Pheochromocytoma associated with adrenocortical adenoma: case report and literature review

The case of a 60-year-old woman with pheochromocytoma and concomitant adrenocortical adenoma in the same gland is presented. She complained of episodic headache, palpitation, nausea, vomiting and sweating. Physical examination revealed that the patient has generalized obesity, wet skin and paroxysmal hypertension, but no signs of Cushing's syndrome. Elevated levels of urinary noradrenaline, adrenaline and total metanephrine were sequentially observed. In addition, urinary 17-OHCS was also slightly elevated, but plasma cortisol was normal and suppressed after oral administration of 0.5 mg of dexamethasone. Abdominal echography and CT scanning demonstrated a left adrenal tumor, which took up both 131I-meta-iodobenzylguanidine and 75Se-scintadoren in the same region. A left adrenalectomy was performed and the tumor was found to consist of two parts, pheochromocytoma (2.5 X 2.5 X 2.5 cm) and cortical adenoma (2.5 X 3 X 5 cm). A total of 23 reported cases showing evidence of hyperfunction of the adrenal cortex and the medulla were noted. So far as we know, this patient was the second case of pheochromocytoma with adrenocortical adenoma in Japan.     

Woodhouse-Sakati syndrome: case report and symptoms review

We report on a 52-year old Caucasian woman exhibiting signs of Woodhouse - Sakati syndrome and review the clinical signs and symptoms in patients reported so far. The syndrome is characterized by alopecia, mental retardation, hypogonadism, diabetes mellitus, hearing impairment, ECG changes, and by autosomal recessive inheritance. We also propose that the limited mobility of the upper extremities is one of the features of the syndrome.     

儿童不典型黄癣1例报告及文献复习

报告1例由许兰毛癣菌(Trichophyton schoenleinii)引起的儿童不典型黄癣,患儿临床症状轻微,缺乏蜡黄色、碟形黄癣痂和特殊的鼠臭味等典型临床症状.病发真菌直接镜检见大量不规则菌丝,间生膨大,大小不等、形态不规则的孢子,真菌培养为许兰毛癣菌生长,经分子生物学测序鉴定证实.E-test药敏结果显示伊曲康唑、两性霉素B、卡泊芬净及伏立康唑均敏感.经口服伊曲康唑胶囊及外用2％舍他康唑乳膏治疗基本痊愈.     

原发肾上腺淋巴瘤1例并文献复习

目的:探讨原发肾上腺淋巴瘤(PAL)的临床特点,提高对该病的认识.方法:对1例原发肾上腺淋巴瘤患者的资料进行分析,并结合国内外相关文献进行总结.结果:原发肾上腺淋巴瘤好发于老年男性,可表现为局部症状如腹痛,腰痛,也可表现为乏力,发热,体重减低等全身症状,50%病人可出现肾上腺功能不全的症状.一些病人是在行影像学检查时因偶然发现肾上腺的肿物,经病理检查而确诊的.该病多累及双侧肾上腺,最常见的病理类型是弥漫大B细胞型.原发肾上腺淋巴瘤的治疗包括手术、化疗、放疗以及它们的不同组合.该病恶性程度高,进展迅速,预后差.结论:原发肾上腺淋巴瘤虽然少见,但在肾上腺肿瘤,尤其是快速增长的肾上腺肿瘤的鉴别诊断中应考虑到它的可能,及时进行病理检查可尽早确诊.     

Testosterone-secreting adrenocortical tumor in a pubertal girl. Case report and review of the literature

A girl aged 12 years and 10 months presented with deepening of the voice first noted 7 months earlier. Pubertal development was almost completed. The girl had regular monthly menses and no signs of hirsutism, clitoris enlargement or Cushing's disease. Serum testosterone was about threefold above normal, whereas dehydroepiandrosterone was in the upper normal range. The 17-ketosteroids as well as the gas-chromatographically analyzed 5 alpha and 5 beta derivatives of testosterone from urine were slightly increased. Other serum and urinary steroids were normal. Dynamic tests of the endocrine function exhibited inconclusive results. Ultrasonography revealed no ovarian cysts. A small, left-sided adrenal mass was identified by computed axial tomographic scan and removed by surgery. There were no signs of local metastasis nor of vascular extension. The histopathological diagnosis was adrenocortical carcinoma. 5 months after surgery, the preoperatively elevated steroid levels had returned to normal.     

