Congenital pyloric atresia and junctional epidermolysis bullosa: a report of long-term survival and a review of the literature.

The coexistence of congenital pyloric atresia (PA) and epidermolysis bullosa (EB) in newborns is a rare but distinct association. Mortality is high. In particular, a universally fatal outcome has been reported in neonates born with the junctional type of EB and PA. This has led some investigators to advocate that surgical correction of PA be withheld to obviate needless suffering. We treated five patients, including one set of siblings. Maternal hydramnios and nonbilious vomiting were constant features. Delayed passage of meconium was found in four. Plain x-rays demonstrated gastric dilatation in an otherwise gasless abdomen. Blistering skin lesions were noted at birth in four and developed soon after in the last patient. All lesions were determined to be junctional EB based on electronmicroscopy. The clinical course for these children has been far better than the literature predicts. Successful repair of PA was performed after appropriate stabilization. One infant died at 4 months of age of staphyloccal septicemia, malnutrition, and sepsis from chronic urinary tract obstruction. Another child, born with dysmorphic features to consanguineous parents, is 9 years old and has a seizure disorder. The remaining three are alive and well at 17 months, and 9 and 16 years. The oldest two are siblings. In all four surviving patients, the blistering nonscarring lesions were found to significantly improve in severity, duration, and occurrence with age. Presently, these lesions are mild and require little therapy. Their nails, initially normal at birth, have become discoloured, thickened, and dystrophic. The management of pitted, carious, and yellow teeth is currently the major problem.(ABSTRACT TRUNCATED AT 250 WORDS)     

Management of urinary tract in children with epidermolysis bullosa.

Epidermolysis bullosa is a group of rare genetic disorders characterized by noninflammatory blistering lesions of the skin occurring after minor mechanical trauma. In association with junctional epidermolysis bullosa, a syndrome of pyloric atresia has occasionally been noted in the literature. Several infants who had this combined disorder have been reported to have severe genitourinary tract involvement. Most of these patients have died at an early age because of severe urinary tract involvement. We describe a rare survivor who was initially treated with urinary diversion. Subsequent attempts at undiversion of this patient were unsuccessful. He is presently stable following rediversion. The entities of e. bullosa and e. bullosa/pyloric atresia are reviewed with emphasis on urologic associations.     

Lepromatous leprosy in a kidney transplant recipient: a case report

Leprosy is a chronic granulomatous disease of the skin and peripheral nerves caused by Mycobacterium leprae. Among mycobacterial infections, leprosy is rare in renal transplant recipients. Here, we report the manifestations of lepromatous leprosy in a 41-year-old renal transplant recipient. Before the renal transplant, the patient had recurrent bullous lesions on his extremities with no systemic complaints. He was on an immunosuppressive regimen that included prednisolone (1 mg/kg/d), cyclosporine (6 mg/kg), and mycophenolate mofetil (2000 mg/d), and had 2 serologically confirmed acute episodes of cytomegalovirus infection that responded favorably to intravenous ganciclovir. The density of his bullous skin lesions decreased after renal transplant. During his regular posttransplant visits, we noticed a decrease in his eyebrow hairs on their lateral margins bilaterally. Later, he developed generalized, symmetric, erythematous papules. With a positive acid-fast bacilli with Fite staining, the results of a skin biopsy showed diffuse foamy histiocyte infiltration in the dermis. These findings are compatible with lepromatous leprosy. After antileprosy therapy, no deterioration of renal allograft function or lepra reactions was noted in a 4-month follow-up. Clinicians should consider leprosy in the differential diagnosis of skin lesions in immunocompromised hosts, and in particular, solid organ transplant recipients in endemic areas.     

Rhabdomyolysis, acute renal failure, and multiple focal neuropathies after drinking alcohol soaked with centipede

Many Chinese like to drink alcohol soaked with creatures for promoting health. This study reports a 49-year-old male who presented with multiple focal neuropathies of the upper limbs, coagulopathy, erythematous swelling of the bilateral upper extremities and trunk with bullous skin lesions, and rhabdomyolysis associated with acute renal failure after drinking alcohol soaked with centipede. Soaking a centipede, Scolopendra subspinipes mutilans, in 53% alcohol, produced the wine. Supportive treatment was administered, and the skin lesions and renal failure improved with subsequent neurologic deficit during the week following initial presentation. Alcohol binge or immobilization was the likely cause of neuropathy, bullous skin lesions and rhabdomyolysis in the patient. However, there is a possibility that centipede venom also contributed to the illness in this patient.     

