Le médulloépithéliome du système nerveux central. À propos de trois cas

Central nervous system medulloepithelioma is a rare, highly malignant childhood tumor. It might be confused with medulloblastoma or other primitive neuroectodermal tumors, but it is quite particular by its clinical, radiological, and pathological features. The mean survival varies depending on whether or not a gross-total resection is possible. Adjuvant radiochemotherapy is often indicated. Only two reported cases in the literature survived beyond 4 years after treatment by gross total resection and radiotherapy without chemotherapy. We report three cases of supratentorial medulloepithelioma occurring in three children aged 11-17 years. Two patients underwent a gross-total resection followed by radiotherapy and survived more than 4 years after treatment. The third case had, however, recurred twice within the 1st postoperative month despite a complete resection each time and metastasis to the lung developed. Chemotherapy was then carried out after the third procedure and the patient died 7 months later.     

Contemporary management of pineocytoma

Pineocytoma is a rare tumor; therefore, assimilating data from case reports and small case series to generate definitive treatment guidelines is difficult. The authors recently systematically reviewed the existing literature on outcomes for patients with pineocytoma. Gross total resection is associated with significantly increased tumor control and survival compared with subtotal resection combined with radiotherapy. When gross total resection is not possible, adding radiotherapy to subtotal resection is not associated with increases in either tumor control or survival. Although aggressive surgery in the pineal region carries the risk of neurologic injury, gross total resection should be attempted for pineocytoma.     

Concurrent benign teratoid medulloepithelioma and pineoblastoma.

Teratoid medulloepithelioma of the ciliary body is a rare tumor usually occurring in young children. Primary tumors of the pineal gland are also quite rare and have a similar age distribution. We report the case of a 2-year-old girl who had a leukocoria of the right eye. Cataract surgery was performed; however, the patient developed a total retinal detachment refractory to treatment. She eventually developed early changes of phthisis bulbi and underwent an evisceration. Histopathologic examination revealed a benign teratoid medulloepithelioma containing islands of hyaline cartilage and rhabdomyoblasts. During the course of her evaluation, a pineoblastoma was discovered and surgically excised. While the association of pineal tumors with retinoblastomas has been well established, no such association has been reported for medulloepitheliomas. To our knowledge, this is the first recorded case of a benign teratoid medulloepithelioma occurring concurrently with a pineoblastoma.     

Significance of lymphangiosis carcinomatosa at the bronchial resection margin in patients with non-small cell lung cancer

BACKGROUND: Treatment options for patients with microscopic residual disease at the bronchial margin (R1-resection) after resection for non-small cell lung cancer include observation, radiotherapy, reoperation, or even systemic therapy. The present study was performed to identify a parameter that would estimate the prognosis of these patients more precisely to permit a well-founded treatment recommendation for the individual patient. METHODS: A total of 1,162 patients with resected non-small cell lung cancer were analyzed in this retrospective study. Fifty-four patients (4.6%) had R1-resections at the bronchial margin. Type of residual disease (mucosal, extramucosal, or involvement of the entire bronchial wall) and occurrence of tumor cells in the lymphatic vessels (lymphangiosis carcinomatosa) were recorded as distinct parameters and analyzed by univariate and multivariate analyses (Log rank test; Cox regression model). RESULTS: Lymphangiosis carcinomatosa at the bronchial margin was detected in 22 patients (40.7%) and was associated with a significantly shortened survival (median survival with lymphangiosis carcinomatosa, 13.3 months; without lymphangiosis carcinomatosa, 20.1 months; p = 0.026). Early stage patients (stage I-II) without lymphangiosis carcinomatosa showed a median survival of 49 months. Multivariate analysis revealed that lymphangiosis carcinomatosa at the resection margin is an independent prognostic parameter (p = 0.038). Even after postoperative radiotherapy the prognosis was still poor if a lymphangiosis carcinomatosa was detected (median survival, 17.1 months). All other parameters (T-stage, N-stage, tumor histology, type of bronchial wall involvement) were not of prognostic significance in R1-resected patients. CONCLUSIONS: Lymphangiosis carcinomatosa at the bronchial resection margin predicts a poor prognosis in patients with non-small cell lung cancer. It is more than questionable whether these patients would benefit from local treatment options like radiotherapy.     

