Bleeding from small bowel neoplasms account for 1–4% of cases of upper gastrointestinal haemorrhage. Renal cell carcinoma constitutes 3% of all adult malignancies and often presents insidiously. Consequently 25–30% of patients have metastases at the time of diagnosis. Gastrointestinal bleeding from renal cell carcinoma metastases is an uncommon and under-recognised manifestation of this disease.In this report we describe two cases of gastrointestinal bleeding from renal cell carcinoma metastases – in one patient bleeding heralded the primary manifestation of disease and in the other signified recurrence of disease following nephrectomy.These cases highlight the importance endoscopic vigilance in cases of undiagnosed upper gastrointestinal haemorrhage, especially in patients with a past history of renal cell carcinoma. It is estimated that in 7–25% of patients who undergo endoscopy for suspected upper gastrointestinal haemorrhage, no source of bleeding is identified . MalignaRenal cell carcinoma (RCC) behaves unpredictably and has a diverse range of clinical manifestations. Patients often present insidiously with vague abdominal symptoms – the classic triad of haematuria, loin pain and abdominal mass is found in only 4–17% ,4. AccorMetastases to the pancreas and small intestine in RCC are rare, but can present as gastrointestinal bleeding ,7. We deA 67 year-old male with a past history of angina and arthritis presented with a one week history of melaena and lethargy. He was found to be anaemic . Endoscopy revealed moderate haemorrhagic gastritis and a Campylobacter-like organism (CLO) -positive duodenal ulcer, but no signs of recent bleeding. His non-steroidal anti-inflammatory medication was stopped and eradication therapy prescribed. He remained an in-patient for one week, during which time seven units of blood were transfused to correct his Hb to 11.6 g/dl.Six weeks later he presented with abdominal pain and further melaena requiring a 2-unit blood transfusion. Endoscopy revealed mild antral gastritis and a normal duodenum but no source of bleeding. Colonoscopy was normal. Two weeks later endoscopy was repeated which showed a small amount of fresh blood in the duodenal bulb, but no obvious lesion was seen to account for the bleeding.Three weeks later, he remained symptomatically anaemic, requiring further blood transfusion. Because of the history of melaena he underwent a fourth endoscopy which revealed a small, highly vascular polyp in the duodenum just beyond the angulus which bled easily on contact. Multiple biopsies were taken. The endoscopist was concerned about the possibility of a pancreatic neoplasm eroding into the duodenum and therefore an abdominal CT was arranged. Surprisingly, this demonstrated a large left renal mass with evidence of left adrenal and lung metastases, and a polypoidal mass in the medial wall of the second part of the duodenum arising from the pancreas Figure . HistoloOver the three month period from initial presentation to diagnosis he required transfusion of a total of twenty units of blood for recurrent symptomatic anaemia. Given the history of recurrent melaena, coeliac angiography was undertaken with a view to trans-catheter embolisation. This was performed successfully via occlusion of the anterior and posterior pancreaticoduodenal vessels using coils Figure  and 2B.He subsequently underwent palliative left nephrectomy and histology from the resected specimen confirmed a grade 2 clear cell variant of RCC. He completed a course of interferon therapy and was able to lead a normal life for approximately 18 months, with no recurrence of anaemic symptoms or melaena. He subsequently developed painful maxillary metastases for which he received a course of radiotherapy, but died at home nearly two years after his initial presentation.A 75 year old male with a past history of left nephrectomy 9 years previously for RCC was referred for out-patient investigation of iron-deficiency anaemia . Gastroscopy revealed a CLO-negative duodenal ulcer. Duodenal biopsies were within normal histological limits. Proton-pump inhibitor and iron therapy was prescribed and the patient was scheduled for review in the out-patient department.He remained anaemic three months later and repeat gastroscopy and a colonoscopy were arranged. Colonoscopy was normal, but gastroscopy revealed a fleshy vascular polyp in the duodenal bulb which was biopsied. A CT scan of the chest and abdomen, and a bone scan revealed a pancreatic mass invading the duodenum Figure  but no mThe patient was unable to tolerate interferon therapy. He became jaundiced due to tumour invasion of the distal common bile duct which was managed successfully by endoscopic biliary stenting. He received a total of 13-units of blood for symptomatic anaemia around the time metastatic disease was diagnosed, but since being commenced on a proton pump inhibitor his haemoglobin has remained stable.RCC accounts for 3% of adult malignancies. It arises from the proximal tubular epithelium of the kidney and has male preponderance (M:F 2:1). Mean age at presentation is 50–70 years. Both sporadic and hereditary forms exist which are associated with genetic abnormalities on the short arm of chromosome 3 (3p). In the United Kingdom the incidence has increased by 22% over 10 years while in the United States there has been an increase of 50% in 30 years . Deaths Multiple risk factors include increased age, smoking, obesity, long-term dialysis, exposure to asbestos, petroleum products and cadmium, and several genetic syndromes including familial clear cell carcinoma, von Hippel-Lindau syndrome, and tuberous sclerosis .Spread in RCC is lymphatic, haematogenous, transcoelomic, or by direct invasion. The most common sites of metastasis in RCC are the lung (75%) and lymph nodes (36%) followed by the bones (20%) and liver (18%) . PancreaAutopsy series suggest only 2% of all tumours metastasise to the small intestine – RCC make up 7.1% of these lesions . A serieOf all intestinal segments involved RCC metastasis to the duodenum occurs least frequently . The oveGastrointestinal bleeding as the presenting symptom of a primary renal cell carcinoma is described rarely in the literature ,24. BleeHere we present a rare case of RCC whose primary manifestation of disease was with symptoms of upper gastrointestinal bleeding related to pancreatic metastases invading the duodenum. The source of bleeding in this case was obscure and initially missed by conventional gastroscopy – earlier diagnosis may have been facilitated using a side-viewing