Aggressive juvenile fibromatosis of the paranasal sinuses: case report and brief review

Desmoid fibromatoses are benign, slow growing fibroblastic neoplasms, arising from musculoaponeurotic stromal elements. Desmoids are characterized by local invasion, with a high rate of local recurrence and a tendency to destroy adjacent structures and organs. Desmoid fibromatoses are rare in children, and though they may occur in the head and neck region, are extremely rare in the paranasal sinuses. Here we report a case of extraabdominal desmoid fibromatosis in a seven-year-old boy involving the sphenoid sinus, one of only six published reports of desmoid fibromatosis of the paranasal sinuses. The expansile soft tissue mass eroded the walls of the sphenoid sinus as well as the posterior ethmoid air cells extending cephalad through the base of the skull. We discuss the clinicopathologic features of this lesion, including structural and ultrastructural characteristics, and we review the literature regarding treatment and outcome.     

Hyperparathyroidism in pregnancy: case report and review of the literature.

The apparent incidence of hyperparathyroidism (HPT) is low in pregnancy but will likely increase now that more asymptomatic HPT is being diagnosed. However, since the serum calcium levels are decreased in pregnant women, mild primary HPT may go unrecognized. In untreated cases of HPT, complications during pregnancy or during the neonatal period have included spontaneous abortion, stillbirth, neonatal death, neonatal tetany and hypercalcemia. A review of the literature indicates a substantial improvement in fetal outcome when parathyroidectomy is done during pregnancy, as in the case reported here. Therefore, parathyroidectomy is the treatment of choice when the diagnosis is made during pregnancy, although oral phosphate therapy may be an alternative if surgery is contraindicated.     

Hypoganglionosis in pregnancy: a case report

ABSTRACT: INTRODUCTION: We report a very rare case of isolated hypoganglionosis first diagnosed during early pregnancy, which should be discussed from an obstetric and a gastroenterological point of view. CASE PRESENTATION: A pregnant 18-year-old Caucasian woman presented at twelve weeks of gestation with lower abdominal pain, mild constipation and a large abdominal mass. Abdominal and pelvic magnetic resonance imaging demonstrated a megarectum and megasigmoid, and our patient was managed with medical therapy during her pregnancy, which occurred without major incidents. At the onset of labor, a fecaloma obstructing the pelvic outlet was detected, which required manual disimpaction. However, during the procedure a sudden continuous fetal bradycardia was detected. An emergency Cesarean section was performed but the fetus suffered hypoxic ischemic encephalopathy. One year after the delivery, our patient underwent a sigmoid resection. A histopathological analysis revealed a reduction of nerve cells in the myenteric and submucous plexus, suggesting hypoganglionosis. CONCLUSION: Although there are some reports of pregnancies complicated by megacolon, they are too few and too old to delineate guidelines for clinical orientation. In our article, we discuss several issues regarding the management of these rare intestinal innervation disorders during pregnancy that we believe will enhance their obstetric and gastroenterological management during pregnancy.     

Diagnosing tuberculosis in pregnancy: a case report

A case of miliary tuberculosis complicated by deciduitis and sub-chorionitis in a pregnant woman manifesting also influenza A/H1N1v infection and urinary tract infection is reported. Diagnosis of tuberculosis was obtained before delivery by examining amniotic fluid for Mycobacterium tuberculosis. Even though maternal symptoms did not suggest TB, diagnosis was early enough to start effective treatment in both the mother and the neonate and prevent in-hospital M.tuberculosis diffusion. A high index of suspicion by health professionals is required to detect and manage tuberculosis in pregnancy and newborns in both the developed and developing word.     

Laparoscopic nephrectomy for pyonephrosis during pregnancy: case report and review of the literature

The maternal and fetal complications of pyonephrosis during pregnancy can be devastating, thus the call for urgent but safe intervention. Laparoscopic nephrectomy has been used safely and effectively in nonpregnant patients with pyonephrotic kidney. We report on a case of a 28-year-old pregnant woman with pyonephrotic kidney that we believe to be the first such case managed by transperitoneal laparoscopic nephrectomy. A review of the reported cases of laparoscopic nephrectomy for different indications and by different approaches during pregnancy is also presented.     

Prosthetic heart valve thrombosis, anticoagulation and pregnancy: a case report and review of literature

In pregnant patients with a prosthetic heart valve (PHV), anticoagulation with warfarin is associated with embryopathy, foetal loss early in pregnancy and maternal bleeding complications in the delivery period. The optimal anticoagulation strategy in the pre-pregnancy period and during pregnancy itself is controversial and a matter of debate. We describe a patient with PHV in the pre-pregnancy period and in a subsequent pregnancy. The optimal anticoagulation treatment strategy in women in their reproductive years with mechanical valve thrombosis is discussed. (Neth Heart J 2007;15: 306-9).     

Abdominal pregnancy in a baboon: a first case report

The abdominal pregnancy is a rare, but life threatening complication of ectopic embryo implantation. Only three cases of abdominal pregnancy have been previously described in primates: in a squirrel monkey, owl monkey and in a rhesus macaque. A 14-year-old wild-caught olive baboon (Papio cynocephalus anubis) was diagnosed at the ultrasound examination with advanced gestational age extrauterine pregnancy. At the initial laparotomy and necropsy the diagnosis of abdominal pregnancy was made on Studdiford's criteria. This case indicates the possibility of developing a model for further study of different types of ectopic pregnancy and indicates a cesarean section as a risk factor for abdominal pregnancy.     