Desmoid tumor of the head and neck

BACKGROUND: [corrected] Desmoid tumors are rare benign tumors but have a tendency toward local recurrence after resection because of their infiltrative growth and frequent entrapment of vital structures in the head and neck region. We report 24 desmoid tumors of the head and neck and propose a reasonable approach in the management of such cases. METHODS: Twenty-four patients (9 male and 15 female; median age, 33 years; range, 0-66 years) with a desmoid tumor of the head and neck (neck, 15 patients; head, 9 patients) treated from 1990 to 2004 were retrospectively analyzed. The size ranged from 0.5 to 13 cm in diameter (mean, 3.6 cm). In the neck, 8 tumors were around the superficial layer of deep cervical fascia, whereas 4 tumors of the neck involved the prevertebral fascia and 2 involved brachial plexus. RESULTS: Twenty patients received complete resection of the tumor, but the section margin was positive in 8 patients, of which 6 patients remained free of disease in a period of 13 to 105 months. Three patients, including 2 with positive section margin and 1 with negative margin, developed recurrences, which were successfully removed again. Two patients underwent partial resection of the tumor because of brachial plexus involvement. One of them achieved regression after postoperative radiotherapy and the other had spontaneous regression. The hypopharygneal tumor in a newborn had spontaneously complete regression, and tracheostomy was closed at the age of 6 years. One patient remained with stable disease for 14 months after biopsy of the tumor without excision. CONCLUSION: The overall prognosis is still good despite frequent incomplete resection. Surgical resection of the tumor with close observation is suggested even if the section margin is positive. If a desmoid tumor cannot be removed grossly, regression or arrested growth of the remaining tumor is expected. Radiotherapy might be reserved for a growing tumor.     

滑膜肉瘤预后相关因素分析

目的 分析影响滑膜肉瘤患者预后的相关因素.方法 回顾性分析1997年9月至2008年9月就诊的66例滑膜肉瘤患者中52例获得随访的患者的临床资料.其中男性28例,女性24例;发病年龄11～71岁,均以无痛性肿块入院.通过随访了解肿瘤学预后,明确3、5年总体生存率及局部复发率.通过回顾病例,分析年龄、性别、肿瘤部位、肿瘤直径、外科边界、病理亚型、局部治疗方式、是否侵及骨与神经血管以及是否化疗9项因素对总体生存率的影响.利用Kaplan-Meier生存分析确定对生存有影响的单个因素,并通过Cox回归分析明确影响预后的独立危险因素.结果 52例患者获得随访,随访率78.8％;随访时间6 ～ 88个月,中位随访时间32个月.患者5年总体生存率为30.3％,局部复发率为32.7％,中位复发时间16个月.单因素分析结果提示:肿瘤直径＜5 cm、取得广泛外科边界、肿瘤位于四肢以及采取广泛切除联合局部放疗的患者预后较好(P＜0.05).多因素分析显示肿瘤直径,部位以及是否取得广泛外科边界是影响预后的独立危险因素.结论 肿瘤直径、部位以及是否取得广泛外科边界是影响预后的独立危险因素.     

Aortico-pulmonary paraganglioma case report and Japanese review

Aortico-pulmonary paraganglioma (APPG) is a rare middle mediastinal tumor. We experienced a case of APPG in a 52-year-old man. Chest computed tomography and magnetic resonance imaging revealed a multicystic mass in the subaortic area. A left thoracotomy was performed without definitive preoperative diagnosis. The tumor was strongly adherent to the pericardium and the surrounding large vessels, but a complete resection was undertaken. Histological and immunohistochemical examination revealed that it was a malignant paraganglioma with a microscopically positive surgical margin. After radiotherapy of 50 Gy for the mediastinum, the patient almost recovered from his hoarseness by thyroplasty. We also reviewed nine Japanese cases of APPG reported previously. Though APPG is rare, we must consider that a middle mediastinal tumor may be APPG, dpreoperative examination and preoperative planning are necessary to prevent massive bleeding and microscopic residual tumor.     

