Mesenteric cystic lymphangioma: a congenital and an acquired anomaly? Two cases and a review of the literature

Mesenteric cystic lymphangioma is an uncommon benign abdominal mass. Two cases of mesenteric cystic lymphangioma are presented, both in combination with malrotation and intermittent volvulus. Both mesenteric cystic lymphangiomas were located near the duodenojejunal junction, the usual area of torsion in case of a volvulus. These findings suggest that mesenteric cystic lymphangioma could have evolved as a consequence of chronic intermittent volvulus. We hypothesize that in patients with malrotation and volvulus, mesenteric cystic lymphangioma may be regarded as an acquired anomaly.     

What lessons can be learned from testicular histology in undescended testes?

Over the last 40-50 years studies involving thousands of testicular biopsies in boys with cryptorchidism have contributed to our knowledge of testicular histopathology and our understanding of the effects of cryptorchidism on the normal development of the germinal epithelium. Growth and maturation of germ cells and Leydig cells are crucial to allow boys to reach normal fertility potential. The following aberrations in testicular development are seen in cryptorchid testes: a decrease in total germ cell numbers, failure of fetal gonocytes (stem cells) to transform into adult dark (Ad) spermatogonia, failure for Ad spermatogonia to mature into primary spermatocytes, Leydig cell hypoplasia, and testicular fibrosis. All of these findings have been found to have a strong negative correlation with a boy’s age at the time of orchidopexy. Some of these findings have prognostic significance in regards to fertility potential especially when coupled with key clinical findings such as hormonal findings, age at orchidopexy, testicle size, laterality and location of cryptorchid testes. This review focuses on key lessons learned from testicular histology in cryptorchid testes.     

Fibroblastic polyp of the colon and colonic perineurioma: 2 names for a single entity?

Fibroblastic polyps of the colon and intestinal perineuriomas are unusual mucosal lesions with identical clinical and histologic features, and apparent different immunohistochemical and ultrastructural characteristics. However, immunohistochemical distinction was solely based on the results obtained with epithelial membrane antigen (EMA), an antibody whose reactivity on perineuriomas is difficult to demonstrate. Likewise, accurate ultrastructural diagnosis may be flawed by sampling error, preservation artifacts, or paucity of specific diagnostic features. In a recent short communication, it was suggested that both lesions may represent the same entity. To further evaluate this hypothesis, 28 colorectal polyps with clinical and histologic features of colonic fibroblastic polyps/perineuriomas (including 10 cases previously reported as fibroblastic polyps) were stained immunohistochemically for 4 markers of perineurial differentiation, that is, claudin-1, GLUT-1, collagen type IV, and EMA (the latter performed using an extended protocol for antigen retrieval and a kit for signal amplification). In addition, electron microscopy was performed in 4 cases. EMA and claudin-1 stained 26 of 28 (93%) polyps whereas GLUT-1 and collagen type IV were expressed in all of them. EMA reactivity was mostly focal and weak whereas the other markers displayed a diffuse and strong signal. Ultrastructural examination revealed elongated cells with features of perineurial differentiation including long, slender cytoplasmic processes with pinocytotic vesicles and an external lamina. Our findings support the hypothesis that fibroblastic polyps and perineuriomas of the colon represent the same entity. We suggest reclassifying fibroblastic polyps reactive to perineurial markers as perineuriomas. To reach an accurate diagnosis, we recommend employing at least 2 markers of perineurial differentiation, and performing EMA immunostaining with high antibody concentration, prolonged incubation time, and/or extended protocol for antigen retrieval.     

Pure alcohol injection of a congenital splenic cyst: a valid alternative?

Congenital splenic cysts are a rare entity. They are more frequent in children and young adults. They are true cysts, lined by epithelium with a typical trabeculation. Aspiration and injection with antibiotics (minocycline-tetracycline) or pure alcohol has been reported sparsely with variable results. The purpose of this case study is to report our experience with ultrasound (US)-guided aspiration and injection of a congenital splenic cyst, which, in our case, did not prove a viable alternative to surgical defenestration.     

Esophageal adenocarcinoma: a long-term complication of congenital diaphragmatic hernia?

A 22-year-old man presented with a lower esophageal adenocarcinoma having been treated for a left-sided congenital diaphragmatic hernia (CDH) as a neonate. There is no known association or similar case reported in the literature. We review the known long-term sequelae of CDH, possible theories for the occurrence of adenocarcinoma, and its implications for follow-up of patients with CDH.     

Puberty stage and spontaneous descent of acquired undescended testis: implications for therapy?

We assessed spontaneous descent of acquired undescended testis (UDT) at puberty. 299 Boys (aged 1.2-16.5 years, mean 9.4) with 350 acquired-UDT were examined annually during a 12.6-year period (mean 3.1). An acquired-UDT was defined as a previously intrascrotal testis which can no longer be manipulated into a stable scrotal position. Each year, position of the testis and pubertal development according to Tanner's stages were assessed. Early puberty was defined as puberty stage G2 (testicular volume 4-9 mL), mid-puberty as puberty stages G3 (testicular volume 10 mL) and G4 (testicular volume 11-15 mL), and late puberty as puberty stage G5 (testicular volume >15 mL). Follow-up was completed if spontaneous descent had occurred, if mid-pubertal orchidopexy (ORP) had to be performed, if the boy was lost for follow-up, or if pre-pubertal ORP was performed in another hospital. In 139 boys with 164 acquired-UDT follow-up was meanwhile completed. Twelve boys with 14 UDT were lost for follow-up. In an additional 16 boys with 21 UDT, ORP was performed in another hospital. In 98 of the remaining 129 (76.0%) acquired-UDT spontaneous descent at puberty occurred. Mean follow-up was 2.5 years (range 0.2-8.5). In 70 of 98 testes (71.4%) descent occurred in early puberty, in 26 of 98 testes (26.5%) in mid-puberty, and in two testes in late puberty. In 31 of 129 testes (24.0%) ORP had to be performed at mid (30 cases) or late (one case) puberty. In this series, 98 of 129 acquired-UDT (76.0%) descended spontaneously at puberty, whereas in 31 of 129 (24.0%) pubertal ORP was performed. If ORP is postponed until puberty stage G3 (testicular volume of 10 mL) three of four acquired-UDT will descend spontaneously.     

