Acholic Stools Research Articles

Home-based screening for biliary atresia using infant stool colour cards: a large-scale prospective cohort study and cost-effectiveness analysis.

Biliary atresia (BA), a leading cause of paediatric liver failure and liver transplantation, manifests by three weeks of life as jaundice with acholic stools. Poor outcomes due to delayed diagnosis remain a problem worldwide. We evaluated and assessed the cost-effectiveness of methods of introducing a BA Infant Stool Colour Card (ISCC) screening programme in Canada. A prospective study at BC Women's Hospital recruited consecutive healthy newborns through six incrementally more intensive screening approaches. Under the baseline "passive" strategy, families received ISCCs at maternity, with instructions to monitor infant stool colour daily and return the ISCC by mail at age 30 days. Additional strategies were: ISCC mailed to family physician; reminder letters or telephone calls to families or physicians. Random telephone surveys of ISCC non-returners assessed total card utilization. Primary outcome was ISCC utilization rate expressed as a composite outcome of the ISCC return rate and non-returned ISCC use. Markov modelling was used to predict incremental costs and life years gained from screening (passive and reminder), compared with no screening, over a 10-year time horizon. 6,187 families were enrolled. Card utilization rates in the passive screening strategy were estimated at 60-94%. For a Canadian population, the increase in cost for passive screening, compared with no screening, is $213,584 and the gain in life years is 9.7 ($22,000 per life-year gained). A BA ISCC screening programme targeting families of newborns is feasible in Canada. Passive distribution of ISCC at maternity is potentially effective and highly cost-effective.

Aetio-Clinical Profile of Cholestatic Jaundice During Infancy- Study of 30 Cases in a Tertiary Care Hospital

Cholestasis in young infants has a varied etiology. There is considerable delay in presentation of cholestatic cases, both in India (average delay of 3 months in referral centers) and Bangladesh (3.5 months). Early diagnosis is important as the effects of cholestasis are profound and wide-spread. EHBA comprises a significant proportion of cases of cholestatic diseases. If treatment of EHBA is delayed beyond the first 90 days of life, the only option thereafter is liver transplantation, which is not presently feasible on a large scale in developing countries. So, the aim and objective of this study is to determine the cause and to categorize the clinical profile and treatment options of conjugated hyperbilirubinaemia in infancy. A total of 30 patients, who fulfilled inclusion and exclusion criteria were included. A detailed history and physical examination was done daily. Complete blood count, liver function tests, HBsAg and TORCH screening, thyroid function test, Urine was tested for non-glucose reducing substances. Ultrasonography of the hepatobiliary system and hepatobiliary scintigraphy was done. Liver biopsy was done in appropriate patients. Patients were followed up daily during hospital stay. The management and its response were also monitored and recorded. Out of 30 patients 24 (80%) were male and 6 (20%) were female. 63% were term and 37% were preterm. Out of 12 patients in BA, 11 were term and only 1 was preterm, whereas in NH group, out of 18 babies, 10 were preterm. Most of the patients were male in both studied groups. Mean value of birth weight in BA was 2.65±0.13 and in NH was 2.40±0.31. Mean age (days) at onset of jaundice in 2 groups were 3.92 ± 2.43 in BA and 6.5 ±4.5 in NH. Most patients of BA (91%) had persistent acholic stool, whereas in Neonatal hepatitis group 83% had intermittent acholic stool. No statistically significant difference was observed when hepatosplenomegaly and ALT values were considered in 2 studied groups. In BA group 33% & In NH group 72% babies had positivity for CMV infection. Normal ultrasonic findings were seen in 2 patients of BA group, and 7 in NH group. No patient had shown contracted gall bladder after meal in BA, Choledocal cyst was found to be responsible for 5 (42%) patients in BA group and none in NH group. HIDA showed 47% had biliary atresia, 43% neonatal hepatitis and 10.0% had normal liver. Liver biopsy revealed that 12 (40.0%) had biliary atresia and 18 (60.0%) had neonatal hepatitis. Out of 12 babies in Biliary atresia group almost all, 11(92%) received surgical management where as in Neonatal hepatitis group; all 18 babies (100%) received medical treatments. Early detection of cholectatic cases by observing stool colour is very important for physicians to direct the very specific investigations to find out the cause and start appropriate treatment immediately. DOI: http://dx.doi.org/10.3329/bmj.v41i2.18804 Bangladesh Medical Journal 2012 Vol. 41 No. 2: 34-39

An Unusual Cause of Painless Jaundice

Question: A 79-year-old man with type 2 diabetes mellitus, hypertension, dyslipidemia, and benign prostatic hyperplasia presented to our institution with a 2- to 3-week history of progressively worsening painless jaundice. The jaundice was associated with anorexia, a 15-lb unintentional weight loss, pruritus, nausea, and acholic stools. The patient otherwise had no complaints. He denied any fevers, chills, night sweats, diarrhea, or vomiting. The patient denied recent travel and had not started any new medications or over-the-counter herbal remedies.