A supratentorial primitive neuroectodermal tumor exhibiting pathological characteristics of medulloepithelioma: a case report

A 50-year-old female presented with headache symptoms and left hemifacial paresis. Brain computed tomography and magnetic resonance images demonstrated a right frontal lobe tumor with hemorrhage and calcification. A gross total resection was carried out. Pathologically, the tumor exhibited three kinds of structures. The first was an astrocytic area which occupied the large part of this tumor. Small, round cells, which were positive for glial fibrillary acidic protein, were randomly distributed. The second was an epithelioid area with the highest MIB-1 labeling index of the three structures. Undifferentiated tumor cells demonstrated a dense proliferation, and short spindle-shaped cells exhibited an epithelial and a partly luminal alignment that resembled the embryonal neural tube. The third was a sarcomatoid area. Long spindle-shaped cells, which were positive for vimentin, were in a bundle-like form. We diagnosed this tumor as a primitive neuroectodermal tumor (PNET) with epithelial differentiation. PNETs are rare brain tumors with a predominance in children. Histologically, undifferentiated tumor cells demonstrate dense proliferations. The tumor arises from germinal matrix cells that differentiate into neuronal, glial, and ependymal cells. Medulloepithelioma, which is classified in the PNET category, is a very rare brain tumor that usually develops in childhood. Pathological studies have demonstrated that papillary, tubular, or trabecular arrangements of neoplastic neuroepithelium mimic the embryonic neural tube predominantly. In our case, the tumor partly demonstrated epithelial differentiation, which is a feature of medulloepithelioma, not only glial and mesenchymal differentiation. This is a rare case of PNET with uncommon histological features.     

Spinal hemangiopericytoma: an institutional experience and review of literature

Purpose

Hemangiopericytoma is a rare tumor of CNS with potential for recurrence and widespread metastasis, even outside CNS with even rare involvement of spinal cord. This case series presents five patients to evaluate the clinical presentation, radiological features, management, pathology and outcome of spinal hemangiopericytomas.

Methods

Between 2004 and 2013, five patients underwent surgery for spinal hemangiopericytoma. Histopathological data were reviewed in all cases and clinical and follow-up details were collected from the data available in our department.

Results

There were three males and two females, including one pediatric patient. Three patients had dorsal spine involvement and two patients had involvement of cervical spine. There were two patients with intradural extramedullary tumors, one patient each with pure intramedullary tumor, pure extradural tumor and both intra and extradural tumor. All of them presented with motor weakness. Gross total resection of the tumor was done in three patients. Four patients received post-operative radiotherapy. Histopathology showed anaplastic tumor in four cases with high MIB-1 LI. Most of them were positive for CD34, mic-2 and bcl-2. Three patients who underwent gross total resection improved significantly in the follow-up period. Two patients who underwent subtotal resection expired due to spread of their disease.

Conclusion

Spinal hemangiopericytoma is a rare tumor. Strong clinical suspicion is required to diagnose it pre-operatively. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors.

     

Treatment of Recurrent and Residual Glomus Jugulare Tumors

Residual and recurrent glomus jugulare tumors are rare but challenging. Treatment options include microsurgical resection, stereotactic radiotherapy, a combination of modalities, and “observation.” Choice of treatment must be made on a case-by-case basis, considering patient age, health status, location and size of tumor, status of the lower cranial nerves, and, of course, patient desire. Surgery is preferred when total resection of the tumor with preservation of function is deemed achievable. When function of the lower cranial nerves has been compromised, total surgical resection may also be possible, provided that the patient's health allows it. Cases where function is still preserved despite presence of a large tumor are more challenging, and a combination modality may be most effective. The goal of treatment is to provide tumor control with low morbidity. Current surgical techniques and the availability of stereotactic radiotherapy make this possible in the majority of cases.     

Local recurrences after subtotal esophagectomy for squamous cell carcinoma.

