肝海绵状血管瘤合并结节性再生性增生误诊为肝癌合并肝硬化1例报告并文献复习

背景与目的：肝海绵状血管瘤是最常见的肝脏原发肿瘤。尽管通过影像学检查,针对该病的诊断程序已经非常成熟,但在罕见疾病组合的情形中仍有被误诊为肝细胞癌的风险。本文通过回顾1例术后被诊断为海绵状血管瘤合并结节性再生性增生的肝移植病例,分析诊疗过程中的关键疑难点,复习海绵状血管瘤和结节性再生性增生的临床病理特征以及其与肝细胞癌和肝硬化的鉴别要点,旨为今后临床工作提供经验与教训。方法：回顾性分析武汉大学中南医院收治的1例肝移植患者的临床病史资料、影像学特点及病理组织学诊断,结合国内外文献中肝海绵状血管瘤和结节性再生性增生的特点,对本病例诊疗过程进行分析和归纳。结果：患者,男性,71岁,因腹水及肝右叶占位性病变入院。影像学检查提示肝硬化、腹水及肝右叶占位性病变。全腹部CT平扫和增强诊断肝硬化合并肝细胞癌。经全科会诊与患者充分沟通后,患者进行了肝移植术。术中检查和术后大体检查发现肝脏呈弥漫结节性改变伴肝右叶占位性病变。组织学发现前者为结节性再生性增生,而占位性病变则为海绵状血管瘤。结论：通常情况下肝海绵状血管瘤不存在影像学诊断困难,但在弥漫结节性背景下,海绵状血管瘤仍可被误诊为肝细胞癌。结节性再生性...     

Lessons from a liver hemangioma registry: subtype classification

PurposeA previously proposed classification of hepatic hemangioma postulated 3 types of lesions: focal, multifocal, and diffuse. A registry (www.liverhemangioma.org) was created to track patients to validate this classification scheme.MethodsRegistry records entered between 1995 and 2010 were reviewed.ResultsOf 121 patients with hepatic hemangioma, 119 were focal (n = 33), multifocal (n = 68), and diffuse (n = 18). Two patients shared features of multifocal and diffuse. The focal group had a balanced sex distribution, whereas multifocal and diffuse were more common in women (66.2% and 70.0%, respectively). Hepatic hemangioma was detected prenatally in 30% (9/30) of patients with focal disease. Detection was not possible in multifocal or diffuse hepatic hemangioma, indicating postnatal proliferation typical of common cutaneous infantile hemangioma. Cutaneous hemangiomas accompanied 77.4% (48/62) of multifocal hepatic hemangioma, 53.3% (8/15) of diffuse hepatic hemangioma, and 15.3% (4/26) of focal hepatic hemangioma. Hypothyroidism was documented in all (16/16) patients with diffuse hepatic hemangioma, 21.4% (9/42) with multifocal hepatic hemangioma, but in no patients with focal hepatic hemangioma (0/17).ConclusionsAnalysis of the Liver Hemangioma Registry confirms that focal lesions are biologically distinct from multifocal and diffuse hepatic hemangioma. Feared diffuse hepatic hemangioma may evolve from previously undetected multifocal hepatic hemangioma. Screening ultrasounds in infants with multiple cutaneous infantile hemangioma may allow for earlier detection and therapy.     

Giant biliary mucinous cystadenoma mimicking mesenchymal hamartoma of the liver in a child: A case report

IntroductionBiliary cystadenomas (BCAs) are rare benign tumors with malignant potential. They are most commonly found in middle-aged women and are quite infrequently reported in children. Even with advanced imaging, diagnosing and distinguishing BCA from other cystic liver lesions remain challenging.Case presentationA 5-year-old boy was brought in by his parents to our hospital with abdominal swelling that had been persistent for a year, along with loss of appetite and weight loss. On examination, the abdomen was distended and dull on percussion. We considered mesenchymal hamartoma of the liver (MHL) as the top differential after an abdominal CT scan with contrast showed a multi-loculated cystic tumour. For both definitive diagnosis and therapy, the patient underwent exploratory laparotomy with excision of the cystic mass. Surprisingly, histopathology examination of the resected specimen revealed biliary mucinous cystadenoma (BCA).ConclusionSince conservative methods are associated with high recurrence rates, biliary mucinous cystic neoplasms require a high index of suspicion and should be handled with total surgical resection. In the post-operative phase, periodic surveillance imaging is recommended due to the risk of recurrence and malignant transformation.     

