Langerhans cell histiocytosis of vulva in adolescent

Langerhans cell histiocytosis (LCH) affecting child vulva alone is a very rare disease. Only 13 cases of primary vulvar LCH have been previously reported in the medical literature. We describe an additional case in which the LCH was confined to the vulva, with review of the literature. A 16.5-year-old girl presented with papulous and ulcerative lesions on her labia majora and minora. The biopsy revealed a typical histopathologic finding consistent with LCH. A metastatic work-up did not reveal any evidence of the disease except on the vulva. Treatment was carried out according to LCH II protocol. The patient was diagnosed with a recurrent disorder in the vulva 8 months after the completion of primary chemotherapy. For this reason, she underwent second line treatment with 2-chlorodeoxyadenosine. Eighteen months after the second line chemotherapy, the patient has no signs of a local or systemic recurrence. Primary LCH of vulva is very unusual, but we have to keep in mind this possibility when an adolescent girl presents with an atypical chronic lesion on the vulva. This patient appears to be the first case of adolescent 16.5 year old having a solely cutaneous lesion of the vulva.     

Isolated Langerhans cell histiocytosis of the vulva: a case report and review of the literature

Isolated Langerhans cell histiocytosis (LCH) of the female genital tract is very rare. A review of the literature revealed that only 15 cases of primary vulvar LCH have previously been published in the English literature. We describe an additional case of confined vulvar LCH. A 49-year-old woman presented with an ulcerous lesion that turned out to be LCH confined to the vulva only. After surgical excision, four recurrences followed, which were treated again by surgery. After the fourth recurrence, adjuvant radiotherapy was applied. When the fifth recurrence occurred, only surgical excision was performed, and the patient has now been disease-free for 51 months. There are no standard treatment options for this rare disease. The most effective treatment options still remain elusive. In our case in the end surgery proved to be effective.     

Mediastinal teratoma as a rare cause of hydrops fetalis and death: report of 3 cases

BACKGROUND: Congenital mediastinal teratomas are rare and may present with nonimmune hydrops. The lesion may be misinterpreted on ultrasound. CASES: A 21-year-old woman, gravida 2, para 0111, was evaluated at 19 4/7 weeks of gestation for suspected fetal death. An ultrasonogram confirmed the death and revealed a posterior encephalocele and possible herniated liver in the chest. At autopsy a 5.2 x 7.5 x 1.0-cm mediastinal teratoma completely compressed the chest organs. No encephalocele was present. A 15-year-old woman, gravida 1, para 0, underwent an ultrasonogram at 27 weeks when fetal heart rate decelerations were detected. The ultrasound revealed hydrops and suggested a calcified left cardiac ventricular wall and diaphragmatic hernia. Autopsy of the stillborn female showed an 8.0 x 6.0 x 4.0-cm teratoma in the mediastinum, with small heart and lungs. A 23 2/7 weeks stillborn female was delivered to a 32-year-old woman, gravida 5, para 2, and noted to be hydropic. Ultrasound had suggested multiple anomalies and hydrops. Autopsy revealed a 23 g, 4.5 x 3.0 x 3.0-cm teratoma that filled the anterior mediastinum. CONCLUSION: Congenital mediastinal teratoma may be associated with fetal death. It is within the differential diagnosis of nonimmune hydrops, particularly if a thoracic mass is detected on ultrasonography.     

Vulvar Lesions in an 8-Year-Old Girl: Cutaneous Manifestations of Multisystem Langerhans Cell Histiocytosis

Background

Langerhans cell histiocytosis (LCH) is a rare localized or systemic disease characterized by proliferation of myeloid-derived dendritic cells. Vulvar lesions might be the herald symptom of LCH and might mimic other cutaneous lesions. Prognosis varies widely on the basis of the extent and spread of disease.

Bilateral pleural endometriosis

The patient was a 40-year-old woman with a 1-year history of catamenial chest pain and a recent bilateral hemothorax. She underwent a left thoracotomy for surgical pleurodesis, and a pleural biopsy. Subsequently, she had a hysterectomy in conjunction with an oophorectomy. The pathologic investigation of the pleura revealed an endometrial implant. The postoperative course was uneventful with no recurrence of hemothorax and chest pain during the first 6 months and at the patient's follow-up. This is a very rare case of a patient with thoracic endometriosis presenting with bilateral hemothorax.     

