nervous system.     

Metastasizing fibrous histiocytoma of the skin: a clinicopathologic and immunohistochemical analysis of three cases.

The clinicopathologic and immunohistochemical features of three metastasizing fibrous histiocytomas of the skin are presented. The first patient had a 1.3-cm nodule in the right thigh, with right inguinal lymph node metastases 19 years later. The second patient, who had a 3-cm nodule excised from his left thigh and inguinal lymph node metastasis after 4 months, had a favorable outcome 14 years after local radiotherapy and chemotherapy. The third had a 2-cm nodule in his neck, which recurred 16 months later. Four months later, cervical lymph node metastases were found. The patient was alive and well 26 months after initial surgery. All three primary skin tumors involved the dermis and subcutis, appeared well-delineated but nonencapsulated, were associated with some degree of epidermal hyperplasia, and showed features of aneurysmal/atypical or cellular fibrous histiocytoma. The number of mitoses ranged from 6 to 11 per 10 high-power fields. Recurrences and metastases showed morphologic features similar to primary lesions. Tumor cells were positive, at least focally, for CD 68, Ki-M1p, and Factor XIIIa, and occasionally for smooth muscle actin. Desmin, CD 34, S-100 protein, and cytokeratin stainings were negative. Primary neoplasms, recurrences, and metastases showed a Mib-1 labeling index of 10% or less. Cellular, aneurysmal, and atypical (pseudosarcomatous) fibrous histiocytomas of the skin can metastasize, yet they often show a protracted clinical course. Risk factors for metastatic dissemination include large size, high cellularity, aneurysmal changes, marked cellular pleomorphism, high mitotic activity, tumor necrosis, and repeated local recurrences.     

Total lung irradiation and chemotherapy in pulmonary metastases from carcinoma of the uterine cervix and endometrium

Despite the radiocurability of stage I and II squamous cell carcinoma (SCC) of the cervix, palliation of patients with pulmonary metastases has been disappointing. Four patients with pulmonary metastases (three from cervical and one from endometrial carcinoma) were treated with total lung irradiation, followed two to three weeks after termination of radiotherapy with combination chemotherapy consisting of vincristine 1.4 mg/m² IV+ adriamycin 50 mg/m² IV + cyclophosphamide 500 mg/m² IV repeated at four-week intervals. Radiotherapy was delivered through a Co⁶⁰ teletherapy unit in a daily dosage of 100 rads in anterior and posterior opposing fields. After completion of irradiation in one lung, the other lung was similarly treated if involved with gross disease. Two out of four patients (both with cervix SCC) obtained complete tumor regression, whereas the other two patients had stabilization of their pulmonary lesions. The survival of the two responders was 216+ and 621 days from the start of irradiation. The two nonresponders survived 56 and 109 days. Except for one patient who died of pulmonary metastases, the remaining three patients died of metastases elsewhere. While only one patient developed radiation pneumonitis, all four had alopecia and leukopenia <4000/mm³ from chemotherapy. Total lung irradiation and combination chemotherapy appear to have activity in the treatment of pulmonary metastases from SCC of the cervix and warrant further exploration.     

Primary gastric inflammatory myofibroblastic tumor: A clinicopathologic and immunohistochemical study of 5 cases

Primary gastric inflammatory myofibroblastic tumors are rare. Here we report on 5 such cases (4 males and 1 female, age range 36–45 years). Their presenting symptoms included abdominal mass (5 patients), abdominal pain (4 patients), and upper gastrointestinal hemorrhage (1 patient). Tumor size ranged from 4.5 to 8 cm in the greatest dimension. Histologically, these tumors showed three patterns: myxoid hypocellular, fascicular, and hyalinized. A lymphoplasmacytic infiltrate was present in all 5 tumors. One to two mitotic figures were recognized in 10 high power fields (HPFs) in 4 patients and focally up to 5 in 10 HPFs in 1 patient. No prominent nuclear atypia or necrosis was observed. ALK, smooth muscle actin, and vimentin staining were observed in all tumors. One tumor focally expressed desmin. S-100, CD21, CD34, CD35, CD68, and CD117 were negative in all IMTs. The patients were followed up for 2–5 years (mean 3.4 years), and none of them had tumor metastasis or died. Only one patient developed local recurrence and is now alive with no evidence of disease after the second surgery (11 months after the second surgery). Our results indicate that primary gastric IMTs have an intermediate behavior as seen at other sites.     