Human pentastomiasis in China: case report and literature review

A 13-yr-old boy was admitted because of persistent fever, abdominal pain and diarrhea for 3 mo. Abdomen CT revealed hepatomegaly and multiple nodular low-density pathological changes. At laparotomy considerable yellow and turbid ascites were seen in the abdominal cavity and miliary nodules were noted on the surface of the omentum majus, liver, and small intestine wall. Histological examination revealed parenchymal tubercles containing several worms. Pathological diagnosis was parasitic granuloma. These parasites were identified as Porocephalus taiwana sp.nov. The patient made an uneventful recovery after therapy and was discharged. Moreover, another 17 cases of human pentastomiasis reported from China were reviewed. Human pentastomiasis is an extremely rare disease and this is only the second case of human Porocephalus taiwana sp.nov infection. Pentastomiasis should be considered in differential diagnosis of patients with a history of abdominal symptoms and eating of poorly-cooked snakes.     

Cutaneous ciliated cyst: case report and literature review

An 18-year-old girl had a cyst excised from the left buttock in 1988. The cyst was diagnosed to be a cutaneous ciliated cyst, since histologic examination using special stains demonstrated its lining to be similar to fallopian tube epithelium. Only 20 such cases have been published to date, and this report is probably the first case in the plastic surgical literature, to the best of our knowledge.     

Lymphoplasmacytic sclerosing pancreato-cholangitis: a case report and review of the literature

Autoimmune pancreatitis is a rare but important cause of pancreatitis that is becoming increasingly recognized in the West. Lymphoplasmacytic sclerosing pancreatitis (LPSP) is a benign form of chronic pancreatitis characterized clinically by infrequent attacks of abdominal pain, jaundice, and weight loss, and pathologically by focal or diffuse chronic or lymphoplasmacytic inflammatory infiltrates centered around pancreatic ducts and ductules, accompanied by obliterative phlebitis, acinar atrophy, and interstitial fibrosis. It has been described alone or as a part of the spectrum of autoimmune gallbladder and biliary tract disease, with clinical, radiological, and pathological overlap reported with primary sclerosing cholangitis. It has been described as "primary sclerosing pancreatitis," "sclerosing cholangitis," "non-alcoholic duct destructive chronic pancreatitis," and "autoimmune pancreatitis." We report a case of LPSP that mimicked pancreatic adenocarcinoma and was subsequently treated with a pylorus-preserving Whipple procedure. This may point towards a primary biliary autoimmune process involving the pancreatic duct, causing a benign form of chronic pancreatitis that may be difficult to characterize pre-operatively to avoid surgery. This case typifies the growing awareness of this relatively recently characterized clinical entity, its similar presentation to pancreatic carcinoma, and the importance for LPSP to be included in the differential diagnosis of pancreaticobiliary disease. Finally, we review the literature.     

Takotsubo syndrome with pulmonary embolism: a case report and literature review

Background

Takotsubo syndrome (TTS) is an acute cardiac condition with reversible heart failure which is often triggered by psychological and physical stressful events. Although pulmonary embolism (PE) was reported as a trigger for TTS, the concurrence of TTS and PE has been rarely reported, let alone that triggered by PE. Here we describe a case of a postmenopausal female presenting with symptoms similar to myocardial ischemia, which may be caused by PE, and review the available literature that may help clinicians with their practice to similar situations since no published guidelines are available.

Case presentation

An 86-year-old female was referred to the emergency department for unrelieved chest tightness, shortness of breath and back pain. Cardiac biomarkers were mildly elevated and electrocardiogram displayed pathologic Q-waves, ST-segment elevation and inverted T-waves. Unexpectedly, coronary angiography was in absence of obstructed coronary atherosclerosis or acute plaque rupture. Chest computed tomography illustrated multiple pulmonary emboli in bilateral pulmonary arteries. She had suffered from long-term right lower extremity pain and experienced a long railway journey with less activity. Both echocardiogram and cardiac magnetic resonance demonstrated regional hypokinesia of left ventricle. She received anticoagulant and diuretic therapies, three-month follow up after discharge revealed uneventful recovery without any pulmonary emboli or regional motion abnormalities, thus she was retrospectively diagnosed with TTS caused by PE.

Conclusion

TTS and PE are scarcely concurrent and PE can exert as a potential trigger for TTS. TTS is easily misdiagnosed, actively seeking possible risk factors of TTS is in favor of early diagnosis and timely intervention. TTS with PE is reversible, timely and effective treatments ensure the best possible outcome.

