A Rare Case of Presacral Paraganglioma Presenting with Hypertensive Encephalopathy

Paragangliomas are exceptionally rare tumors in children of neural crest origin. Our case report represents an extremely rare location for paraganglioma including its imaging features, perioperative challenges and histopathological characteristics. In the present case, a 10-year-old boy had headache and blurring of vision for four years but was not diagnosed until he came with malignant hypertension and hypertensive encephalopathy. The symptoms raised urinary catecholamines and CT findings confirmed the diagnosis of a functioning paraganglioma in presacral region. Challenges during surgery are to control the fluctuating blood pressure which may suddenly rise during tumor handling and suddenly fall following its removal. The unusual location of the tumor in pararectal region also made surgical resection difficult. Paragangliomas are rare and should be kept in mind while evaluating a child with hypertension and complete excision is the treatment of choice.

Giant Cauda Equina Schwannoma with Dystrophic Calcifications : Case Report and Review of the Literature

Giant spinal schwannoma of the cauda equine involving many nerve roots is rare, and ossification is usually not observed in the schwannoma. A 21-year-old man presented with a 12-month history of urinary dysfunction and numbness below the buttocks. Plain radiography showed scalloping of the posterior surface of the vertebral bodies from L4 to the sacrum, and magnetic resonance imaging and computed tomography revealed a giant cauda equina tumor with dystrophic calcification. The tumor was completely removed, with intraoperative neurophysiologic monitoring. Histopathologic examination showed that the tumor was a schwannoma. The patient's postoperative course was uneventful, with urinary function and numbness gradually improving. Although a giant schwannoma accompanied by dystrophic calcification is extremely rare, such a tumor can be removed safely and completely by meticulous dissection and careful neuromonitoring of the cauda equina spinal nerves involved in the tumor.

Eccrine Porocarcinoma

Eccrine porocarcinoma is an extremely rare malignant cutaneous neoplasm. This tumour has an indolent behavior and misleading clinical diagnosis often leading to a delay in definitive treatment. The definitive diagnosis is made by histopathology since clinical findings often vary. The tumour must be considered in the differential diagnosis of elderly patients presenting with long standing skin lesion with a recent history of accelerated growth rate. Because of its reported high rate of local recurrence, a wide excision of the tumour with clear resection margins is mandatory. Close long term follow up is required. We present herein two cases of eccrine porocarcinoma.

Surgical Treatment for Idiopathic Granulomatous Mastitis

PURPOSE: Idiopathic granulomatous mastitis is a rare benign inflammatory breast disease of an unknown etiology and the optimal treatment remains controversial. The aim of this study is to evaluate the efficacy of surgically complete excision in patients with idiopathic granulomatous mastitis. METHODS: Between March 2005 and November 2008, we treated 14 cases that were diagnosed with idiopathic granulomatous mastitis. Prospectively, we treated the cases with complete surgical excision with or without steroid therapy in all patients. RESULTS: The mean age of the patients was 36 years (range 30 to 53 years). All cases performed were complete excision with or without steroid therapy. The median follow up period was 26 months (range 5 to 50 months) and all cases had no recurrence. 13 patients out of the 14 were satisfied with the cosmesis of the treated breast. CONCLUSION: We conclude that the treatment of choice for idiopathic granulomatous mastitis is surgically complete excision.

A Study of Reconstruction Methods for 186 Cases after Complete Excision ofNonmelanoma Skin Cancers / 대한피부과학회지

BACKGROUND: Skin cancer is divided into two categories, melanoma and nonmelanoma skin cancer, by its malignant potential. The former is prone to be metastatic and it often requires regional lymph node dissection and chemotherapy, while the latter is rarely metastatic with no need for such further treatment. We report here on the reconstruction methods after surgical removal of skin cancers, with excluding melanoma. OBJECTIVE: Our purpose was to analyze the reconstruction methods according to the location and size of the lesions after surgical removal of nonmelanoma skin cancers and we report on the clinical findings. METHODS: We analyzed 186 cases of nonmelanoma skin cancers that were treated Between January, 2000 and December, 2006 at the Department of Dermatology, Chonbuk University Hospital. The cases were analyzed according to the reconstruction methods for the defects after surgery, the gender ratio, the age range, the lesion site and the lesion size. RESULTS: The mean age of onset was 67.7 years old. The ratio of men to women was 1:1.02. The most common nonmelanoma skin cancer was basal cell carcinoma (66.7%), followed by squamous cell carcinoma (22.1%), and Bowen's disease (4.3%). The most common site of all the nonmelanoma skin cancers was the face (68.8%), followed by the trunk (7.5%) and scalp (6.5%). The most common reconstruction method was local flap (40.3%), followed by primary closure (38.7%), skin graft (18.8%) and secondary intention (2.2%). In terms of location, the most commonly used method was local flap for the face (50.0%) and primary closure for the trunk (53.3%), scalp (83.3%) and the upper (44.4%) and lower extremities (63.6%). According to the lesions' size, the most common method was flap for tumor between 10 mm and 30 mm in diameter (95.1%), primary closure for tumor less than 10 mm in diameter (66.7%) and graft for tumor greater than 30 mm in diameter (66.7%). CONCLUSION: We offer this data for the reconstruction methods of nonmelanoma skin cancers and their clinical findings in Korea.

Desmoplastic Fibroma of the Skull

Desmoplastic fibroma is one of the uncommon osseous tumors that present in the skull. Although classified as benign tumor, desmoplastic fibroma exhibits local aggressiveness and has a high potential for recurrence. The most common sites include metaphysis of long bones and mandible. Only 15cases have been described in the skull. We report the 16th case of desmoplastic fibroma of the skull.