One-staged or two-staged surgery for perforated choledochal cyst with bile peritonitis in children? A single center experience with 27 cases

To report the authors' experience in the management of perforated choledochal cyst with bile peritonitis (PCC) in children. Medical records of all children undergoing surgery for PCC at our hospital from May, 2005 to January, 2013 were reviewed. Twenty seven patients were identified, with a median age of 2 years. The clinical manifestations were abdominal pain (96.3 %), abdominal distention (92.6 %), vomiting (88.9 %), jaundice (74.1 %), fever (70.4 %), acholic stool (51.8 %), abdominal tenderness (92.6 %), peritoneal signs (74.1 %), and palpable mass (18.5 %). Duration of the acute symptoms ranged from 1 to 30 days (median 3 days). Median size of the choledochal cysts was 3 cm (range 1.5-18 cm). Before January 2008, four cases were treated by two-staged surgery and only one case by one-staged surgery. Since then, all remaining 22 cases were treated by the one-staged surgery. There was no anastomotic leakage and all patients were discharged in good health. The mean postoperative stay was 7.6 ± 2.5 days for the one-staged group. No complication was noted at a median follow-up of 24 months. One-staged definitive repair is feasible, safe and should be the treatment of choice for most cases of childhood PCC in experienced centers.

Biopsy-driven diagnosis in infants with cholestatic jaundice in Iran

To determine the frequencies of diagnoses confirmed by liver biopsy in infants with cholestasis in an Iranian pediatric hospital. This was a retrospective study conducted in a tertiary referral children's hospital in Iran. We retrieved all pathology reports of liver biopsies from children less than two years of age who had presented for evaluation of cholestatic jaundice from March 2001 to March 2011. Additional specimen samples obtained from archived pathology blocks were reviewed by a pathologist blinded to the final diagnosis. These results were compared with the pathology reports from chart records to ensure consensus and eliminate any inconsistencies in final diagnoses. A structured checklist was used to gather information on multiple variables including age, sex, gestational age at birth, birth weight, age at which hyperbilirubinemia manifested, presence and identification of associated anomalies, clinical manifestations, and histological findings from liver biopsies. The baseline data are reported using descriptive statistics, and differences between groups were assessed by Fisher's exact test and Student's t test when indicated. Fifty-five cases (28 females; 27 males) of infantile cholestasis (IC) were included in this study. The mean serum total bilirubin and direct bilirubin at presentation were 13.6 ± 5.9 and 7.3 ± 3.4, respectively. Forty cases (72.7%) were the product of term pregnancies. Common associated clinical findings were acholic stool in 33 cases (60.0%), hepatomegaly in 30 cases (54.5%), and dark-colored urine in 21 cases (38.2%). Biliary atresia (BA) was the most frequent diagnosis, found in 32 cases (58.2%), followed by intrahepatic bile duct paucity found in 6 cases (10.9%), metabolic disease in 6 cases (10.9%), idiopathic neonatal hepatitis in 5 cases (9.1%), choledochal cyst in 2 cases (3.6%), liver cirrhosis in 2 cases (3.6%), and progressive familial intrahepatic cholestasis and portal fibrosis each in 1 case (1.8%). The mean times for jaundice onset and liver biopsy were 43.8 and 102.0 d, respectively. In BA, the mean age at jaundice presentation was 21 d and for liver biopsy was 87.5 d, representing a mean delay of 66.5 d. A significant delay was found between IC presentation and liver biopsy, which is detrimental in conditions that can cause irreversible liver damage, such as BA.

