Annular pancreas in adults: embryological development, morphology and clinical significance

Three cases (two male, one female) of annular pancreas are presented, which were found and operated on within the last three decades. Diagnosis was made using imaging techniques, especially ultrasonography and computed tomography. In two cases there was incomplete obstruction, while in the other obstruction was complete. It is characteristic that in the case of complete obstruction the annular portion of the pancreas attached to a circular band of connective tissue, while in the two cases of incomplete obstruction there was a complete ring of pancreatic tissue. In all cases surgical intervention was needed in order to create a bypass. In the first case gastroenterostomy and truncal vagotomy was performed, in the second latero-lateral duodeno-jejunostomy and in the third latero-lateral antropyloroduodeno-jejunostomy. The embryology and morphology of the annular pancreas, the morphology of its duct system, diagnosis, differential diagnosis and the surgical treatment of this rare condition are reviewed.     

Annular pancreas in adults (2 case reports)

Two cases of annular pancreas in adults are being reported. In the first case, a middle aged lady presented with duodenal obstruction and gastric ulcer. Anterior H. S. V. with posterior truncal vagotomy and isoperistaltic duodeno-jejunostomy relieved her symptoms. In the second case, a young girl presented with duodenal obstruction, severe wasting and gross dehydration. She had an uneventful recovery following a duodeno-jejunostomy.     

Annular pancreas: A review of its molecular embryology,genetic basis and clinical considerations

The annular pancreas remains an intriguing congenital anomaly. Many theories have sought to explain its embryological basis; however, no consensus has yet been reached regarding the exact mechanism of aberration. More recently, molecular investigations have shed light on some of these theories, confirming the origination of annular tissue from the ventral pancreatic bud. These studies highlight the role of the hedgehog signaling pathway in the development of this anomaly. Overexpression of the ventral-specific gene transmembrane 4 superfamily member 3 (tm4sf3) has also been associated with annular formation. Moreover, isolated case reports of familial annular pancreas have also been documented, suggesting a genetic basis for the development of this anomaly. Annular pancreas can initially present in childhood or adulthood with symptoms of duodenal obstruction and is diagnosed using a variety of imaging modalities. Treatment usually involves surgical correction, with bypass procedures (e.g. duodenoduodenostomy, gastrojejunostomy or duodenojejunostomy) currently favored over annular resection due to complications associated with the latter approach.     

胃、胰外周孤立性异位胰腺—附16例(18枚)分析

在937例胃癌根治术清除的淋巴结中,发现16例(18枚)孤立性异位胰腺结节.直径最大2.2 cm、最小0.2 cm.主要异位於脾动脉干(5/18枚)、脾门及幽门下(各 3/18).其中组织结构较完全的Ⅰ型(含分叶、腺泡、导管及胰岛)有11枚,组织结构不全的Ⅱ型(仅有腺泡及导管)7枚.胰岛直径最大者500μm、最小者60μm.有胃癌转移1枚.文内除详细介绍了每例的病理形态外,尚对其组织发生进行了讨论.     

Secondary tumors of the pancreas: an analysis of a surgical and autopsy database and review of the literature

The vast majority of pancreatic carcinomas are primary, and, among these, more than 90% are of ductal origin. However, a variety of extrapancreatic tumors may involve the pancreas secondarily and may manifest different clinicopathological characteristics and outcomes. In this study, pathology material from 973 surgical specimens and 4955 adult autopsy cases was reviewed to identify the tumors metastatic to or secondarily involving the pancreas. Biliary and periampullary neoplasms and tumors confined to peripancreatic soft tissue were excluded. In the autopsy series, the pancreas was involved by tumor in 190 cases, and 81 of these were secondary tumors. These were predominantly of epithelial origin, most commonly from lung (34), followed by GI tract (20), kidney (4), breast (3), liver (2), ovary (1), and urinary bladder (1). In addition, there were six tumors of hematopoietic origin, two melanomas, two sarcomas, and two mesotheliomas. Among the 973 surgical specimens, 38 cases contained metastatic tumors to the pancreas. Of these, 11 were lymphomas, and the others were carcinomas of stomach (7), kidney (6), lung (2), liver, prostate, ovary, uterus (1 case of each), and a Merkel cell carcinoma. In addition, there were three malignant gastrointestinal stromal tumors and one retroperitoneal leiomyosarcoma. In conclusion, lung cancer is the most common source of metastasis to pancreas, followed by gastrointestinal carcinomas and lymphomas. These tumors are usually seen in patients with disseminated disease and are detected mainly in autopsies. Secondary tumors constitute about 4% of pancreatic specimens in the authors surgical database. Approximately one-third of them are clinically mistaken as primary tumors of the pancreas. These are predominantly hematopoietic malignancies or carcinomas of renal or gastric origin. Secondary tumors should be entertained in both the clinical and pathological differential diagnosis of pancreatic neoplasia.     