Intra-abdominal Follicular Dendritic Cell Sarcoma (FDCS): Series of 18 cases of a rare entity from Pakistan

ObjectivesFollicular Dendritic Cell Sarcoma (FDCS) is a rare neoplastic proliferation of dendritic cells which are immune accessory cells found in both lymphoid and non-lymphoid organs. FDCS can thus occur in lymph nodes as well as non-lymphoid organs. Intraabdominal FDCS is even rarer.Our aim was to describe the clinical and morphological features of intra-abdominal FDCSs diagnosed in our practice and to review published literature on FDCSs including intra-abdominal FDCSs.MethodsAll cases of FDCSs diagnosed between January 1, 2008 and December 31, 2019 were included in the study. Slides of the cases were reviewed and clinical follow up was obtained.ResultsA total of 18 cases of intraabdominal FDCS were diagnosed during the study period. Age range was 17 to 55 years. Mean and median ages were 28 and 29 years respectively. Of the 18 patients, 11 were male and 7 were females. Colon was involved in 9 cases and appendix in 2 cases. 9 cases were received as resection specimens while 9 cases were received as slides and blocks for second opinion. Tumor size ranged from 2.7 to 26 cm. Average tumor size in these 9 cases was 8.2 cm and in 6 of these 9 cases, tumor size was greater than 6 cm in largest dimension. Grossly, tumors were nodular or polypoid and had a fleshy, grey white, homogeneous cut surface. Histologically, all 18 cases showed proliferation of plump to spindle shaped cells arranged in a fascicular or storiform pattern. Tumor cells had mild to moderately pleomorphic spindle to ovoid vesicular nuclei with fine chromatin and inconspicuous to variably conspicuous nucleoli, and moderate amount of pale eosinophilic cytoplasm. Mitotic activity was usually brisk. CD21 and CD23 were positive in all 18 cases. Resection margins were negative in all 9 resection specimens. Lymph nodes positive for metastases were seen in 4 cases. Follow up was available in 13 cases. Recurrence was seen in 6 patients, out of which 3 patients died of disease 15, 17- and 24-months following resection. 1 patient with appendiceal FDCS was free of disease almost 12 years after surgery but recently developed recurrence and is currently undergoing chemotherapy. 6 patients were alive and well at the time of follow-up 5 to 68 months after resection. None of them had developed recurrence or metastases at the time of follow up. 8 of the 13 patients received chemotherapy and/or radiotherapy post-surgical resection.ConclusionColon was involved in 9 of our 18 cases. Lymph nodes were positive for metastases in 4 out of 9 resection specimens. All cases were diagnosed based on morphology supported by positivity for immunohistochemical stains CD21 and CD23. Histological factors associated with aggressive behavior were seen in 14 cases. Majority of patients had an aggressive clinical course.     

Pulmonary artery sarcoma: a clinicopathologic and immunohistochemical study of 12 cases

We report 12 cases of pulmonary artery sarcoma. The mean age at diagnosis was 48.4 years. Based on histomorphologic features and immunohistochemical findings, 2 tumors were classified as rhabdomyosarcoma, 4 as leiomyosarcoma, 1 as osteogenic sarcoma, 1 as angiosarcoma, and 4 as high-grade sarcoma. All patients underwent surgery. In addition, 7 patients received neoadjuvant or adjuvant therapy. Five patients died 3 to 23 months after surgery. Three patients were still alive at 8, 27, and 68 months at last follow-up. Another 3 patients were alive at 2, 15, and 40 months and then lost to follow-up. The 2 patients with the longest survival (40 months and 68 months) had a diagnosis of leiomyosarcoma. Both patients with rhabdomyosarcoma died at 3 months after surgery. Pulmonary artery sarcoma is an uncommon entity with a poor prognosis. The role of early diagnosis, histologic classification, surgical treatment, and adjuvant therapy in patient outcome is discussed.     

Poorly differentiated ('insular') carcinoma of the thyroid

Three cases of unusual poorly differentiated ('insular') carcinoma of the thyroid gland are presented. These three thyroid carcinomas were large; the tumors from patients 1 and 3 were encapsulated, and that from patient 2 showed invasive growth. Microscopically the tumors were characterized by welldefined solid nests (insulae), which were composed of rather small and uniform tumor cells with round to oval nuclei. Formation of small and colloid-containing follicles was associated with these nests to varying degrees. The tumors of patients 1 and 3 were composed entirely of insular components, but that of patient 2 was associated with small areas of welldifferentiated follicular carcinoma. The metastatic tumors of patients 1 and 2 were essentially similar to the primary with small foci of follicular carcinoma.
Patient 1 is alive with local and mediastinal node recurrences, but patient 2 died of the disease with local recurrences and metastases to lungs, bones and skin. Patient 3 had no recurrences and died of unrelated disease 5 years after surgery. The present study indicates that insular carcinomas have characteristic histologic features and a less favorable prognosis, confirming the findings of previous studies.     

