Hemangioma of the rectum - How misleading can hematochezia be?

We present the case of an 18-year-old male patient that was referred to our gastrenterology department with history of intermittent painless hematochezia since childhood. During such instances, he was diagnosed with bowel intussusception, eosinophilic gastroenteritis and inflammatory bowel disease at 4, 6 and 8 years old, respectively. He underwent treatment with 5-aminosalicylic acid for two years, without improvement of symptoms. He was then lost to follow-up until our observation. His physical examination was unremarkable except for digital rectal examination which found a nodular compressible mass by the palpating finger. Blood tests revealed a mild iron deficiency anemia. The colonoscopy showed an extended reddish and bluish multinodular submucosal mass in the rectum, suggesting diffuse cavernous hemangioma of the rectum (DHCR). The Magnetic Resonance Imaging, showed diffuse thickening of the entire rectum extending into the distal sigmoid with the mesorectum revealing multiple serpiginous structures, corresponding to abnormal blood vessels. After discussion, we considered to perform a sphincter-sparing procedure, namely pull through transection and coloanal anastomosis. However, intervention was ruled out by the patient because of his fear of anal incontinence and permanent colostomy. We adopted a conservative strategy with clinical surveillance and iron supplementation. At the present, the patient remains with intermittent rectal bleeding, referring poor quality of life due to his ongoing symptoms. This is a rare case of DHCR. Despite of being a benign disease, the management of DHCR requires a sphincter mucosectomy and pull-through coloanal sleeve anastomosis which has become the first-line procedure. The surgical outcomes are non-expectable in 32% with permanent sphincter lesion or with incomplete DHCR removal. As in this case, the surgeons or patients refusal to perform the intervention is common which represents a challenge to the clinical follow-up.

En face mode of swept-source optical coherence tomography in circumscribed choroidal haemangioma.

PURPOSE: To describe the findings in circumscribed choroidal haemangioma (CCH) using en face swept-source optical coherence tomography (SS-OCT). METHODS: En face images were obtained employing DRI-1 Atlantis OCT (Topcon, Tokyo, Japan), using a three-dimensional volumetric scan of 12×9 mm. Images were obtained from the retinal pigment epithelium to 1000 µm in depth of the tumour. RESULTS: Twenty-two eyes from 22 patients with the clinical diagnosis of CCH were included. In 20 eyes (90.9%), a characteristic pattern was visualised in the en face image across the vascular tumour. A multilobular pattern, similar to a honeycomb, with hyporeflective, confluent, oval or round areas corresponding with the lumen of the tumour vascular spaces, and hyper-reflective zones, which may represent the vessels walls and connective tissue of the tumour. Ten eyes (45.4%) showed a hyper-reflective halo surrounding the tumour. Seventeen tumours (77.2%) showed small diameter vessels at the inner zone and larger vessels in the outer area. Twelve patients (54.5%) had previously received treatment (photodynamic therapy, transpupillary thermotherapy, dexamethasone intravitreal implant or brachytherapy with ruthenium-106). No differences were found between treated and untreated patients in any of the measured parameters. CONCLUSIONS: En face SS-OCT is a rapid, non-invasive, high-resolution, technology, which allows a complementary study to cross-sectional scans in CCH. A characteristic multilobular pattern, with a hyper-reflective halo surrounding the tumour, was found in en face SS-OCT images. No morphological differences were found between naïve patients and patients who received previous treatment.

Primary hepatic angiosarcoma: multi-institutional comprehensive cancer centre review of multiphasic CT and MR imaging in 35 patients.

