An unusual presentation of aggressive fibromatosis (retroperitoneal desmoid tumour) in pelvic cavity presented as obstructed labour

Retroperitoneal desmoid tumours are uncommon tumours usually occurring in young females in their reproductive age group and commonly have a benign course but sometimes they can become malignant. These tumours belong to family of myofibroblastic fibromatosis. These tumours are composed of fibroblastic cells with bands of collagen and ill defined cytoplasm and rarely show mitosis. Recurrence is common especially after incomplete excision. Retroperitoneal desmoid is an uncommon tumour among desmoid group which led to an obstructed labour. Here a 28-year-old female diagnosed to have a pelvic desmoid, was missed during the antenatal check-up, presented as an obstructed labour which necessitated a caesarean section. The patient underwent a definitive surgery three months later for excision of tumour. The tumour was excised through a combined abdominal and gluteal incision. Histopathological report revealed an aggressive fibromatosis (retroperitoneal desmoid).     

臀部巨大韧带样纤维瘤一例及文献复习

目的 探讨韧带样纤维瘤发病机理、临床特点、影像学特征及治疗方法.方法 报告1例这种罕见病例,并进行了有关文献复习.结果 报道臀部巨大韧带样纤维瘤一例并复习相关文献.本病例患者,女,45岁,临床表现为左臀部胀痛、肿块和跛行,无创伤史和家族性遗传病史.病变位于左侧股骨后方自髋臼顶水平至股骨髁水平,呈条索状大小约28cm×4...     

Extra-abdominal fibromatosis invading the mandible: case report

Extra-abdominal fibromatosis (desmoid tumour) is a rare aggressive neoplasm with a tendency to infiltrate local structures but rarely metastasises or undergoes spontaneous malignant transformation. The treatment of choice is surgery, however, recurrences have been reported even after wide-field resection. This article presents a case of extra-abdominal fibromatosis that had extensively invaded the mandible.     

Giant retroperitoneal desmoid tumour

A giant retroperitoneal desmoid (30 x 15 cm), in a 16-year old girl arising from psoas fascia is reported. Despite debulking surgery, adjuvant radiotherapy, anti-oestrogen agents and non-steroidal anti-inflammatory agents, 3 years later she died from tumour invasion of major blood vessels and bowel, leading to massive gastrointestinal bleeding.     

腹壁韧带样纤维瘤的诊断与临床病理分析

目的总结腹壁韧带样纤维瘤的诊断及治疗特点。方法收集经手术病理证实为腹壁韧带样纤维瘤的21例患者的临床资料,包括临床症状、包块特征、影像学资料、病理(常规及免疫组化)、手术及术后辅助治疗等,并进行回顾性分析。结果所有病例均有明确的手术、外伤史或妊娠史,而无明显症状,包块平均直径4.5cm,女性18例。MRI提示边界不清,T1WI常为等信号或稍低信号,T2WI提示稍高信号,增强后明显强化。肿瘤位于肌肉内或与筋膜相连处,质地坚韧,由梭形细胞和胶原纤维束组成,胶原纤维成分特别丰富,无明显包膜,边缘不清,向周围组织浸润,常侵及横纹肌,无病理性核分裂象。免疫组化检查梭形细胞波形蛋白(Vimentin)5例均阳性反应;平滑肌抗体SMA灶性阳性5例;ki-67指数均阴性或不高于5%。术中需切除周围2～3cm组织,手术后局部复发2例。结论腹壁韧带样纤维瘤是一种交界性肿瘤,临床及病理有一定的特征,为其诊断及治疗提供依据。     

Multiple carcinomas of the large and small bowel in childhood

A case is reported of multiple metachronous carcinomas of the small and large intestine in a child. The latter were associated with multiple adenomatous polyps in the colon. A desmoid tumour and multiple keloids developed in the laparotomy scars. The relationship of this case to Gardner''s syndrome and polyposis coli is discussed and the literature on intestinal carcinoma in childhood is briefly reviewed.     