From July 1982 to June 1985, 100 patients with squamous cell carcinoma of the thoracic esophagus had esophageal resection and reconstruction using an abdominal and right thoracotomy approach (Lewis-Tanner operation). Five patients died within 30 days. The remaining 95 patients were studied prospectively for evidence of local recurrences. It was found that anastomotic recurrences occurred in eight patients, and mediastinal recurrences involving the intrathoracic stomach occurred in seven patients over a mean follow-up period of 13 months. The total local recurrence rate was 16% (15 of 95 patients). The incidence of anastomotic recurrence was shown to be related only to the length of the proximal resection margin and not related to tumor differentiation or lymph node metastases. A proximal resection margin of less than 5 cm measured at operation had a 20% risk of developing an anastomotic recurrence, and a margin of between 5 to 10 cm had an 8% risk. Mediastinal recurrences that encroached on the intrathoracic stomach were found to be related more to the extent of lateral spread of the primary tumor in the mediastinum than to the length of the resection margins. Postoperative radiotherapy in patients with palliative resections decreased the incidence of local recurrences. To reduce the incidence and consequences of local recurrence after esophagectomy, it is suggested that in patients with tumors in the upper thorax, a more complete esophagectomy is warranted, postoperative radiotherapy should be given to patients with short resection margins, and in patients with extensive mediastinal spread, use of the retrosternal route for reconstruction is preferred.     

Long-term outcome following radical temporal bone resection for lateral skull base malignancies: a neurosurgical perspective

OBJECT: Primary temporal bone malignancy is a rare form of tumor for which the therapeutic strategy remains controversial. In this study, the authors reviewed their experience with radical temporal bone resection (TBR) of such lesions and analyzed the long-term results to provide treatment recommendations. METHODS: Between 1994 and 2006, 17 patients (10 men and 7 women) underwent total or subtotal TBR for primary temporal bone malignancies. Tumors were graded according to the University of Pittsburgh system. The effects of surgical margins and tumor extensions on patient survival were analyzed using the Kaplan-Meier method. RESULTS: All tumors, except 1, were graded T4 (most advanced). Subtotal TBR was performed in 14 patients, and total TBR was performed in 3. The surgical margin was tumor negative in 10 patients and tumor positive in 7. For large tumors extending into the infratemporal fossa or encroaching on the jugular foramen, orbitozygomatic (3 patients) and posterior transjugular (4 patients) approaches were combined with the standard approach, and en bloc resection with a negative margin was achieved in all cases but 1. The follow-up time ranged from 0.3-11.6 years (mean 3.3 years). The 5-year recurrence-free and disease-specific survival rates were 67.5 and 60.1%, respectively. When a negative surgical margin was achieved, the survival rates improved to 100 and 89%, respectively. CONCLUSIONS: The neurosurgical skull base technique could improve the probability of en bloc resection with a tumor-free margin for extensive temporal bone malignancies, which would cure a subset of patients. The active participation of neurosurgeons would improve patient care in this field.     

Breast angiosarcoma that is not related to radiation exposure: A comprehensive review of the literature

Breast angiosarcomas that are not related to previous radiotherapy are very rare. Surgical resection is the primary treatment for these tumors, but there is no general agreement on the extent of surgery. The role of multimodality adjuvant treatment also remains controversial. The aim of this study was to summarize the available data from the largest published series of patients in terms of management and outcome. We also sought to identify prognostic factors influencing patient survival. We have included studies presenting detailed data on multimodality therapy and survival of patients with breast angiosarcoma. Ten studies presenting data on 280 patients were included in the review. Seventyfive percent of patients underwent a total mastectomy and 25% had breast-conserving treatment (BCT). In 42% of patients, an axillary node dissection was combined with mastectomy or BCT. Thirty-six percent of patients received chemotherapy and 35% were treated with radiotherapy in an adjuvant or neoadjuvant setting. Survival varied significantly according to tumor size and grade. Adjuvant multimodality therapy may improve the outcome in selected patients with breast angiosarcoma. Tumor size, grade, and margin status are the most important prognostic factors for survival.     

Chondrosarcoma of the chest wall

We report 2 patients with chondrosarcoma of the chest wall. They were a 67-year-old woman (case 1) with an anterior chest wall tumor and a 68-year-old woman (case 2) with a painful rib tumor. Their computed tomography (CT) both revealed calcified tumors. Case 1 underwent a wide resection by partial sternectomy, with free surgical margins. Case 2 underwent tumor resection with the posterior part of the 3rd rib, with positive surgical margin in the vertebral site, and received adjuvant radiotherapy. Both patients were pathologically diagnosed as having grade II chondrosarcoma. In their postoperative courses, they are free from recurrence. Wide resection is likely to be the key to successful management of chest wall chondrosarcoma.     

