Extra‐medullary haematopoiesis: A pictorial review of its typical and atypical locations

Extra‐medullary haematopoiesis (EMH) occurs in many haematological disorders and is secondary to insufficient bone marrow function or ineffective erythropoiesis. It usually presents as haematopoietic masses in several typical and atypical body locations. This pictorial review briefly discusses the common EMH locations encountered in clinical practice, including the liver, spleen, lymph nodes and paravertebral regions. Unusual presentation as focal hepatic and splenic masses is also discussed. Some atypical EMH locations are then presented together with their pathophysiology and relevant illustrations. These include EMH in the intra‐spinal canal, pre‐sacral region, nasopharynx and paranasal sinuses. Intra‐spinal EMH can cause cord compression and present with neurological symptoms. In these cases, urgent treatment may be required. Haematopoietic masses in the other atypical locations can present with obstructive symptoms or may be diagnosed incidentally on imaging. EMH in unusual locations need to be monitored with follow‐up imaging to ensure their stability. In some circumstances, tissue biopsy is appropriate to confirm the diagnosis and exclude other sinister pathology (e.g. malignancy). Treatment is only required where symptoms are present. Management options include blood transfusion, radiotherapy, hydroxyurea or surgical resection in selected cases.     

Isolated left brachiocephalic vein aneurysm presenting as a symptomatic mediastinal mass

Thoracic venous aneurysms arising from the mediastinal systemic veins are very rare conditions. We report a case of a 42-year-old female who presented with dull aching pain in the left infraclavicular region, dyspnea and palpitation since 4-5 month. Chest roentgenogram revealed superior mediastinal widening secondary to a mass. Contrast enhanced CT scan revealed a homogenously enhancing superior mediastinal mass. Selective left brachiocephalic vein venography confirmed the diagnosis of an isolated large left brachiocephalic vein saccular aneurysm. The patient is being followed up without surgical treatment. Although rare a diagnosis of innominate vein aneurysm should be considered when a uniformly attenuating mediastinal mass is seen on CT so that unnecessary biopsy and surgery can be avoided.     

Conclusions concerning the symptomatic treatment of cancer of the pancreas]

Symptomatic treatment is the only possibility in the large majority of patients with pancreatic cancer since a curative surgical excision of the tumor is only possible in less than 5% of cases. In most cases, symptomatic treatment must take short survival rate into consideration. Symptoms managed, in the aim to improve the quality of remaining life include weight loss and anorexia, abdominal pain, jaundice secondary to biliary compression, and sometimes digestive consequences of pancreatic surgery. Weight loss can be treated with nutritional support (particularly enteral nutrition) but the indications of such a treatment are rare. Abdominal pain should be treated by potent analgesics (opiates) and sometimes by percutaneous block of the coeliac ganglion rather than abdominal radiotherapy. Jaundice can be treated by surgical biliary drainage or better still by palliative biliary endoprothesis. Finally, digestive consequences of pancreatic surgery require symptomatic treatment.     

Combined modality therapy for mediastinal Hodgkin's disease. Prognostic significance of constitutional symptoms and size of disease

Combined modality therapy was used in an attempt to increase the complete remission rate and survival of previously untreated patients with Hodgkin's disease. MOPP (nitrogen mustard, vincristine, procabazine, prednisone) chemotherapy was followed by radiotherapy. The median duration of follow-up exceeded 5 years. Complete remissions were achieved in 63 of 71 patients (89%) with mediastinal masses. The actuarial survival of 10 years was 72% for patients with small mediastinal masses (mediastinal mass ratio less than 0.35) and 46% for patients with large mediastinal masses (P less than 0.05). The corresponding disease-free survival figures were 69% and 46%, respectively (P less than 0.05). A small mediastinal mass did not affect prognosis. Systemic symptoms especially affected the prognosis in patients with a large mediastinal mass, since in symptomatic patients the actuarial survival and freedom from relapse were 19%, whereas all asymptomatic patients survived without relapse. In patients with a small mediastinal mass, systemic symptoms had no significant effect on the actuarial survival or disease free survival. Age and stage did not affect the prognosis in patients with large mediastinal masses. It was concluded that MOPP chemotherapy followed by radiotherapy was an adequate treatment for asymptomatic patients with large mediastinal disease. However, in symptomatic patients with large mediastinal masses, this treatment was clearly inadequate.     