endoscope as described previously in a case of RCC metastasizing to the duodenal ampulla . BleedinWe also outline a case presenting 9 years after nephrectomy for RCC with isolated pancreatic and duodenal metastases causing intestinal bleeding. This is unusual as a recent study suggests the majority (83%) of tumours recur within 2 years of curative surgery, with higher recurrence rates in tumours of increasing size . HoweverPalliative nephrectomy may alleviate local symptoms and can be undertaken in selected cases, but should be weighed against the burden of surgical morbidity and mortality, and is not justifiable for inducing disease regression which occurs in < 1% of patients . OutcomeGastrointestinal bleeding from intestinal metastasis may present with overt haematemesis or signs of occult blood loss. Due to its rarity, small intestinal metastasis is often not suspected as a cause of gastrointestinal bleeding. In this report, we hope to alert clinicians to the possibility of intestinal metastasis, or local small bowel invasion from pancreatic metastasis, as a cause for gastrointestinal bleeding in patients with a past history of RCC, regardless of the time since nephrectomy. We present a rare case of RCC whose primary manifestation was with gastrointestinal bleeding. We highlight the importance of endoscopic vigilance in correctly diagnosing the source of bleeding from the upper gastrointestinal tract, and demonstrate the effective use of selective angiographic embolisation in controlling intestinal bleeding from metastatic RCC.The author(s) declare that they have no competing interests.GJS has drafted the manuscript. MA has reviewed the manuscript. PGW has reviewed the manuscript and was consultant in charge of care of the two patients. MSM performed the radiological investigations and angiographic embolisation and has reviewed the manuscript.The pre-publication history for this paper can be accessed here:


Leiomyomatosis peritonealis disseminata (LPD) is a rare disease in which multiple smooth muscle or smooth muscle-like nodules develop subperitoneally in any part of the abdominal cavity. No reports of multiple congenital malformations associated with LPD have been found in the English literature.A 27 year-old patient referred to our gynaecology unit for pelvic pain, amenorrhoea, stress incontinence, chronic constipation and recurrent intestinal and urinary infections. Multiple congenital malformations had previously been diagnosed. Most of these had required surgical treatment in her early life: anorectal malformation with rectovestibular fistula, ectopic right ureteral orifice, megadolichoureter and hemisacrum.An ultrasound scan and computed tomography performed in our department showed an irregular, polylobate, complex 20 cm mass originating from the right pelvis that reached the right hypochondrium and the epigastrium. The patient underwent laparotomy. The three largest abdominal-pelvic masses and multiple independent nodules within the peritoneum were progressively removed. The histological diagnosis was of LPD.The case we report is distinctive in that a rare acquired disease, LPD, coexists with multiple congenital malformations recalling a particular subgroup of caudal regression syndrome: the Currarino syndrome. Leiomyomatosis peritonealis disseminata (LPD), also known as diffuse peritoneal leiomyomatosis, is a rare disease in which multiple smooth muscle or smooth muscle-like nodules develop subperitoneally in any part of the abdominal cavity . These ne.g. pregnancy, prolonged exposure to oral contraceptives and/or combined hormonal replacement therapy, granulomatous cell tumours of the ovary) [The aetiology is thought to be smooth muscle metaplasia of the subperitoneal mesenchyme . About 1e ovary) ,6, indice ovary) ,7. Altere ovary) .We report a case of a patient affected by LPD in whom multiple congenital malformations recalling Currarino syndrome  were previously diagnosed. CS is a rare form of caudal regression syndrome (CRS), consisting in anorectal malformation (ARM), sacral bone deformity and presacral mass . The genA 27 year-old nulliparous Caucasian female was referred to our Gynaecology Unit with a 7-month history of pelvic pain and amenorrhoea. She also complained of stress incontinence, chronic constipation and recurrent intestinal and urinary infections. Her previous history was characterised by several congenital malformations, most of which had required surgical treatment. Neonatally, she had undergone anoplasty for an anorectal malformation with a rectovestibular fistula. Two months after birth, a laparotomy showed a hydronephrosis caused by obstruction of the ectopic right ureteral orifice, which had also caused megadolichoureter and recurrent urinary sepsis. A cutaneous ureterostomy was performed to improve renal function. However, two months later, she underwent a further laparotomy following an urography showing decreased renal function. Right nephrectomy was performed. The histological diagnosis was chronic pyelonephritis.Furthermore, she had been diagnosed with right hand thumb torsion of the metacarpal-phalangeal articulation with hypotrophy of the tenar eminence, valgus and hemisacrum  , and a bAn ultrasound performed in our department showed a complex mass originating from the right pelvis that reached the right hypochondrium and the epigastrium. The transverse extension was more than 20 cm. Fluid was noted in the Douglas pouch. Computerized tomography (CT) of the abdomen and pelvis confirmed a suspicious irregular polylobate complex mass of 24 × 19.5 × 13.5 cm; uterus and adnexa were not identified. Chest X-ray and CT were negative for pleural effusion or lung metastasis.The patient underwent laparotomy. The three largest abdominal-pelvic masses and multiple smaller independent nodules within the peritoneum were progressively removed Figure . The thrHistological examination of the pelvic mass exhibited multiple smooth muscle-like nodules with low cellularity, no cytological atypia and few mitoses Figure . In someThe final diagnosis, confirmed by two independent pathologists with expertise on smooth-muscle cell tumours, was LPD.NM 005515), except for the variant *164^65insC in the 3' UTR. This variation was also present in pooled DNA from 10 healthy individuals, demonstrating that it is a benign polymorphism.Among the features typical of CS, our patient presented only hemisacrum and anorectal malformation. These findings suggested that the patient might have harboured a pathological mutation in the HLXB9 gene. We therefore performed