Fetal and neonatal hepatic tumors

Background/Purpose

Although hepatic tumors are uncommon in the perinatal period they are associated with significant morbidity and mortality in affected patients. The purpose of this review is to focus on the fetus and neonate in an attempt to determine the various ways liver tumors differ clinically and pathologically from those found in the older child and adult and to show that certain types of tumors have a better prognosis than others.

Methods

The author conducted a retrospective review of perinatal hepatic tumors reported in the literature and of patients treated and followed up at Children's Hospital San Diego and Children's Hospital Los Angeles. Only fetuses and infants younger than 2 months with adequate clinical and pathologic data ere accepted for review. The period of patient accrual was from 1970 to 2005. Length of follow-up varied from 1 week to more than 5 years. Elevated α-fetoprotein level was defined as one significantly higher than that of the reporting institution's normal level for age group; laboratory values for this protein vary from one institution to the next and therefore it was not possible to assign one figure as a standard reference number. Discussion of the differential diagnosis and pathologic findings of hepatic tumors in the fetus and neonate are described elsewhere and will not be discussed here in detail (Perspect Pediatr Pathol 1978;4:217; Weinberg AG, Finegold MJ. Primary hepatic tumors in childhood. In: Finegold M, editor. Pathology of neoplasia in children and adolescents. Philadelphia, PA: WB Saunders, 1986; Am J Surg Pathol 1982;6:693; Pediatr Pathol 1983;1:245; Arch Surg 1990;125:598; Semin Neonatol 2003;8:403; Pediatr Pathol 1985;3:165; Isaacs H Jr. Liver tumors. In: Isaacs H Jr, editor. Tumors of the fetus and newborn. Philadelphia, PA: WB Saunders, 1997; Isaacs H Jr. Liver tumors. In: Isaacs H Jr, editor. Tumors of the fetus and infant: an atlas. Philadelphia, PA: WB Saunders, 2002).

Results

One hundred ninety-four fetuses and neonates presented with hepatic tumors diagnosed prenatally (n = 56) and in the neonatal period (n = 138). The study consisted of 3 main tumors: hemangioma (117 cases, 60.3%), mesenchymal hamartoma (45 cases, 23.2%), and hepatoblastoma (32 cases, 16.5%). The most common initial finding was a mass found either by antenatal sonography or by physical examination during the neonatal period. Overall, hydramnios was next followed by fetal hydrops, respiratory distress, and congestive heart failure, which were often related to the cause of death. Half of the fetuses and neonates with hepatoblastoma had abnormally elevated serum α-fetoprotein levels compared with 16 (14%) of 117 of those with hemangioma and 1 neonate with mesenchymal hamartoma. There were 76 (65%) examples of solitary (unifocal) hemangiomas and 41 (35%) of multifocal (which included the entity diffuse hemangiomatosis) with 86% and 71% survival rates, respectively. Of 45 patients with mesenchymal hamartoma, of the 29 (64%) who had surgical resections, 23 (79%) survived. Patients with hepatoblastoma had the worst outcome of the group, for only 8 (25%) of 32 were alive. Half of patients with either stage 1 or 3 hepatoblastoma died; no patient with stage 4 survived. There was some relationship between histologic type and prognosis. For example, half of the patients with the pure fetal hepatoblastoma histology survived compared with those with fetal and embryonal histology where 30% survived. Fifteen of 32 hepatoblastoma patients received surgical resection with or without chemotherapy, resulting in 7 (47%) of 15 cures. The 56 fetuses and 138 neonates with hepatic tumors (hemangioma, mesenchymal hamartoma, and hepatoblastoma) had survival rates of 75%, 64%, and 25%, respectively. The overall survival of the entire group consisting of 194 tumors was 125 or 64%.