OBJECTIVE: To assess the imaging features of primary hepatic angiosarcoma on multiphasic CT and MR. METHODS: Multi-institutional review identified 35 adults (mean age, 57.1 years; 22M/13F) with pathologically proven hepatic angiosarcoma and pretreatment multiphasic CT (n = 33) and/or MR (n = 7). RESULTS: Multifocal hepatic involvement was seen in all 35 cases, with at least 10 lesions in 74.3% (26/35). Mean size of the dominant mass was 8.9 ± 4.7 cm (range, 2.6-20 cm). Individual nodules were typically circumscribed. Arterial-phase foci of hypervascular enhancement without washout were seen in 89.7% (26/29). Heterogeneously expanding foci of enhancement generally followed blood pool in 88.6% (31/35). Progressive centripetal (n = 16) or diffuse "flash-fill" (n = 4) enhancement pattern resembling cavernous haemangiomas predominated in 20 cases, whereas a "reverse haemangioma" centrifugal pattern predominated in 11 cases. Rapid interval growth was seen in 24 (96.0%) of 25 cases with serial imaging. Vascular invasion was not seen in any case. Underlying cirrhotic morphology was seen in 42.3% (15/35). CONCLUSION: Primary hepatic angiosarcomas typically manifest as aggressive multifocal tumors containing small heterogeneous hypervascular foci that progressively expand and follow blood pool. The appearance can mimic cavernous haemangiomas, but distinction is generally possible. In the setting of cirrhosis, lack of tumour washout and vascular invasion argue against multifocal hepatocellular carcinoma. KEY POINTS: • Hepatic angiosarcoma manifests on CT and MR as rapidly progressive multifocal tumours • Multiphasic imaging demonstrates hypervascular foci that progressively expand and follow blood pool • Enhancement pattern can resemble cavernous haemangiomas or show a "reverse" centrifugal pattern • Lack of tumour washout of hypervascular lesions argues against multifocal hepatocellular carcinoma • Careful assessment of the cross-sectional imaging findings may suggest the diagnosis.

Exophytic hypothalamic cavernous malformation mimicking an extra-axial suprasellar mass.

We report the case of a 31-year-old male who presented to the ER with a 1-week history of progressively worsening, throbbing, left retro-orbital headache, ptosis, and subjective worsening of short-term memory function. Initial review of systems and laboratory data were noncontributory. Non-contrasted CT demonstrated a large hyperdense mass centered in the suprasellar cistern without evidence of dissecting extra-axial hemorrhage. Though the initial appearance mimicked a basilar tip aneurysm or another primary extra-axial suprasellar pathology such as a hemorrhagic or proteinaceous craniopharyngioma, germinoma, or optic glioma, a second smaller, clearly intra-axial, hyperdense lesion was observed in the left periventricular forceps major white matter. Consideration for multiple cavernomas versus hypervascular metastatic disease such as renal malignancy, thyroid malignancy, or melanoma was raised. CTA confirmed normal intracranial vasculature. Subsequent MRI images showed an acutely hemorrhagic mass centered at the left paramedian hypothalamus and tuber cinereum with numerous secondary foci, demonstrating mature hemorrhagic elements and confirming the diagnosis of multiple cavernomas.

Vertebral and spinal cavernous angiomas associated with familial cerebral cavernous malformation.

BACKGROUND: Cerebral cavernous malformations are vascular malformations that affect the CNS and have been associated with cutaneous, retinal, and hepatic lesions. Until now, vertebral hemangiomas associated with CCM have been described only in one case. The coexistence of intracranial and spinal cavernous angiomas in familial CCM is extremely rare. In addition to previous studies, the occurrence of spinal, vertebral, and cutaneous cavernous angiomas is now described in different members of a large family with CCM. CASE DESCRIPTION: Our study reports a previously described family (IFCAS-07) with 12 members affected by autosomal dominant cavernous angiomas: 11 had CCM either alone or associated with hepatic or retinal angiomas, and one had only hepatic angioma. In all 11 members affected by CCM, the mutation of CCM1 gene was detected. During the follow-up, 8 subjects underwent a spinal MRI: 2 because they were symptomatic (thoracic paresthesias, enuresis, back pain) and 6 as a screening examination. Spinal MRI showed in 5 subjects spinal cavernous angiomas either alone or associated with vertebral hemangiomas. CONCLUSIONS: To our knowledge, this is the largest family reported with different subjects affected by CCM associated with multiple cavernous angiomas throughout (brain and spinal cord) and besides (retina, skin, liver, and vertebral column) the CNS. Comprehensive care of patients with familial CCM includes screening of all the tissues that can be affected and appropriate management by specialists. We emphasize the importance of spinal MRI in the diagnosis of spinal and vertebral cavernous angiomas in all patients affected by familial CCM.

