Solid pseudo papillary tumor of the pancreas: case report and literature review

Solid pseudopapillary neoplasm (SPT) of the pancreas is a rare exocrine tumor, for the first time described from Frantz et al. in 1959. Despite the increasing recognition of the tumor in this last year, its pathogenesis remain unclear. It occurs predominantly in young woman and behave in an indolent fashion, even when distant metastasis are present. The Authors report the case of a 24 years-old woman with an abdominal mass localized in retro-peritoneum, removed with body-tail of the pancreas and spleen, diagnosed as pancreatic SPT after histological examination.     

Non-functioning neuroendocrine tumor of the pancreas: report of a clinical case and review of the literature

The authors report the case of a 26-year-old woman, with a palpable abdominal mass, dyspepsia, pain and weight loss. These symptoms were caused by a non-functioning or biologically inactive neuroendocrine tumour (BINT), weighing 510 g and located in the tail of the pancreas. The treatment opted for was a surgical resection consisting in a distal pancreatectomy. The results of the operation were satisfactory and the woman is now alive and in good health, without recurrence, after 7 years. The authors then go on to analyse the concept of non-functioning neuroendocrine tumours and the problems relating to their symptoms, location and nature. These tumours, which arise, from the pancreatic islet cells, fail to produce a clinical syndrome owing to insufficient peptide production or insufficient release, or concurrent secretion, of inhibitory peptides by the tumour or production of biologically inactive molecular forms of the peptides (without clinical effects). The value of tumour markers and the indications and type of surgery are also discussed according to the different patterns of tumour spread (local, locoregional and metastatic disease). It is stressed that a complete surgical resection is the only curative treatment for these tumours. Encouraging results have been recently obtained by adjuvant treatment with somatostatin analogues, chemotherapeutic agents and/or inteferon.     

Synchronous prostate stromal sarcoma and gastrointestinal stromal tumor of rectum: case report and review of the literature

We report a case of prostate stromal sarcoma with initial presentation of obstructive voiding symptoms. The synchronous rectal gastrointestinal stromal tumor was also finally discovered. The patient underwent pelvic exenteration as the choice of treatment. Synchronous prostate stromal sarcoma and rectal gastrointestinal stromal tumor is an extremely rare disease in the literature view.     

Gastrointestinal stromal tumours: a case report and review of the literature

Gastrointestinal stromal tumours are a heterogeneous group of neoplasms of the digestive apparatus characterised by a complex histogenesis and lines of differentiation that make it difficult to predict their biological behaviour. Expression of the c-kit receptor in about 99% of gastrointestinal stromal tumours suggests they may originate from a common mesenchymal element with a variable possibility of phenotypical differentiation. The Authors report a case of small bowel gastrointestinal stromal tumour referred to them for severe bleeding and treated surgically. They then go on to analyse the diagnostic and therapeutic problems that such tumours entail. Surgery is currently the only therapeutic option capable of improving survival in patients with gastrointestinal stromal tumours. Further studies are needed to assess the efficacy of laparoscopy and new chemotherapeutic agents.     

Primary extragastrointestinal stromal tumor arising in the pancreas: report of a case

Primary extragastrointestinal stromal tumors (EGISTs) arising in the pancreas are extremely rare, with only ten cases documented to our knowledge. We report a further case of EGIST of the pancreas. The patient was a 55-year-old man who presented with postprandial abdominal discomfort. Abdominal computed tomography and magnetic resonance imaging showed a lobulated heterogenous enhancing mass, 11 cm in diameter, in the abdominal cavity. No regional lymphadenopathy, ascites, or metastasis was seen radiologically. There was no obvious lesion in the stomach or small intestine. The initial diagnosis was a solid pseudopapillary tumor or serous cystic neoplasm. The patient underwent distal pancreatectomy with splenectomy. Microscopically, the tumor consisted of spindle cells arranged in short fascicles. Mitotic figures were seen in 7/50 high-power fields. Immunohistochemical examination revealed strongly positive staining for CD117. Based on these findings, the final pathologic diagnosis was a primary EGIST of the pancreas. This case consolidates the possibility that this rare tumor can involve the pancreas as a primary site and should be included in the differential diagnosis of cystic lesions in this site.     

Solid pseudopapillary tumor of the pancreas in children: report of a case and review of the literature

Solid pseudopapillary tumor (SPT) of the pancreas is an infrequent neoplasm of low malignant potential, first described by Frantz in 1933 and representing less than 3% of all exocrine tumors. SPT is rare in children, accounting for 6% of all cases and shows different clinical features compared with adults. Here, a pediatric case is reported and a review of the Literature is provided. A 15-year-old girl with a 12 × 14 × 10 cm solid mass growing from the tail and the body of the pancreas, involving spleen, left adrenal gland and kidney, stomach and some bowel loops, was referred for surgical treatment. A distal pancreasectomy with splenectomy was performed. Histopathological examination revealed that the tumor was a 14-cm well-circumscribed solid mass, with pseudopapillary cell architecture, showing strong cellular immunoreactivity for alpha-1 antitrypsin, vimentin, neurone-specific enolase, progesterone receptors and in part to CD10 and CAM 5.2, but not to sinaptofisin and chromogranin. A 24-month post-surgical follow-up after successful surgical resection showed no evidence of recurrent disease. SPT shows different clinical features in childhood. High survival rates can be achieved in most cases, warranting aggressive treatments even in metastatic disease.     