Diagnosis of Biliary Atresia Can Not Be Excluded by Declining Trend of Serum Direct Bilirubin

The aim of this paper is to investigate the clinical courses of patients with biliary atresia (BA) during neonatal period. We examined 19 patients with BA, who underwent blood tests including direct bilirubin (D-Bil) within 20 days of age, in 3 tertiary hospitals in Japan. The first blood sample was collected at 8.4±6.5 days of age. The acholic stool was observed within 2 weeks of age in 16 cases (84.2%). Decrease of T-Bil was observed in all the subjects, with a range of reduction of 6.5±3.3 mg/dL, from 10.4±7.5 to 29.8±9.1 days of age. Decrease of D-Bil was also observed in 17 out of 19 cases (89%), with a range of reduction of 1.1±1.0 mg/dL, from 15.5±8.0 to 24.9±9.6 days of age. A significant decrease of D-Bil was observed in 2 cases of biliary atresia splenic malformation syndrome. We therefore conclude that clinicians treating icteric infants should not exclude a diagnosis of BA even if the level of D-Bil has a declining tendency.

AugmentinTM-induced DILI: A Case of Severe Cholestatic Jaundice with Spontaneous Resolution

Introduction: Drug-induced liver injury (DILI) is a major cause of iatrogenic illness, and may present with an array of histologic signatures. Establishing a diagnosis of DILI requires a careful exclusion of other etiologies and an awareness of the hepatotoxicity profile of suspected agents. Case: A 70-year-old man with a history of HTN, CAD, AF, and BPH was referred for a six-day history of intense pruritus, associated with jaundice, dark urine, and acholic stool. He denied any abdominal pain, weight loss, recent travel, fevers/chills, or sick contacts. Physical exam revealed a jaundiced patient with no cutaneous stigmata of chronic liver disease, and no hepatosplenomegaly, ascites, or asterixis. On presentation, his LFT's were as follows: T bili 7.5, D bili 4.6, AST 34, ALT 45, ALP 232, GGTP 63, alb 3.8, and normal INR. Serologies revealed a negative HAV IgM, HBSAg, HB core IgM, HCV PCR, ANA, SMA, AMA, anti-LKM, P-ANCA, ACE, and normal immunoglobulins. Doppler U/S revealed a normal liver morphology with a small hepatic cyst, patent hepatic vessels, normal gallbladder, no biliary ductal dilation and no hepatosplenomegaly or ascites. MRCP revealed a normal biliary tree with no pruning. Within 10 days of presentation, his T bili peaked at 20.9 with an ALP of 274. On further inquiry, the patient recalled a two-week course of amoxicillin/clavulanate (AugmentinTM) for a URI, with his symptoms beginning one week after discontinuation of the antibiotics. A liver biopsy revealed mild cholestasis, spotty necrosis/apoptosis, lymphocyte, neutrophil, and eosinophil infiltration consistent with a drug-induced hepatitis. There was no bile duct damage, ductopenia, granulomas, or fibrosis. The patient was prescribed cholestyramine four g BID for pruritus, and urso 250 mg BID. Within eight weeks, his LFT's had completely normalized. Discussion: Amoxicillin/clavulanate- induced DILI is estimated to occur with an incidence of 17/100,000 prescriptions, and the clavulanic acid component is the likely injurious agent. This idiosyncratic reaction is thought to be immunoallergic in origin, and autoantibody formation is uncommon. Men over 50 years of age and prolonged/repeated courses of AugmentinTM are associated with an increased risk of DILI. Although cases of acute liver failure and chronic ductopenia have been described with AugmentinTM, a delayed cholestatic or a mixed cholestatic-hepatocellular liver injury pattern is the norm, and usually resolves within two months. Despite our patient being an elderly male who developed marked jaundice, his course was ultimately benign, with full resolution of symptoms.

Spontaneous perforation of a choledochal cyst, clues for diagnosis

Aim: Spontaneous perforation is a rare complication of a choledochal cyst that is difficult to diagnose because of its nonspecific clinical presentation. These patients usually present with an insidious course characterized by progressive abdominal distension, vomiting, and pain. Here, we present a series of three cases for clues for diagnosis. Materials and methods: All patients with spontaneous perforation of a choledochal cyst who were presented to our institute from January 2009 to December 2011 were included in the study and symptoms and signs at the time of presentation were analyzed. Results: All patients had clinical jaundice and acholic stools at the time of presentation; pain and vomiting was not a consistent feature. Abdominal paracentesis helped us diagnose these cases. Conclusion: Abdominal paracentesis is very useful in cases of suspected spontaneous perforation of the bile duct. Simple drainage with a T-tube, if possible, is safe, efficacious, has low morbidity, and aids in the establishment of the diagnosis as well as delineation of the anatomy of the concerned region for definitive surgery. Keywords: Choledochal Cyst, Spontaneous Perforation of Bile Duct, T-tube