Imaging of a small bowel cavernous hemangioma: report of a case with emphasis on the use of computed tomography and enteroclysis.

Hemangiomas of the small bowel are rare benign tumors, that are dangerous since they may cause massive or occult gastrointestinal bleeding. We describe a case of a jejunum cavernous hemangioma detected by computed tomography (CT) and barium studies. An abdominal CT scan (with intravenous contrast agent) depicted a pronounced contrast enhanced lesion arising from the front wall of a loop of the proximal ileum. Enteroclysis revealed a small intramural nodular defect.

Palinacousis - auditory perseveration: two cases and a review of the literature.

Palinacousis is an auditory illusion rarely reported in cases of temporal lobe dysfunction. Detailed observations where made by Jacobs et al. in 1973. Since that time, only a few other cases have been described in the literature. After reviewing the literature and comparing our clinical experience, we believe that palinacousis can occur as an aura, a simple partial seizure, a complex partial seizure, and/or a postictal event. Within one academic year, we observed two patients who experienced palinacousis. Palinacousis maybe more common than recognized in patients with receptive aphasias or diffuse cerebral dysfunction, whose language deficits preclude adequate description. It is important to differentiate palinacousis from auditory hallucinations seen in psychotic and psychiatric patients. Identification of palinacousis as an aura, simple partial seizure, complex partial seizure, and/or postictal phenomenon can help localize potential lesions and improve patient care.

Histopathological findings in a surgically resected thalamic cavernous hemangioma 1 year after 40-Gy irradiation.

Stereotactic radiosurgery is a controversial treatment modality in the management of cerebral cavernous hemangiomas (CHs), and results vary from center to center. Even the interpretation of treatment failure is controversial. It is suggested that the systematic pathological investigation of irradiated specimens could help to resolve the controversy. A hemorrhagic lesion in the posterior part of the thalamus had been diagnosed as a tumor and was treated with 40-Gy fractionated radiotherapy. One year after this treatment the case was reconsidered based on new imaging evidence, and the lesion was removed by conventional craniotomy. Histopathological examination revealed a CH with postirradiation changes. Compared with nonirradiated control CH tissue samples, there was endothelial cell destruction and marked fibrosis with scar tissue formation in the stroma of the treated lesion. The histopathological findings in this specimen were similar to those described in arteriovenous malformations after gamma knife surgery. The results of light microscopic investigations suggest that the ionizing effect of radiation energy evokes vascular and connective tissue stroma changes in CHs as well.

Diffuse cavernous hemangioma of the rectum: an atypical cause of rectal bleeding.

OBJECTIVE: cavernous hemangioma of the rectosigmoid colon is a rare disease, with no more than 200 cases reported in the literature. The rectosigmoid is the most common site of this disease in the gastrointestinal tract. CASE REPORT: we report the case of a 31-year-old male with recurrent episodes of rectal bleeding, who was finally diagnosed of diffuse cavernous hemangioma of the rectum. The tumor, of 12 x 10 x 9 cm in size, occupied the rectum to the margin of the anal sphincter. A surgical procedure was ruled out because of the inability to carry out a safe anastomosis while preserving anal sphincters. DISCUSSION: rectal hemangiomas are less frequent vascular malformations. The clinical presentation of a cavernous hemangioma of the rectum is usually acute, recurrent or chronic rectal bleeding. Other symptoms stem from the possible compression or invasion of adjacent structures, such as lumbar or perianal pain, metrorrhage, hematuria, etc. This diagnosis is commonly made in younger patients. Colonoscopy is without doubt the diagnostic technique of choice, and it allows to establish the localization, morphology, and total extension of the lesion; its characteristic image is a red-purplish nodule with great vascular congestion. According to the opinion of most authors, biopsy is not advisable during colonoscopy, since imaging techniques are sufficient for an accurate diagnosis, and the risk of bleeding while manipulating this lesion is not negligible. Computed tomography and particularly magnetic resonance imaging, given their high precision to delimit the lesion and its relations to adjacent structures, are imaging studies that are mandatory before surgical treatment. Other techniques such as selective angiography, barium enema, gastrointestinal transit, and upper-tract endoscopy may be supplementary and help locate more lesions along the gastrointestinal tract. Failure to recognize the exact diagnosis and extent of diffuse cavernous hemangioma may lead to failed surgical treatment and severe complications. Complete surgical excision of the lesion with a sphincter-saving procedure is the primary mode of treatment: conservative proctectomy with coloanal anastomosis.

