Appendiceal Goblet Cell Carcinoma: Role of Cytoreductive Surgery (CRS) and Hyperthermic Intraperitoneal Chemotherapy (HIPEC).

Goblet cell carcinoma (GCC) encompasses a separate entity in appendiceal neoplasms with mixed glandular and neuro-endocrine pathological features. GCC mostly presents as an acute appendicitis duo to luminal obstruction or as an incidental finding on the surgical appendectomy specimen. In case of tumour perforation or presence of other risk factors, guidelines suggest additional treatment with a completing right hemicolectomy or cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC). We report the case of a 77-year-old male with symptoms of appendicitis for which an appendectomy was performed. The appendix was ruptured during the procedure. There was an incidental finding of GCC on the pathological specimen. Because of possible tumour soiling, the patient received a prophylactic CRS-HIPEC. A literature review was performed to investigate the potential role for CRS-HIPEC as a curative treatment in patients with GCC. GCC of the appendix is an aggressive type of tumour with a high risk of peritoneal and systemic dissemination. CRS and HIPEC is a treatment option: both in a prophylactic setting and in patients with established peritoneal metastases.

Hepatic rhabdomyosarcoma in an adult: a rare primary malignant liver tumor. Case report and literature review.

Rhabdomyosarcomas are malignant tumors that display features of striated muscle differentiation. They are the most common soft-tissue sarcomas among children and young adults. In mature adults however there are very rare. The liver as a primary site in adults has only been described in 12 cases. We report a case of a primary alveolar rhabdomyosarcoma of the liver in a 59 year old female, confirmed by histological examination using immunohistochemical analysis (positive actin, desmin, vimentin and myogenin staining) and fluorescent in situ hybridization (FISH) analysis (positivity for PAX3/FOXO1A fusion). The patient underwent primary surgical resection, but presented a few weeks after surgery already with recurrent disease in the abdomen and bone metastasis. Despite initial good response to chemotherapy (doxorubicin/ifosfamide) and stable disease at 12 months after diagnosis, the patient died 31 months after the first presentation secondary to complicated abundant abdominal recurrent disease. We further present a review of the literature on published similar cases since 1979.

Interlobar venous flow is different between left and right kidney in uncomplicated third trimester pregnancy.

AIM: To investigate Doppler flow patterns of renal interlobar veins (RIV) in left (L) and right (R) kidney in relation to gestational age and to intrarenal pyelocalyceal diameters (PCD). METHODS: Cross-sectional study involving 6 groups of women: (1) non-pregnant women, women pregnant between weeks (2) 11-14, (3) 18-23, (4) 28-33, (5) 37-40 and (6) women <5 days post delivery. During standardized renal duplex ultrasound scan, PCD (mm) was measured and venous impedance index (VI = [peak flow (PV) - presystolic flow]/PV) of RIV was defined. Comparison between L and R kidney was performed by repeated-measures analysis of variance for VI and by t tests for PCD. RESULTS: A total of 150 women were examined, 25 in each group. In the early third trimester of pregnancy, L VI was significantly higher than R VI (0.38 +/- 0.01 vs. 0.33 +/- 0.01, p < 0.0001) and L PCD was significantly lower than R PCD (3.90 +/- 3.68 vs. 6.25 +/- 4.03, p = 0.03). CONCLUSION: RIV Doppler flow is different between left and right kidney only in the third trimester of pregnancy. This difference is inversely related to PCD, which may be explained by pelvic compression from the gravid uterus.

Oro-facio-digital syndrome type 1: imaging findings in an 11-year-old girl.

We describe a uniquely illustrated case of orofaciodigital syndrome type 1. We document many of the possible features of this disorder in a young girl using multiple imaging modalities. Furthermore, we prove that the cysts in kidney and liver are not in contact with the excretory system using MRI, something that to our knowledge has not been previously reported.

Fat containing adrenal cavernous haemangioma: CT and MRI findings.

We report a case of fat containing adrenal cavernous haemangioma. Abdominal computed tomography images showed a large adrenal mass with calcifications and small well demarcated islets of intralesional fat. Magnetic resonance images confirmed the presence of fat and furthermore demonstrated the peripheral spotty contrast enhancement with centripetal enhancement, assuming the possibility of cavernous haemangioma.The histological specimen confirmed cavernous haemangioma of the adrenal gland.

Esophageal intramural pseudodiverticulosis complicated by pneumomediastinum.