Histological and immunohistochemical characterization of extranodal diffuse large-cell lymphomas with prominent spindle cell features

AIMS: To describe five cases of diffuse large-cell lymphoma with prominent spindle cell components involving skin, nasal-ocular mucosa, and soft tissue. Because of the spindle cell morphology, such cases must be differentiated from true sarcomas arising in or metastasizing to soft tissue, skin, bone, lymph node, or other organs and sites. METHODS AND RESULTS: Formalin-fixed paraffin-embedded archival tissue from five consultation cases of diffuse large-cell lymphoma with prominent spindle cell features involving the skin, nasal-ocular mucosa, and soft tissue in three male and two female patients was studied by histology and immunohistochemistry. Clinicopathological findings were also reviewed for all the patients. By morphology, initial evaluation of the cases suggested spindle cell sarcoma in two cases, inflammatory pseudotumour in one case, large-cell lymphoma in another case, and one case was considered suspicious for malignant lymphoma. Immunohistochemistry demonstrated a B-cell lineage in four of the spindle cell lesions, with a diagnosis of primary cutaneous CD30+ anaplastic large cell lymphoma made for the fifth case. Four of five cases also showed actin reactivity. CONCLUSIONS: Although extremely rare, lymphomas with prominent spindle cell morphology can be encountered in daily surgical pathology practice, and should be included in the differential diagnosis of spindle cell lesions in skin and soft tissue. The observed actin reactivity in four of the five spindle cell lymphomas may lead to a misdiagnosis of leiomyosarcoma if lymphoid markers are not included in the immunohistochemical panel.     

Association of annular pancreas and duodenal obstruction – evidence for Mendelian inheritance?

This report presents a family with two individuals in two successive generations who were affected by annular pancreas and high duodenal obstruction. An argument for autosomal dominant transmission and implications for appropriate team management are discussed.     

Secondary neoplasms of the male genital tract with different patterns of involvement in adults and children

AIMS: The incidence, presentation and macroscopic and histological features of secondary neoplasms of the male genital tract are described with reference to their differential diagnosis. METHODS AND RESULTS: A retrospective study of cases from the Royal London Hospital yielded a total of 31 secondary neoplasms involving the testis: 14 at postmortem examination and 17 surgical specimens. Nine cases were leukaemias: six acute lymphoblastic and two acute myeloid leukaemias in children, and one chronic lymphocytic leukaemia in an adult. The commonest primary sites of metastases to the testis were prostate (six cases), stomach (five cases) and lung (three cases). There were two malignant melanomas and isolated examples of metastases from the adrenal gland (neuroblastoma), cerebellum (medulloblastoma), soft tissue (alveolar rhabdomyosarcoma), pancreas and rectum. Of the metastases from solid tumours, 12 involved the right testis only, three involved the left and four were bilateral. In seven of these cases there were multiple testicular nodules, in seven there was a single mass, and in the rest there was diffuse involvement. Secondary neoplasms represented 4.6% of all testicular neoplasms at autopsy, and 1.6% in surgical specimens. There were five secondary penile neoplasms: two each from the pancreas and prostate and one from the bladder. Two neoplasms metastatic to the spermatic cord, both from a gastric primary, were included in the series. CONCLUSIONS: Secondary neoplasms of the testis occur with a frequency comparable to other sites in the genitourinary tract, and metastases to the spermatic cord, epididymis, and penis, are rare in comparison. Disseminated neoplasms rarely present initially at this site and are histologically distinctive in adults, but in children they must be distinguished from primary small round blue cell tumours.     

Autosomal dominant inheritance of annular pancreas

This report describes a family with apparent autosomal dominant transmission of congenital annular pancreas. Four individuals in two generations were affected; all developed duodenal obstruction. The implications for genetic counseling are discussed.     

Localized amyloidosis of seminal vesicles: report of three cases in surgically obtained material

Localized amyloidosis of the seminal vesicles (ASV) is reported as an incidental finding in surgical specimens from three elderly men. In two cases, the amyloid deposits were bilateral, subepithelial, and clinically inapparent, features similar to other cases in the literature. In one case, the diagnosis was made on a transrectal prostatic needle biopsy that included a small portion of seminal vesicle; to our knowledge, this has not been previously reported. Electron microscopy in one case demonstrated nonbranching fibrils characteristic of amyloid, and pretreatment of tissue sections using the permanganate method in two cases showed almost complete ablation of congophilia. Evidence suggests that ASV is a permanganate-sensitive, non-AA (amyloid, protein A) type of amyloid that may be different from all other types of amyloid previously characterized.     

Fine-needle aspiration cytology in fibromatoses.