胰腺混合性腺泡- 神经内分泌癌1例报道及文献复习

目的:探讨胰腺混合性腺泡-神经内分泌癌(MANEC)的临床病理特征、鉴别诊断和治疗方法。方法:回顾性分析收治的1例胰腺MANEC患者的临床资料,结合国内外文献,总结该病的临床特点、病理特征、鉴别诊断和治疗方法。结果:该患者术前影像学检查提示十二指肠降部肿瘤,行手术治疗,术后病理提示胰腺MANEC。术后患者顺利出院,20个月后复查CT提示肿瘤无复发转移。结论:胰腺MANEC是非常罕见的肿瘤,常无典型的症状,最终需要病理确诊。预后至今不清楚,但是早期的手术治疗能显著提高总的生存期。     

Solid-pseudopapillary tumors of the pancreas: case report and literature review

Solid pseudopapillary tumors (SPT) of the pancreas are rare neoplasms of low malignant potential that mostly affect young women. These tumors are of unclear pathogenesis, are slow growing, and can become considerably large before causing symptoms. Complete resection is curative in most cases. This is the case of a 39-year-old African-American woman undergoing evaluation for Roux-en-Y gastric bypass, who was found to have a pancreatic mass. Image-guided biopsy revealed SPT. The patient underwent complete excision of the tumor and had an open Roux-en-Y gastric bypass performed concurrently. The patient had an uneventful postoperative course. A review of the literature is presented.     

高危原发前列腺胃肠道外间质瘤诊疗体会及文献复习

目的探讨前列腺原发胃肠道外间质瘤诊治要点。 方法回顾性分析我院2017年9月诊治的1例高危原发性前列腺胃肠道外间质瘤临床病理特征资料、随访情况，总结现有文献讨论总结本病诊治心得。 结果65岁男性，因"前列腺电切术后2年，反复血尿3个月余"入院。术前MRI考虑为来源不清的盆腔巨大实性占位（115 mm×105 mm×85 mm），经直肠穿刺诊断为梭形细胞来源的肿瘤。行盆腔肿瘤切除+膀胱前列腺腺切除+盆腔淋巴结清扫术+Bricker术。术后病理提示为前列腺原发胃肠道外间质瘤[CD117（+）；Dog1（+）；CD34（+）；PSA（+）；AR（+）；P504s（+）；Ki-67（2%）]。术后肿瘤组织全外显子测序提示为C-Kit基因（Exon 11 p.Q556-V560del）存在明显临床意义突变，筛选靶向药物甲磺酸伊马替尼+比卡鲁胺（PSA平稳后停用）口服，术后随访18个月无肿瘤复发及不良并发症。 结论前列腺原发胃肠道外间质瘤罕见，需与前列腺其他良恶性肿瘤相鉴别诊断。全外显子测序了解其发病高危基因，同时筛选药物辅助治疗可使患者生存获益。     

Merkel cell tumor: a case report and literature review

Merkel cell tumor (MCT) is extremely rare, being discovered so far about 400 cases in literature. It is classified among neuroendocrine tumors. We report a case of MCT in the subclavicular region in a 93 years old woman. We confirm the efficacy of radiotherapy associated with octreotide, which these tumours express specific receptors for.     

Primary melanoma of small intestine masquerading as gastrointestinal stromal tumor: a case report and literature review

Malignant melanoma of the gastrointestinal tract is a rare entity among intestinal neoplasms. Primary intestinal melanoma is difficult to differentiate from metastatic melanoma, especially given that the primary cutaneous lesion has the potential to regress and disappear. In addition, melanoma by itself is a great mimicker of other neoplastic conditions and may create a major diagnostic challenge when presenting at an intra-abdominal location. Here we report a case of small intestinal melanoma in a 74-year-old female who presented with symptoms of intestinal bleeding and a preoperative clinical and radiological diagnosis of gastrointestinal stromal tumor. The initial frozen section diagnosis also favored gastrointestinal stromal tumor, however furthermore histological and immunohistochemical stain evaluation confirmed the diagnosis of intestinal melanoma.     