We report a rare case of esophageal intramural pseudodiverticulosis with perforation of the esophagus. A 32-year-old male presented with acute thoracal pain after a period of vomiting. Computed tomography revealed an important amount of mediastinal free air and small outpouchings in the wall of the esophagus. During the following thoracic surgery procedure no macroscopic site of rupture could be identified. Pseudodiverticulosis was detected during a barium swallow exam of the esophagus 4 weeks later.

Hemangiopericytoma of the ovary.

We report on a case of hemangiopericytoma in the pelvis occurring in the left ovary. The tumor was found in a 33-year-old woman with left iliac fossa pain. The lesion was well demarcated and had a diameter of approximately 10 cm. It was surgically resected. Computed tomography and ultrasound revealed an aspecific lesion with round lesion with a smooth contour and intratumoral necrosis. To our knowledge, radiological features of hemangiopericytoma of the ovary have not been reported yet.

Ureteral ectopy in the seminal vesicle associated with cyst formation and renal dysplasia: contribution of 3D-MRI.

A case of a congenital anomaly of the urinary tract in a 25-year-old Caucasian male is presented. Preoperative and pathologic findings demonstrated an ectopic ureter which terminated in a seminal vesicle cyst. Both structures formed an abortive common duct at the bladder base. The ipsilateral kidney was dysplastic. Findings on voiding cytourethrography, spiral CT, and MRI are discussed and correlated with pathologic findings. The authors suggest that MRI, especially with the use of 3D sequences, is the examination of choice in the evaluation of a complex congenital urogenital anomaly as presented.

Cystic insulinoma of the pancreas in a patient with myotonic dystrophy: correlation of imaging and pathologic findings.

We present a case of cystic islet cell tumor of the pancreas in a 60-year old male with myotonic dystrophy. A cystic lesion was found with a thick peripheral rim, a few intralesional septae and a fluid-debris level on CT and MRI. Image characteristics are compared with the pathological findings. MRI demonstrated the internal architecture of the lesion more clearly.

Midgut volvulus in an adult patient.

The authors report on a case of midgut volvulus in a 27-year-old man who presented with bilious vomiting and acute abdominal pain. US demonstrated a reversal of the normal relationship between the superior mesenteric artery (SMA) and superior mesenteric vein (SMV). A clockwise whirlpool sign, diagnostic for midgut volvulus, was not visualised. In a further assessment, upper gastrointestinal series demonstrated obstruction in the second part of the duodenum highly suspicious of Ladd's bands. Malpositioning of bowel structures, as already suggested by the reversal of the SMA and SMV on ultrasound, and a distinctive whirl pattern due to the bowel wrapping around the SMA was demonstrated on CT. Furthermore angiography revealed focal twisting of the SMA. US is the first imaging modality to perform in suspicion of midgut volvulus. When inconclusive, CT is in our opinion the next stage in the diagnostic work-up.

Proximal pulmonary artery aneurysm.

The authors report on a case of proximal pulmonary artery aneurysm detected on a routine chest X-ray. The scintigraphic and angiographic features of the condition are described and the importance of including the entity in the differential diagnosis of lung masses is stressed.

Undescended ovary and unicornuate uterus: simplified diagnosis by the use of clomiphene citrate ovarian stimulation and magnetic resonance imaging (MRI).

BACKGROUND: Although the association between unicornuate uterus and undescended ovaries is well established, little information is available on this entity, suggesting the possibility that many cases are not recognized at all. Consequently, important clinical information is missed in many patients worldwide. METHODS: During a period of 5 years, eight cases of unicornuate uterus were observed in our infertility clinic. The first three patients received magnetic resonance imaging (MRI) after mild clomiphene citrate (CC) stimulation due to a discrepancy between estradiol levels and follicular growth and because a suspected ectopic ovary could not be visualized on ultrasound. Based on this experience, five consecutive patients were offered MRI after CC stimulation as part of this study. RESULTS: In five out of eight patients with unicornuate uterus (62.5%) an undescended ovary could be visualized in the upper abdomen. Abdominal ultrasound revealed the ectopic ovary in only two cases. The use of CC stimulation undoubtedly facilitated the diagnosis of the undescended ovary on MRI. CONCLUSION: It is suggested that MRI after CC stimulation is an excellent non-invasive method to diagnose undescended ovaries in women with a unicornate uterus.