Conclusions

The study shows that clinical findings in fetuses and neonates with hepatic tumors are less well defined than in older children. Survival rates are much lower as well. When the clinical course is complicated by associated conditions such as stillbirth, fetal hydrops, congestive heart failure, severe anemia, or thrombocytopenia, the mortality rate is much greater. If the patient is mature enough and in a clinical condition where he or she can be operated on, survival figures approach those of the older child. Some hepatic tumors have a better prognosis than others. Neonates with focal (solitary) hepatic hemangiomas have the best outcome and fetuses with hepatoblastoma the worst. Although infantile hemangioma undergoes spontaneous regression, it may be life threatening when congestive heart failure and/or consumptive coagulopathy occur. Mesenchymal hamartoma is a benign lesion best treated by surgical resection, which usually results in cure. However, there are fatal complications associated with this tumor, ie, fetal hydrops, respiratory distress, and circulatory problems owing to a large space occupying abdominal lesion and sometimes stillbirth, all contributing to the death rate. Hepatoblastoma, the major malignancy of the fetus and neonate, is treated primarily by surgical resection. Pre- or postoperative chemotherapy is reserved for those patients with unresectable tumors or metastatic disease. The survival rate is much lower than that reported by multigroup prospective trials. Patients die from the mass effect caused by the tumor, which lead to abdominal distension, vascular compromise, anemia, hydrops, respiratory distress, and stillbirth. Metastases to the abdominal cavity, lungs, and placenta are other causes of death. Because of the danger of labor-induced rupture of the tumor and potentially fatal intraabdominal hemorrhage, cesarean delivery is recommended when a hepatic tumor is found on prenatal ultrasound.     

Liver Transplant for Adult Recurrent Hepatic Mesenchymal Hamartoma and a Feasible Treatment Modality: A Case Report and Literature Review

BackgroundAdult hepatic mesenchymal hamartoma (HMH) is an extremely rare hepatic tumor. Recurrence following complete resection is uncommon. Liver transplantation (LT) is described as a possible treatment option in nonresectable HMH.We conducted a systematic review investigating LT in adult HMH followed by a case report describing evidence of extensive recurrence following complete resection of large right-sided HMH requiring LT.Case ReportA 46-year-old woman with symptomatic large right-hepatic HMH underwent right hemi-hepatectomy with histologic evidence of complete resection. Two and a half years postresection, she presented with abdominal pain and distension; imaging revealed large multi-septated hepatic cystic lesions within the liver suggestive of extensive recurrence of disease with concerns of malignant sarcomatous transformation. After a multidisciplinary team discussion, the lesion was deemed unresectable and the patient was referred for LT. Findings on transplantation included giant multiple hepatic cystic lesions occupying the entire abdomen and histopathological analysis confirmed recurrent HMH with no malignancy.The 6-month follow-up was unremarkable with no signs of postoperative complications or rejection.ConclusionWe identified only 3 reported adult unresectable HMH cases in the English literature requiring LT, with good clinical outcome and no rejection on a 1-year follow-up. To our knowledge, we report the first recurrent HMH that required LT in the English literature. Current evidence suggests possible malignant sarcomatous transformation of those lesions.No guidelines exist on postresection surveillance for HMH; however, given their malignant potential, we suggest a benefit of imaging-based surveillance following HMH resection. Offering LT for nonresectable or recurrent HMH is a feasible treatment modality with a reported good outcome.     

Malignant transformation of mesenchymal hamartoma of the liver: case report and review of the literature

Here the first case in the literature of both mesenchymal hamartoma and malignant mesenchymoma occurring in a 6-year-old male child, at different times and at different sites in the liver, and also the possible malignant transformation of a mesenchymal hamartoma is reported. The tumor developed from a lesion in the right lobe that was overlooked initially during a left lateral segmentectomy at 18 months of age for a mesenchymal hamartoma. Malignant mesenchymoma is a rare and aggressive tumor. The origin of this tumor is not well understood. There has been no direct support to the hypothesis that malignant mesenchymoma may be the malignant counterpart of mesenchymal hamartoma. The authors provide clinical and histopathologic evidence in our case that suggests the possibility of malignant mesenchymoma arising from a mesenchymal hamartoma. This case emphasizes the need for complete removal of mesenchymal hamartoma and the need for long-term follow-up to detect multifocal lesion or malignant transformation.     

Management of Hemangioma of the Liver: Surgical Therapy or Observation?

Background

Elective surgery for liver hemangioma is controversial. We reviewed long-term outcomes following elective hepatectomy or observation only for symptomatic and asymptomatic liver hemangioma.

Methods

All patients (n = 307) with liver hemangioma referred to our hospital for surgical evaluation from January 1988 to December 2009 were identified, and imaging results, tumor characteristics, surgical indication, surgical mode, outcome of observation, clinical and/or postoperative outcome, and adverse events were retrospectively evaluated.

