GI-Associated Hemangiomas and Vascular Malformations.
ABSTRACT: Hemangiomas and vascular malformations of the gastrointestinal tract, rare clinical entities, present as overt or occult bleeding. They can be distributed throughout the intestinal digestive system, or present as a singular cavernous hemangioma or malformation, which is often located in the rectosigmoid region. Misdiagnosis is common despite characteristic radiographic features such as radiolucent phleboliths on plain film imaging and a purplish nodule on endoscopy. Adjunctive imaging such as computed tomography and magnetic resonance imaging are suggested as there is potential for local invasion. Endorectal ultrasound with Doppler has also been found to be useful in some instances. Surgical resection is the mainstay of treatment, with an emphasis on sphincter preservation. Nonsurgical endoscopic treatment with banding and sclerotherapy has been reported with success, especially in instances where an extensive resection is not feasible.
Project description:Vertebral hemangiomas are benign lesions accounting for 2 to 3% of all spinal tumors. They are usually asymptomatic and found incidentally on imaging. Uncommonly, vertebral hemangiomas with significant epidural extension can result in radiculopathy or spinal cord compression. Decompressive surgery with or without stabilization is often required when neurological deficits are present. However, surgery can be associated with massive hemorrhage as these tumors are hypervascular. Preoperative embolization and sclerotherapy are well-known management strategies used to minimize intraoperative bleeding and improve symptoms. Recently, the use of sclerosants such as ethanol has decreased, due to reported complications such as Brown-Sequard syndrome. We describe the use of sodium tetradecyl sulfate (Fibro-Vein™, STD Pharmaceutical, Hereford, UK) as an effective alternative to ethanol in the preoperative management of vertebral hemangiomas. To our knowledge, this has not been previously reported. In three patients, we demonstrated minimal intraoperative blood loss using a combination of preoperative embolization of arterial feeders and sclerotherapy with sodium tetradecyl sulfate to control and secure venous drainage. No patients developed complications related to the procedure. In addition to minimal blood loss, a clear dissection plane was also noted intraoperatively.
Project description:Renal capillary hemangiomas are rare and benign vascular tumors which are typically incidentally discovered on imaging. Surgical excision is often performed, as imaging appearance is similar to malignant lesions. Renal hemangiomas are typically solitary and unilateral. We present a rare case of multiple renal capillary hemangiomas in a patient with end-stage renal disease. Two hemangiomas were detected on imaging and 2 smaller hemangiomas were detected upon pathological evaluation, suggesting there may be a wider prevalence of smaller, radiographically-occult renal hemangiomas.
Project description:Infantile hemangiomas are the most common infantile benign vascular tumor. While most infantile hemangiomas proliferate then involute, some may persist and require treatment for reasons including risk of disfigurement or functional impairment. Treatments currently include observation, pharmacological therapy, laser, cryosurgery, surgery and radiotherapy. Although pharmacological therapy is a well accepted treatment option, limited studies have evaluated the efficacy of different drug therapies. In this study, we compare different pharmacological modalities in the management of infantile hemangiomas. The study included 853 infants with proliferative infantile hemangiomas who were treated with topical timolol, oral propranolol, intralesional pingyangmycin, or intravenous vincristine from 2009 to 2012. Treatment stratification was based on clinical severity of the tumor. Response to the treatment was clinically evaluated and graded as: excellent, good, poor, or no response. Response to pharmacological therapies was excellent in almost all infantile hemangiomas. In addition, patients younger than 8 months responded highly to pharmacological treatment (89.1%), while patients older than 8 months were less responsive to treatment (36.3%). There were no instances of life-threatening complications. Overall, these findings support the efficacy of timolol, propranolol, pingyangmycin and vincristine in the treatment of infantile hemangiomas, especially in the youngest patient cohort (8 months or younger).