Role of myeloid differentiation factor 88 in Rhesus rotavirus-induced biliary atresia

BackgroundBiliary atresia (BA) is a unique neonatal disease resulting from inflammatory and fibrosing obstruction of the extrahepatic biliary tree. Previous studies have demonstrated the critical role of innate immunity and the Th1 response to activated inflammatory cells and overexpressed cytokines in the pathogenesis of BA. Myeloid differentiation factor 88 (MyD88) is a critical adaptor molecule that has been shown to play a crucial role in immunity. We investigated the role of MyD88 in the inflammatory response and development of cholangiopathy in murine BA. MethodsMyD88 knockout (MyD88−/−) and wild-type (WT) BALB/c pups were injected with Rhesus rotavirus or saline on day 1 of life. The mice were monitored for clinical symptoms of BA, including jaundice, acholic stools, bilirubinuria, and death. The liver and extrahepatic bile ducts were harvested for histologic evaluation and the quantification of viral content, determination of cytokine expression, and detection of inflammatory cells. ResultsRhesus rotavirus infection produced symptoms in 100% of both MyD88−/− and WT pups, with survival of 18% of WT and 0% of MyD88−/− mice. Histologic analysis demonstrated bile duct obstruction in both MyD88−/− and WT mice. Viral titers obtained 7 d after infection and expression of interferon-γ and tumor necrosis factor-α at day 3, 5, 8, and 12 after infection revealed no significant differences between the WT and MyD88−/− mice. Flow cytometry demonstrated similar levels of activated CD8+ T cells and natural killer cells. ConclusionsThe pathogenesis of murine BA is independent of the MyD88 signaling inflammatory pathway, suggesting alternative mechanisms are crucial in the induction of the model.

Persistent Hyperbilirubinemia in a Newborn

172 Volume 28 Number 2 of a tertiary children’s hospital. Jaundice was originally noted at birth; she was believed to have physiologic jaundice of the newborn and was discharged home on day 2 of life. The mother reported that the newborn had evidence of conjugated hyperbilirubinemia on day 5 of life. Subsequently, she began passing acholic stools. The patient was the product of an uncomplicated full-term pregnancy. She was delivered vaginally and had a birth weight of 3.54 kg. The patient’s family had a history of asthma (mother) and elevated liver enzymes of an unknown cause (father). The patient had not yet received any vaccinations.

Early detection of neonatal cholestasis by stool color card screening.

The objective of this study was to identify, by early screening, one-month old infants with acholic or hypocholic stools for the early detection of biliary atresia and other causes of neonatal cholestasis. The study was essentially exploratory (observational, prospective, statistically underpowered and with no clinical intervention) and used a color card screening of all newborns attending the first month of life checkup from 1999 to 2002 in a public hospital in Argentina. Of 12 484 newborn infants, 4239 were examined at their first month of life checkup visit with the stool color card. Eighteen infants were identified with hypocholic stools, of whom only four were proven to have cholestasis. Although no case of biliary atresia was detected, screening by stool color cards proved useful for the detection of other causes of neonatal cholestasis.

Neonatal spontaneous bile duct perforation presenting as giant intraabdominal cyst

We present a case of neonatal spontaneous bile duct perforation in a previously healthy neonate presenting acutely at 2 weeks of age followed by abdominal distention, acholic stools and obstructive jaundice. Ultrasound and CT scan showed a giant intraabdominal cyst of unknown origin. Exploratory laparotomy revealed a tense lesser sac pseudocyst containing bile with compression and displacement of adjacent structures. The patient underwent partial excision and drainage of the cyst uneventfully with full recovery.

Conjugated Hyperbilirubinemia in Children

A variety of anatomic, infectious, autoimmune, and metabolic diseases can lead to conjugated hyperbilirubinemia, both in the newborn period and later in childhood. The pediatric practitioner is most likely to encounter conjugated hyperbilirubinemia in the neonatal period.It is crucial to maintain a high degree of suspicion for cholestasis in the persistently jaundiced newborn. The goal is recognition of conjugated hyperbilirubinemia between 2 and 4 weeks after birth, allowing for the prompt identification and management of infants who have biliary atresia, which remains the most common cause of neonatal cholestasis.