胸骨后甲状腺肿瘤的外科治疗

  目的   探讨胸骨后甲状腺肿瘤外科治疗的手术入路和围手术期处理方案。   方法   回顾分析天津医科大学附属肿瘤医院自2004年1月至2012年10月经手术治疗的104例胸骨后甲状腺肿瘤患者的临床资料。   结果   104例患者中, 102例通过颈部低位领式切口入路切除, 2例患者加用垂直胸骨劈开切口, 无手术死亡患者, 所有患者中结节性甲状腺肿52例, 甲状腺腺瘤41例, 甲状腺乳头状癌10例, 甲状腺滤泡癌1例。   结论   大多数胸骨后甲状腺肿瘤可经颈部低位领式切口入路切除, 加强术前影像学检查、娴熟的手术技巧、完善的术后管理可减少手术并发症。      

Thoracic CT-scanning follow-up of residual mediastinal masses after treatment of Hodgkin's disease

Sequential thoracic CT-scanning was performed for 14 patients presenting with residual mediastinal masses on chest X-ray after treatment of stage I and II Hodgkin's disease (HD). Eleven patients initially presented with bulky mediastinal involvement. No mediastinal biopsy was performed. No local recurrence was detected at a 48 month median follow-up. Early evaluation by CT-scanning showed cystic degeneration in three cases. Sequential CT-scans on longer follow-up demonstrated continuous regression of residual masses in six cases and no or limited volume reduction in six other cases. In two patients, part of the residual mass remained stable and the other part showed reduction in size. In addition, late calcifications developed in four cases. The results of sequential CT-scanning suggest that residual mediastinal masses do not generally represent active disease. Hence additional, potentially toxic, therapy can be avoided.     

胸骨后甲状腺肿经颈部低领切口切除的诊治体会（附33例）

目的:探讨经颈部低领切口行胸骨后甲状腺肿切除的诊治体会。方法:回顾性分析我院33例经颈部低领切口行胸骨后甲状腺肿切除的临床资料。结果:本组病例全部经颈部低领切口入路顺利完成手术,无围手术期死亡、呼吸道阻塞及大出血病例,无永久性喉返神经损伤和甲状旁腺功能低下病例。并发症发生率为15.15%(5/33),2例（6.06%）出现暂时性声音嘶哑,术后3周恢复正常,3例（9.09%）出现手足麻木,给予补钙处理后,出院时症状消失。结论:应用一定的手术技巧,经颈部低领切口行Ⅰ型和Ⅱ型胸骨后甲状腺肿切除是安全可行的。     

Hodgkin's disease: a reassessment of prognostic factors following modification of radiotherapy

Between 1970 and 1982, 175 patients with Stage IA, B, IIA, B, or IIIA Hodgkin's disease were treated with curative radiotherapy following surgical staging. The patients treated prior to 1975 received either regular extended or total nodal field treatments (Treatment Group 1, N = 65). Unsatisfactory results from this treatment program led to treatment modification in 1975. The modified protocols consisted of low-dose lung irradiation in patients having large mediastinal masses and/or hilar disease, and low-dose liver irradiation for Stage IIIAS+ patients (Treatment Group 2, N = 110). Recurrence-free survival rates improved significantly for various risk groups. Univariate analysis indicated that age, stage, symptoms, mediastinal mass size, number of sites involved, hilar disease, stage, and symptoms were significant risk factors in Treatment Group 1. In Treatment Group 2, only sex was a statistically significant risk factor. Stepwise Cox regression analysis for risk factors selected mediastinal mass size and stage as the most significant prognostic factors in Treatment Group 1. In Treatment Group 2, number of initial disease sites and sex were the most significant risk factors. The results of the study show significant improvement in recurrence-free survival rates in Treatment Group 2. It is concluded that these improvements are due to the modification in treatment.     

Postchemotherapy Surgery for Germ Cell Tumors—What Have We Learned in 35 Years?

Postchemotherapy surgery for advanced testicular cancer has evolved over the last couple of decades. Patients with nonseminomatous germ cell tumors and residual retroperitoneal mass ≥1 cm should undergo postchemotherapy retroperitoneal lymph node dissection (RPLND). For seminoma, RPLND is considered in those patients with masses ≥3 cm that are also positron emission tomography positive. Masses that occur outside of the retroperitoneum should be completely resected with the possible exception of bilateral lung masses when resection of the first mass shows necrosis. The role of surgery in patients with extragonadal germ cell tumors is most vital in those with primary mediastinal nonseminomatous germ cell tumors. Importantly, patient selection, surgical planning, and consideration of referral to centers with this expertise are important to optimize success.     