Rhabdomyolysis,Acute Renal Failure,and Multiple Focal Neuropathies After Drinking Alcohol Soaked with Centipede

Many Chinese like to drink alcohol soaked with creatures for promoting health. This study reports a 49‐year‐old male who presented with multiple focal neuropathies of the upper limbs, coagulopathy, erythematous swelling of the bilateral upper extremities and trunk with bullous skin lesions, and rhabdomyolysis associated with acute renal failure after drinking alcohol soaked with centipede. Soaking a centipede, Scolopendra subspinipes mutilans, in 53% alcohol, produced the wine. Supportive treatment was administered, and the skin lesions and renal failure improved with subsequent neurologic deficit during the week following initial presentation. Alcohol binge or immobilization was the likely cause of neuropathy, bullous skin lesions and rhabdomyolysis in the patient. However, there is a possibility that centipede venom also contributed to the illness in this patient.     

A study of tricuspid-tailoring annular constriction in autopsy cases--cases which appear to embody limitation of tricuspid-tailoring annular constriction

We our department have performed tricuspid-tailoring annular constriction (T-TAC) as salvage operation for secondary tricuspid regurgitation (TR) with gratifying results. Of those cases undergoing MVR and T-TAC for mitral valve regurgitation (MR) and secondary TR with an associated severe cardiac cachexia, 2 autopsy cases in which an associated severe cardiac cachexia, 2 autopsy cases in which residual tricuspid regurgitation culminated in patient's death long after operation were investigated. In Case 1, the patient was discharged asymptomatic 2 months after operation, but later developed severe left heart failure and marked tricuspid regurgitation as precipitated by upper respiratory tract infection and died from exacerbation of LOS before undergoing a scheduled reoperation. T-TAC was found successful on autopsy. In Case 2, the patient died of long-persisting left heart failure and severe residual tricuspid regurgitation after following a similar postoperative course to that in Case 1. On autopsy stitches made through the tricuspid anterior leaflet, septum and posterior leaflet were found untied and loosened and the tricuspid annulus diameter increased. In severe valvular heart disease, even if T-TAC has proven successful, aggravation of cardiac failure may lead to the development of TR. Because of the fragility of myocardial tissue due to severe myocardial damage in such compromised patients, sophisticated operative techniques need to be devised. Cases illustrative of a limited long-term success of T-TAC used alone are presented.     

Perioperative management of two neonates with severe Ebstein's anomaly with pulmonary atresia

Ebstein's anomaly appearing during the neonatal period carries a high mortality rate. We report the perioperative management of two neonates with severe Ebstein's anomaly associated with pulmonary atresia. Their chest radiography revealed massive cardiomegaly, with cardiothoracic ratio of 90% and 100%, respectively. Their tracheas were intubated immediately after birth because of severe respiratory distress. The babies were laid in the prone position and one of them was managed with high frequency oscillatory ventilation (HFO) for prevention of pulmonary barotrauma and extensive atelectasis in the preoperative period. On the 4th and 3rd day after birth, respectively, they underwent Starnes procedure which consists of closure of tricuspid valve, enlargement of atrial septal defect, reduction of right atrium and creation of aortopulmonary shunt reported to be a useful palliative treatment for critically ill neonates with Ebstein's anomaly. Case 1 baby could not be weaned from cardiopulmonary bypass because of low cardiac output. Case 2 could not be weaned from extracorporeal membrane oxygenation due to hypoxia. We consider, however, treatment of respiratory failure and lung protection after birth are important for the prognosis. Perioperative use of HFO may be advantageous for the neonate with severe Ebstein's anomaly with pulmonary atresia.     

The spectrum of renal involvement in epidermolysis bullosa dystrophica hereditaria: report of two cases

Epidermolysis bullosa dystrophica Hallopeau-Siemens (EBDH) is one of the most severe inherited epidermolyses, a group of mechanobullous dermatological disorders. We observed two patients presenting with a severely multilating type of EBDH who developed biopsy-proven renal disease, which substantially altered the evolution and pathogenesis of their disease. In a boy, chronic postinfectious glomerulonephritis developed, most probably due to recurring superinfections of bullous skin lesions. He also experienced acute oliguric renal failure due to severe diarrhea during exacerbation of EBDH. A female patient developed a nephrotic syndrome due to secondary amyloidosis. Hypoalbuminemia caused further fluid losses through bullous skin lesions, aggravating intravascular hypovolemia and leading to rapid renal failure secondary to bilateral renal vein thrombosis. The study shows that, although rare, renal complications may alter the natural course of EBDH.     