Nasobiliary Drainage in an Episode of Intrahepatic Cholestasis in a Child With Mild ABCB11 Disease

Nasobiliary Drainage in an Episode of Intrahepatic Cholestasis in a Child With Mild <i>ABCB11</i> Disease

Common bile duct stones in infancy: A medical approach

Symptomatic choledocholithiasis in infancy is not common. It usually presents with jaundice and acholic stools and is diagnosed on abdominal ultrasonography. Favourable outcome of conservative management has been reported, but specific management guidelines are not well defined in the literature. We describe three cases using a combination of ursodeoxycholic acid and antibiotics as a treatment paradigm, which could potentially negate more invasive treatment. All three patients had ultrasonography proven choledocholithiasis with concomitant obstructive liver function test. They were treated with a combination of ursodeoxycolic acid and antibiotics. Patient 1 had an Escherichia coli urinary tract infection and was treated with oral bactrim. Intravenous amoxicillin, gentamicin and metronidazole were used for the other two patients. All three patients responded with a return to normal-coloured stools within 48 h of combination treatment. Repeat ultrasonography done within 11 days after the first study for all three patients confirmed complete resolution of choledocholithiasis. It is postulated that this improvement is as a result of a reduction in inflammation and oedema, associated with a low-grade cholangitis, following antibiotic treatment. This is coupled with improved bile flow with ursodeoxycholic acid therapy. The findings suggest the potential application of this safe, non-invasive therapeutic strategy as initial management in infants with this condition. A follow-up prospective randomised controlled trial may be an answer to prove the validity of this observation but due to the rarity of this problem, it would be a challenge to recruit sufficient number of patients.

A 42–Year–Old Woman With a New Diagnosis of Sclerosing Cholangitis

A 42-year-old woman is referred to you for severe pruritus, dark urine, and acholic stools. These symptoms have gradually increased during the past 7–10 days. She had previously been in good health with no history of hepatobiliary dysfunction. She complains of right upper quadrant discomfort and a general sense of malaise. Blood tests reveal a normal complete blood count, but her liver enzymes are elevated in a cholestatic pattern. Her alkaline phosphatase level is approximately 3 times the upper limit of normal (ULN) at 428 U/L, the aspartate aminotransferase level is 1.5 times the ULN at 116 U/L, and the total serum bilirubin is 6.4 mg/dL, with a direct bilirubin of 4.8 mg/dL.

Infants With Cholestasis: Diagnosis, Management And Outcome

Objective: Infants with cholestatic jaundice were evaluated retrospectively in terms of etiologies, diagnostic methods, laboratory findings, treatment procedures and longterm prognosis. Patients and Methods: The study consisted of 70 children (52.8% male, 47.1% female) with cholestasis ranging in age from 15 days to 8 months (mean age, 60±26 days). Patients were divided into three groups according to the diagnosis: (i) patients with extrahepatic biliary atresia, (ii) patients with intrahepatic biliary hypoplasia, and (iii) patients with hepatocellular disease. Their clinical parameters were evaluated. Results: In the group with extrahepatic biliary atresia the onset of jaundice was significantly earlier and the presence of acholic stool and total bilirubin levels were remarkably higher than in the groups with intrahepatic biliary hypoplasia or hepatocellular disease. Serum gamma-glutamyl transpeptidase (GGT) and alkaline phosphotase (ALP) levels were found to be significantly higher in the groups with extrahepatic biliary atresia and intrahepatic biliary hypoplasia than the group with hepatocellular disease (p<0.001 and p<0.01, respectively). The contribution of technetium-99m (99mTc) scintigraphy to the diagnosis was significantly higher in the group with extrahepatic biliary atresia than the groups with intrahepatic biliary hypoplasia and hepatocellular disease (p<0.002). Conclusion: It was found that cholestasis, acholic stool and elevated GGT are better markers for extrahepatic biliary atresia than for intrahepatic biliary hypoplasia or hepatocellular disease in infants. The contribution of scintigraphy to the diagnosis was found to be higher in the group with extrahepatic biliary atresia than in the other groups. (Marmara Medical Journal 2012;25:83-6)