Gastrointestinal tract

Surgical site infection associated complications in colorectal cancer patients;Endoscopic interventional treatment of malig. nant gastrointestinal obstruction; Induction of apoptosis in human gastric carcinoma cell line SGC-7901 by anti-Fas monoclonal antibody;Disciplinarian of lymph node metastasis and effect of paraaortic lymph nodes dissection on clinical outcomes in advanced gastric carcinoma;Comparison of two reconstruction methods of alimentary canal after total gastrectomy; Diagnosis and therapy of familial adenomatous polyposis with desmoid tumour; The role of CD2 in acute rejection after small bowel transplantation in rats;     

Surgical extirpation of a chest wall desmoid tumor: a case report

A case is described of an anterior chest wall desmoid tumor in a 20-year-old Micronesian male that had been previously incompletely resected one year prior to presentation. A radical chest wall resection was performed with reconstruction accomplished using a gortex patch and latissimus dorsi myocutaneous flap. The patient developed a massive local recurrence within eight months following surgery. This report illustrates the local aggressiveness of these benign tumors. A historical perspective, etiology, and treatment principles are discussed.     

小剂量甲氨蝶呤联合长春瑞滨治疗不可手术切除的硬纤维瘤的疗效及预后因素分析

目的研究硬纤维瘤的临床特点,探讨对于不可手术切除的硬纤维瘤患者进行系统性治疗的有效性并分析其预后因素。
方法纳入2009年8月~2013年12月间北京大学肿瘤医院骨与软组织肿瘤科连续收治的52例不可手术切除的硬纤维瘤患者,
治疗方法为长春瑞滨联合小剂量甲氨蝶呤化疗。结果确诊的52例患者中,男性22例,女性30例。发病年龄2~46岁,平均发病
年龄18.7岁。发病部位以下肢最多,占36.5%（19/52）。肿瘤最大径2.7~37 cm,平均9.5 cm。随访时间7~64月,中位随访时间
29月。化疗持续时间4~30月,中位化疗时间12月。化疗结束时评效：1例CR,18例PR,27例SD,6例PD。总有效率（CR+PR+
SD）为88.5%。PFS时间为4~63月,中位PFS时间26.5月。2年PFS为76.7%,5年PFS为41.9%。化疗持续时间大于12月者预
后较好,不同性别、发病年龄、化疗时年龄、肿瘤部位、肿瘤大小组间PFS无统计学差异。结论足够疗程的长春瑞滨联合小剂量
甲氨蝶呤的化疗,在肿瘤反复复发、不可切除且肿瘤持续进展的情况下,是一种安全满意的治疗选择。
     

Benign breast lesions mimicking carcinoma at mammography

Many benign breast lesions pose diagnostic challenges. These lesions include abscess, haematoma, radial scar, post surgical scar, diabetic mastopathy, focal fibrosis, sclerosing adenosis, granular cell tumour, extra-abdominal desmoid tumour, medial insertion of pectoralis muscle and sternalis muscle, and axillary lymphadenopathy (due to HIV infection, collagen vascular lesions, tuberculous and bacterial lymphadenitis). Radiologists should be familiar with the characteristic imaging features of these benign lesions, and should include these benign lesions in the differential diagnosis whenever malignant-appearing findings are encountered. Correlation of the patient's clinical features with the mammographical findings and additional use of ultrasonography, fine-needle aspiration biopsy or core biopsy are helpful in establishing the final diagnosis and obviating unnecessary surgical intervention. In some of these lesions, surgery may be avoided while in others, the appropriate surgical procedure may be planned. This pictorial essay aims to illustrate the mammographical features of these lesions in a group of proven cases.     