Serum visceral protein levels reflect protein-calorie repletion in neonates recovering from major surgery.

Protein catabolism resulting from acute metabolic stress causes significant postoperative decreases in visceral proteins, including albumin (Alb) and prealbumin (PA). Although clinical trials have suggested an advantage of PA over Alb in monitoring the visceral protein response to nutritional supplementation following surgery, the capability of the neonate to generate such a response has yet to be evaluated. Therefore, this study was undertaken to determine if PA is superior to Alb in assessing postoperative repletion of the visceral protein pool in neonates. Serum Alb and PA levels were measured and energy balance (EB) and protein intake (PI) were recorded in 10 neonates less than 48 hours after major surgery and again following 4 consecutive days of positive EB. Resting energy expenditure (REE) was measured using indirect calorimetric methodology. Mean PI (g/kg/d) was lower (0.78 +/- 0.78) and mean EB (kcal/kg/d) was negative (-2.92 +/- 10.05) less than 48 hours postoperatively compared with mean PI (2.52 +/- 0.57; P = .0006) after 4 consecutive days of positive EB (34.84 +/- 16.5; P = .0004). Mean percent change (mean% delta) from negative EB to positive EB was significantly greater for PA (100%; P = .0002) as compared with Alb (18.5%). These data appear to support the conclusion that serial serum PA levels are superior to Alb to monitor the visceral protein response to nutritional supplementation in neonates following surgery.     

A novel association of alveolar capillary dysplasia and duodenal atresia with paradoxical dilatation of the duodenum

Background/purpose

The authors experienced 3 cases of persistent pulmonary hypertension of the neonate (PPHN) associated with duodenal atresia with paradoxical dilatation of the distal blind end of the duodenum. The aim of this study was to clarify the characteristics of this novel association.

Methods

The medical charts of the patients were reviewed retrospectively.

Results

Case 1 was a 2,862-g male infant with a prenatal diagnosis of duodenal atresia. The dilated intestine was identified as the distal blind end of the duodenum during duodeno- duodenostomy. PPHN developed after surgery, and the patient died despite treatment with inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). Lung biopsy results showed alveolar capillary dysplasia (ACD) with misalignment of pulmonary vessels. Case 2 was a 2,244-g female infant with duodenal atresia. Surgery could not be performed because of refractory PPHN, which resulted in death. Autopsy results showed ACD with misalignment of pulmonary vessels and duodenal atresia associated with dilatation of the distal blind end. Case 3 was a 2,462-g female infant with a prenatal diagnosis of duodenal atresia associated with dilatation of the distal blind end. Enlarged echogenic fetal lungs were noted antenatally. PPHN developed after surgery, and she was extubated successfully under combined therapy with iNO and intravenous prostacyclin. Unfortunately, she finally died of refractory PPHN. A retrospective review of the prenatal ultrasonographic findings showed enlarged echogenic fetal lungs as in the first 2 cases.

Conclusions

The characteristics of this novel association are enlarged echogenic fetal lungs, duodenal atresia associated with paradoxical dilatation of the distal blind end, and refractory PPHN resistant to iNO.     

Calciphylaxis in secondary hyperparathyroidism. Diagnosis and parathyroidectomy

Calciphylaxis is a rare, severe complication of secondary hyperparathyroidism. Patients present with painful, violaceous, mottled skin lesions of the upper and lower extremities, which become necrotic and produce nonhealing ulcers. Gangrene of fingers and toes frequently requires amputation, produces nonhealing wounds, and can lead to sepsis and death. We reviewed the clinical course of five patients with calciphylaxis treated in our institution. The three men and two women (aged 47 to 72 years) had secondary hyperparathyroidism from chronic renal failure. All patients had severe pruritus, painful ulcers, and severe hyperphosphatemia with elevated serum calcium-phosphate product (greater than 12 mmol2/L2), but the serum parathyroid hormone levels were only moderately elevated. Most patients had medical calcification of medium and small blood vessels, and some had soft-tissue calcification visible on roentgenography. Treatment consisted of local wound care, antibiotics, phosphate-binding agents, and parathyroidectomy. Two patients died of uncontrollable sepsis. The three survivors had dramatic improvement of pain and ulcers after parathyroidectomy. Calciphylaxis is a limb- and life-threatening complication of secondary hyperparathyroidism. Diagnosis can be made by recognizing the characteristic painful skin lesions, ulcers, and gangrene of the digits, and patients should be treated with subtotal parathyroidectomy.     