Project description:Introduction:Intramedullary lesions and tumors are generally accessed by a posterior approach. However, if the lesion is located on the ventral side of the spinal cord, a posterior resection with myelotomy poses technical difficulties. We report two cases of complete resection of a cervical ventral intramedullary cavernous hemangioma using an anterior approach. Case Report:Two cases of intramedullary cavernous hemangioma located on the ventral side of the spinal cord were successfully treated by total resection with anterior cervical corpectomy followed by anterior spinal fusion with an autologous bone strut from the iliac crest. In both cases, the postoperative course was uneventful, and there was no neurological deficit. Bony fusion was achieved, and there was no recurrence or complication during a follow-up period of at least two years. Conclusions:Here, we describe an anterior approach for total resection of cavernous hemangiomas on the ventral side of the cervical spinal cord. Outcomes were stable two years after the operations. Although the method should be assessed with more patients and a longer follow-up time, this anterior approach may be useful for the radical resection of a vascular malformation or tumor.
Project description:Some infantile hemangiomas called in literature "minimal or arrested growth hemangiomas" or "abortive hemangiomas" are present at birth and have a proliferative component equaling less than 25% of its total surface area. Often, they are mistaken for vascular malformation. We present five patients (three girls and two boys) with abortive hemangiomas diagnosed between January 2010 and December 2015 localized in acral part of the extremities. They were congenital lesions resembling precursor of hemangiomas but did not show proliferation phase. Immunohistochemical Glut-1 was performed in all of them as a way to confirm the abortive hemangioma diagnosis. The most common appearance was a reticulated erythematous patch with multiple fine telangiectasias on the surface. We remark that one of them presented a segmental patch with two different morphologies and evolutions. The proximal part showed pebbled patches of bright-red hemangioma and presented proliferation and the distal part with a reticulated network-like telangiectasia morphology remained unchanged. We detected lower half of the body preference and dorsal region involvement preference without ventral involvement. The ulceration occurred in three patients with two different degrees of severity.
Project description:Introduction Hemangiomas are vascular malformations, with slow blood flow, that can occur in any part on the body. They are more common in women and, predominantly, are isolated lesions. The malformation does not spontaneously regress. Subcutaneous hemangioma is a rare variant with an aggressive growth pattern that sometimes recurs after excision. Objective Case report of a subcutaneous cavernous hemangioma in the nasal dorsum treated with endoscopic rhinoplasty. Case Report A 27-year-old woman had a fibroelastic tumor mass in the midline of the nasal dorsum, which was pulsatile; she had obstruction and nasal congestion with associated rhinorrhea, with evolution and worsening over the previous 2 years. Computed tomography showed a tumor demarcated in the nasal dorsum without evidence of intracranial communication. Endoscopic rhinoplasty with septoplasty and associated paranasal sinus sinusectomy was performed without arteriography embolization, sclerotherapy, or laser. Pathologic diagnosis showed cavernous hemangioma. Postoperative follow-up shows no recurrence at 3 years. Discussion This case presented with atypical features, thus making the diagnosis a challenge. Imaging studies were required to confirm the vascular nature of the tumor. Excisional biopsy is the procedure of choice for pathologic examination. Subcutaneous hemangiomas never involute and always need treatment. The surgical approach is exceptional because there was no preoperative diagnosis. In addition, the closed technique provided best aesthetic results in this case. Conclusion Endoscopic rhinoplasty is suitable for nasal dorsum tumor resection and has superior aesthetic result to open techniques.
Project description:Current treatments for infantile hemangiomas have unpredictable outcomes. The authors' aim was to develop a nanoporphyrin-delivered, high-efficacy treatment for infantile hemangiomas using a mouse hemangioendothelioma model.The authors injected mouse hemangioendothelioma cells intradermally to axillary regions of 5-week-old, female, nude mice (n = 19) to induce hemangioendothelioma growth. They documented nanoporphyrin accumulation in hemangioendotheliomas using positron emission tomography. For the treatment study, the authors randomized hemangioendothelioma-bearing nude mice (n = 9) into three groups (n = 3 each). Group I received only saline injections. Group II received only laser treatment after saline injection, and group III received laser treatment after nanoporphyrin injection through the tail vein. The authors followed up the treatment response with digital caliper measurements.Hemangioendotheliomas started to grow approximately 1 week after inoculation and resembled infantile hemangiomas histologically. Nanoporphyrin uptake in hemangioendotheliomas was 19.7 ± 2.2, 16.7 ± 2.02, 8.4 ± 0.3, and 4.9 ± 0.6 percent injected dose per gram of tissue at 3, 6, 24, and 48 hours after injection, respectively. Nanoporphyrin uptake was significantly higher than in blood at 24 and 48 hours after injection (p < 0.05). Results of ex vivo biodistribution study were consistent with positron emission tomographic imaging. Hemangioendotheliomas in group III started to regress 1 day after the treatment and disappeared totally by day 21. The difference between tumor volumes in group III and other groups was significant on days 17 and 21 (p < 0.05).Nanoporphyrin accumulated in hemangioendotheliomas at high concentrations, enabling a high-efficacy photodynamic therapy. Given the similarities between hemangioendotheliomas and infantile hemangiomas, this treatment potentially can be a high-efficacy treatment for infantile hemangiomas.