     

Rhinosporidiosis in Egypt: A case report and review of literature

Rhinosporidiosis is an infection caused by Rhinosporidium seeberi that frequently presents as a polypoidal nasal lesions. Here, we report the first indigenous case of tumoral rhinosporidiosis in Egypt. In this case, a 25-year-old male patient from a rural background of Assuit City presented with epistaxis and a nasal polyp. The patient had not traveled abroad. The diagnosis was established on the morphological basis by the identification of 5- to 10-m endospores and 50- to 1000-m sporangia. The clinicopathological and immunologic features were discussed and the literature was reviewed. To the best of our knowledge this is the first case of this disease to be reported in Egypt in the human literature.     

圣乔治教堂诺卡菌引起的肺部感染1例

诺卡菌肺部感染可引起肺诺卡菌病,因其无特异性的临床表现,容易误诊、漏诊。因此,临床实验室的培养鉴定能力非常重要,若不能及时诊治,则会导致病死率较高。诺卡菌病临床较为少见,为引起临床实验室对诺卡菌的鉴定和药敏试验的重视,本文报道了上海市嘉定区中心医院2019年2月收治的1例由圣乔治教堂诺卡菌(Nocardia cyriacigeorgica)感染引起的肺诺卡菌病病例,针对其临床特征、实验室检测及治疗等进行分析,期望对临床诊治诺卡菌病有所帮助。     

尖端赛多孢子菌引起的真菌性鼻窦炎1例并文献复习

目的 探讨尖端赛多孢子菌的实验室检测方法,了解其对5种常用抗真菌药物的体外敏感性.通过对相关文献的复习,熟悉真菌性鼻窦炎的临床表现和诊治方法.方法 将1例尖端赛多孢子菌引起的真菌性鼻窦炎患者经鼻内腔镜手术切除的团块用10％ KOH压片镜检、涂片革兰染色镜检、沙堡弱培养基培养、分子生物学方法鉴定到种.分离株应用E-test进行体外药敏试验.结果 鉴定为尖端赛多孢子菌.5种药物对其MIC范围分别为:伏立康唑0.064 μg/mL,卡泊芬净1.500 μg/mL,氟康唑16.000 μg/mL,两性霉素B＞32.000 μg/mL,5-氟胞嘧啶＞32.000 μg/mL.结论 尖端赛多孢子菌引起的真菌性鼻窦炎国内少见报道,易与肿瘤相混淆;实验室检测对正确诊断起决定性作用;行鼻内腔镜下手术治疗效果较好.了解该菌的耐药性对指导抗真菌治疗尤为关键.     

Alstrom syndrome (OMIM 203800): a case report and literature review

Hereditary chronic pancreatitis (HCP) is a very rare form of early onset chronic pancreatitis. With the exception of the young age at diagnosis and a slower progression, the clinical course, morphological features and laboratory findings of HCP do not differ from those of patients with alcoholic chronic pancreatitis. As well, diagnostic criteria and treatment of HCP resemble that of chronic pancreatitis of other causes. The clinical presentation is highly variable and includes chronic abdominal pain, impairment of endocrine and exocrine pancreatic function, nausea and vomiting, maldigestion, diabetes, pseudocysts, bile duct and duodenal obstruction, and rarely pancreatic cancer. Fortunately, most patients have a mild disease. Mutations in the PRSS1 gene, encoding cationic trypsinogen, play a causative role in chronic pancreatitis. It has been shown that the PRSS1 mutations increase autocatalytic conversion of trypsinogen to active trypsin, and thus probably cause premature, intrapancreatic trypsinogen activation disturbing the intrapancreatic balance of proteases and their inhibitors. Other genes, such as the anionic trypsinogen (PRSS2), the serine protease inhibitor, Kazal type 1 (SPINK1) and the cystic fibrosis transmembrane conductance regulator (CFTR) have been found to be associated with chronic pancreatitis (idiopathic and hereditary) as well. Genetic testing should only be performed in carefully selected patients by direct DNA sequencing and antenatal diagnosis should not be encouraged. Treatment focuses on enzyme and nutritional supplementation, pain management, pancreatic diabetes, and local organ complications, such as pseudocysts, bile duct or duodenal obstruction. The disease course and prognosis of patients with HCP is unpredictable. Pancreatic cancer risk is elevated. Therefore, HCP patients should strongly avoid environmental risk factors for pancreatic cancer.     

Acute pancreatitis after major spine surgery: a case report and literature review

Background

Acute pancreatitis has been described as potential complication of both abdominal and non-abdominal surgeries. The pathogenetic mechanism underlying acute pancreatitis in spine surgery may include intraoperative hemodynamic instability causing prolonged splanchnic hypoperfusion, as well as mechanical compression of the pancreas due to scoliosis correction, with a higher risk in cases of more extended fusions, especially in young adults with lower body mass index (BMI).