Hyperhomocysteinemia and Arterial Aneurysm

Hyperhomocysteinemia (HCY) is an independent risk factor for atherosclerosis. Arterial aneurysm has rarely been described in association with heterozygous HCY. Here we report two cases of this association. Case 1 was 32-Year-old man who presented with distal trophic manifestations of the lower extremities. Upon investigation, occlusive arterial disease with fusiform aneurysm of both popliteal arteries and occlusion of the left cubital artery were found. Laboratory findings indicated HCY due to homozygous methylene tetrahydrofolate reductase (MTHFR) deficiency. Case 2 was 38-year-old man with no history of trauma who presented with repeated ischemic events involving the right hand in association with isolated aneurysm of the right cubital artery. Histological study demonstrated extensive dystrophic changes in the aneurysmal vessel wall, including sclerohyalin deposits. The only abnormality was homozygous MTHFR deficiency. Pathologic changes induced by HCY in vessel walls may be implicated in early arterial aneurysm. The association of anatomic lesions, young age, and absence of other causes suggests that the relationship between HCY and arterial aneurysm observed in these two patients was not coincidental.     

胸脐皮瓣的临床应用

应用胸脐皮瓣游离移植修复颈部、手部严重烧伤瘢痕畸形８例，胸脐岛状皮瓣修复先天性阴道闭锁及硫酸烧伤后遗严重会阴部瘢痕、阴茎大部分软组织缺损伴严重漏尿各１例均获成功。该皮瓣解剖恒定、血管口径粗，易于吻接，皮瓣面积大、色泽与颈部皮肤相似是修复颌颈部及四肢软组织缺损的理想供区。皮瓣血管蒂长、血运丰富、移转方便，适合作逆行岛状瓣修复会阴部组织缺损、阴茎再造或阴道成形术。     

Bullous Lesions in a Patient with Systemic Lupus Erythematosus

Bullous eruptions in patients with lupus erythematosus can be difficult to diagnose as bullous lesions can develop in lupus-specific lesions, and primary blistering disorders can also occur. Additionally, these patients tend to have multiple co-morbidities making them more likely to be on many medications that can lead to bullous drug reactions. A thorough history, the clinical presentation, and histopathological findings along with direct immunofluorescence can be helpful in diagnosing most cases. The authors report the case of a woman with a long history of systemic lupus erythematosus who initially presented in their clinic for diagnosis and management of erythema dyschromicum perstans and one year later developed bullae in atypical targetoid lesions on the extremities and trunk. They discuss several blistering disorders that have been reported in patients with lupus erythematosus with a focus on features that help distinguish erythema multiforme, fixed drug eruption, and lupus erythematosus from Stevens-Johnson syndrome/toxic epidermal necrolysis. In the patient described herein, the authors favor a diagnosis of Stevens-Johnson syndrome, but the classification between erythema multiforme major and Stevens-Johnson syndrome/toxic epidermal necrolysis cannot be made in some cases.The diagnosis of bullous eruptions in patients with lupus erythematosus (LE) can be difficult to make as several different primary blistering disorders have been reported to occur in association with LE, including bullous pemphigoid, pemphigus vulgaris, dermatitis herpetiformis, epidermolysis bullosa acquisita, linear immunoglobulin A (IgA), porphyria cutanea tarda, Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN).1-3 Bullous lesions can also occur in erythema multiforme (EM). These conditions must be differentiated from the bullous lesions that can occur in cutaneous lesions of LE, which can be due to extensive vacuolar degeneration of the basement membrane (BM) or from antibodies to type VII collagen in bullous systemic lupus erythematosus (BSLE).4 Patients with LE also tend to have multiple comorbidities, making them more likely to be on multiple medications that can lead to bullous drug eruptions. To add to the difficulty, many of these conditions may mimic SJS/TEN, which can be associated with significant morbidity and mortality. A thorough history, the clinical presentation, and histopathological findings along with direct immunofluorescence (DIF) can be used to diagnose most cases, but there are some cases where a clear diagnosis cannot be made. The authors report a case of a patient with systemic lupus erythematosus (SLE) who presented with a bullous eruption and focus on a discussion of features that help differentiate fixed drug eruption (FDE), LE, and erythema multiforme (EM) from SJS/TEN.     

