• The Journal of the Korean bone and joint tumor society
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• v.11 no.2
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• pp.208-212
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• 2005

Maffucci syndrome was first reported by Maffucci, an Italian, in 1881. This rare, nonhereditary syndrome is characterized by multiple enchondromas combined with hemangiomas and less commonly lymphangiomas. Maffucci syndrome is sometimes confused with Ollier disease, which was described in 1899 and consists of multiple enchondromas without hemangiomas. Although the prevalence of malignant change in skeletal lesions in Maffucci syndrome and Ollier disease is quite similar, it is important to differentiate between these two conditions because of the greater risk of developing nonmusculoskeletal malignancies in the former. We experienced a case of this rare syndrome and because of it's rarity, we report with review of literature.

• Archives of Plastic Surgery
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• v.45 no.6
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• pp.517-524
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• 2018

Background Infantile hemangioma (IH) is a common vascular tumor in pediatric patients, and is commonly treated with propranolol. We describe our experiences with dosage, response to treatment, and side effects in 23 IH patients treated with propranolol. Methods For this nonrandomized comparative cohort study, the authors enrolled 23 patients treated with propranolol. Photographs were taken before propranolol administration and at 3, 6, 9, and 12 months after treatment. Treatment responses were objectively analyzed with a computer program. Results There were three male and 20 female patients. Common tumor locations were the head and neck (13 cases, 56.5%), trunk (four cases, 17.4%), extremities (three cases, 13.0%), and combined locations (three cases, 13.0%). The response to propranolol was significantly lower in patients with two or more lesions than in patients with a single lesion in terms of both color fading (P<0.001) and size reduction (P<0.001). In male patients ($42.2{\pm}3.9$), the change in a-values, indicating coloration, was higher than in female patients ($19.8{\pm}13.8$) (P<0.001). In patients who started treatment before 6 months after birth, the size reduction was greater than in their counterparts (62.3%; range, 3.0%-93.0% vs. 15.8%; range, 1.0%-79.0%; P<0.001). Conclusions Propranolol is an efficacious treatment with a good safety profile. In patients with a single lesion, the response to treatment was better in terms of color fading and size reduction. Furthermore, male patients responded better to propranolol treatment in terms of color fading than female patients, and starting treatment before 6 months after birth was more advantageous for size reduction.

• Clinical and Experimental Pediatrics
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• v.58 no.12
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• pp.484-490
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• 2015

Purpose: To determine the efficacy and safety of oral propranolol as a first-line treatment for superficially located infantile hemangioma (IH) and propose an assessment tool to measure treatment response. Methods: Patients with superficial IH under 1 year of age were prospectively recruited between May 2012 and December 2013 at the Department of Pediatrics of Chungbuk National University Hospital. Propranolol was administered to 12 infants (median age, 3.8 months) while monitoring cardiovascular and adverse metabolic effects. If a patient showed no adverse events, the dosage was gradually increased up to 3 mg/kg/day and maintained for 1 year. We used our own scoring system to assess treatment response using parameters like change in color, and longest diameter, and thickness of the IH. Results: Eleven out of 12 patients completed the protocol with consistent improvement of hemangiomas during therapy. Patients on propranolol showed a more than 50% involution in the first 3 months, with additional steady involution until 1 year. Patients with the highest scores at 1 month maintained their score and showed better responses until treatment termination. The patient with the lowest score at 1 month did not show any further regression and stopped propranolol treatment 4 months after initiation. In two children with recurrences after successful therapeutic regression, propranolol was effective after being reintroduced. Propranolol treatment was not interrupted in any patient due to adverse events. Conclusion: Oral propranolol at 3 mg/kg/day showed a consistent, rapid, and therapeutic effect on superficial IHs without significant adverse events.