肾类癌1例报道并文献复习

目的探讨肾类癌的临床病理特征。方法对1例肾类癌的临床资料进行分析并结合文献复习。结果 59岁女性患者体检时偶然发现无症状左肾肿物,患者无类癌综合征。CT示左肾下极肿物,直径约3.8 cm,边界清楚,强化不明显。患者行经腹膜后途径根治性左肾切除术。免疫组织化学染色显示突触素,CD56,神经元特异性烯醇酶,波形蛋白,CK18呈阳性。病理诊断：左肾类癌T1a期。术后未行辅助治疗,随诊17个月未见肿瘤复发或转移。结论肾类癌极其罕见,确诊依赖病理学及免疫组织化学检查。局限性肾类癌的治疗首选完整的外科切除,其预后相对较好。     

Sarcoidosis of the pancreas: case report and review of the literature

Sarcoidosis involving the pancreas is rare. Patients can present with symptoms that mimic pancreatic cancer. We report a case of a male patient with clinical and radiographic findings suggestive of pancreatic cancer as the initial manifestation of sarcoidosis.     

Paraspinal desmoid tumor: a case report and a literature review

Aim  Paraspinal desmoid tumors are rare and only few cases have been reported in the literature. This report describes a case of successful excision of a parasacral desmoid tumor in a 34 years old female. Methods  The patient presented with a tender mass over her right sacral region. Radiological examination with CT and MRI demonstrated two adjacent well circumscribed masses measuring 4 × 3 and 2.5 × 3 cm, in the subcutaneous fat of the right paravertebral region. At surgery the patient underwent a wide excision of the lesion, and two tumor masses were dissected away from the gluteus and the multifidus muscles. Histopathologic examination of the specimens confirmed the diagnosis of a desmoid tumor. Results  After surgical resection without adjuvant therapy, the patient is recurrence free after 36 months. Conclusions  Despite their benign microscopic appearance, and their insignificant metastatic potential, the tendency of desmoid tumors for local infiltration is potentially significant causing deformity, morbidity and even mortality, especially when present at the paraspinal area, due to the pressure effects to the close neural structures and the spinal canal as well. Early detection of the neoplasm is crucial, in order to achieve radical excision of the neoplasm before it penetrates vital structures.     

Lymphoepithelial cyst in the pancreas: a case report and review of the literature

BACKGROUND: Lymphoepithelial cysts of the pancreas constitute a rare clinicopathologic entity. CASE REPORT: We report a case of lymphoepithelial cyst of the pancreas and review the world literature. RESULTS: Lymphoepithelial cysts are true pancreatic cysts lined by squamous epithelium and surrounded by mature lymphoid tissue. The cyst arises typically in middle aged men, and is usually asymptomatic or causes nonspecific abdominal complaints. There is no specific serologic marker for this entity. None of its radiologic characteristics can help differentiate it from other cystic lesions of the pancreas. Fine-needle aspiration cytology may be able to suggest its benign nature and identify it as a true cyst of the pancreas. The outcome after surgical excision is uniformly good with good symptom control and no recurrences. RECOMMENDATIONS: In the symptomatic patient or the asymptomatic patient with acceptable surgical risk a simple cyst excision should be performed after verification of the diagnosis with frozen section. In the asymptomatic patient with a high surgical risk, in whom fine-needle aspiration suggests the diagnosis of a lymphoepithelial cyst, observation of the lesion is recommended. When simple cyst excision is technically not possible, extensive resections/reconstructions should be avoided and drainage/bypass procedures may be considered.     

Intracerebral epidermoid tumor: a case report and review of the literature

BACKGROUND: Intracerebral epidermoid cysts are rare lesions and may account for only 1.5% of intracranial epidermoid tumors. Cell entrapment from the mesectodermal origin of the neural crest within the primitive cerebral hemisphere may lead to the formation of such rare intracerebral lesions. METHODS: An intracerebral epidermoid cyst located in the right temporal lobe that was surgically treated is presented. The origin, clinical course, radiological features, and surgical treatment of such uncommon lesions are discussed based on a review of the literature.RESULTS: Intraoperative findings revealed an epidermoid tumor. The postoperative course was uneventful and the patient was discharged with no neurological deficits. On long-term follow-up (2 years), there were no signs of recurrence. CONCLUSIONS: Truly intracerebral epidermoid tumors are rarely found. Cells originating from mesectodermal lines may migrate and remain trapped within the primitive cerebral hemisphere while the neural tube is closing, leading to the formation of such intraparenchymal epidermoid tumors. Accurate preoperative diagnosis can be very difficult due to the radiological similarities to other common intracerebral cysts (e.g., astrocytomas or gliomas). Magnetic resonance imaging (MRI) studies, especially with diffusion-weighted images, allow greater accuracy in the preoperative differential diagnosis. Radical surgical removal should be attempted, but a less aggressive surgical strategy should be considered if there is strong adherence between the tumor capsule and the brain tissue, particularly in eloquent areas.