Primitive neuroectodermal tumor of the ovary

A 31-year-old woman presented to King Abdulaziz University Hospital complaining of an abdominal pain and a rapid increase in abdominal girth. An ultrasound and MRI, revealed a huge cystic ovarian mass without ascites. Ovarian tumor markers were all within normal range. Exploratory laparotomy showed huge right ovarian mass with omental mass. Frozen section from the omentum showed metastatic malignant neoplasm. Total abdominal hysterectomy was carried out with bilateral salpingooophorectomy and omentectomy with residual tumor of less then one centimeter. Final pathology assessment showed primitive neuroectodermal tumor arising from the right ovary. She received post- operative chemotherapy. Four months later she had recurrence and was given second line chemotherapy, but she did not respond and died 15 months after the diagnosis due to obstructive uropathy.     

Primitive neuroectodermal tumor of lumbar spine: case report

Primitive neuroectodermal tumors （PNETs）, derived from the primitive neural crest, are highly malignant and mainly exist in the central nervous system （CNS）, chest wall, lower extremities, trunk, kidney, and orbit but rarely in the spine. Though multidisciplinary treatments have been well established as the standard therapy for intracranial PNETs, little is known about the optimal treatment strategy and therapeutic results for intraspinal PNETs. The following report illustrates the operative and non-operative management of an extradural PNET at the level of L5 in a young girl.     

Primitive neuroectodermal tumor of the kidney with inferior vena cava tumor thrombus during pregnancy response to sorafenib

Primitive neuroectodermal tumor （PNET） most often presents as a bone or soft tissue mass in the trunk or axial skeleton in adolescents and young adults.1 It is highly aggressive and rarely arises in the kidney.2 A combined therapy, consisting of surgical resection,chemotherapy and radiotherapy, is needed to treat this tumor but long-term survival remains poor.3,4 In the current study, we describe a case of 26-year-old woman （primigravida） who presented with a PNET of the right kidney with inferior vena cava （IVC） tumor thrombus.     

Melanotic neuroectodermal tumour of infancy

Melanotic neuroectodermal tumour in infancy is rare, mainly benign with little tendency to recur after excision or effective curettage. This pigmented neoplasm of neural crest origin occurring in infants before 1 year of age. The most common site of occurrence is the anterior maxillary alveolar ridge (70%), following by the skull, brain and mandible. The genital organ is the most frequent extra cranial site. We report a 6 months old male baby with a similar tumour arising from right half of cheek involving the maxilla. We diagnosed the case after histological report. We remove the tumour through a sub-labial incision. The mass was blackish in colour, and was mobilized from all side including from the maxillary sinuses. The author thought that this should be reported for improving the clinical awareness and treatment of pigmented soft tissue mass in children. Almost one year follow up of the patients showed no recurrence.     

类似脑膜瘤的原始神经外胚叶肿瘤3例

1临床资料例1: 男,6岁.头痛伴癫痫1 mo,复视10 d.神志情,双侧视乳头水肿,右侧动眼神经不全麻痹,左侧巴氏征阳性.     

Peripheral type of primitive neuroectodermal tumour arising from the left orbital floor

Primitive neuroectodermal tumours (PNETs) are rare tumours that originate from primitive neural crest cells. They are usually found in children below ten years of age. Peripheral PNETs (pPNETs) occur in soft tissues of the body, but have the same genetic changes as Ewing's sarcoma of the bone (now called soft tissue Ewing's sarcoma). They commonly present in the thoracopulmonary region, abdomen, pelvis and the extremities. The head and neck regions may also be involved. Our case demonstrates a PNET in the peripheral tissue arising from the left orbital floor and spreading locally to involve the left maxillofacial region, cheek and gum. The incidence of pPNETs is likely to be under-reported in the literature. Recent diagnostic advances, including cytogenetic and immunohistochemical analysis, have allowed these tumours to be distinguished from other small, poorly differentiated round cell tumours such as rhabdomyosarcoma, lymphoma and poorly differentiated synovial sarcoma.     

儿童盆腔原始神经外胚叶瘤1例

1 临床资料患儿,女,14岁。主因排便困难3个月,发现肛周肿物2个月,左下肢肿胀1个月入院。患儿于3个月前无明显诱因出现排便困难,进行性加重。2个月前发现肛周有一肿物,如花生米大小,渐增大。1个月前出现左下肢浮肿。在当地医院诊断为“肛瘘”并行手术,术后排便困难不缓解,肛周肿物渐增大,左下肢肿胀渐蔓延至双侧腹股沟及外阴区,于当地上级医院行腹部B超示:(1)盆腔实性占     

A childhood malignant rhabdoid tumour of the kidney

A case report is presented of a malignant rhabdoid tumour occurring in the kidney of a 23-month-old boy. Important differences between this and the conventional Wilms' tumour include the histological demonstration of sheets or cords of large cells resembling myoblasts, a tendency to frequent clinical relapse, and a high mortality rate despite multimodal therapy. In this child, an encouraging initial response to pre-operative chemotherapy, followed by surgical excision and postoperative triple chemotherapy, was not sustained. Recurrence of pulmonary metastases did not respond to further chemotherapy and whole lung irradiation.     

Melanotic neuroectodermal tumour of infancy (progonoma) treated by radical maxillary surgery

Two cases of melanotic neuroectodermal tumour of infancy (MNTI), otherwise known as melanotic progonoma, both arising from the maxilla are presented here. The two infants both presented within three months of each other and though clinically Burkitt's lymphoma was stated as a possible diagnosis, the correct diagnosis was made on biopsy specimens. Radical surgery consisting of wide resection of the tumour with margins of healthy tissue via hemi-maxillectomy was performed in each case as an initial method of treatment. At follow up four months later, both infants appeared clinically well. One infant was then lost to follow up thereafter but the other was reviewed three years later, and apart from slight facial asymmetry, there was no evidence of tumour recurrence.     

Carcinoid tumour of the kidney in a Chinese woman presenting with loin pain.

Renal carcinoid tumours are uncommon. The aetiology is not yet fully understood and there is still no useful diagnostic tool for detecting them. We report our experience managing a Chinese woman with a primary renal carcinoid tumour.     

Carcinoid tumour of the cervix.

A case of non-metastatic carcinoid tumour of the cervix is described from a patient without the carcinoid syndrome. The rarity of carcinoid tumour involving gynaecological structures other than the ovaries is discussed and a bried review of carcinoid disease presented.     

Benign chondrolipomatous tumour of the breast.

Cartilage associated with malignant neoplasms of breast has been known and documented for over 200 years. Benign breast tumours containing cartilage are rare. A case of such a tumour was encountered. Histologically it comprised multiple foci of mature benign cartilage in benign fibrous and adipose stroma. Other cases of similar tumours in the literature are reviewed.     

盆腔原始神经外胚层瘤一例报告

1临床资料患者男性,33岁,因"尿频、尿急1月余,B超发现盆腔肿块6 d"于2003年11月18日入院.患者1个月前无明显诱因出现尿频、尿急,无明显尿痛,无畏寒、发热,在当地医院给予抗生素抗感染治疗(具体不详)症状无缓解,后患者出现肛门坠胀,大便次数增多(每日3次以上,大便形状变扁).