Polymerase Chain Reaction (PCR) amplification of the three exons, intron-exon boundaries, intron 2, and the entire 5' and 3' untranslated regions (UTR) of the gene. The amplified PCR products were sequenced and analysed using a CEQ2000XL DNA Analysis System . We identified no variations with respect to the reference sequence . Sometimes patients may present with mostly non-specific symptoms, such as irregular, heavy uterine bleeding and pain or a mass in the lower abdomen , discomfSonographic and CT findings reported in the literature include non-specific, solid, and complex soft tissue masses that are often large and mimic a leiomyomatous uterus. In some cases, the masses grow in a fashion similar to that of normal uterine parenchyma, whereas others demonstrate heterogeneous enhancement. Diagnosis may be confused with peritoneal carcinomatosis if the masses are present diffusely throughout the abdomen and pelvis. Peritoneal carcinomatosis, however, is often associated with tumour cake, ascites and liver metastases, which have not been reported with LPD .Magnetic Resonance (MR) findings include masses similar in signal intensity to skeletal muscle or uterine parenchyma, and when sarcomatous transformation occurs these are not significantly different from the features of benign implants. If the masses are located in the pelvis adjacent to the iliac vessels, they may be confused with lymphadenopathy ,14,30-33Sometimes, LPD may recur in patients taking HRT ,24,25 evMalignant transformation of LPD is uncommon and only ten cases have been documented in the English literature ,29. Of tOn the basis of these observations, we can affirm that no established guidelines exist regarding the management of LPD. However, therapy needs to be individualised according to the patient's age, hormonal and reproductive status and symptomatology. Different drugs  have been considered in some cases but with poor results. If intestinal and bladder mass effect symptoms are prominent, a surgical approach is indicated .We report a case of LPD diagnosed in a young lady who has never used oral contraceptives and was referred to our department because of pelvic pain, amenorrhoea, stress incontinence and chronic constipation. This case is unusual in the coexistence of this rare acquired condition with multiple congenital malformations, which had been diagnosed in our patient during her early life. A MEDLINE search of the English language literature revealed no report of associations between multiple malformations and LPD.Most of the congenital malformations diagnosed in our patient seem to indicate a particular subgroup of the caudal regression syndrome (CRS): the Currarino syndrome (CS). CRS is a congenital heterogeneous constellation of caudal anomalies that include varying degrees of agenesis of the spinal column, anorectal malformations, genitourinary anomalies and pulmonary hypoplasia. The combination of hemisacrum, anorectal malformation (ARM), and presacral mass constitutes CS . The hemCurrarino's syndrome is phenotypically very variable. It has been classified into three different types :- Complete: full expression, presenting with hemisacrum, anorectal malformation and presacral mass;- Mild: hemisacrum and only one of the other malformations ;- Minimal: only hemisacrum is present.Girls are more commonly affected (80% of cases). Complete CS is commonly diagnosed in the first decade of life, whereas mild or minimal CS is diagnosed predominantly in adults ,42-44. GThe absence of mutations within the coding sequence of the HLXB9 gene does not totally exclude the possibility that our patient might represent an atypical sporadic case of CS. First, the presence of hemisacrum, anorectal malformation, long-term chronic constipation and hydroureteronephrosis represent typical features of mild CS. Second, in contrast to the familial form of CS, in which almost all patients show mutations in the HLXB9 gene, mutations are much rarer in sporadic cases -47. MutaAlternatively, since previous studies excluded the involvement of HLXB9 in CRS  and we fThe case we report is distinctive in that a rare acquired disease, LPD, coexists with multiple congenital malformations recalling a particular subgroup of the caudal regression syndrome: the Currarino syndrome (CS). Whether this association is coincidental or the expression of a common aetiopathogenetic mechanism remains to be ascertained.LPD: Leiomyomatosis peritonealis disseminataCS: Currarino SyndromeCRS: Caudal Regression SyndromeARM: Anorectal MalformationCT: Computerised TomographyER: Oestrogen ReceptorPR: Progesterone ReceptorEMA: Epithelial Membrane AntigenPCR: Polymerase Chain ReactionHRT: Hormone Replacement TherapyLH: Luteinizing HormoneMR: Magnetic ResonanceThe author(s) declare that they have no competing interests.CN performed surgical treatment for LPD and wrote the manuscript. ADSS conceived of the case report, reviewed the literature and wrote the manuscript. VDM conceived of the case report, reviewed the literature and wrote the manuscript. GB performed surgical treatment for LPD and wrote the manuscript. EM carried out the cytogenetic analysis and wrote the genetic section of the manuscript. AS performed surgical treatment for some of the patient's congenital malformations and provided data on her childhood. CM carried out the histological and immunohistochemical examinations. VC carried out the cytogenetic analysis and wrote the genetic section of the manuscript. MG performed surgical treatment for LPD and reviewed the literature.All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Vacuum-assisted closure (VAC) is an acknowledged method of treating wound healing disorders, but has been viewed as a contraindication in therapy of intraabdominal fistulas.th postoperative day a leakage of the ureteroileal anastomosis appeared that cannot be managed by surgical means. To stopp the continued leakage we tried a modified VAC therapy with a silicon covered polyurethane foam under a suction of 125 mmHg. After 32 days with regularly changes of the VAC foam under general anesthesia the fistula resolved without further problems of ureteroileal leakage.We present the case of an 83-year old patient with ureteroileal anastomotic insufficiency following cystectomy and urinary diversion by Bricker ileal conduit due to urothelial bladder cancer. After developing an open abdomen on the 16We present the first report of VAC therapy successfully performed in urinary tract leakage after surgical treatment of bladder cancer. VAC therapy of such disorders requires