Results

Complete median follow-up for 246 patients was 124 months. Elective surgery was performed in 103 patients (symptomatic [n = 62] and asymptomatic [n = 41]). Postoperative morbidity occurred in 17 % of the patients and was significantly lower in asymptomatic patients (p = 0.002). No perioperative mortality was registered. Surgery relieved complaints in most (88 %) patients. In the observation group (n = 143), 56 % of patients had persistent or new onset of hemangioma-associated symptoms. Major hemangioma-related complications occurred in 12 patients (9 %) during the follow-up period, and 2 patients died after traumatic hemangioma rupture. Overall the rate of adverse events was by trend lower in the surgical group than in the observation group (35 versus 57 %; p = 0.08).

Conclusions

The majority of patients with liver hemangioma can be safely managed by clinical observation. In a subset of patients, especially those with giant hemangioma and/or occurrence of symptoms, surgical treatment could be considered and is justified in high-volume centers.     

成人免疫性血小板减少性紫癜合并肝血管瘤1例报告并文献复习

背景与目的 成人免疫性血小板减少性紫癜（ITP）合并肝血管瘤临床罕见,容易误诊误治。笔者报告的1例成人ITP合并肝血管瘤患者的诊治经过,以期为临床提供参考和借鉴。方法 回顾性分析桂林医学院第二附属医院收治的1例成人ITP合并肝血管瘤患者的临床资料,并结合文献总结该病的临床诊治特点。结果 患者为46岁女性,因“全身皮肤黏膜出血1个月余,血小板减少1 d”入住血液内科。血细胞分析血小板计数4.0×10⁹/L,凝血功能正常,骨髓穿刺活检提示巨核细胞相对增多,血小板少见,高度疑似ITP。经激素等治疗后皮肤黏膜出血好转,血小板升高至59×10⁹/L。上腹部增强CT提示右肝巨大血管瘤,转至肝胆胰外科行肝血管瘤切除,术后术区渗血明显,阴道流血,血小板逐步降低至3.0×10⁹/L,右侧胸腔大量积液,但凝血功能正常。经止血、输血、调节免疫、抗感染、胸腔穿刺引流处理后逐步恢复。术后第13天患者血小板升高至220×10⁹/L。随访32个月,患者皮肤黏膜不再出血,血小板计数维持在正常范围。结论 成人ITP,需警惕合并肝血管瘤,处理血管瘤可使患者获得良好的远期效果,但需做好围术期管理以使患者顺利恢复。     

Giant mammary hamartoma in a middle aged female. Case report and review of literature of the last 15 years

BackgroundMammary hamartoma is a benign rare tumour occurring in both sexes, with size range mostly between 2–4 cm. Giant breast hamartoma (GMH) is very rare and can reach unexpected sizes in women.Presentation of the caseA 26 year old Egyptian female presented with left breast lump since 3 years, gradually increasing in size, with no other associated complaints. No family history of breast cancer, she did not smoke or consume alcohol, and had no past medical history. Examination revealed a large soft freely mobile mass (12 × 9 cm) in the lower outer quadrant of the left breast at the 3–6 o’clock position. There were no palpable axillary lymph nodes in both sides. Nipples and right breast were normal.DiscussionThe diagnosis of GMH can be made by examination and imaging only. The specific features that appear in mammogram and ultrasound can be used to reduce the need for core biopsy in hamartoma. Wide local excision is curative. We include a review of the literature of cases of GMH > 10 cm published during the last 15 years.ConclusionA non-invasive mammogram and ultrasound provide sufficient evidence of the tumour, hence core biopsy might not be critically required. However, if a breast hamartoma is still clinically suspected but with inconclusive or unequivocal mammographic and ultrasonographic features or if there is suspicion of dysplasia, then invasive core biopsy is justified. Recurrence is low and prognosis is good.     