Rapidly fatal Langerhans' cell histiocytosis in an adult.

Langerhans' cell histiocytosis (LCH) is a disorder of unknown etiology, characterized by abnormal proliferation of cells of the dendritic cell lineage. The acute disseminated form of the disease characteristically occurs in children younger than 3 years of age. More indolent forms of LCH occur primarily in older children and young adults. The prognosis for patients with LCH is good. We report a 28-year-old man with acute disseminated LCH who initially presented with fever, chills, and dry cough. Diagnosis of LCH was confirmed by bronchoalveolar lavage fluid cytology, and skin and bone marrow biopsies. Conventional chemotherapy was not given, because of the patient's poor clinical condition, including respiratory failure, unstable vital signs, and impaired consciousness. Salvage thalidomide therapy was started on the 12th day of hospitalization. The patient died from multisystem LCH with septic shock and multiple organ failure on the 22nd day of hospitalization. This report illustrates the diagnostic features and treatment of LCH in a rapidly fatal case of adult LCH.     

Small-cell lung cancer presenting with Lambert-Eaton myasthenic syndrome and respiratory failure.

Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular disorder characterized by defective neurotransmitter release at presynaptic terminals. It is caused by an IgG autoantibody reacting against voltage-gated calcium channels. Severe LEMS complicated by ventilatory failure is rare. We report a case of small-cell lung cancer (SCLC) presenting with LEMS and ventilatory failure in a 67-year-old man who initially presented with progressive limb weakness for 6 months and tachypnea with shallow breathing for 1 week. LEMS was diagnosed through electrophysiologic studies. Chest radiography and computerized tomography showed a huge mass lesion over the left anterior and middle mediastinum with an encasement of the left pulmonary artery. Cytologic examination of ultrasound-guided fine needle aspiration disclosed SCLC. Successful treatment in combination with plasma exchange and chemotherapy resulted in dramatic tumor regression and LEMS remission, which were confirmed by chest radiography and electrophysiologic studies. This case suggests that plasma exchange and chemotherapy can be effective in treating SCLC with severe LEMS that produces ventilatory failure.     

Coronary dissection and myocardial infarction following blunt chest trauma.

Myocardial infarction (MI) following blunt chest trauma is rarely diagnosed because the ensuing cardiac pain is commonly attributed to contused myocardium or the traumatic injuries in the local chest wall. There are only scattered reports on the coronary pathology associated with MI secondary to blunt chest trauma. Because differentiation of the pathology is difficult but important, we report here three cases of acute anterior MI secondary to coronary dissection following blunt chest trauma. Coronary dissection was demonstrated by coronary angiography. Two of the patients had intimal tears at the proximal left anterior descending artery (LAD) with normal flow, and the other patient had nearly total occlusion of the LAD associated with filling defects probably caused by an intracoronary thrombus. All three patients received conservative treatment without major complications and remained free from angina or heart failure throughout a 5-year follow-up period. In order to exclude associated MI in cases of blunt chest trauma, electrocardiography is necessary, and coronary angiography may be indicated to demonstrate coronary arterial pathology. Dissection of the coronary artery with subsequent thrombus formation is one of the possible pathophysiologic mechanisms of MI following blunt chest trauma.     

Apical hypertrophic cardiomyopathy: clinical, echocardiographic and angiographic features in 3 Chinese patients

We present 3 cases of apical hypertrophic cardiomyopathy. The presenting symptoms were dyspnea on exertion in 2 of 3 patients, typical anginal pain in 1 patient, atypical chest pain in 1 patient, and fatigue in 1 patient. One patient was asymptomatic with an abnormal electrocardiogram. Sustained apical impulse was noted in all patients, Grade 1/6 systolic murmur was audible in 2 patients. Electrocardiogram showed T wave inversion in the left precordial leads in all patients with amplitudes of 1.4, 0.8 and 2.0 mV, respectively. Isolated apical hypertrophy was noted in all patients. Two-dimensional echocardiogram and the left ventriculogram revealed a "spade-shaped" configuration of the left ventricular cavity at endo-diastole in only one patient. Left ventricular end diastolic pressures were elevated and coronary angiograms were normal in all 3 cases. We conclude that this disease entity should be considered in patients whose electrocardiogram shows a large inverted T wave in the left precordial leads, either accompanied by symptoms such as dyspnea on exertion or chest pain, or even when asymptomatic. Echocardiogram is the most useful screening tool in the diagnosis of apical hypertrophic cardiomyopathy.     