greater care than of superficial application to avoid mechanical alterations of internal organs but opens new opportunities in cases without surgical alternatives. In 1995 Morykwas and Argenta introduced vacuum-assisted closure (VAC) into the management of complex wound healing disorders . The prerd postoperative day, wound drains showed fecal secretion and relaparotomy was undertaken. Intraoperatively, massive adhesions and ileoileal anastomotic insufficiency were found. Extensive lavage of the abdomen was performed, the former ileoileal anastomosis resected and reconstructed. Severe wound healing disorder occurred resulting in an open abdomen. A further relaparotomy and extensive lavage were performed on the 9th postoperative day; despite massive adhesions full overview of the surgical site could be obtained. There was no macroscopic sign of anastomotic leakage at this point. As primary wound closure could not be achieved, open wound care was initiated consisting of wet saline dressings changed every two days following minor abdominal lavage. On the 16th postoperative day, leakage of urine was suspected due to increased secretion of the wound and confirmed by radiographic imaging. Relaparotomy was mandated, ureteroileal anastomosis reconstructed and two nephrostomies placed. Persistent exudation led to the assumption of continued anastomotic insufficiency, which was confirmed by flexible endoscopy and radiologic control  is a recognized tool in the management of complex wound healing disorders expanding the spectrum of surgical strategies. While enteral leakage has been viewed as a contraindication for VAC, previous singular reports of successful treatment of intestinal anastomotic insufficiency -10 disprWe present the first report of vacuum-assisted closure therapy successfully performed in urinary tract leakage after surgical treatment of bladder cancer. Appreciated in superficial wound healing disorders VAC even may be considered in the face of lacking surgical alternatives for the closure of complex anastomotic insufficiencies.The author(s) declare that they have no competing interests.SD and WO drafted the manuscript, LL treated the patient and provided the Case report, MB and SK helped to draft the manuscript. WFW supervised treatment and draft of the manuscript.


We report an extremely rare case of concomitant huge exophytic GIST of the stomach and Kasabach-Merritt phenomenon (KMP).4; tumor resection was performed at 16 days after admission. Laparotomy revealed a huge extraluminal tumor arising from the greater curvature of the stomach that measured 25 × 30 cm and had not ruptured into the peritoneal cavity or infiltrated other organs. Partial gastric resection was performed. The resected mass measured 25 × 25 × 20 cm. In cross section, the tumor appeared hard and homogenous with a small polycystic area. Histopathology of the resected specimen showed large spindle cell GIST with >5/50 HPF (high-power field) mitotic activity. The postoperative course was uneventful, and the coagulopathy improved rapidly.The patient was a 67-year-old man experiencing abdominal distension since September 2006. A physical examination revealed a 25 × 30 cm hard mass that was palpable in the middle and lower left abdomen minimal intrinsic mobility and massive ascites. Since the admitted patient was diagnosed with DIC, surgery could not be performed. The patient received a platelet transfusion and the DIC was treated. Due to this treatment, the platelet count recovered to 7.0 × 10Since the characteristic of tumor in this case was hypervascularity with bleeding and necrotic lesions, coagulopathy was thought to be caused by the trapping of platelets within a large vasculized tumor mass. Gastrointestinal stromal tumors (GISTs) are mesenchymal tumors of the digestive tract with various clinical and biological characteristics. The expression of c-kit distinguishes GISTs from true leiomyomas, leiomyosarcomas, and other mesenchymal tumors of the GI tract -3. The sAn association between a vascular lesion and life-threatening coagulopathy is termed as the Kasabach-Merritt phenomenon (KMP). It includes thrombocytopenia, microangiopathic hemolytic anemia, and disseminated intravascular coagulopaty (DIC). Generally, KMP is the most characteristic symptom of giant hemagiomas -7. To ouThe patient was a 67-year-old man experiencing abdominal distension since September 2006. In October 2006, the abdominal fullness became progressive, and he had hard general fatigue. There was no history of vomiting, fever, or gastrointestinal bleeding. Renal failure occurred due to diabetes mellitus (DM). On admission in November 2006, a physical examination revealed a 25 × 30 cm hard mass that was palpable in the middle and lower left abdomen minimal intrinsic mobility and massive ascites.An upper gastrointestinal barium study and gastric endoscopic examination indicated no anomalies. Tumor findings of Doppler ultrasonography showed a hypoechoic lesion with a hypervascular area Figure . An abdo3, FDP: 135.4 μg/ml, PT: 1.20, SIRS score: over 3 heads) surgery could not be performed. The patient received a platelet transfusion of 20 units each day 6 times, and the DIC was treated with nafamostat mesilate and fresh-frozen plasma. Due to this treatment, the platelet count recovered to 7.0 × 104; tumor resection was performed at 16 days after admission. Laparotomy revealed a huge extraluminal tumor arising from the greater curvature of the stomach that measured 25 × 30 cm and had not ruptured into the peritoneal cavity or infiltrated other organs  was administered orally 300 mg per day because the patient displayed renal dysfunction : 41.5)The definition of a GIST has been changing with immunohistochemical and molecular technical advances . It is wet al., reported a classification for malignancies that is based on tumor size and the number of mitotic divisions ., 18.17],Since the characteristic of tumor in this case was hypervascularity with bleeding and necrotic lesions, coagulopathy was thought to be caused by the trapping of platelets within a large vasculized tumor mass.The author(s) declare that they have no competing interests.TW designed the study and participated in the writing process. KS, TS, HK and TE designed the study, carried out the data and picture acquisition as well as bibliographic research, drafted and revised the manuscript. YJ, SK, NT, JS and TS participated in manuscript revision process. TS, TA, HN and TO they participated in the editing process. All authors read and approved the final manuscript.