不消退型先天性血管瘤的诊断和治疗研究

目的 研究一种特殊的婴幼儿血管肿瘤的病史、临床表现、影像学和组织病理学特征及其治疗.方法 回顾分析了2004年7月至2008年7月在我科被诊断为不消退型先天性血管瘤的12例患者(女性5例,男性7例,年龄18个月～26岁)的临床资料、影像学和组织学特征及治疗情况.结果 病灶通常是单发性,呈圆形或椭圆形,平坦或高出皮面,平均大小约5 cm×6 cm.表面皮肤通常有较粗的毛细血管扩张,中央或边缘发白,皮温较高.MBI、计算机断层扫描血管造影和数字减影血管造影影像学特征和普通婴幼儿血管瘤相似.组织病理学检查表现为结节状聚集的小的薄壁的血管和较大的通常呈星状的中央静脉,小叶内血管壁衬以"钉头样"内皮细胞,有时可见小动脉和结节内血管或结节外静脉直接交通.病灶可轻易切除,随访未见复发.结论 不消退型先天性血管瘤是一种特殊的婴幼儿血管肿瘤,认识该类疾病将有利于防止误诊误治的发生.     

聚桂醇瘤内注射硬化治疗肝血管瘤的临床疗效

目的探讨超声引导下经皮经肝穿刺聚桂醇瘤内注射硬化治疗肝血管瘤的安全性及有效性。方法将56例肝血管瘤患者,随机分为对照组29例(34个瘤灶),行平阳霉素瘤内注射硬化治疗;研究组27例(33个瘤灶),行聚桂醇瘤内注射硬化治疗。观察治疗前和治疗后3、6个月时患者临床症状、瘤体大小变化及不良反应。结果对56例患者均顺利实施硬化治疗,手术成功率100%。对照组与研究组患者治疗前及治疗后3、6个月血管瘤平均最大直径的差异无统计学意义(P均0.05);治疗3、6个月的有效率分别为58.82%、70.59%和54.54%、66.67%,差异均无统计学意义。两组患者治疗中及治疗后均未发生严重并发症。结论超声引导下经皮穿刺瘤内注射聚桂醇硬化治疗肝血管瘤安全有效。     

Intestinal failure-associated liver disease in surgical infants requiring long-term parenteral nutrition

PurposeOur aim was to determine incidence, severity, and outcome, as well as predisposing factors and underlying diagnoses, of intestinal failure-associated liver disease (IFALD) in surgical infants requiring long-term parenteral nutrition (PN).MethodsWe retrospectively studied surgical infants receiving PN for at least 28 days for congenital or acquired intestinal anomalies over a 5-year period (January 2006 to December 2010). Intestinal failure-associated liver disease was defined as type 1 (early)—persistent elevation of alkaline phosphatase for 6 weeks or longer; type 2 (established)—additional elevated total bilirubin (≥50 μmol/L); and type 3 (late)—additional clinical signs of end-stage liver disease.ResultsEighty-seven infants required PN for at least 28 days. Intestinal failure-associated liver disease occurred in 29 infants (33%). Intestinal failure-associated liver disease was managed medically in all but 2 patients who underwent intestinal elongation. None were referred for intestinal or liver transplant. Intestinal failure-associated liver disease has been reversed in 17 (59%) of cases to date. Sixty-one children receiving long-term PN (70%) have achieved enteral autonomy, whereas 12 (14%) require home PN. Severity of IFALD was significantly associated with duration of PN and female sex.ConclusionIntestinal failure-associated liver disease remains a fairly common but rarely life-threatening complication of intestinal failure in surgical infants. Intestinal failure-associated liver disease can be reversed in more than half of these children, and enteral autonomy was achieved in more than two thirds, even with minimal use of intestinal elongation. This is the first study to demonstrate an association between the severity of IFALD in surgical infants and female sex.     

Chronic excruciating forearm pain in a child with intra-neural hemangioma: A challenging case report

Introduction and importanceCavernous hemangioma is a rare form of hemangioma. It usually arises in the central nervous system, but the tumor has also been reported in the liver, retina and skin with a lower prevalence. Its occurrence into the peripheral nerves has only been reported a few times. Herein, we report an extremely rare case of intra-neural hemangioma in the ulnar nerve and discuss the complications we faced following surgery.Case presentationWe present a 6-year-old boy with history of severe progressive left forearm pain in the last two years. Imaging studies revealed a soft tissue mass and histopathological exam was in favor of a cavernous hemangioma. Patient underwent surgery to excise the tumor. Despite temporary response, he began to experience excruciating pain shortly after surgery which caused him to adopt bizarre postures.Clinical discussionIn the more common form of nerve involvement in a hemangioma, the nerve is displaced and surrounded by the tumor. However, in cases with intra-neural involvement, the nerve would have to be sacrificed. This case report brings some rare but important characteristics of a hemangioma in to attention, such as the intra-neural location, possibility of recurrence and aggravating pain with bizarre positions as a result.ConclusionIn cases of intra-neural hemangioma, there is a chance that the patient experiences recurrence and/or excruciating pain following surgery. The orthopedic surgeon should be prepared for the possibility of nerve transfer, repeat surgeries and the need for prolonged palliative pain suppression modalities in the approach to intra-neural hemangioma.     