以脊髓压迫为首发症状的髓系肉瘤5例临床分析

目的 探讨以脊髓压迫为首发症状脊柱髓系肉瘤患者的临床表现、影像学特征及治疗方法。方法 采用回顾性横断面研究方法。纳入2014年1月—2017年12月长征医院骨肿瘤科收治的经骨髓穿刺及病理证实的脊柱髓系肉瘤患者5例,其中男3例、女2例,年龄15~54岁;肿瘤位于胸椎3例、腰椎2例。4例采取开放性外科手术治疗,出院后根据骨髓穿刺及病理结果,予以化疗及血液系统肿瘤规范化治疗;另1例行内科保守治疗(消炎镇痛、营养支持、化疗等)。分析患者治疗前后各项观察项目。结果 5例患者均有腰背部疼痛,伴下肢无力3例、双下肢不全瘫1例;X线检查均未见异常,CT和MRI表现为椎体骨质破坏或占位性病变。骨髓穿刺及术后肿瘤病理结果显示为急性粒细胞白血病4例,慢性粒细胞白血病1例;5例患者均经治疗后症状有所缓解。5例患者均获随访:1例术后行去甲氧柔红霉素+阿糖胞苷方案化疗5个疗程,并行异基因造血干细胞移植治疗,目前已随访至术后28个月,患者一般状态良好、无肿瘤复发;另外4例经化疗后出现复发,均死于感染,生存期5~26.5个月。结论 以脊髓压迫为首发症状的髓系肉瘤病例临床罕见,其影像学表现缺乏特异性,易出现误诊,骨髓穿刺及病理检查可确诊;当出现脊髓压迫表现时,建议早期行肿瘤切除神经减压术,术后辅以异基因造血干细胞移植和全身化疗。     

Liposarcoma of the pleural cavity: clinical and pathologic features of 4 cases with a review of the literature

BACKGROUND: Primary liposarcomas of the pleura are extremely rare malignancies, and relatively few reports appear in the world literature. DESIGN: We compiled a small series of 4 cases of primary pleural liposarcoma from the files of the Armed Forces Institute of Pathology (Washington, DC) and compared the histopathologic and clinical features of these 4 cases with those of 9 previously published cases. RESULTS: Our investigation included the case studies of 9 men and 4 women, aged 19 to 80 years (average, 49 years). Histologic subtypes in the 9 cases with available information included 5 myxoid liposarcomas, 1 well-differentiated liposarcoma, and 3 liposarcomas with mixtures of histologic types. Surgical resection with or without chemotherapy appeared to be the most common form of treatment, although radiation therapy was used in some cases and seemed beneficial. Survival information was available for 11 cases; 4 patients died of disease at 7, 9, 12, and 19 months; 1 died of heart failure 2 days after presentation; 1 died of unknown causes 16 months after presentation; and 3 patients were alive without tumor at 5, 16, and 66 months after diagnosis. One patient had local recurrence at 2 years. A second surgical resection in this patient failed, and he died of disease 9 years after initial presentation. A second patient experienced recurrence at 4 years and was free of disease 4 years after the second surgical resection. CONCLUSIONS: Primary pleural liposarcomas occur predominantly in older men, and the myxoid histologic subtype is the most common. Radiographic or surgical evaluation is important to distinguish primary pleural liposarcoma from chest wall or mediastinal sarcomas, as well as metastases from other sites. Although further investigation is needed, evidence from the cases reviewed here indicates that surgical resection with adjuvant radiation therapy may benefit patients with primary pleural liposarcoma.     

肺肠型腺癌6例诊疗分析并文献复习

目的：肠型腺癌是非小细胞肺癌的一种少见病理类型,本文主要讨论肺肠型腺癌的临床和病理特征,诊断和治疗方法。方法：回顾性分析2013年至2016年5月间诊治的6例肺肠型腺癌患者的临床资料。结果：男2例,女4例,发病年龄25~78岁,以“咳嗽胸闷”起病4例,伴“颈部包块”4例,以“头晕恶心呕吐”起病1例;影像学主要表现为肺部、锁骨上及纵隔占位,病理形态及免疫组化均提示肿瘤来自于结直肠癌,但胃肠镜均未见异常占位;根治性和姑息性手术各1例,其中1例根治术后复发转移;1例行脑单发转移灶射波刀治疗后未再接受治疗,余5例患者均接受了全身化疗。截止随访结束,4例患者死亡,生存时间最短9个月,最长32个月。结论：肺肠型腺癌易与结直肠癌肺转移相混淆,确诊需要排除肠道病变,早期治疗以手术治疗为主,关于系统治疗方案有待临床进一步研究。     

Lymphangiomyomatosis: A report of three cases treated with tamoxifen

Summary Three cases are reported of lymphangiomyomatosis with pulmonary and abdominal manifestations. Two had a chylous pleura effusion, while the third presented a retroperitoneal manifestation, which was completely resectable. Antiestrogen therapy with tamoxifen was administered in all three cases. Two patients died of pulmonary progression after 4 months of therapy. The third is still alive, with stable disease for more than 6 years, and has been receiving tamoxifen for 66 months. These observations indicate that antiestrogen treatment may be as effective as oophorectomy when started at an early stage of the disease.