The prognostic significance of large mediastinal masses in the treatment of Hodgkin's disease. The experience of the Institut Gustave-Roussy

At the Institut Gustave-Roussy we undertook a study of 154 patients with clinical stages I and II Hodgkin's disease treated by irradiation to evaluate the prognostic significance of the mediastinal mass size. The population under study included those patients treated at our institute and entered into the H2 and H5 E.O.R.T.C. trials between 1972 and 1981. Patients were divided into three groups for purposes of analysis; large mediastinal masses (MT ratio greater than or equal to 0.35) were noted in 20 cases (13 per cent), moderate mediastinal invasion (MT ratio less than 0.35) was observed in 60 cases (39 per cent), and 74 patients (48 per cent) had no mediastinal involvement on presentation. Intrathoracic relapses were more frequent in those patients with mediastinal involvement at presentation (p less than 0.001) but there was no statistically significant difference between those patients with 'large' masses and patients with 'small' masses. Additionally multivariate analyses showed that neither the presence nor the size of mediastinal disease adversely affected relapse free survival or overall survival. These results can perhaps be linked to the technique of radiotherapy where a 'split course' technique was habitually used for large masses and the radiation fields were routinely modified during treatment according to the tumour response. Consequently we do not advocate the routine use of extensive primary treatment such as combined modality therapy utilizing MOPP chemotherapy for all patients presenting with bulky mediastinal masses. Rather chemotherapy should be reserved for those tumours which are particularly voluminous (MT ratio greater than 0.50) where primary radiotherapy may potentially result in unsatisfactory late pulmonary complications. We also advise the use of combined modality therapy for those patients who may additionally have certain unfavourable prognostic factors which we have previously identified.     

Successful chemotherapy in undescended testicular and extragonadal germ cell tumors: report of 2 cases

Two patients with advanced germ cell tumor who entered complete remission following intensive combination chemotherapy, radiation therapy and surgical intervention are reported. A 28-year-old businessman presented with abdominal pain and masses associated with an elevated HCG level for which he underwent exploratory laparotomy. Large retroperitoneal masses were found and microscopical examination of the masses were revealed seminoma. Three courses of combination chemotherapy consisting of CDDP, VLB and PEP were given to the patient followed by radiation therapy to the parailiac, paraaortic, mediastinal and supraclavicular lymph nodes with boost irradiation to the paraaortic lymph nodes where the large masses were located. The other patient was a 21-year-old student who developed sharp precordial chest pain which proved to be due to a large mediastinal mass accompanied by an elevated AFP level. He was treated with radiation therapy to the mediastinum, surgical resection and combination chemotherapy. However, he showed recurrence in the lungs associated with rising AFP levels, and was given a salvage chemotherapy consisting of 3 courses of CDDP, ADR, PEP and Etoposide. Both patients were successfully treated with combined modalities of treatment including intensive chemotherapy and have been off therapy without recurrence for over 12 and 4 months, respectively.     

Minimally-invasive surgery in cancer children

Minimally-invasive surgery, or video-assisted surgery, includes laparoscopy, retroperitoneoscopy and thoracoscopy, can be used for diagnosis (biopsies) and treatment (resections) in various malignant solid tumours in children. Potential advantages of MIS techniques include a decreased parietal trauma (cosmetic benefit, bowel adhesions) and less postoperative discomfort (postoperative pain, analgesics requirement, postoperative ileus, length of hospital stay). Main indications of MIS techniques are represented by diagnostic biopsies (mediastinal or lung tumors, retroperitoneal extrarenal masses), resection of the primary tumor in thoracic and abdominal neuroblastic tumors and in post-treatment residual mass in lymphoma.     

Superior vena cava syndrome

Opinion statement Lung cancer is the most common cause of superior vena cava syndrome (SVCS) and requires timely recognition and management. The syndrome is rarely an oncologic emergency in the absence of tracheal compression and airway compromise. Treatment depends on the etiology of the obstructive process. Treatment should also be individualized and should not be undertaken until a diagnosis is obtained. Most patients with SVCS secondary to lung cancer can be treated with appropriately directed chemotherapy or radiotherapy. With the refinement of endovascular stents, percutaneous stenting of the SVC is being increasingly used as primary treatment modality. Thrombotic occlusion can be treated with appropriate lytic agents. In rare circumstances, surgical decompression can be performed; bypass or replacement of the SVC results in immediate improvement in the majority of cases and can be accomplished with low morbidity.     