An oral combined fluoropyrimidine anticancer drug, tegafur/gimeracil/oteracil potassium (S-1), has recently been used alone or in combination for colon cancer.2 was administered by oral route from day 1 to day 14. Irinotecan (CPT-11) at 40 mg/m2 was administered by intravenous day 1 and 15. This treatment was followed by 2 weeks absent period, and repeated every 4 weeks. Six cycles of administration were performed in total. Following this treatment, the multiple costal metastases resolved. Down-staging to T3, M0, stage IIA was achieved, and curative resection was judged to be possible.The patient was a 42-year-old man with sigmoid colon cancer with direct invasion of the urinary bladder and multiple costal metastases. A diagnosis of T4, M1, stage IV sigmoid colon cancer was made, and curative resection was considered impossible. S-1 at 50 mg/mOccasional cases in which S-1/CPT-11 therapy was effective have been recently reported. The patient's tumor became resectable despite the discovery of colon cancer associated with bone metastasis at the initial examination, offering hope for cancer patients. The standard chemotherapy for non-resectable advanced colon cancer is combination chemotherapy with 5-fluorouracil/leucovorin (5-FU/LV) and irinotecan CPT-11) or with 5-FU/LV and oxaliplatin (L-OHP) 1 or with. An oralThe patient was a 42-year-old man with dysuria and fecaluria from late January 2004, who attended the Urology Department of our hospital. Cystoscopy and pelvic CT suggested a tumor of digestive tract origin invading the urinary bladder. The patient was referred to the Department of Digestive Surgery.2. Performance status was grade 0. A fist-size tumor was palpable in the lower abdominal region. There was no particular past medical history or familial medical history.At the initial examination, height, was 160 cm; body weight, 63.5 kg; and body surface area, 1.89/m3/μl; hemoglobin, 16.4 g/dl; AST, 19 IU/l; ALT, 11 IU/l; creatinin clearance, 185.9 ml/min, C reactive protein, 2.19 mg/dl; CEA, 4.3 ng/ml; and CAl9-9, 7.3 U/ml. Bacterial culture of urine detected Escherichia coli and Klebsiella pneumoniae. No malignant cells were identified on urine cytoanalysis. Pelvic computerized tomography (CT) revealed a mass lesion measuring 8 cm was present in the pelvis, with direct invasion of the posterior wall of the urinary bladder. Abdominal CT detected no space-occupying lesion in the liver or swelling of peritoneal lymph nodes.At the initial examination, white blood cell count, was 7,600/μl; red blood cell count, 509 × 10Colonoscopy revealed a 1/2-circumferential ulcerated tumor in the sigmoid colon, and a protuberant tumor was noted on the anal side of the main tumor. Histopathologically, both tumors were well-differentiated adenocarcinoma.99mTc-HMDP bone scintigraphy revealed many lesions with accumulation in the left ribs, which were diagnosed as multiple costal metastases , and curative resection was considered impossible.2 was administered orally from day 1 to day 14. CPT-11 at 40 mg/m2 was administered intravenously day 1 and 15. This treatment was followed by a 2 week rest, and was repeated every 4 weeks [2 after their improvement, and 6 cycles of administration were performed in total . This therapy resulted in resolution of the multiple costal metastases  declare that they have no competing interests.KM reviewed the literature and prepared the draft manuscript.YI participated in the design of the study and helped in drafting the manuscript.TM, MU and HM were involved in performing surgery.TT supervised preparation of the manuscript and edited the final version for publication.All authors read and approved the manuscript.


Renal cell carcinoma is the most common kidney tumor in adults and accounts for approximately 3% of adult malignancies. An increased incidence of second malignancies has been well documented in a number of different disorders, such as head and neck tumors, and hairy cell leukemia. In addition, treatment associated second malignancies  have been described in long term survivors of Hodgkin's lymphoma (HL), Non Hodgkin's lymphoma and in various pediatric tumors.We present the case of a 66 year-old woman with abdominal pain and dyspnea. We performed a thorax CT scan that showed lymph nodes enlargement and subsequently by presence of abdominal pain was performed an abdominal and pelvis CT scan that showed a right kidney tumor of 4 × 5 cms besides of abdominal lymph nodes enlargement. A radical right nephrectomy was designed and Hodgkin's lymphoma was diagnosed in the abdominal lymph nodes while renal cell tumor exhibited a renal cell cancer. Patient received EVA protocol achieving complete response.We described the first case reported in the medical literature of the coexistence between Hodgkin's lymphoma and renal cell cancer. Previous reports have shown the relationship of lymphoid neoplasms with solid tumors, but they have usually described secondary forms of cancer related to chemotherapy. Renal cell carcinoma is the most common kidney tumor in adults and accounts for approximately 3% of adult malignancies. The incidence is higher in male and appears to be increased with cigarrete use and with obesity.,2 There We present the case of a 66 year-old woman with history of intense smoking (pack years of 50), diabetes mellitus detected a prior year and COPD (Chronic obstructive pulmonary disease) diagnosed 8 prior months. She came at the emergency department to receive medical attention by presenting fever, loss of weight and dyspnea. During the initial evaluation was performed a thorax CT scan that showed lymph nodes enlargement and subsequently by presence of abdominal pain was performed an abdominal and pelvis CT scan that showed a right kidney tumor of 4 × 5 cms besides of abdominal lymph nodes enlargement. A radical right