Primary Non-Hodgkin’s Lymphoma of the Extrahepatic Bile Duct

Since the first reported case of primary non-Hodgkin’s lymphoma (NHL) of the extrahepatic bile duct in 1982, only 20 cases have been reported in the English literature [1–21]. Moreover, the follicular subtype of lymphoma was noted in only three of these cases [10, 19–21]. The previous 20 patients presented with signs of biliary obstruction such as jaundice, pruritis, dark urine, or acholic stools. In the majority of cases, a computed tomogram (CT) of the abdomen prior to diagnosis documented either a mass in the right upper quadrant or biliary dilatation. Subsequently, treatment options included palliative and curative procedures including endoscopic stents, percutaneous drainage, and surgery. Chemotherapy and radiation treatment have been offered as well. This report describes the 21st case of primary lymphoma of the extrahepatic biliary system and reviews the previous 20 cases as well. Case Report

Bupivacaine Induced Hepatotoxicity after Continuous Infusion for Pain Control

Purpose: Background: Bupivacaine is increasingly used as a local anesthetic. Hepatotoxicity from this medication is not well recognized. We present a case report of drug-induced liver injury from bupivacaine use. Case: 51 year old white male without a significant PMH except dyslipidemia was admitted to the hospital for an elective hernia repair with mesh placement. Prior to hospital admission he was taking simvastatin 20 mg for about 10 years without clinical or laboratory side effects. He denied alcohol use. On admission, complete blood cell count, basic metabolic panel and liver function tests were within normal range. A Q-pump with continuous bupivacaine infusion was placed at the site of surgery and continued for 3 days to control pain. A total dose of 770 mg was received. After discharge, the patient experienced dizziness, diaphoresis and hypotension. Symptoms resolved after the Q-pump was stopped. Ten days after, he presents with increasing fatigue, pruritus, dark urine and acholic stools. CBC and LFTs were abnormal (Table). Simvastatin was discontinued. Liver ultrasound and CT scan plus serologic markers for acute viral hepatitis were negative. Symptoms and LFTs continued to worsen (Table). Additional work up for chronic liver disease was negative. A liver biopsy showed mild mixed portal inflammation, low grade ductular reaction, hepatocanalicular cholestasis and mild portal and periportal fibrosis. Repeat LFTs returned to normal at 7 weeks and symptoms resolved. Discussion: Bupivacaine hydrochloride is a local anesthetic, increasingly used in ambulatory operative anesthesia and in the treatment of chronic pain due to low incidence of transient neurological syndrome and improved analgesic effect. It is metabolized in the liver via conjugation with glucuronic acid. We present a case of bupivacaine induced hepatotoxicity based on the temporal association of symptoms with the introduction of bupivacaine, the exclusion of other risk factors for hepatotoxicity, and the improvement with withdrawal of the medication. Lack of laboratory or clinical side effects to simvastatin prior to surgery and the laboratory pattern of cholestatic hepatitis makes simvastatin an unlikely cause of this liver injury. Development of dizziness, hypotension and esoinophilia suggest an allergic mechanism. Local infiltration of the medication in the wound area may explain its prolonged toxicity. Competitive inhibition of CYP3A4 may account for bupivacaine-simvastatin interaction and enhanced toxicity. A previous case report of cholestatic hepatitis from continuous epidural block with bupivacaine suggest that bupivacaine causes cholestatic hepatitis pattern of liver injury when used locally and epidurally.Table 1: Liver function test at 3 weeks and 4 weeks post bupivacaine infusion

Chest Wall Ewing Sarcoma Presenting as Abdominal Pain in a 12-Year-Old Boy

A 12-year-old boy presented with a 3-month history of right upper quadrant abdominal pain, a 15%-20% loss of body weight, and a 3-week history of cough and dyspnea. He did not have jaundice, fever, acholic or melanotic stools, or nausea/vomiting. Respirations were 40/minute and regular, and heart ratewas 125 beats/minute. Heart sounds and point of maximum impulse were displaced to the left. He exhibited increased work of breathing, with absent breath sounds on the right side and diffuse crackles on the left side. Examination revealed a palpable 10 8 cm, firm, immobile, nontender subcutaneousmass protruding from the right side of the back. Laboratory studies were significant only formild normocytic anemia (hemoglobin, 9.4 g/dL, mean corpuscular volume, 75 fL) and mildly elevated serum lactic dehydrogenase (422 U/L) and uric acid (7.3 mg/dL) levels. Imaging revealed a large mass in the right hemithorax with associated pleural effusion, mediastinal shift, and protrusion posteriorly through the ribcage (Figure, A, B, and C). Biopsy analysis showed a small, round blue cell tumor strongly positive for CD99