Successful perinatal management of a very low birthweight infant with congenital complete atrioventricular block

We report a very low birthweight infant with congenital complete atrioventricular block (CCAVB) who underwent delayed implantation of a permanent pacemaker without temporary pacing. The female infant was born at 30 weeks gestation and weighed 1422 g. After birth, the infant showed respiratory failure due to pleural effusion and respiratory distress syndrome at birth. The heart rate ranged between 50 and 55 bpm. The chest x-ray demonstrated dilated heart, but echocardiogram showed good systolic ventricular function. Respiratory failure was resolved after supportive treatment without temporary pacing. Mild heart failure due to low heart rate persisted, but was successfully managed by conventional heart failure therapy combined with nasal continuous positive airway pressure. She achieved a body weight gain to 1856 g at the age of 49 days and underwent implantation of a permanent pacemaker. We conclude that it is important to determine the timing of both delivery and pacemaker implantation for successful perinatal management of infants with CCAVB.     

Cavernous hemangioma of the heart: report of a case

Cavernous hemangioma of the heart is a very rare disease. A 57-year-old male patient was admitted due to frequent onset of dull chest pain, which had been occurring for about 1 year. The pain was not related to exercise and was not relieved with nitroglycerin. On echocardiographic examination, a tumor was shown in the outflow tract of the right ventricle and was confirmed with computer tomography. He underwent open heart surgery for resection of the tumor. After a median sternotomy and opening of the pericardial cavity, a reddish-brown-colored tumor, 3 cm in diameter, was found protruding from the epicardial layer of the right ventricular outflow tract. The tumor involved all layers of the ventricle and could be resected only with the help of the cardiopulmonary bypass technique. The defect in the right ventricle was repaired with a woven dacron patch. The patient recovered without incident after the operation, and experienced no chest pain during 7 months of follow-up. Histology showed it to be a cavernous hemangioma.     

Tako-Tsubo cardiomyopathy caused immediately following cesarean section delivery of triplets: a case report

The name 'tako-tsubo' cardiomyopathy was initially used to describe a unique 'short-neck round-flask'-shaped form of left ventricular apical ballooning, resembling a Japanese tako-tsubo, a jar (tsubo) used for capturing octopus (tako). Tako-tsubo cardiomyopathy exhibits acute onset, transient left ventricular apical wall motion abnormalities with chest symptoms and minimal myocardial enzymatic release, mimicking acute myocardial infarction in patients without angiographic stenosis on coronary angiography. There have been few case reports on tako-tsubo cardiomyopathy, and this disorder is especially rare in pregnant women. A 30-year-old woman who was pregnant with triplets, and had been treated with ritodrine hydrochloride for 12 weeks for threatened premature delivery, underwent cesarean section with spinal anesthesia at 30 weeks' gestation. Three hours later, she complained of acute chest pain, dyspnea and episodes of unconsciousness. She was transferred to the intensive care unit and intubated for ventilatory support. We diagnosed heart failure due to tako-tsubo cardiomyopathy based on heart ultrasonography, blood tests, chest X-ray, electrocardiogram and myocardial scintigraphy. She was extubated from the ventilator after 3 days of catecholamine, furosemide and carperitide administration. She was discharged from the hospital on day 53 without symptoms.     

Isolated Langerhans cell histiocytosis of the vulva: a case report

We present a case of isolated vulval Langerhans cell histiocytosis (LCH) that was initially treated with an excision biopsy but recurred 2 months later. Local vulval radiotherapy resulted in complete resolution of the lesion and there was no evidence of recurrence after 24 months of follow-up.     

Internal mammary artery to pulmonary artery fistula associated with sick sinus syndrome: report of a case.

A 77-year-old woman presented with general weakness, dizziness and slow, irregular heart beats. An electrophysiologic study revealed a markedly prolonged sinus node recovery time of up to 5,900 msec, accompanied with dizziness. A permanent pacemaker with an AAIR mode was implanted. An abnormal shadow in the right lower lung field was found on chest x-ray. Selective arteriography revealed a right internal mammary to right pulmonary artery fistula. No significant hemodynamic abnormalities were found. This anomaly is extremely rare. Our patient is the oldest among 20 cases reported in the literature, and is the first one associated with sick sinus syndrome. Most reported cases have involved surgical ligation and excision, due to the risk of rupture of the malformation, endarteritis and congestive heart failure. Our patient refused surgical intervention and has done well during the past year.     