Chronic granulomatous disease: two members of a single family with different dermatologic manifestations

Case 1: A 33-year-old man with a 14-year history of localized skin disease on the face and scalp was evaluated at the department of dermatology. The physical examination revealed plaques with papules, pustules, and a golden yellow crusting on the forehead, cheeks, upper lip, and chin (Figure 1). The scalp presented fine, whitish scales. At the beginning of his disease, the patient presented large red and painful purulent boils. The 14-year clinical course of these lesions was characterized by partial remissions and recurrences, but he did not specify any treatment related to improvement. The clinical diagnosis given for the scalp lesions was seborrheic dermatitis. For the facial lesions, many differential diagnoses were considered, among them: seborrheic dermatitis, acneiform dermatitis, impetigo, folliculitis, seborrheic pemphigus, and demodicidosis. The histopathologic study of a biopsy taken from the cheek (Figure 2) showed superficial spongiform dermatitis with neutrophils and folliculitis that are compatible with the diagnosis of seborrheic dermatitis. Both Gram and periodic acid-Schiff stains were negative. Follow-up of the patient was not possible since he did not come back. The disease in this patient initially manifested at age five by the presence of recurrent ganglionic abscesses. At age 15, he presented a pulmonary abscess of a left lobule that was surgically removed; at this point the diagnosis of chronic granulomatous disease was established. At age 28, an exploratory laparotomy was performed due to peritonitis and multiple hepatic abscesses. At that time, he was treated with antibiotics (mainly trimethoprim-sulfamethoxazole) and interferon-g. The patient had two brothers who died due to complications of chronic granulomatous disease. In addition, both his mother and sister presented a history of discoid lupus-like lesions. Case 2: Coincidentally, his 27-year-old sister was seen in our department of dermatology 5 years before, presenting infiltrated and erythematous plaques with fine scales (Figure 3) on the right side of the nose and the left annular finger. No other cutaneous or mucous lesions were seen. She referred onset in childhood with similar lesions on sun-exposed areas that disappeared without scarring. A biopsy was performed and the results were compatible with the diagnosis of discoid lupus erythematosus (Figure 4). Direct immunofluorescence was not available. At that time, she did not mention the family history of chronic granulomatous disease. Clinical follow-up was not possible, but his brother referred that she afforded complete remission only with sun protection.     

Pyloric atresia: five new cases, a new association, and a review of the literature with guidelines

BACKGROUND/PURPOSE: Pyloric atresia is an uncommon condition occurring in 1 of 100,000 live births. When occurring in isolation, the clinical course usually is uncomplicated after surgical treatment. However, it may occur in association with other congenital abnormalities. The authors present 5 new cases, 3 of associated abnormalities including 1 of esophageal atresia and 2 of agenesis of the gall bladder and malrotation. Agenesis of the gall bladder has not been described previously in combination with pyloric atresia. The literature has been reviewed and guidelines are suggested for the management. METHODS: The case records of 4 neonates who presented to the author's institution between January 1998 and June 1999 and 1 who presented at another center in 1991 were reviewed. A Medline literature search was performed, and guidelines were developed for the management of this condition based on our cases and the literature review. RESULTS: Patients 1 and 5 had no associated anomalies. Patient 2 had associated esophageal atresia, tracheoesophageal fistula, atrial septal defect, crossed renal ectopia, malrotation, and absent gall bladder. Patient 3 had a rectovestibular fistula, vaginal atresia, atrial septal defect, malrotation absent gallbladder, and absent extrahepatic portal vein. Patient 4 had epidermolysis bullosa. Patients 2 and 5 had unremarkable recoveries, patients 2 and 3 had markedly delayed gastric emptying that responded to cisapride. Patient 3 has portal hypertension and remains under close follow-up. Patient 4 died at 22 days of age of pseudomonas sepsis. CONCLUSIONS: Based on our cases and literature review, we have adopted the following guidelines: (1) All children with pyloric atresia should be screened for multiple anomalies. (2) Delayed gastric emptying should be considered early and may respond to prokinetic agents. (3) Association with Epidermolysis bullosa should not preclude surgical treatment. (4) A skin biopsy specimen should be taken at the time of surgery for electron microscopy if there is a family history of epidermolysis bullosa.     