• Journal of the korean academy of Pediatric Dentistry
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• v.25 no.2
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• pp.458-466
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• 1998

Non-odontogenic tumors can be classified as malignant or benign. Most oral tumors in children are benign. In the Belfast series only 7.5 percent of soft tissue tumors were malignant and Bhaskar(l963) found only 9 percent of 293 oral tumors of all kinds to be malignant. Benign tumors may be classified as epithelial and mesenchymal. The most common tumor of surface epithelium is the squamous papilloma. These are easily recognized clinically as cauliflower-like lesions. Fibrous lesions are very common in children's mouths. Many of these are not true neoplasms but are related to fibrous hyperplasia. Another common oral tumor in children is angiomatous tumors. Hemangioma occurred more frequently than lymphangioma. Cystic hygroma, a cystic subtype of lymphangioma, is a developmental tumor of lymphatic origin. It is a considered to be a relatively rare lesion. About 50 percent of cystic hygroma are present at birth, and most of the remaining 50 percent appear in the early years of life during the period of active lymphatic growth. The preferred treatment for these lesions, except for hemangioma, is complete surgical excision. With proper surgical techniques, recurrence is not expected.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.43 no.2
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• pp.125-133
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• 2017

Intramuscular hemangioma (IMH) is a rare vascular disease involving skeletal muscle, comprising only 0.8% of hemangiomas. About 10% to 15% of IMHs occur in the head and neck region, mostly involving the masseter muscle. IMH occurs mostly in childhood, but is often not found until unexpected enlargement, pain, or cosmetic asymmetry occurs in adulthood. Several non-surgical treatments including cryotherapy, sclerosant injection, and arterial ligature have been described, but complete surgical resection is the curative intervention. In this report, we present two rare cases of IMH. One IMH case in a 48-year-old male occurred in the masseter muscle feeding from the transverse facial artery. Embolization of the distal branch of the facial artery was first conducted, and then the buccal mass was removed surgically via the intraoral approach. A second IMH case in a 58-year-old female occurred in the orbicularis oris muscle feeding from the superior labial artery, and the mass was excised surgically without embolization.

• Archives of Plastic Surgery
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• v.37 no.6
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• pp.758-762
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• 2010

Purpose: It is difficult to provide accurate diagnosis and proper treatment for vascular anomalies, because vascular anomalies have a wide spectrum of lesion with various symptoms. Furthermore, vascular anomalies often require the services of a wide range of specialists. So, many patients have been discouraged by the lack of expertise and treatment options available to them. In this study, we aimed to describe the scope of the Vascular Malformation clinic (VAM clinic), a multidisciplinary clinic for hemangioma and vascular malformation patients and investigate of diagnostic accuracy of VAM clinic. Methods: 68 patients have included in a retrospective, medical record review study in the VAM clinic from may 2002 to august 2009. Data included the types of diagnoses of patients seen in the clinic, locations of lesions, comparison of accuracy of diagnoses at other clinic and initial diagnoses at VAM clinic and types of recommended treatments. Results: Initial diagnosis at VAM clinic showed 94.2% accuracy, while diagnosis at other clinic only showed 12.5% accuracy. Head and neck is most frequently occurred lesions in patients of VAM clinic. And VAM clinic is able to provide various and proper treatments to patients. Conclusion: Hemangiomas and vascular malformations can present with various medical problems. VAM clinic, the multidisciplinary clinic provided accurate diagnoses and proper treatments to patients.

• Journal of Chest Surgery
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• v.37 no.5
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• pp.460-463
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• 2004

Hemangiomas of mediastinum are rare lesions comprising less than 0.5% of all mediastinal masses. Posterior mediastinal hemangiomas are less common than anterior mediastinal. A 21 year old female was refered to our hospital because of abnormal mediastinal shadow in simple chest X-ray. Chest CT scan and T-spine MRI revealed a posterior mediastinal dumbbell-shaped mass with extradural extension. Surgical excision was performed and pathologic diagnosis was confirmed as 3${\times}$4${\times}$2 cm sized capillary hemangioma.