Case presentation

We report here a case of postoperative acute pancreatitis with benign evolution in a young female patient after the first and second surgery of a two-stage correction of right thoracic idiopathic scoliosis.In December 2017, the patient underwent first-stage T4-L3 posterior arthrodesis with T7-T12 osteotomies and temporary magnetic bar. Intraoperative blood loss required massive transfusion. In the immediate postoperative period, the patient started reporting nausea/vomiting, abdominal pain at pressure, moderate meteorism, abdominal distension, hypoactive bowel sounds, and fever. Laboratory tests indicated a progressive increase in aspartate aminotransferase, alanine aminotransferase, serum amylase, lipase, phospho-creatine kinase, and reactive C-protein. A CT scan showed free abundant abdominal fluid in the hepatic, renal, pancreatic, and pelvic regions. After the diagnosis, a hypolipidic diet was initiated, and good hydration per os was maintained. After gastroenterologic consultation, somatostatin, rifaximin, and ursodehoxycholic acid were initiated and maintained for 8 days. In the following days, laboratory tests showed a slow but consistent decrease in liver and pancreatic enzymes until normalization. In January 2018, the patient underwent second-stage surgery with removal of magnetic bar, definitive posterior fusion, and instrumentation T4-L3. Laboratory tests showed a second, even more significant, increase in the amylase and lipase level and a moderate increase in the reactive C-protein. Therapy was maintained until complete normalization of amylase and lipase levels.

Conclusions

Early recognition of symptoms plays a key role in preventing severe morbidity after scoliosis surgery. When symptoms suggest abdominal complication, pancreatic and liver enzymes are to be evaluated for posing prompt diagnosis. Gastroenterologic consultation and eventual imaging are further steps in differential diagnosis and treatment of this rare complication.

     

Malignant struma ovarii: report of a case and review of the literature

Malignant struma ovarii is a very rare disease and therefore there is neither common agreement on treatment regimens nor sufficient follow-up experience. The case of a 38-year-old woman with malignant struma ovarii is described. The patient presented with a clinically silent ovarian neoplasm discovered incidentally during investigations for metrorrhagia. The ovarian mass was resected and the tumor was found to be a teratoma. Within the teratoma a papillary thyroid carcinoma of the follicular variant was found. Subsequently, in order to make follow-up of the patient possible by thyroglobulin measurement and radioiodine whole body scintigraphy, near-total thyroidectomy was performed and the thyroid was found to be normal on histology. Whole body radioiodine scintigraphy with (131)I and ablation of the thyroid remnant by the administration of 80 mCi (131)I was performed. The patient recovered uneventfully and is now well.     

Twin fetus in fetu in a child: a case report and review of the literature

Introduction

Necrotizing fasciitis is a rare condition with a mortality rate of around 34%. It can be mono- or polymicrobial in origin. Monomicrobial infections are usually due to group A streptococcus and their incidence is on the rise. They normally occur in healthy individuals with a history of trauma, surgery or intravenous drug use. Post-operative necrotizing fasciitis is rare but accounts for 9 to 28% of all necrotizing fasciitis. The incidence of wound infection following saphenofemoral junction ligation and vein stripping is said to be less than 3%, although this complication is probably under-reported. We describe a case of group A streptococcus necrotizing fasciitis following saphenofemoral junction ligation and vein stripping.

Case Presentation

A 39-year-old woman presented three days following a left sided saphenofemoral junction ligation with long saphenous vein stripping at another institution. She had a three day history of fever, rigors and swelling of the left leg. She was pyrexial and shocked. She had a very tender, swollen left groin and thigh, with a small blister anteriorly and was in acute renal failure. She was prescribed intravenous penicillin and diagnosed with necrotizing fasciitis. She underwent extensive debridement of her left thigh and was commenced on clindamycin and imipenem. Post-operatively, she required ventilatory and inotropic support with continuous veno-venous haemofiltration. An examination 12 hours after surgery showed no requirement for further debridement. A group A streptococcus, sensitive to penicillin, was isolated from the debrided tissue. A vacuum assisted closure device was fitted to the clean thigh wound on day four and split-skin-grafting was performed on day eight. On day 13, a wound inspection revealed that more than 90% of the graft had taken. Antibiotics were stopped on day 20 and she was discharged on day 22.

Conclusion

Necrotizing fasciitis is a very serious complication for a relatively minor, elective procedure. To the best of our knowledge, this is the first report in the English-language literature of this complication arising from a standard saphenofemoral junction ligation and vein stripping. It highlights the need to be circumspect when offering patients surgery for non-life-threatening conditions.