Relevant risk factors and prognostic impact of positive resection margins after endoscopic submucosal dissection of superficial esophageal squamous cell neoplasia

Background

Endoscopic submucosal dissection (ESD) for the treatment of esophageal mucosal lesions has a risk of resection margin residues. The related risk factors and prognosis of post-ESD resection margin residues have not been fully evaluated. The aim of this study was to investigate the associated risk factors and the prognostic impact of resection margin residues after ESD of superficial esophageal squamous cell neoplasia.

Methods

We conducted a retrospective analysis of medical records at our hospital, including the clinical, endoscopic, and pathological data from patients who underwent ESD for the treatment of superficial esophageal squamous cell neoplasia from January 2008 to December 2012. In addition, we conducted a statistical analysis of the following factors: sex, age, location, the proportion of circumferential extension, the maximum diameter of the resected specimen, macroscopic type, the depth of invasion, and the endoscopists.

Results

A total of 145 patients were included in the study. Overall, 148 lesions were completely resected. There were 17 patients (17 lesions) presenting with positive resection margin after ESD and the positive rate was 11.5 %. A total of 16 patients were followed-up. Among the patients who had resection margin residues, three underwent surgical esophageal resection, one underwent radiotherapy, two received ESD, and one received endoscopic mucosal resection. The remaining nine patients were periodically followed-up, and no recurrences were found. The results of a one-dimensional analysis suggested that there were significant differences in the maximum diameters of the resected specimens, macroscopic type, and the depth of invasion between the positive resection margin group and the negative resection margin group. The results of a multivariate regression analysis suggested that the maximum diameter of the resected specimens and the depth of invasion were risk factors for resection margin residues.

Conclusions

The maximum diameter of the resected specimens and the depth of tumor invasion are risk factors for post-ESD positive resection margins, which suggests that larger lesions and a greater depth of invasion increases the chance of residual tumor after ESD.     

Do Close but Negative Margins in Radical Prostatectomy Specimens Increase the Risk of Postoperative Progression?

Purpose

When tumor extends close to the margin of resection yet does not extend to the inked edge of the gland, it is unclear whether patients have an adverse prognosis compared to cases with greater distance between the tumor and margin.

Materials and Methods

Among radical prostatectomy specimens with negative margins the distance between the most peripheral tumor and the surgical margin of resection was measured in 52 cases with and 49 without progression. All patients had clinically confined disease (stages T1 or T2) with subsequent progression or a minimum 5-year followup without evidence of disease. No patient received preoperative or postoperative radiotherapy or hormonal therapy until progression occurred. All men underwent a postoperative serum prostate specific antigen test to evaluate progression. Seminal vesicles and lymph nodes were pathologically free of tumor. All prostates were serially sectioned, completely embedded and assessable regarding margins of resection.

Results

Patients with progression were no more likely to have tumor close to the margin than those without progression. In a regression analysis analyzing the effect of Gleason score, distance between tumor and margin, location of closest margin and pathological stage as related to progression, only grade was predictive of progression (p <0.00001).

Conclusions

It is not necessary for pathologists to designate these margins as close, since biologically this finding has no significance. Furthermore, physicians who are involved in treatment of patients after radical prostatectomy for prostate cancer should not alter therapy depending on whether margins are reported as close.     

Matched group study of surgical resection versus cobalt-60 plaque radiotherapy for primary choroidal or ciliary body melanoma

We report the results of a nonrandomized, matched-group, survival and visual preservation study of patients with a choroidal or ciliary body melanoma managed by microsurgical resection of the tumor versus cobalt-60 episcleral plaque radiotherapy. Each treatment group consisted of 30 patients. All patients were matched on a case-by-case basis in terms of tumor size (largest linear tumor dimension), location of the anterior tumor margin relative to the ora serrata, location of the posterior tumor margin relative to the equator, and age at the time of treatment. Although the estimated actuarial 5-year survival probability was slightly greater in the resection group (85.2%) than in the cobalt plaque group (81.8%), this difference was neither clinically impressive nor statistically significant. In contrast, there was a substantially higher rate of early posttreatment severe visual loss in the resection group (P = .0008, Mantel-Haenszel test).     