nephrectomy was designed, prior to surgery we performed an spirometer study that revealed a moderate obstruction pattern. The surgical procedure included lymph nodes remotion. Histopathology study revealed Hodgkin's lymphoma involving right kidney, abdominal lymph nodes, ureter and liver. Immunostaining showed positiveness for CD15, CD30 and LMP-1 while CD3, EMA and ALK were negative. Histological study also reported a clear renal cell cancer, degree II, involving only the right kidney without extension to any other structure. Surgical resection was complete and margins were negative for cancer, diffuse glomerulosclerosis also was found. After surgical procedure we did a bilateral bone marrow biopsy that was negative for lymphoma. Staging studies showed an IV b stage and 4 unfavorable factors  at diagnosis. We performed an echocardiography study that revealed light enlargement of right cavities, diastolic dysfunction and moderate pulmonary hypertension. The patient received chemotherapy with a protocol called EVA . After third cycle we documented complete response, fact that was obtained after completed the chemotherapy scheme. At present she continues asymptomatic without evidence of disease activity. We present a figure We described the first case reported in the medical literature of the coexistence between Hodgkin's lymphoma and renal cell cancer. Previous reports have shown the relationship of lymphoid neoplasms with solid tumors, but they have usually described secondary forms of cancer related to chemotherapy. Nishikubo et al, reported a case of a 63 year-old man that developed renal cell cancer 8 years after treatment for Hodgkin's lymphoma.  Due to tThe author(s) declare that they have no competing interests.VJ participated designing the study, collecting data, reviewing and writing the article.The pre-publication history for this paper can be accessed here:


Tuberculosis of the appendix remains a rarity despite the frequency of intestinal tuberculosis. We report a case of acute appendicitis that underwent appendectomy at our hospital, and the histopathology of the specimen revealed tuberculosis. Although the ileocaecal region is the most commonly affected part of the intestine in intestinal tuberculosis, involvement of the appendix is rare, occurring in only about 1.5 to 3 % of cases. The appendix may either be involved secondary to ileocaecal tuberculosis, or to tuberculosis at another site within the abdomen, or, may occur in the even rarer "isolated" form, without evidence of the disease elsewhere.Tuberculosis of the appendix presenting with the signs and symptoms of acute appendicitis is an even rarer entity. Even in areas where tuberculosis is common, it is not possible to make the correct diagnosis because the clinical picture is that of acute appendicitis, without any signs suggestive of tuberculosis infection of the organ. Therefore, the diagnosis of appendicular tuberculosis is usually made on histopathological examination of the appendectomy specimen, often received well after the patient has been discharged.A 12-year-old female patient presented with clinical signs and symptoms of acute appendicitis over a duration of one day. General examination revealed tenderness and rebound localised to the McBurney's point. Routine hematological and biochemical investigations were within normal limits except for raised total leucocyte count . She was diagnosed as acute appendicitis and taken up for appendectomy in the emergency. Surgery was unremarkable, the appendix turgid, and showing signs of inflammation. She was discharged on the very next day.On OPD follow up, she was detected to have wound infection that was managed conservatively. She subsequently developed an incisional hernia at the appendectomy site that was repaired electively later.Histopathology analysis of the appendectomy specimen revealed the presence of caseating granulomas and Langhan's giant cells, suggesting tuberculosis of the appendix Figure .She was started on Anti Tubercular Therapy on OPD follow up. Efforts to detect a primary focus of tuberculosis elsewhere in the body were unsuccessful. The patient is presently well on a follow up of two and a half years.Despite gastrointestinal tuberculosis being common in India, affliction of the appendix with the disease remains a rarity . The repThe exact mechanism of involvement of the appendix remains unclear. The various ways by which the appendix can be involved are – hematogenous, by infected intestinal contents, and, by extension of disease from neighbouring ileocaecal or genital tuberculosis . A few aThe disease can present either as a chronic disease (commonest presentation) with recurrent episodes of right iliac fossa pain, vomitings and diarrhoea, as acute appendicitis, or as a latent type that is detected incidentally ,2. The aAs there are no pathognomic clinical features of appendicular tuberculosis, a pre-operative diagnosis is difficult. The diagnosis is usually made after histopathological examination of the appendectomy specimen. Pre-operative diagnosis does not alter the management of these patients as treatment in patients presenting with signs and symptoms of appendicitis remains appendectomy. However, anti-tubercular therapy must be started in the post-operative period if the biopsy reveals tuberculosis.The author(s) declare that they have no competing interests.SG participated in the surgery and was involved in the drafting of the manuscript.RK performed the surgery, and was involved in drafting of the manuscript, and revising it critically for the intellectual content.A carried out the histopathological examination and helped in drafting the manuscript.AKA helped in the revision of the intellectual content and gave final approval of the version to be published.Authors have read and approved the final manuscript.