Development of pseudarthrosis in a dysplastic clavicle: A variant of congenital pseudarthrosis?

The development of a pseudarthrosis is reported in a child's right clavicle, which was previously noted to have been morphologically abnormal. Such pseudarthroses are uncommon, but they usually appear on the right side and may rarely be bilateral. The differential diagnosis is of a postpartum fracture, a posttraumatic pseudarthrosis, cleidocranial dysostosis, or neurofibromatosis. A review of the development of the clavicle and the hypotheses for such anomalous development are given.     

Adenocarcinoma and gastro-intestinal stromal tumor: fortuitous association or a single carcinogenic agent? A report of 2 cases

The synchronous development of epithelial and stromal tumors of the digestive tract was rarely reported in the literature. Two such cases are described in this article. One is of a 44-year-old man presenting a high grade gastric stromal tumor associated with an adenocarcinoma of the large bowel. The other is of a 68-year-old man presenting an adenocarcinoma of the head of the pancreas associated with a low grade gastric stromal tumor. The hypothesis that the association is due to a simple coincidence particularly in areas with high rates of digestive cancer is proposed. A possibility of a genetic mutation or a single carcinogenic agent interacting with two tissues in the digestive tract and inducing the development of tumors of different histotype cannot be discarded.     

Congenital absence of cartilaginous tracheal rings associated with esophageal atresia and trifurcated carina: a novel anomaly?

Tracheomalacia associated with esophageal atresia (EA) is a well-known condition. However, complete absence of tracheal rings (TRs) is extremely rare. Our aim is to describe a novel triad of conditions and to discuss the best treatment. An expremature male operated for EA presented with severe respiratory distress. The diagnosis of absent cartilage rings, suspected on bronchoscopy, was confirmed by optical coherence tomography. The absence of TRs was localized to a short tracheal segment, and the carina trifurcated into right upper lobe, right intermediate, and left main bronchus. The patient was treated with resection and anastomosis with a completely satisfactory course. Absence of TRs was previously reported by us in 2 other cases, both with associated EA and trifurcation of the carina. One child was treated with tracheostomy and the other with a stent, but the outcome was far from optimal. The patient with tracheostomy eventually underwent resection and anastomosis with tracheostomy closure. Congenital absence of TRs is extremely rare. Although localized, it is responsible for severe symptoms owing to complete tracheal collapse and may be misdiagnosed as tracheomalacia. In our experience, it has been associated with EA and trifurcated carina. Our limited experience suggests resection of the abnormal segment and tracheal anastomosis as the best treatment.     

Cervical and upper-third thoracic oesophageal carcinoma: a single pathological entity?

AIM OF THE STUDY: Cervical and upper-third thoracic oesophageal carcinomas are considered as a single pathological entity. The aim of this study was to compare postoperative and oncological results after surgical resection in these two locations. MATERIAL AND METHODS: Postoperative and oncological results were compared retrospectively in 155 patients who underwent surgery for carcinoma of the cervical (C group, n = 21) or upper-third thoracic (TS group, n = 134) oesophagus. RESULTS: The two groups were comparable regarding the pre-, peroperative and histological data. Postoperative mortality and morbidity rates in the C and TS groups were 4.8% and 10.4% (P= 0.413) and 57.1 and 50.7% (P= 0.585), respectively. R0 resection and recurrence rates were 61.9% and 73.1% (P= 0.289) and 50.0% and 51.1% (P= 0.941), respectively. Five-year survival rates were 0% and 35% in the overall population (P= 0.098) and 0 and 49% in the R0 population (P= 0.047), respectively. By multivariate analysis, cervical location of the tumour was found to be an independent factor of poor prognosis (relative risk= 3.1, 95% confidence interval= 1.3-7.8, P= 0.014). CONCLUSION: Prognosis after surgical resection of cervical oesophagus carcinoma is very poor. Surgery in this location should be proposed in case of chemoradiation failure.     

Ectopic Pheochromocytoma: Does the Rule of Tens Apply?

Introduction The rule of “tens” is often stated to reflect the distribution and histology of pheochromocytomas, with 10% being bilateral, 10%; ectopic in origin, and 10%; malignant. The objective of this study was to review the ectopic pheochromocytomas in a tertiary endocrine center and to establish whether the rule of tens holds true. Methods Retrospective data were collected on all adrenalectomies and ectopic pheochromocytoma resections performed between 1993 and 2005 at our institution. Results In total, 77 patients had pheochromocytomas: 75%; (58/77) adrenal and 25%; (19/77) ectopic. Of the adrenal pheochromocytomas, 10%; (6/58) were bilateral. The anatomic locations of the ectopic pheochromocytomas were as follows: 26%; (5/19) adjacent to the adrenals, 53%; (10/19) in the organ of Zuckerkandl, 11%; (2/19) in the bladder, 5%; (1/19) in the mediastinum, and 5%;(1/19) in the neck. Conclusions Our series demonstrates an incidence of 10%; for bilateral pheochromocytoma, which is similar to that in the published reports. However, 25%; of the pheochromocytomas were ectopic. Zuckerkandl pheochromocytomas were the most common among the ectopic lesions. Rarely, these tumors present outside the abdominal cavity An erratum to this article is available at .