Project description:OBJECTIVES:The aim of this study was to evaluate the technical and clinical outcomes of using laparoscopic radiofrequency (RF) ablation for treating large subcapsular hepatic hemangiomas. METHODS:We retrospectively reviewed our sequential experience of treating 124 large subcapsular hepatic hemangiomas in 121 patients with laparoscopic RF ablation. RESULTS:The mean diameter of the 124 hemangiomas was 9.1 ± 3.2 cm (5.0-16.0 cm). RF ablation was performed successfully in all patients. There were 55 complications related to the ablation in 26 patients, including 5 of 69 (7.3%) patients with hemangioma <10 cm and 21 of 52 (40.4%) patients with hemangiomas ?10 cm (P < 0.001). No injuries to abdominal viscera occurred in all the 121 patients. According to the Dindo-Clavien classification, all the complications were minor in 26 patients (Grade I). Out of 124 hepatic hemangiomas, 118 (95.2%) were completely ablated, including 70 of 72 (97.2%) lesions < 10 cm and 48 of 52 (92.3%) lesions ? 10 cm (P = 0.236). CONCLUSION:Laparoscopic RF ablation therapy is a safe, feasible and effective procedure for large subcapsular hepatic hemangiomas, even in the hepatic hemangiomas ? 10 cm. Its use avoids thermal injury to the abdominal viscera.
Project description:Propranolol is a widely used beta blocker that consists of a racemic mixture of R and S stereoisomers. Only the S stereoisomer has significant activity against the beta-adrenergic receptor. A fortuitous clinical observation was made in an infant who received propranolol for cardiac disease, and regression of a hemangioma of infancy was noted. This has led to the widespread use of propranolol for the treatment of large and life-threatening hemangiomas of infancy. Infants receiving propranolol require monitoring to ensure that they do not suffer from side effects related to beta blockade. The exact mechanism of activity of propranolol in hemangioma of infancy is unknown. In this study, we treated hemangioma stem cells with both beta blockade active S- and inactive R-propranolol and looked for genes that were coordinately regulated by this treatment. Among the genes commonly downregulated, Angiopoietin-like 4 (ANGPTL4) was among the most regulated. We confirmed that propranolol isomers downregulated ANGPTL4 in endothelial cells, with greater downregulation of ANGPTL4 using the beta blockade inactive R-propranolol. ANGPTL4 is present in human hemangiomas of infancy. Finally, R-propranolol inhibited the growth of bEnd.3 hemangioma cells in vivo. The implication of this is that hemangioma growth can be blocked without the side effects of beta blockade. Given that humans have been exposed to racemic propranolol for decades and thus to R-propranolol, clinical development of R-propranolol for hemangiomas of infancy and other angiogenic diseases is warranted.
Project description:Primary vaginal endometrioid adenocarcinoma is a rare cancer that is often associated with chronic endometriosis. We present the case of a 72-year-old female who underwent right salpingo-oophorectomy followed by hysterectomy with benign pathology 25 years prior to her cancer diagnosis. She had an extensive surgical history in the intervening years and several complicating factors including a history of endometriosis as well as a recurrent peritoneal inclusion cyst treated with ethanol sclerotherapy, followed by formation of a peritoneal-vaginal fistula. Endometriosis is associated with malignant transformation to endometrioid adenocarcinoma through genomic alteration, oxidative stress, inflammation, and hyperestrogenism. Frequency of surveillance examinations and imaging prior to diagnosis were based on patient symptoms, and ultimately a vaginal cuff mass was detected with invasion of the rectosigmoid colon, bladder and levators at time of diagnosis, necessitating infralevator total pelvic exenteration for removal.