Intrachromosomal insertions: a case report and a review

Summary We describe the phenotype of a child having a recombinant chromosome 3 with a duplication 3q13.2 q25 derived from a paternal inv ins(3)(p25.3q25q13.2). A review of 27 reported cases of intrachromosomal insertions has revealed that for a carrier of intrachromosomal insertion the risk of a child with an unbalanced karyotype is 15%. This risk may be higher for particular insertions. The recombinant chromosome can have a duplication or a deletion of different segments depending on whether the insertion is direct or inverted, paracentric or pericentric, and whether there is meiotic crossing over in the inserted or the interstitial non-inserted segment. Several of the insertions have been difficult to interpret and some of them have been mistaken for paracentric inversions. Caution is therefore indicated in interpreting parental karyotypes of a child with a deletion or a duplication, particularly if it is interstitial. This is because, whereas a risk of recurrence of a child with an unbalanced karyotype is low in de novo cases and for carriers of paracentric inversions, it is high for carriers of insertions.     

Termination of unwanted pregnancy in a llama with cloprostenol and subsequent pregnancy: a case report

A 5-yr-old female llama was presented by its owner for an elective abortion. The llama was accidentally bred to an unknown, and possibly related, male about 2.5 mo prior to presentation. The pregnancy was first confirmed by ultrasonography then cloprostenol (150 microg im) was administered once. Cloprostenol, an analogue of prostaglandin F2alpha, was chosen in preference to natural PGF2alpha due to reported adverse reactions in llamas to this abortifacient. Blood serum progesterone levels decreased rapidly from 5.7 to < 0.2 ng/ml at 0 to 60 h post injection, respectively. The aborted fetus was expelled at approximately 108 h after the injection. Twenty days post abortion the llama was rebred. At 27 and 87 d post breeding, pregnancy was indicated first by male refusal and then by elevated serum progesterone concentrations and was confirmed by ultrasonography. Following a 355 d gestation period, a male cria was born. This case provides evidence that an abortion can be induced with cloprostenol without an adverse effect on future fertility in the llama.     

Peripheral primitive neuroectodermal tumor of the vulva: report of a case with imprint cytology.

BACKGROUND: Peripheral primitive neuroectodermal tumor (PNET) of the vulva is an extremely rare disease, and, to our knowledge, only two cases have been previously reported. CASE: A 45-year-old woman presented with a mass in the right labium major. Three years after removal of the tumor, she noticed a new lesion in the same place and underwent a partial vulvectomy. The imprint cytology of the recurrent tumor showed a monomorphic appearance, composed of small round cells with scant cytoplasm against a hemorrhagic background. These tumor cells were loosely connective, but rosettelike structures were observed focally. On pathologic examination, the neoplasm was composed of small round tumor cells showing sinusoidal, diffuse or micropapillary growth. Immunohistochemically, the neoplastic cells stained positively for neuron-specific enolase, vimentin and HBA 71 and negatively for cytokeratin, HBA 45 and muscle-specific actin. The morphologic characteristics of the disease were well expressed in the imprint cytology, and this influenced the selection of immunohistochemical studies. CONCLUSION: Cytologic examination for vulvar tumors, even imprint cytology, can be a useful tool in obtaining an accurate pathologic diagnosis of a rare disease, such as peripheral PNET.     

Aggressive low grade middle ear adenocarcinoma with multiple recurrences: a case report

Background

Special stains, such as reticulin stain and CD34 immunostain, are very helpful in the diagnosis of well differentiated hepatocellular carcinoma (HCC). Most studies have shown that absent or decreased reticulin stain or an abnormal reticulin pattern with widened trabeculae is reliable for the diagnosis of well-differentiated HCC.

Case report

We report here two cases of well differentiated HCC with an unusual reticulin staining pattern. A strongly positive reticulin network was preserved within the tumor, which surrounded individual tumor cells in a monolayered trabecular pattern. At the same time, an increased CD34 stain was present in the tumor.

Conclusions

This unusual reticulin pattern represents part of the diverse reticulin staining patterns seen in HCC. Although this staining pattern is rare, it should be recognized when diagnosing well-differentiated HCC in small samples such as cellblock of fine needle aspiration or small core biopsies.     

Urethral foreign body management: a case report

The management of urethral foreign bodies may require the use of various surgical techniques in a urologist's armamentarium. We report a unique case of a urethral foreign body requiring percutaneous and endoscopic techniques for removal.     

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.     

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.     

Failed medical management in ovarian pregnancy despite favorable prognostic factors--a case report

Primary ovarian pregnancy is a rare form of ectopic pregnancy that must be demonstrated with use of 4 Spiegelberg criteria. It is usually diagnosed at laparotomy or laparoscopy, although it may resemble a hemorrhagic corpus luteum. Successful conservative management of ovarian pregnancy with methotrexate has been reported only occasionally. This may be partly because of the rarity of this condition and partly because when medical treatment is successful, the patient does not need to undergo laparotomy or laparoscopy, and an occasional ovarian pregnancy may have been diagnosed as a tubal pregnancy. We present a case of ovarian pregnancy (diagnosed at laparotomy) for which initial medical management with methotrexate failed despite favorable prognostic factors. Whether the unusual location (ovary) could have contributed toward treatment failure is unknown.