Endoscopic intranasal control of hemorrhage from unexpected ethmoidal hemangioma during conchoplasty: A case report

Introduction and importanceHemangiomas of paranasal sinuses are rare. Unexpected hemangiomas of this region can bleed profusely leading to operative morbidity and mortality. Hemangiomas of paranasal sinuses may be associated with concha bullosa.Case presentationWe report a case of 41-year-old female who presented with difficulty in nose breathing and headache since two years. She was diagnosed with concha bullosa with deviated nasal septum and was planned for endoscopic septoplasty and conchoplasty. During the procedure, profuse, unexpected hemorrhage resulted from the undiagnosed hemangioma which was managed by endoscopic intranasal cauterization of anterior ethmoidal artery.DiscussionHemangiomas of paranasal sinuses such as ethmoidal sinus are rare. These are slow growing tumors and may be radiologically silent. Due to high vascularity of these lesions, it may be very difficult to manage associated bleeding. A sound knowledge of vascular anatomy of this area is important in managing intraoperative complications. Until now, only a few cases of ethmoidal hemangiomas have been reported in association with concha bullosa. Screening for these lesions may be important in patients with concha bullosa.ConclusionSound knowledge of surgical anatomy forms the basis of managing intraoperative complications in endoscopic sinus surgery. Further research should be carried out to find out the association between concha bullosa and ethmoidal hemangioma and patients with concha bullosa should be screened with advanced imaging techniques for such vascular lesions where feasible.     

椎体成形术治疗症状性椎体血管瘤:影像学随访评价

目的探讨经皮椎体成形术（PVP）治疗症状性椎体血管瘤及影像学随访评价的临床应用价值。方法回顾性分析接受PVP治疗的24例（共29节椎体）椎体血管瘤患者的资料,对其术前、术后1周及术后1年随访时的x线平片、CT及MR检查结果进行统计学分析。结果24例患者PVP均成功,骨水泥充填良好,未出现严重并发症;术后1周所有病变椎体前、后缘高度比（A／B）为0．83士0．15,术后1年A／B为0．83土0．20（t=-1．984,P〉0．05）。随访未见新发椎体压缩性骨折,血管瘤无复发。结论PVP是一种比较安全且有效的微创治疗椎体血管瘤的方法;影像学检查可用以评价PVP治疗效果。,     

Treatment of infantile subglottic hemangioma with electrocautery.

Laryngeal subglottic hemangioma is a rare but potentially life-threatening congenital lesion that can compromise that infant airway. A multitude of medical and surgical modalities have been used in the treatment of this lesion but no single approach has received universal acceptance. Three infants with subglottic hemangiomas were treated successfully with electrocautery; each was managed without tracheostomy. Bronchoscopic electrocautery appears to be a safe, effective, inexpensive way to manage infantile subglottic hemangioma.     

Mediastinal hemangioma mimicking an invasive tumor growth: A case report

Introduction and importanceMediastinal hemangiomas are a rare entity frequently developing in the anterior mediastinum. They may exhibit an infiltrating appearance into the surrounding tissue, and extensive surgery is often required despite its benign nature. We report a case of a mediastinal hemangioma mimicking an invasive tumor growth requiring a combined resection of the lung and diaphragm.Case presentationAn asymptomatic 73 year-old-man presented with a 50 mm-sized mass on his chest radiography. A combined resection of the mediastinal mass with the part of the lower lobe of left lung and diaphragm not necessitating a patch repair was performed. The pathological findings were compatible with a diagnosis of a mediastinal hemangioma. Only fibrous adhesions were observed between the tumor and resected lung and diaphragm without any histological invasion.Clinical discussionWhile imaging examinations play a key role in determining a preoperative diagnosis, mediastinal hemangiomas may pose a diagnostic challenge mainly due to its rarity. With a clinical suspicion of a hemangioma during the diagnostic work up, a dynamic CT might be helpful.ConclusionsIn mediastinal hemangiomas, a preoperative diagnosis is essential in order to avoid extensive surgery. A hemangioma should be raised as a differential diagnosis for anterior mediastinal tumors, especially in cases with an infiltrative appearance that suggests the necessity for a combined resection of the surrounding organs.     