A case of cystic lymphangioma of the broad ligaments / 대한산부인과학회잡지

Cystic lymphangioma of the broad ligament is very rare benign cystic lesion. They rarely present in adult life. We present the case of a 37-year-old female with a history of chronic recurrent left lower abdominal dull pain over a period of several months. Clinical presentation of these tumors varies greatly from an asymptomatic mass to serious complications. The therapy of choice is a complete excision, unless vital structures were involved. Once excised, the prognosis is excellent. Our patient had complete excision of her lesion with a good outcome.

Diagnosis and Arthroscopic Treatment of Symptomatic Medial Patellar Plica Syndrome of the Knee / 대한정형외과학회잡지

PURPOSE: To evaluate the efficacy of physical examination and dynamic arthroscopy in the diagnosis and treatment of medial patellar plica syndrome which is likely to be overlooked. SUBJECTS AND METHODS: 23 cases diagnosed as medial patellar plica syndrome from August 1998 through December 1999 were evaluated. Diagnosis was made by a symptom of a pain and a click sound during knee flexion, a physical examination of mass palpation and dynamic arthroscopy. RESULT: A dynamic rotation test was an useful physical examination having 96% rate of sensitivity. The results of excellent and good cases were in 19 cases (83%) and there was no worsened case symptomatically after arthroscopic resection. Postoperative complications were 3 cases including incomplete excision (1 case), postoperative hemarthrosis (1 case) and medial meniscus instability due to incorrect arthroscopic technique (1 case). In patients younger than 30 year old, the result of arthroscopic excision was better than that in older ones. CONCLUSION: Careful physical examination with dynamic arthroscopy is a useful method to diagnose the medial patellar plica syndrome which is likely to be overlooked, and arthroscopic complete excision result in excellent results which is of benefit to fast rehabilitation.

Recurrence and Extraneural Metastasis in 31 Meningeal Hemangiopericytomas

Purpose: Meningeal hemangiopericytoma(M-HPC), characterized by a high local recurrency and metastatic potential, is a rare neoplasm arising from perivascular pericytes. A retrospective study was performed to identify the recurrence and extraneural metastasis in M-HPC. MATERIALS AND METHODS: We reviewed the records of 31 M-HPC patients treated from 1982 through 1999 at our institution. The time to recurrence and the various parameters affecting recurrence were determined. Extreneural metastasis was also analyzed. RESULTS: The rate of local recurrency was 38.7%(12/31). The overall average recurrence-free period(RFP) before the first recurrence was 104 months, with overall recurrence-free rates(RFRs) at 5 and 10 years after first surgery of 59.2% and 33.6%, respectively. Of the 12 patients who experienced local recurrence, 4 had recurrences 5 years later after the first surgery. Complete excision at the first operation significantly extended the average time before first recurrence from 43 to 111 months. The 5-year RFRs for the groups of complete excision and incomplete excision were 72.7% and 20.8%, respectively(p=0.0060). Although there was no statistical significance, complete excision followed by adjuvant radiotherapy of more than 50Gy extended the RFP. The 5-year RFRs for the groups of complete excision and complete excision with adjuvant radiotherapy were 70.3% and 100%, respectively(p=0.3359). Four patients(12.9%) presented one or more extraneural metastases that were developed at an average of 107 months after the first operation with the 5- and 10-year metastasis rates of 4.4% and 24.9%, respectively. Conclusions: M-HPC has a propensity to recur either locally or at distant sites after surgical resection. Complete excision is the most important factor to reduce recurrence. However, even with complete excision, adjuvant radiotherapy of more than 50Gy significantly reduces the risk of recurrence. Local and distant recurrences may occur after a prolonged disease-free interval, emphasizing the need for long-term follow-up.

Hemangiopericytoma On Buccal Mucosa

Hemangiopericytoma is uncommon vascular neoplasm that arises from pericytes arround the capillary walls. It was first described as a distinct vascular neoplasm by Stout and Murray in 1942 The anatomic distribution is widespread throughout the body, with approximately one third occur in the head and neck. No sex predilection has been found. Although middle age appears to be the most prevalent time of onset, this neoplasm has been found in all age groups. The differentiation between benign and malignant hemangiopericytoma can be difficult. Although the majority of these tumors are benign, there are malignant variants that can metastasize. Metastasis of seemingly benign tumors may appear year of decade later, so long term close follow-up is needed The treatment of choice is complete surgical excision of the tumor. Despite their vascular origin, these tumors are relatively radioresistant. Radiation therapy is reserved for inoperable metastases or treatment of postoperative surgical fields. Here we present a case of hemangiopericytoma occuring on the Lt. buccal mucosa.

Papillomatosis of the Nipple: A case report

Papillary lesions of the nipple are extremely rare. These lesions are not associated with a positive family history of or other risk factors for breast carcinomas. Papillary lesions of the nipple tend to assume one or more of three distinct morphologic growth patterns. These patterns include sclerosing papillomatosis, papillomatosis adenosis, and adenosis. A nipple papilloma initially presents itself as a bulge just beneath the nipple and enlarges slowly over months to years before causing symptoms. Patients present with nipple erosions, ulcerations, or masses and often have serous or bloody discharge. Because of this constellation of symptoms, it is often a challenge to differentiate a papilloma of the nipple from Paget's disease or eczematous dermatitis. Complete excision of the papillary lesion is sufficient. The author experienced papillomatosis of nipple and got good results postoperatively. A 55-year-old woman presented with multiple lobulated small masses and erosion of the left nipple. At first, we diagnosed the lesion as Paget's disease or eczematous dermatitis. Later, a wedge biopsy revealed the papilloma. A complete excision of the papillary lesion was performed.