胸脐皮瓣的临床应用

应用胸脐皮瓣游离移植修复颈部、手部严重烧伤瘢痕畸形8例,胸脐岛状皮瓣修复先天性阴道闭锁及硫酸烧伤后遗严重会阴部瘢痕、阴茎大部分软组织缺损伴严重漏尿各1例均获成功。该皮瓣解剖恒定、血管口径粗,易于吻接,皮瓣面积大、色泽与颈部皮肤相似是修复颌颈部及四肢软组织缺损的理想供区。皮瓣血管蒂长、血运丰富、移转方便,适合作逆行岛状瓣修复会阴部组织缺损、阴茎再造或阴道成形术。     

CHARGE and esophageal atresia.

CHARGE association was diagnosed in 61 infants, 20 of whom died, mainly during the first 2 years of life. Esophageal atresia and/or tracheoesophageal fistula were present in 10 neonates. Axial skeletal anomalies occurred in 7 of the 10, but none had preaxial limb defects typical of the VATER association. All had major cardiac anomalies, predominantly tetralogy of Fallot. The majority of patients had primary repair of the esophagus. The postoperative course was stormy in all patients, with a high incidence of complications and 70% died. The recognition of features of the CHARGE association is important because it has major prognostic and therapeutic implications.     

Multiple myeloma diagnosed during hormonal therapy for prostate cancer: report of two cases

Case 1: A 73-year-old man presented with a serum prostate specific antigen (PSA) level of 30.2 ng/ml, and was diagnosed with prostate cancer (cT3aN0M1, stageD2), for which hormonal therapy (maximal androgen blockade : MAB) was commenced. Nine months later he developed back pain, and osteolytic bone lesions progressed despite a stable, low PSA level (0.087 ng/ml). He was diagnosed with multiple myeloma on the basis of positive M protein on immunoelectrophoresis. MP combination therapy (melphalan and prednisolone) was commenced, but the patient died of multiple myeloma 33 months later. Case 2: A 70-year-old man was diagnosed with prostate cancer (PSA 19 ng/ml) at another hospital 5 years ago, and underwent hormonal therapy (luteinizing hormone-releasing hormone (LHRH) agonist only). He was referred to our hospital and underwent bicalutamide+MAB combination therapy due to a raised PSA level (58 ng/ml) and multiple bone metastases. His PSA level dropped to around 20 ng/ml, but 2 years later he developed back pain, and bone metastases with osteolytic change were seen in the skull, ribs, and limbs. Needle aspiration biopsy of a fist-sized soft tissue mass in the chest wall showed multiple myeloma. Although chemotherapy with melphalan was commenced, the patient died of multiple myeloma 8 months after its diagnosis. Both these cases exhibited rapidly progressing bone lesions, regardless of an absence of any large fluctuations in serum PSA levels, during hormonal therapy for prostate cancer. Further investigations yielded the diagnosis of multiple myeloma. If progression of bone lesions does not match the status of prostate cancer as surmised from the serum PSA level, we should consider the possibility of multiple myeloma, and biopsy of one of the bone lesions.     

Surgical treatment of primary pulmonary artery tumor

We report two cases of rare primary pulmonary artery (PA) tumor. Case 1 was a 65-year-old man with malignant fibrous histiocytoma which caused severe stenosis over the main PA and right PA. Case 2 was a 49-year-old woman with leiomyosarcoma which caused severe stenosis from the right ventricular outflow tract (RVOT) to the main PA. Both cases underwent endarterectomy, tumor resection, and PA reconstruction under cardiopulmonary bypass. The symptoms of right heart failure improved postoperatively. Tumor recurrences were however recognized in both cases in the early postoperative period, and both patients died of the disease 12 (case 1) and 21 (case 2) months after operation respectively. Tumor resection by endarterectomy techniques may be incomplete because of frequent local recurrence. Postoperative adjuvant therapy in addition to radical resection and reconstruction of the PA and tumor might increase the length of survival.