Intraneural lipomatous tumor of the median nerve: Three case reports with a review of literature

INTRODUCTIONIntraneural lipoma and fibrolipomatous hamartoma of the nerve are rare soft tissue tumors that most commonly occur in the forearm and the wrist, and particularly within the median nerve. When the lesions are large enough, they may cause progressive compression neuropathy. They are distinct entities each other with different clinical and radiological findings and thereby need different surgical treatments.PRESENTATION OF CASEWe report here 3 cases of intraneural lipomatous tumors of the median nerve (1 case of intraneural lipoma and 2 cases of fibrolipomatous hamartoma).DISCUSSIONAll patients were surgically treated successfully with complete excision for intraneural lipoma and with carpal tunnel releases for the both fibrolipomatous hamartomas.CONCLUSIONA careful preoperative planning is necessary for the optimal treatment by distinguishing whether it is a resectable or non-resectable tumor based on the clinical and radiological findings, because they have characteristic findings each other.     

Mediastinal tumors in children: Experience with 196 cases

Background: Mediastinal masses are relatively common in infants and children. These lesions are often neoplastic in origin and have a high risk of malignancy. Methods: This report concerns 196 infants and children with mediastinal tumors. Fifty-five cases (28%) were benign, and 141 (72%) were malignant. Diagnosis included Hodgkin's disease (47), neuroblastoma (46), non-Hodgkin's lymphoma (37), teratoma (18), ganglioneuroma (14), cystic hygroma (11), Schwannoma (five), germ-cell tumors (three), lipoma (three), thymic tumor (three), malignant histiocytosis (two), neurofibroma (two), mesenchymal sarcoma (one), rhabdomyosarcoma (one), peripheral neuroectodermal tumor (one), hamartoma (one), and hemangioma (one). Diagnoses were usually made by assessing the patient's age, radiologic evidence of tumor location, the presence of calcium in the tumor, and the presence of tumor markers (α-fetoprotein, vanillmandelic acid, human chorionic gonadotropin). Diagnoses were verified by histologic evaluation. Resection was the only treatment for benign tumors. Biopsy and chemotherapy (and/or radiation) were employed for lymphoid tumors, and resection and adjuvant therapy were used for other solid malignancies. Results: Survival was achieved in 53 of 55 (96.3%) patients with benign tumors and 105 of 141 (74.4%) patients with malignant tumors. Conclusions: Seventy-two percent of mediastinal tumors in this study were malignant. Early diagnosis followed by biopsy and chemotherapy for lymphoid tumors or resection of nonlymphoid tumors along with aggressive adjuvant therapy result in high survival rates (74.4%). Children with benign tumors almost always survive (96.3%) after resection. Presented at the 46th Annual Cancer Symposium of the Society of Surgical Oncology, Los Angeles, California, March 18–21, 1993.     

Spinal epidural cavernous hemangioma in an HIV-positive patient

BackgroundSpinal epidural cavernous hemangioma is rare.PurposeHere, we report a case of spinal epidural cavernous hemangioma in an HIV-positive patient.Study designSingle case report.MethodA 40-year-old known HIV-positive woman presented with a 6-month history of slowly progressive bilateral leg weakness and pain. Neuroimaging showed an epidural mass lesion, which was isointense on T1–WI, hyperintense on T2–WI, and homogenously enhanced with gadolinium diethylenetriamine pentaacetate (Gd-DTPA), from T8 to T10.ResultsThe patient underwent T8–T10 laminectomy and removal of this lesion. Pathological diagnosis was cavernous hemangioma.ConclusionAlthough soft-tissue tumors such as Kaposi's sarcoma and lymphomas have been well documented in association with HIV infection, this is the first reported case of spinal epidural cavernous hemangioma. This may be an incidental finding or perhaps a causal relationship exists, suggesting that cavernous hemangioma could be a differential diagnosis of a spine lesion in HIV-positive patients.