Fine-needle aspiration (FNA) cytology was performed in seven cases of fibromatosis of variable types with tumorous clinical presentation. These included: four cases of musculoaponeurotic fibromatosis, two in posterior neck muscles, one in anterior neck muscles and one in intercostal muscles; one case of fibromatosis of the breast; and two cases of fibromatosis colli in neonates. In all cases the specimens contained connective tissue with many fibroblast-like cells, lacking features which could indicate a malignant lesion. The findings in these cases indicate that, although by FNA cytology in fibromatoses a specific diagnosis for each pathologic entity may not be easily reached, in the proper clinical setting the cytologic findings can be of sufficient relevance to offset the need for an open tissue biopsy, where there are valid reasons against a surgical intervention.     

Chronic ovarian torsion after vaginal hysterectomy: a case with metastatic serous ovarian cancer

Ovarian torsion refers to partial or complete rotation of the ovary on its ligamentous support, often resulting in partial or complete obstruction of its blood supply. It is one of the most common gynecologic surgical emergencies and may affect females of all ages, but it is relatively rare in postmenopausal women. Chronic adnexal torsion with complete occlusion of the ovarian blood supply results in necrosis and loss of ovarian function. Clinical symptoms and signs are not specific and definitive diagnosis is often challenging. In this case report, we present a 65-year-old woman with chronic ovarian torsion that was detected 7 years after vaginal hysterectomy. Approximately 3% of postmenopausal cases with adnexal torsion are associated with malignancy. Ovarian torsion incidence is low in postmenopausal ovarian cancer due to the progression of accompanying inflammation, which causes immobility of the ovarian mass.     

体外培养人退变髓核与纤维环细胞增殖能力的研究

目的 探讨体外培养的人退变髓核与纤维环细胞的增殖能力,比较2种退变细胞体外培养中所表现的不同生物学行为,为椎间盘退变疾病的预防与治疗提供新的理论依据.方法 采集临床腰椎间盘突出症患者手术取材的椎间盘组织标本,病理学诊断评估其退变程度,酶消化法原代培养髓核与纤维环细胞,并鉴定.每例病例的髓核细胞与纤维环细胞分别体外培养至第5代,各代次细胞传代接种密度控制为1×105个.同条件培养48 h后,观察每一代细胞的形态学变化,同时应用流式细胞仪检测2种细胞的增殖能力.结果 体外培养的退变髓核与纤维环细胞生长状态良好.随着体外传代的进行,退变髓核与纤维环细胞S期细胞百分比和增殖指数(PI)均呈上升趋势,髓核细胞PI值于第3代时达到峰值,而纤维环细胞PI值于第5代时最高;且第2～4代的退变髓核细胞的增殖活性均高于退变纤维环的增殖活性(P＜0.05).结论 体外培养的人退变髓核与纤维环细胞有着不同的增殖特点,髓核与纤维环细胞对体内退变微环境有着不同的响应机制,并影响着整个椎间盘退变的进展.     

Need for invasive diagnostic procedures in the management of superior vena cava syndrome

Radiation therapy is considered the first treatment of choice for thoracic malignant diseases causing superior vena cava syndrome (SVCS). However, its use is poorly documented in cases in which intravascular tumor thrombosis is the major cause of obstructive symptoms. We report two cases of SVCS caused by thoracic malignancy. The first case failed to respond to the initial course of mediastinal radiation therapy. Contrast and radionuclide venography revealed two sites of tumor thrombosis located outside the radiation portal. Irradiation of these areas resulted in prompt relief of symptoms and complete relief of obstruction. In the second case, superior venacavogram and transluminal biopsy established the diagnosis and location of superior vena cava obstruction.     

Mesenteric fat necrosis after recent surgery causing bowel obstruction: a case report and review of the literature

Mesenteric fat necrosis causing bowel obstruction is a rare occurrence with only one case reported in humans. It is due to accidental or surgical trauma to the adipose tissue with extracellular liberation of fat or enzymatic lysis of fat due to the release of lipases resulting in fibrosis. Preoperative imaging may often be misleading and fail to identify fat necrosis as the cause of bowel obstruction. As surgical intervention is the only suitable treatment option in cases of failed conservative treatment, the diagnosis is made postoperatively. There is no published advice on the management of mesenteric fat necrosis. We recommend safe operating techniques to minimize the risk of developing fat necrosis and its potential harmful consequences.     

Lipoma-like tumor mass probably arising in the retroperitoneal heterotopic pancreas: a previously undescribed lesion

Heterotopic pancreatic tissue is found in several locations of the body. However, to the best of our knowledge, there have been no reports on heterotopic pancreas in the retroperitoneum. A case of retroperitoneal lipoma-like large tumor mass probably arising in the heterotopic pancreas is reported. A 45-year-old Japanese woman was admitted to hospital because of back pain. Imaging modalities showed an abnormal mass in the retroperitoneum separate from the surrounding organs, including the pancreas and kidney. Histologically, the mass consisted of mature adipose tissue and scattered ductal and acinar elements. Although there was no islet tissue in this fatty mass, the epithelial elements suggested heterotopic pancreatic tissue in origin. The present case is very unusual; however, heterotopic pancreas should be considered in the differential diagnosis of retroperitoneal tumors.     