ACTH overproduction within the pituitary gland or ectopically leads to hypercortisolism. Here, we report the first case of Cushing' syndrome caused by an ectopic ACTH-secreting neuroendocrine carcinoma of the mesentery. Moreover, diagnostic procedures and pitfalls associated with ectopic ACTH-secreting tumors are demonstrated and discussed.111In-pentetreotide helped identify the mesenteric neuroendocrine tumor. After removal of this carcinoma, the patient improved clinically. Laboratory testing confirmed remission of hypercortisolism. An octreotide scan 7 months after surgery showed normal results.A 41 year-old man presented with clinical features and biochemical tests suggestive of ectopic Cushing's syndrome. First, subtotal thyroidectomy was performed without remission of hypercortisolism, because an octreotide scan showed increased activity in the left thyroid gland and an ultrasound revealed nodules in both thyroid lobes one of which was autonomous. In addition, the patient had a 3 mm hypoenhancing lesion of the neurohypophysis and a 1 cm large adrenal tumor. Surgical removal of the pituitary lesion within the posterior lobe did not improve hypercortisolism and we continued to treat the patient with metyrapone to block cortisol production. At 18-months follow-up from initial presentation, we detected an ACTH-producing neuroendocrine carcinoma of the mesentery by using a combination of octreotide scan, computed tomography scan, and positron emission tomography. Intraoperatively, use of a gamma probe after administration of radiolabeled 111In-pentetreotide and a gamma probe.This case underscores the diagnostic challenge in identifying an ectopic ACTH-producing tumor and the pluripotency of cells, in this case of mesenteric cells that can start producing and secreting ACTH. It thereby helps elucidate the pathogenesis of neuroendocrine tumors. This case also suggests that patients with ectopic Cushing's syndrome and an octreotide scan positive in atypical locations may benefit from explorative radioguided surgery using  Endocrine tumors can develop from a variety of tissue types and are often under- or misdiagnosed which underscores the difficulties to classify them. Ectopic ACTH overproduction not only leads to hypercortisolism but is also associated with various diagnostic and therapeutic pitfalls despite performing extensive diagnostic procedures.111In-pentetreotide scan showed radiotracer enrichment in the left thyroid gland consistent with the region of the autonomous adenoma and faint focal octreotide enhancement in the abdomen which was interpreted to be nonspecific because of lacking correlation on CT scan and changing location of enhancement between early and late scan  and colonoscopy showed normal results. Thyroid antibodies were negative, supporting the scintigraphic finding that hyperthyroidism in this patient was caused by an autonomous adenoma. Given the scenario of EAS and an octreoscan being reliably positive only in the left thyroid gland, we decided to perform a left-sided hemithyroidectomy and subtotal resection of the right gland after treatment with methimazole, although basal calcitonin was normal and pentagastrin stimulation could not be performed because it was not available. Extensive pathological workup of the thyroid specimen did not reveal an ACTH-producing endocrine tumor but a 2 mm sized benign nodule on the right side, and 2 benign nodules  within the left thyroid gland.In September 2003, a 41-year-old man presented with unexplained progressive weight gain, facial plethora, tremor, sweating, newly-diagnosed hypertension, hypokalemia, and diabetes mellitus. Physical examination revealed a blood pressure of 170/110 mm Hg, pulse of 88 bpm, thyroid enlargement, and cushingoid features including striae, moon face, central obesity, and a buffalo hump. Laboratory testing showed hyperthyroidism , free T3 of 9 pmol/L ) and increased 24 h urinary free cortisol . Thyroid ultrasound showed a multinodular goiter. A Tc-99m-pertechnetate thyroid scan revealed increased uptake in an area of the left thyroid gland corresponding to an autonomous adenoma. Serum calcitonin was within the normal range. Basal serum cortisol and plasma ACTH were significantly elevated at 1307 nmol/l  and 22.51 pmol/l (1.98–11.4), respectively. After a 2-mg overnight dexamethasone suppression test, serum cortisol was still at 1132 nmol/l . Midnight cortisol was elevated at 847 nmol/l. After an overnight 8-mg dexamethasone suppression test, serum cortisol decreased by 38 % of basal values suggesting ectopic ACTH-secretion (EAS). CRH stimulated serum cortisol and plasma ACTH to less than 4 % of basal values. Inferior petrosal sinus sampling (IPSS) did not show significant central-to-peripheral ACTH gradients. Ectopic ACTH syndrome was diagnosed. Computed tomography (CT) scans at first presentation did not show pulmonary and/or abdominal tumors, however, both adrenals appeared enlarged with the left gland showing a tumor of 1 cm size. A magnetic resonance imaging (MRI) scan of the head demonstrated a small 3 mm hypointense and hypoenhancing lesion of the neurohypophysis  combined with hydrocortisone replacement  to achieve normal 24 h urinary cortisol values. Regular follow-up examinations at 3, 6, 9, and 12 months after initial presentation confirmed the pituitary lesion within the posterior lobe, while CT scans of thorax and abdomen remained negative except for the left-sided adrenal tumor. 24 h urinary catecholamines including fractionated metanephrines were negative. Over the following months, control of hypercortisolism by metyrapone became difficult and the question arose whether bilateral adrenalectomy should be performed. Therefore, we consulted a neuropathologist/pathologist (A.O.V.) and neurosurgeon (E.H.O.) at the National Institutes of Health whether they ever had seen an ACTH-producing pituitary adenoma located in the posterior lobe (see ref. 1). Subsequently, transsphenoidal surgery was performed to explore the pituitary lesion in our patient. Despite complete removal of this lesion, hypercortisolism persisted and metyrapone therapy was continued. Histological workup of the removed pituitary tissue revealed normal posterior pituitary. Postoperatively, diabetes insipidus developed and persisted. An extensive re-evaluation was performed 18 months after initial presentation. An octreoscan revealed tracer accumulation in projection of the upper and middle part of the right abdomen  months after surgery showed a normal 2 mg dexamethasone suppression test and 24 h urinary cortisol levels. Plasma ACTH levels were normal . NSE was 10.7 ng/ml , chromogranin A and calcitonin were undetectable. In addition, the insulin requirement to treat the patient' s type 2 diabetes declined by more than 50%. At 7 month follow-up, the patient had regained strength and muscle mass, did not need further potassium replacement, and felt fine. An octreoscan showed normal results.We present for the first time a case of EAS caused by a neuroendocrine carcinoma of the mesentery. Among ectopic ACTH-producing lesions, approximately 50% originate from intrathoracic tumors, usually small cell carcinomas -6. OtherDiagnosing EAS is often difficult ,40. None18F-FDG PET at initial presentation and during follow-up. Except for PET, these tests are commonly used to locate the cause of EAS [111In-pentetreotide scan provided the decisive clue for locating a malignant