A case of Turner's syndrome complicated with desmoid tumor of the transverse colon

The association of Turner's syndrome and endometrial carcinoma has been previously established, but has never been described in conjunction with a desmoid tumor of the colon. A case is described of a colonic desmoid tumor developing in a 38-year-old female with Turner's syndrome. The association has not been previously reported. In this report, we describe a 38-year-old woman who has Turner's syndrome with a colonic desmoid and review the literature.     

胰腺纤维瘤破裂出血导致失血性休克1例报道——罕见急腹症病因

我们发现一例罕见的24岁年轻男性因胰腺纤维瘤破裂出血导致失血性休克病例。患者急诊行外科手术治疗，最终病理检查证实诊断。虽然胰腺纤维瘤不发生转移，但因其会侵犯血管出血导致生命危险，需行外科手术治疗。     

腹壁硬纤维瘤的治疗

探讨自体阔筋膜移植或结合腹直肌前鞘翻转成形加自体阔筋膜移植，在修复硬纤维瘤切除后腹壁大缺损的作用。方法；在大腿外侧面作平行的二个皮肤纵形切口，皮下潜行分离后用切割器切除阔筋膜，并进行０．５厘米重叠的褥式缝合修补缺损。     

腹壁韧带样纤维瘤的临床特点及治疗

目的总结腹壁韧带样纤维瘤的临床特点及治疗方法。方法回顾性分析广东医学院附属医院13年来34例腹壁韧带样纤维瘤诊治情况,并参阅相关文献。结果34例患者均经手术及病理证实为腹壁韧带样纤维瘤,以腹壁肿块为首发症状,特点为浸润性生长,术前诊断考虑该病者19例,切除范围2cm以上与2cm以下（包括侵犯周围脏器）的复发率（1／19vs6／15）比较差异有统计学意义（P〈0．05）,放疗也能减少术后复发率,复发患者再次手术均能切除或部分切除,无远处广泛转移。结论腹壁韧带样纤维瘤病术前诊断有一定难度,确诊主要依靠病理检查,根治性手术切除是主要治疗方法。     

Extra-abdominal desmoid fibromatosis in the foot: a case study

Extra-abdominal desmoid tumors account for 0.03% of all neoplasms and rarely present in the foot. They are benign but locally aggressive, and wide local surgical excision is the treatment of choice owing to the high rate of recurrence in the lower extremities. Invasiveness into the surrounding soft-tissue structures often makes wide excision difficult without compromise of function. We describe a 34-year-old woman with a large, pedal, extra-abdominal desmoid fibroma treated by wide local surgical excision with minimal complications postoperatively and no clinical evidence of recurrence at 28 months.     

腹壁韧带样瘤的CT与MRI征象分析

目的回顾性分析腹壁韧带样型纤维瘤病的cT和MRl表现,提高CT和MRI对该病的认识和诊断水平。方法回顾性分析经临床手术病理证实5例腹壁韧带样型纤维摘病的临床、病理、CT和MRI特征。5例中2例行CT平扫和增强扫描：2例行MRI平扫及增强扫描;1例行CT、MRI平扫及增强扫描检查。分析其cT和MRI表现特征并与病理结果对照。结果5例腹壁韧带样瘤均发生于前腹壁腹直肌内,4例位于左侧腹直肌内,l例位于右侧。CT、MRI表现：边祭多不清,呈浸润性生长,无包膜;坏死或钙化少见：密度、信号可均匀或不均匀。注射造影剂后,4例较均匀强化,1例不均匀强化。,CT表现缺乏特征性,而MRI~／-J,N可见T2wI稍高信号及致密胶原纤维所致的低信号,具有一定特征性。结论cT增强扫描检查可满足病变的检出与定位的要求;而MRl的优势在于其表现表现较具有特征性,对腹壁韧带样瘤病的术前诊断、鍪别诊断具有重要价值。     