Cavernous haemangioma of the rectum: an uncommon cause of rectal bleeding.

Cavernous haemangioma of the rectum is an uncommon cause of rectal bleeding. Initial diagnosis is often elusive because of lack of awareness. For accurate diagnosis, investigations such as endoscopy, plain X-ray of the abdomen, barium enema and selective angiography of the inferior mesenteric artery are required. Complete surgical excision of the haemangioma and colo-anal sleeve anastomosis is the most favoured operative procedure to eradicate the disease. We report a case of cavernous haemangioma of the rectum and discuss its salient clinical features, investigations and management.

Cavernous hemangioma of the foot and antecubital fossa: an alternative therapeutic option.

We present two cases of recurrent cavernous hemangiomas of the extremities. These conditions were initially treated unsuccessfully with surgical resection. After recurrence, the hemangiomas were treated by percutaneous transcatheter embolization. To the best of our knowledge, this is the first case report of treatment of cavernous hemangioma involving the extremities using an angiographically directed vascular embolization technique.

[Recurrent bleeding of thalamic cavernous angioma under hormonal treatment. A case report]. / Saignements itératifs d'un angiome caverneux sous traitement hormonal.

A case of recurrent bleeding from a probable left thalamic cavernoma in a 26 year old woman taking hormonal treatment is reported. Four episodes of bleeding were clinically and radiologically documented, prior to her referral to our institution. Interestingly, each episode occurred three weeks after starting hormonal treatment, dydrogesterone, desogestrel ethinylestradiol, chlormadin, nomegestrel acetate). The patient was not operated because of the deep situation of the cavernoma which was remote from the thalamic surface within the third ventricle. There was no recurrent bleeding after the onset hormonal treatment was discontinued. Although no similar case has been found in the literature, we believe that this case gives further argumentation in favor of a role of hormonal factors influencing the biological behavior of cavernous angiomas which has been previously suggested in pregnant females with bleeding cavernous angiomas.

Diffuse cavernous hemangioma of the rectosigmoid colon: imaging findings.

Diffuse cavernous hemangioma of the rectosigmoid colon (DCHR) is an uncommon disease that affects mainly young adults. As the main symptom is non-specific chronic rectal bleeding, the clinical diagnosis is difficult and often delayed. In this essay, we illustrate the imaging features of DCHR, including plain radiograph, barium enema, CT and MR study, and echoendoscopy. Pitfalls in diagnosis and the role of imaging study in the diagnosis, in the preoperative staging, and in the follow-up of the patients are defined.

Cavernous hemangiomas of the rectum: report of three cases.

Cavernous hemangioma of the rectum are uncommon benign vascular lesions. Misdiagnosis often occurs because of lack of awareness of classic clinical features. Endoscopy, plain x-ray abdomen, barium enema, and selective angiography are useful means of investigation for accurate diagnosis. Three cases of rectal haemangioma are presented that were earlier misdiagnosed as ulcerative colitis.

Mixed cavernous angioma and glioma (angioglioma) in the hypothalamus--case report.

A 54-year-old female presented with a huge mixed cavernous angioma and astrocytoma in the hypothalamus manifesting as headache, visual field defect, gait disturbance, and convulsion. Radiological studies revealed a huge suprasellar tumor encasing all the major cerebral vessels. Craniotomy disclosed a hemorrhagic tumor poorly demarcated from the surrounding brain which was partially removed. Histological examination of the operative specimen revealed cavernous angioma with low grade glioma in the periphery. The residual tumor responded to radiation therapy remarkably well. An autopsy conducted 3 years later revealed a small hypothalamic astrocytoma with abundant vasculature.

MR findings of hepatic cavernous hemangioma after intraarterial infusion of iodized oil.