胸骨后甲状腺未分化癌1例报告并文献复习

目的:探讨胸骨后甲状腺未分化癌的临床特征和预后。方法:结合文献对1例胸骨后甲状腺未分化癌进行分析。结果:胸骨后甲状腺未分化癌,术前确诊较难,手术可解除压迫症状,但难以完整切除,预后差。结论:胸骨后甲状腺未分化癌应及时行影像学检查,尽早手术切除,并辅以放射等综合治疗。     

Potential complications of the growing teratoma syndrome in patients with advanced germ-cell tumors: description of three cases

In order to illustrate potential complications of the growing teratoma syndrome (GTS), three out of 180 pa- tients with germ-cell tumors from a single institution were identified. Despite surgical resection of recurrent mature teratoma, one patient developed a renal failure due to structure compression, one patient presented malignant transformation to squamous cell lung cancer, and a third patient had an unresectable mediastinal mass. Although the treatment of choice for GTS is complete surgical resection, it is not always possible and then recurrence is common.     

原发性恶性纵隔肿瘤的诊断及治疗

纵隔肿瘤种类繁多 ,鉴别肿瘤为原发性或继发性、良性或恶性 ,对制定治疗方案极为重要 ,作者综述了常见原发恶性纵隔肿瘤的常规诊断方法及纵隔镜检查、纵隔穿刺活检的临床价值 ,介绍了常见原发性恶性纵隔肿瘤外科处理以及放疗、化疗的临床应用     

Postmenopausal palpable ovary symdrome: A retrospective survey

　Objective A retrospective study of the postmenopausal palpable ovary (PMPO) on their incidence, characteristics of masses and treatment selected were carried out. Methods 97 postmenopausal women over age 50 with ovarian masses diagnosed by the manual pelvic palpation were analyzed. The age, size and characteristics of tumor, symptoms, as well as pathological findings and treatment were discussed. Results 86.6% of cases had tumor masses, and 13.4% were physiological in nature. 23.7% of the masses were malignant. 72 patients (74.2%) visited doctors because of existing symptoms. Among them 82.5% cases had the masses discovered by manual pelvic examination, and 91.3% found by abdominal ultrasound screening. Transvaginal sonography can diagnose all the palpable masses. Conclusion All PMPO patients should be operated as soon as diagnosis is established.     

Systematic review of the diagnosis and management of malignant extradural spinal cord compression: the Cancer Care Ontario Practice Guidelines Initiative's Neuro-Oncology Disease Site Group.

PURPOSE: This systematic review describes the diagnosis and management of adult patients with a suspected or confirmed diagnosis of extradural malignant spinal cord compression (MSCC). METHODS: MEDLINE, CANCERLIT, and the Cochrane Library databases were searched to January 2004 using the following terms: spinal cord compression, nerve compression syndromes, spinal cord neoplasms, clinical trial, meta-analysis, and systematic review. RESULTS: Symptoms for MSCC include sensory changes, autonomic dysfunction, and back pain; however, back pain was not predictive of MSCC. The sensitivity and specificity for magnetic resonance imaging (MRI) range from 0.44 to 0.93 and 0.90 to 0.98, respectively, in the diagnosis of MSCC. The sensitivity and specificity for myelography range from 0.71 to 0.97 and 0.88 to 1.00, respectively. A randomized study detected higher ambulation rates in patients with MSCC who received high-dose dexamethasone before radiotherapy (RT) compared with patients who did not receive corticosteroids before RT (81% v 63% at 3 months, respectively; P = .046). There is no direct evidence that supports or refutes the type of surgery patients should have for the treatment of MSCC, whether surgical salvage should be attempted if patient is progressing on or shortly after RT, and whether patients with spinal instability should be treated with surgery. CONCLUSION: Patients with symptoms of MSCC should be managed to minimize treatment delay. MRI is the preferred imaging technique. Treatment for patients with MSCC should consider pretreatment ambulatory status, comorbidities, technical surgical factors, the presence of bony compression and spinal instability, potential surgical complications, potential RT reactions, and patient preferences.     