Brain herniation in a neonate

Brain herniation is generally thought to be unlikely to occur in newborns due to the presence of the patent fontanelles and cranial sutures. A review of the literature published from 1993 to 2008 via MEDLINE search revealed no reports on neonatal brain herniation from intracranial tumour. We report a preterm Malay male infant born via elective Caesarean section for antenatally diagnosed intracerebral tumour, which subsequently developed herniation. Cerebral magnetic resonance imaging showed features that were compatible with a large complex intracranial tumour causing mass effect and gross hydrocephalus. Tumour excision was scheduled when the infant was two weeks old. Unfortunately, on the morning of the surgery, he developed signs of brain herniation and had profuse tumour haemorrhage during the attempted excision. Histopathological examination revealed an embryonal tumour, possibly an atypical rhabdoid/teratoid tumour. This case illustrates that intracranial tumours in newborns can herniate and should therefore be closely monitored.     

家族性腺瘤性息肉病大肠外病变22例分析

目的总结家族性腺瘤性息肉病（familial adenomatous polyposis，FAP）的临床表现特征，探讨FAP的大肠外病变。方法回顾1995～2000年22例FAP患者上消化道及腹部影像学检查资料，分析其大肠外病变特征。结果22例FAP患者中伴胃窦部增生性息肉19例，占86．3％；十二指肠各段有息肉样病灶18例，占81.8％（其中腺瘤性息肉12例，无1例癌变）。22例中有3例分别在大肠切除术后5年内发生腹壁、腹盆腔和小肠系膜硬纤维瘤．仅1例手术完整切除治愈。结论上消化道息肉是FAP常见大肠外病变，国人十二指肠腺瘤癌变罕见；硬纤维瘤严重影响FAP患者大肠切除术后的生存质量。     

Recurrence of Phaeochromocytoma and Abdominal Paraganglioma After Initial Surgical Intervention

BackgroundClinical and biochemical follow up after surgery for phaeochromocytoma is essential with long term studies demonstrating recurrence frequencies between 6% and 23%.AimTo examine the characteristics and frequency of tumour recurrence in a regional endocrine referral centre, in patients with surgical resection of phaeochromocytoma (P) and abdominal paraganglioma (AP).MethodsWe identified a cohort of 52 consecutive patients who attended our Regional Endocrinology & Diabetes Centre and retrospectively reviewed their clinical, biochemical and radiological data (between 2002 and 2013). After confirmation of early post-operative remission by negative biochemical testing, tumour recurrence was defined by demonstration of catecholamine excess with confirmatory imaging.ResultsPhaeochromocytoma was confirmed histologically in all cases (43:P, 9:AP, mean-age:53years). Open adrenalectomy was performed in 20 cases and laparoscopically in 32. Hereditary phaeochromocytoma was confirmed by genetic analysis in 12 (23%) patients. Median follow up time from initial surgery was 47 months, (range: 12 - 296 months), 49 patients had no evidence of tumour recurrence at latest follow-up. Three patients (6%) demonstrated tumour development, one in a patient with VHL which occurred in a contralateral adrenal gland, one sporadic case had local recurrence, and an adrenal tumour occurred in a patient with a SDHB gene mutation who had a previous bladder tumour. After initial surgery, the tumours occurred at 8.6, 12.0 and 17.7 years respectively.ConclusionIn this study tumour development occurred in 6% of patients. Although tumour rates were low, careful and sustained clinical and biochemical follow up is advocated, as new tumour development or recurrence may occur long after the initial surgery is performed.     

Hyperthyroidism during pregnancy due to coexistence of struma ovarii and Graves' disease

A patient with hyperthyroid Graves' disease and struma ovarii is described. She had pre-existing Graves' disease and positive thyrotrophin receptor antibody. She was treated with radioactive iodine 5 months before she became pregnant. Paripartum she had torsion of an ovarian cyst with histological evidence of a functional struma ovarii. Immediate exacerbation of her thyrotoxic state was observed after operation as a result of release of thyroid hormone from the tumour. It is postulated that the tumour was stimulated by circulating thyrotrophin receptor antibody.