• The Korean Journal of Nuclear Medicine
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• v.21 no.2
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• pp.191-197
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• 1987

39 patients with focal hepatic lesions were evaluated by $^{99m}Tc-RBC$ liver scan. The diagnosis of focal hepatic lesions were made by percutaneous needle biopsy, angiography, surgery, or clinical courses. Thses diagnoses included 24 cases of hemangioma, 7 hepatomas, 6 metastatic disease, 1 abscess, and 1 cyst. 19 hemangiomas showed focal hot activity on delayed static planar images. 3 small deep seated hemangiomas were diagnosed by SPECT that would have been missed by planar images alone. 2 large hemangiomas had no radioisotope uptake within the lesions on delayed images and at surgery cavernous hemangioma with thrombosis, calcification, and fibrosis were found. For hepatic hemangiomas in our series, the sensitivity was 91.7% and the specificity was 100%. The remaining 15 patients including hepatomas, metastatic lesions, cyst and abscess showed cold defect on delayed blood pool images. It is concluded that $^{99m}Tc-RBC$ liver scan should be the choice of primary diagnostic procecure for clinically suspected hepatic hemangioma since it's inexpensive, non-invasive, and readly available.

• Korean journal of dermatology
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• v.56 no.10
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• pp.631-635
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• 2018

Microvenular hemangioma (MVH) is a rare acquired benign vascular neoplasm, which presents commonly as a solitary purple-to-red nodule or plaque measuring approximately 10 mm in diameter. MVH occurs primarily on the extremities or the trunk. Most lesions are solitary, and multiple lesions are rare. Histopathological features of MVH include numerous, scattered, thin and irregularly branching small vessels in the dermis and endothelial cells without atypia. Owing to similarities in clinical morphology and histopathological features, MVH may often be indistinguishable from the early patch stage of Kaposi sarcoma. Immunohistochemical (IHC) analysis helps differentiate between the 2 diseases. The results of IHC tests in patients with MVH show positive staining for CD31 and smooth muscle actin and typically, negative staining for the human herpes virus 8 antigen. We report a rare case of multiple MVH clinically mimicking the early patch stage of Kaposi sarcoma in a 63-year-old woman who presented with a 3-year history of slowly growing, compressible, soft, bluish-purple macules and plaques on the trunk and right arm.

• The Korean Journal of Nuclear Medicine Technology
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• v.12 no.1
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• pp.39-43
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• 2008

Purpose: There are various methods to diagnose hemangioma, such as ultrasonography (US), computed tomography (CT), magnetic resonance imaging (MRI) and nuclear medicine. However, by development of SPECT imaging, the blood-pool scan using $^{99m}Tc$-labeled red blood cell has been used, because it was non-invasive and the most economical method. Therefore, in this study, we proposed that the usefulness of $^{99m}Tc$-RBC scan and SPECT of the head and neck to diagnose unlocated hemangiomas. Materials and Methods: $^{99m}Tc$-RBC scan and SPECT was performed on 6 patients with doubtful hemangioma (4 person, head; 1 person, neck; 1 person, another). We labeled radiopharmaceutical using modified in vivo method and then, centrifuged it to remove plasma. After a bolus injection of tracer, dynamic perfusion flow images were acquired. Then, anterior, posterior, both lateral static blood-pool images were obtained as early and 4 hours delayed. SPECT was progressed 64 projections per 30 seconds. Each image was interpreted by physicians, Nuclear medicine specialist, and technologist blinded to patient's data. Results: In 5 patients of all the radioactivity of doubtful site didn't change in flow images, but, in blood-pool, delayed and SPECT images, it was increased. So, it was a typical hemangioma finding. The size of lesion was over 2 cm, and it could discriminate as comparing to the delayed and SPECT imaging. On the other hand, in 1 patient, the radioactivity was increased in blood-pool images, but, not in delayed and SPECT images, so, it was proved no hemangioma. Conclusion: Using $^{99m}Tc$-RBC Scan and SPECT, we could diagnose the hemangiomas in head and neck, as well as, liver, more non-invasive, economical, and easy. Therefore, it considered that $^{99m}Tc$-RBC scan and SPECT would offer more useful information for diagnosis of hemangioma, rather than otherimaging such as US, CT, MRI.