Sphincter-saving resection for all rectal carcinomas: the end of the 2-cm distal rule

OBJECTIVE: To assess oncologic outcome of patients treated by conservative radical surgery for tumors below 5 cm from the anal verge. SUMMARY BACKGROUND DATA: Standard surgical treatment of low rectal cancer below 5 cm from the anal verge is abdominoperineal resection. METHODS: From 1990 to 2003, patients with a nonfixed rectal carcinoma at 4.5 cm or less from the anal verge and without external sphincter infiltration underwent conservative surgery. Surgery included total mesorectal excision with intersphincteric resection, that is, removal of the internal sphincter, to achieve adequate distal margin. Patients with T3 disease or internal sphincter infiltration received preoperative radiotherapy. RESULTS: Ninety-two patients with a tumor at 3 (range 1.5-4.5) cm from the anal verge underwent conservative surgery. There was no mortality and morbidity was 27%. The rate of complete microscopic resection (R0) was 89%, with 98% negative distal margin and 89% negative circumferential margin. In 58 patients with a follow-up of more than 24 months, the rate of local recurrence was 2% and the 5-year overall and disease-free survival were 81% and 70%, respectively. CONCLUSIONS: The technique of intersphincteric resection permits us to achieve conservative surgery in patients with a tumor close to or in the anal canal without compromising local control and survival. Tumor distance from the anal verge is no longer a limit for sphincter-saving resection.     

Cerebellar liponeurocytoma with an unusually aggressive clinical course: case report

OBJECTIVE AND IMPORTANCE: Liponeurocytomas are rare cerebellar neoplasms in adults, with benign histological features and a favorable clinical prognosis. Current clinical opinion is based on a total of less than 20 published cases and suggests that gross total resection and long-term follow-up monitoring, with possible additional surgery and radiotherapy for treatment of recurrent tumors, represent the best treatment approach for this relatively benign tumor type. CLINICAL PRESENTATION: A 51-year-old Caucasian woman presented with worsening unsteady gait and headaches, suggesting increased intracranial pressure. INTERVENTION: The patient underwent subtotal resection of a cerebellar liponeurocytoma, followed by fractionated radiotherapy (total dose of 54 Gy). She experienced a local recurrence of the tumor 12 months later and underwent additional surgery for removal of the cerebellar mass. A second recurrent tumor was diagnosed on magnetic resonance imaging scans 3 months later and was surgically resected. The tumor histological findings were consistently devoid of atypical features, apart from leptomeningeal invasion noted in the first surgical specimen. CONCLUSION: This unusual case demonstrated an atypical clinical course of a highly aggressive and radiation-resistant tumor, despite the consistent absence of aggressive histological features. Cerebellar liponeurocytomas may not be as benign as the current literature and typical low-grade cytological and histological features suggest.     

A Prospective, Multi-Institutional Study of Adjuvant Radiotherapy After Resection of Malignant Phyllodes Tumors

Background  Malignant phyllodes tumors of the breast are unusual neoplasms, with an incidence of approximately 500 cases annually in the United States. Published local recurrence rates after margin-negative breast-conserving resections of borderline malignant and malignant phyllodes tumors are unacceptably high, at 24 and 20%, respectively. It is uncertain whether radiotherapy after resection of phyllodes tumors is beneficial. Methods  We prospectively enrolled patients who were treated with a margin-negative breast-conserving resection of borderline malignant or malignant phyllodes tumors to adjuvant radiotherapy. The primary endpoint was local recurrence. Results  Forty-six women were treated at 30 different institutions. The mean patient age was 49 years (range, 18–76 years). Thirty patients (65%) had malignant phyllodes tumors; the rest were borderline malignant. The mean tumor diameter was 3.7 cm (range, .8–11 cm). Eighteen patients had a negative margin on the first excision. The median size of the negative margin was .35 cm (range, <.1–2 cm). Twenty-eight patients underwent a re-excision because of positive margins in the initial resection. Two patients died of metastatic phyllodes tumor. During a median follow-up of 56 months (range, 12–129 months), none of the 46 patients developed a local recurrence (local recurrence rate, 0%; 95% confidence interval, 0–8). Conclusions  Margin-negative resection combined with adjuvant radiotherapy is very effective therapy for local control of borderline and malignant phyllodes tumors. The local recurrence rate with adjuvant radiotherapy was significantly less than that observed in reported patients treated with margin-negative resection alone.