Magnetic resonance (MR) imaging was performed on five tumors of three patients who had hepatic hemangiomas. Four tumors were given an intraarterial infusion of 3-8 ml of iodized oil, while one tumor was not. MR images were obtained at 2.0 or 0.5 T. A single spin echo sequence with TE of 30 ms and TR of 500 ms and a double echo sequence with TEs of 60 and 150 ms and TR of 2000 ms, were used to produce relatively T1-, T2-weighted, and heavily T2-weighted images, respectively. Follow-up MR imaging was done 1-5 months after infusion of iodized oil. On relatively T1-weighted images, hemangiomas showed iso- or hypointensity. On T2-weighted images, all tumors showed hyperintensity. However, on heavily T2-weighted images, tumors with iodized oil showed heterogeneous, slight hyperintensity, while tumors without iodized oil showed characteristic appearance of marked hyperintensity in hemangiomas. In hepatic cavernous hemangiomas with intraarterial infusion of iodized oil, familiarity with this unusual MR intensity of tumors on heavily T2-weighted images is useful to avoid the incorrect diagnosis and to reduce the frequency of inappropriate hepatic resection.

[Interventional therapy of hepatic cavernous hemangioma].

From March. 1991 through Dec. 1993, 39 patients with hepatic cavernous hemangioma were treated by interventional therapy. After 1-3 periods of therapy 28 were cured, in 8 case the mass reduced by 50%, and in the remaining 3 cases the tumor reduced by 30%. Symptoms disappeared in all patients. The diagnosis of hepatic cavernous hemangioma, its differential diagnosis from malignant tumors, indications for interventional therapy, the use of embolizing materials were discussed.

A case of polypoid cavernous hemangioma of the sigmoid colon excised by colonoscopic polypectomy.

A 28-year-old Japanese man was admitted to our institute because of sharp anal pain and a mass that prolapsed from the anus after defecation. After spinal anesthesia for emergency operation, the mass spontaneously withdrew into the anus and the pain disappeared. Surgery was postponed. Barium enema and colonoscopy revealed a pedunculated polyp in the sigmoid colon, which was removed by snare polypectomy with electrocautery. The resected polyp was granular and reddish, and measured 33 x 22 x 14 mm. Histological examination of the polyp revealed a cavernous hemangioma. Hemangioma of the colon is rare; only 52 cases have been reported in Japan. Of these patients, eight underwent endoscopic polypectomy. The present lesion is the largest thus treated that has been documented. Endoscopic polypectomy seems to be a safe and preferable procedure for the diagnosis and treatment of small, solitary polypoid hemangiomas of the colon.

Colorectal haemangioma and its relationship to haemorrhoids in childhood.

A case of diffuse cavernous haemangioma of the rectum and sigmoid colon is presented to exemplify the propensity for this tumour to be misdiagnosed as haemorrhoids in the paediatric age group. In view of the prolonged morbidity and high mortality associated with this tumour we suggest that, in any child referred for management of haemorrhoids, a diagnosis of colorectal haemangioma be considered. Appropriate investigations include plain abdominopelvic X-rays, sigmoidoscopy and colonoscopy with biopsy, and barium enema. Selective inferior mesenteric angiography and computerized tomography may be helpful. Excision and coloanal sleeve anastomosis is the most favoured operative procedure for this tumour.

Cause of signal loss in MR images of old hemorrhagic lesions.

Old hemorrhagic lesions in the brain are characteristically surrounded by a band of hemosiderin-containing tissue. This region is typically of low signal intensity on long-echo-time (TE) radio-frequency (RF) spin-echo magnetic resonance (MR) images and on gradient-echo MR images. To determine the cause of signal loss in this band, the authors measured the signal that arises from imaging such a region with use of an RF spin-echo technique with a 180 degrees pulse incrementally displaced from TE/2. The incremental loss of signal was small. Using an agar phantom containing iron particles, the authors also showed that signal loss results primarily from diffusion in magnetic gradients. They conclude that most signal loss in the dark band surrounding areas of late-stage hemorrhage arises from diffusion in areas of magnetic inhomogeneity.