A case of intraductal tubulopapillary neoplasm of pancreas with severe calcification,a potential pitfall in diagnostic imaging

We experienced a case of intraductal tubulopapillary neoplasms (ITPN) of the pancreas with severe calcification, which complicated image diagnosis. A pancreas head tumor was detected in a Japanese female in her 50s. Early enhancement by contrast‐enhanced CT and coarse calcification suggested a neuroendocrine tumor, although the obstruction and dilation of the main pancreatic duct appeared to be an intraductal tumor. An endoscopic ultrasound‐guided fine needle aspiration biopsy specimen revealed adenocarcinoma tissue. Pancreaticoduodenectomy was performed, and the patient has been well without evidence of recurrence for over 10 months. Pathological examination on the resected specimen revealed that the tumor showed papillary and tubulo‐cribriform growth patterns. Together with typical immunohistochemical results, the final diagnosis of ITPN was made. Characteristically, this case showed extensive calcification of both psammoma body‐type and non psammoma body‐type with foamy macrophage aggregation. This is the first report of ITPN with two types of calcification and macrophage. Since calcification might be one of the characteristic histological findings in ITPN as shown in our case, the possibility of ITPN should be also considered when calcification is detected in pancreatic lesions by various imaging modalities.     

Benign solitary fibrous tumor of the pancreas: a rare location of extra-pleural fibrous tumor. Single case report and review of the literature

BACKGROUND/AIMS: Extra pleural solitary fibrous are very rare, but occasionally they appear in extraserosal soft tissues or parenchymatous organs, where their diagnosis is often a challenge. In this report we describe the case of a patient with a single primary solitary fibrous tumor of the pancreatic head with a review of the literature. METHODS/RESULTS: A 62 years old woman underwent a Traverso-Longmire procedure in November 2004. Sixteen months after resection there is no evidence of recurrence. The tumour showed immunoreactivity for CD34, CD99, bcl-2, vimentin and smooth muscle actin. MIB-1 proliferating activity was < 5%. CONCLUSIONS: Extra pleural solitary fibrous tumor are often benign lesions. In the pancreas only 2 cases have been described so far. Other mesenchymal tumours that may occur in the pancreas include leiomyosarcoma, tumours of the peripheral nerve sheath, fibrous histiocytic tumours and rare vascular tumours. The differential diagnosis is mainly based on immunohistochemistry. The surgical approach is fundamental for the treatment of solitary fibrous tumour.     

A rare case of nasal Schneiderian (inverted) papilloma associated with basaloid squamous cell carcinoma

Sinonasal inverted papilloma (IP) is a benign, locally aggressive epithelial neoplasm. In less than 9% of cases it is associated with malignancies, typically conventional squamous cell carcinomas (SCC), while other histological variants have been less frequently reported. We describe the third case of basaloid squamous cell carcinoma (BSCC) arising in nasal IP.An 81-year-old female patient presented with a pinkish irregular lesion on the nasal septum. Biopsy was consistent with IP and carcinoma in situ. Two surgical procedures were needed to obtain radical excision. Histology on the surgical specimen revealed BSCC. Seven months after surgery, there was no evidence of disease recurrence.Although IP is more frequently associated with conventional SCC, other malignancies should be considered. The histological differential diagnosis should be supported by immunohistochemistry. The generally-recommended treatment for sinonasal BSCC is complete surgical resection, although this may be a problem in multifocal distributions, as in the present case.     

成人肾盂输尿管连接部梗阻的手术治疗

目的 总结成人肾盂输尿管连接部梗阻的诊治经验。方法 对我院1990年7月至2003年6月收治的56例成人肾盂输尿管连接部梗阻患者的病因、诊断及治疗结果进行回顾性分析。结果 肾盂输尿管连接部狭窄引起梗阻者46例(82．1％)，其中3例为双侧；粘连带压迫者3例(5．3％)，输尿管肾盂高位连接者4例次(7．3％)，异位血管压迫者3例次(5．3％)。共行手术59例次，Anderson—Hynes离断式肾盂成形术47例次，肾盂Y—V成形术4例，纤维条索松解3例，异位血管切断3例，肾切除术2例。本组病例除2例肾切除者外，余54例均于术后3个月至1年进行复查随访，50例痊愈。总治愈率92．8％。4例(7．2％)发生再狭窄，二次行肾盂成形术后均痊愈。结论 IVU和RU是诊断成人肾盂输尿管连接部梗阻的主要方法，Anderson-Hynes离断式肾盂成形术是治疗成人肾盂输尿管连接部梗阻的首选术式。