CT and MR imaging in the evaluation and staging of lung cancer

Cancer of the lung is one of the most frustrating yet important challenges facing medicine today. Despite screening programs and education of the public concerning the established link of lung cancer and cigarette smoking, the overall incidence of lung cancer continues to rise. Improved imaging has led to more accurate staging. Expanded treatment has yielded improving survivals of certain specific tumors. Accurate diagnosis and staging of lung cancer is important in detecting therapy and prognosis. Computed tomography (CT) has been established as an important component of the staging process. More recently, applications of magnetic resonance imaging (MRI) are ideally suited to evaluate tumor extent and nodal disease. We reviewed the uses and limitation of CT and MRI. Compared with CT, the relatively low signal in the lung limits the detection of pulmonary nodules and other lung parenchymal diseases, and noise due to motion has been a frequent and significant problem in thoracic MRI. Because of its superior spatial resolution and ability to detect calcification, CT is better than MRI for the detection and evaluation of lung nodules and mediastinal adenopathy when assessing lung cancer. For the detection of mediastinal invasion or lymph node metastases, CT and MRI generally provide similar information. However, volume averaging problems, which may occur on trasaxial CT, can be avoided or clarified using MRI, and nodes can sometimes be more clearly distinguished from vessels using this technique. In the diagnosis of hilar masses or lymphadenopathy, CT and MR provide similar information in the majority of cases, but occasionally MR may more clearly indicate the presence or absence of a mass. Because of superb vascular imaging capability (without the need for exogenous contrast agents), exquisite soft tissue contrast, the ability to image the chest directly in multiple planes, and the potential to characterize certain tissues, MRI appears to be superior to CT in defining the extent of chest-wall invasion. In general, CT is superior to MRI as an all-around tool for imaging the wide range of thoracic abnormalities that can be present in patients with lung cancer. Limited availability, and longer examination time of MRI compared with CT has restricted the use of thoracic MRI. If MRI is used selectively as a secondary imaging study to answer specific questions raised or unanswered by CT, its value can be optimized.     

Unusual functioning endocrine tumors

Opinion statement Endocrine surgeons should maintain a high index of suspicion when patients are diagnosed with clinical signs or symptoms of parathyroid carcinoma. Although rare, the best chance for cure of these patients is at the time of the initial operation. Surgical resection of recurrent disease can provide effective palliation and can sometimes be assisted using gamma-probe directed dissection of sestamibi-labeled tumor tissue. Treatment of hyper-parathyroidism in the setting of multiple endocrine neoplasia type 1 (MEN-1), particularly in the reoperative setting, can be aided by using the rapid intraoperative parathyroid hormone assay to judge the adequacy of parathyroid debulking. In addition, in selected cases, the gamma probe can assist in identifying the location of ectopic or autografted sestamibi-labeled parathyroid tissue. Patients with incidental adrenal masses rarely require fine needle aspiration to exclude metastatic cancer. Fine needle aspiration, if performed, should never precede hormone evaluation to exclude pheochromocytoma. Patients who are diagnosed with incidental adrenal masses in the setting of a prior or concurrent cancer diagnosis are equally likely to have a primary adrenal mass as they would be to have metastatic cancer in the adrenal gland. Pheochromocytomas occasionally develop in patients with MEN-1. In suspicious cases, molecular identification of an MEN-1 mutation can be used to confirm the diagnosis. Preoperative hormone evaluation of a patient with an adrenal incidentaloma should include evaluation for subclinical Cushing’s syndrome through an overnight 1-mg dexamethasone suppression test. Identification of this condition allows for safe peri- and postoperative steroid hormone replacement, with very slow withdrawal of exogenous steroids to allow the opposite adrenal gland to recover and avoid postoperative Addisonian crisis. Paragangliomas are more commonly multifocal and malignant compared to pheochromocytomas. Evaluation of patients with paragangliomas should include radiographic staging for multifocality and metastatic disease, and postoperative hormone and radiographic follow-up evaluation should be performed. Consideration should be given to genetic testing for von Hippel-Lindau and succinate dehydrogenase mutations. Surgical treatment of rare functioning pancreatic and duodenal endocrine tumors, such as metastatic sporadic insulinoma and MEN-1-associated gastrinoma, can provide effective palliation. Surgical treatment should be integrated into a comprehensive treatment scheme that recognizes the natural history of the disease and incorporates appropriate adjunctive therapies and follow-up strategies.