Primary langerhans cell histiocytosis of the vulva.

Langerhans cell histiocytosis (LCH) of the female genital tract is very rare. A review of the literature revealed that only 12 cases of primary vulvar LCH have previously been published. We describe an additional case of LCH in which the disease was confined to the vulva. A 64-year-old woman was admitted to our hospital with a nodular mass on her left labium minus, and complete surgical excision was performed. On histological and immunohistochemical examination, the tumor fulfilled the criteria of LCH. A metastatic workup did not reveal any evidence of disease beyond the vulva. The patient received local radiotherapy, and 22 months later she is in excellent condition without local recurrence or metastatic disease.     

An Unusual Case of Shock Following an Elective Caesarean Delivery

BackgroundThe differential diagnosis of peripartum chest pain and cardiogenic shock is broad and includes pulmonary embolism, amniotic fluid embolism, peripartum and Takotsubo cardiomyopathy, myocardial infarction, and anaesthetic complications.CaseA 31-year-old woman with Addison's disease underwent an elective caesarean section that was complicated by chest pain and cardiogenic shock. After initial resuscitation, she was transferred to a tertiary hospital, and urgent transthoracic echocardiography revealed severe systolic dysfunction. She was treated with an increased dose of hydrocortisone and intravenous furosemide and improved. Follow-up imaging showed improvement of left ventricular systolic function.ConclusionIn patients with cardiogenic shock after delivery, early transthoracic echocardiography is a non-invasive tool that can rapidly narrow the differential diagnosis.     

A late post-traumatic diaphragmatic hernia revealed during pregnancy by post-partum respiratory distress

Introduction Diaphragmatic hernia during pregnancy is uncommon and is usually traumatic in origin, epigastric pain, and vomiting could be the initial symptoms of herniation of gastrointestinal contents, with a risk of strangulation and ischaemia, leading to respiratory distress due to collapse of the lung. Methods Case report. Results A 27-year-old woman, with undiagnosed traumatic diaphragmatic hernia who presented, at 32 weeks’ gestation, epigastric pain, vomiting and tachycardia, immediate post-partum course was complicated by respiratory failure. A chest X-ray showed an air fluid level in the left lung which was wrongly diagnosed as an hydropneumothorax, in front of respiratory symptoms exacerbation, an inappropriate thoracic drainage tube was accidentally placed into the herniated stomach leading to perforation of this last. An emergency laparotomy discovered a 2/3 of the stomach, transverse colon and greater omentum herniated in the left hemithorax through a defect of the left hemidiaphragm. Conclusion The diagnosis should then be considered early, and chest radiography with a nasogastric tube is the first technique to prefer and may be helpful to confirm the diagnosis.     

Esophageal Intramural Pseudodiverticulosis Complicated With Stricture

We report a rare case of diffuse esophageal intramural pseudodiverticulosis in a 35-year-old man complaining of severe dysphagia and vomiting for several months. The advanced morphological change in the esophagus caused irregular track formation, mimicking an ulcerative lesion on esophagogram. Endoscopic examination revealed an esophageal stricture with intact mucosa. Endoscopic ultrasonography and chest computed tomography showed multiple hyperechoic lesions of unknown nature and multiple air collection sites in the esophageal wall, respectively, making diagnosis difficult. The patient finally received a subtotal esophagectomy because of severe symptoms. The lesion was pathologically proven to be intramural pseudodiverticulosis with marked submucosal fibrosis. Our experience suggests that awareness of this rare pathology and the related image changes will be helpful for early diagnosis and treatment in the future.     

Cutaneous metastasis of gestational choriocarcinoma.

A 40-year-old woman presented with subcutaneous masses on her chest wall, abnormal vaginal bleeding and an enlarged uterus. Chest X-ray and liver ultrasound revealed metastatic disease to these sites, respectively. A urine human chorionic gonadotrophin assay was positive. A biopsy of the chest wall lesion and endometrium revealed choriocarcinoma. Treatment with methotrexate, actinomycin-D and cyclophosphamide led to complete resolution of the disease on examination, X-ray and ultrasound scans. The urinary pregnancy test became negative.