ACTH-producing tumor of the ileum in a patient reported by Segu et al. [111In-pentetreotide and a gamma probe.However, in the case presented, correct localization of tumor causing the EAS had been difficult. The percentage of tumors not identified despite extensive evaluation is between 12% and 19% ,6,42. Ine of EAS ,43-47. Fe of EAS ,49. PET e of EAS . In the e of EAS . Howevere of EAS . Furthere of EAS . In our u et al. . This suComplicating the management of our patient with EAS and clearcut negative IPSS, a small lesion of the neurohypophysis was demonstrated at first presentation and during 1-year follow-up. False-negative results of IPPS in patients who do have an ACTH-producing pituitary tumor are reported to occur in up to 13% 7/127) . Given t7 . Given18F-FDG PET. Here, tracer accumulation in projection of the upper and middle part of the right abdomen was detectable in the octreotide scan and PET, for the first time corresponding to a 21 × 15 mm and 26 × 15 mm tumor mass on CT and MRI scans, respectively. These results support the view that regular follow-up of occult EAS may result in localization of ACTH production at later time points due to tumor growth [111In-pentetreotide within hours before surgery facilitates localization of the tumor during surgery by use of radiolabeled 111In-pentetreotide and a gamma probe similar to recent reports [At 18-months after first presentation, another extensive diagnostic procedure was performed including CT scans of chest, abdomen, and pelvis, MRI head, octreotide scan, and  reports ,56.111In-pentetreotide. Unfortunately, we were not able to assess mRNA for somatostatin receptor expression.To further elucidate whether patients with neuroendocrine tumors that are (supposedly) completely resected, may benefit from adjuvant therapy including somatostatin analogs, we performed a MIB-1 proliferation index of the tumor in our patient and immunohistochemical analyses of somatostatin receptor expression ,58. We fWe report for the first time a patient with EAS due to a neuroendocrine carcinoma of the mesentery. This case not only may help elucidate the pathogenesis of (ACTH-producing) endocrine tumors, but also illustrates diagnostic pitfalls in the management of patients with EAS, and suggests that patients with EAS and an octreoscan positive in atypical locations may benefit from explorative radioguided surgery using a gamma probe.CT – computed tomography; EAS – ectopic ACTH-secretion; FDG – fluorodeoxyglucose; IPSS – inferior petrosal sinus sampling; MRI – magnetic resonance imaging; PET – positron emission tomographyNovartis Pharma GmbH will carry the costs of the article-processing charge in conjunction with a grant provided to Prof. Koch (CAK) on the topic "Neuroendocrine tumors: Nachuntersuchung aller operierten (R0/komplett resezierten) Patienten mit gastroenteropankreatischen neuroendokrinen Tumoren mittels Octreoscan". The authors declare that they have no other competing interests.MF provided clinical care for this patient and drafted the paper. TL provided clinical care for this patient and helped draft and interpret the results, especially of studies related to nuclear medicine. HW provided clinical care for this patient and operated on the patient. RK provided clinical care for this patient and helped draft and interpret the results, especially of studies related to nuclear medicine. AT provided important insights into the histopathological diagnosis of this patient, delivered macroscopic, microscopic, and immunohistochemical images of this patient's tumor, and helped interpret the results. MM provided clinical care for this patient and helped interpret radiological images performed in this patient. MB conducted transsphenoidal surgery in this patient and helped interpret the clinical course and results of various tests. SP carried out immunohistochemical studies on somatostatin receptor types and helped interpret the results and draft the paper. JK delivered important insights into the laboratory and clinical chemistry aspects of this patient's history. RP helped interpret the results of all studies performed in this patient and provided intellectual input. CAK designed this study, interpreted the results, drafted the paper, and finalized the manuscript after input from the other authors.All authors read and approved the final version of the manuscript.The pre-publication history for this paper can be accessed here:


Malignant schwannoma of the upper mediastinum originating from the vagus nerve is extremely rare.A 46-year-old female was admitted for a left cervical mass which was associated with both hoarseness and Horner's syndrome. Chest computed tomography showed a mass extending from the left upper mediastinum to the left supraclavicular area. A fine needle aspiration cytological examination suggested primary lung cancer stage IIIB large cell carcinoma. After administering induction chemo-radiotherapy, a complete surgical resection was performed. The tumor was found to involve both the left vagus nerve and the left sympathetic nerve. Histological examination of the resected specimen revealed the tumor to be malignant schwannoma.Despite incorrect preoperative diagnosis, the multimodality treatment administered in this case, including induction chemo-radiotherapy and surgery, proved to be effective. According to a collected series of 2399 cases of mediastinal tumors reported in the literature , 496 cas2 for days 8 and 36 + UFT 400 mg/m2, both on days 1–14 and on days 29–42 plus radiotherapy, 2 Gy/day on days 1–20 for a total of 40 Gy) [A 46-year-old female with symptoms of hoarseness and Horner's syndrome presented with a left cervical mass that was diagnosed to be undifferentiated carcinoma based on the findings of aspiration cytology Figure . The patTumors of vagus nerve origin are observed in about 2% of all neurogenic tumors of the mediastinum , howeverMalignant peripheral nerve sheath tumors (MPNST) including malignant schwannoma are the malignant variants of schwannomas and neurofibromas. Although the 5-year survival rates have been reported to be up to 75 % in MPNST's patients, MPNST often advance locally and can also occasionally metastasize to the lung or other organs . TherefoWe initially misdiagnosed this patient's disease to be non-small cell lung cancer. The reason for this was partly due to the cytological findings which indicated undifferentiated carcinoma. In general, an exact diagnosis cannot always be made based on the findings of aspiration cytology alone. The second reason for a misdiagnosis in this case was due to the patient's symptoms which included hoarseness and Horner's syndrome. Apical lung cancer involving both the vagus and the sympathetic nerve is occasionally observed. However, to the best of our knowledge, the present case is considered to be the first case demonstrating malignant schwannoma of the vagus nerve involving the sympathetic nerve.Malignant schwannoma of the upper mediastinum arising from the vagus nerve is rare. The multimodality treatment administered in this case, including induction chemo-radiotherapy and surgery, proved to be effective.The author(s) declare that they have no competing interests.FS: conceived of the study, participated in its design and coordination and drafted the manuscript.RM and TO: carried out the literature search and helped in drafting the manuscript.HW: participated in the design of the study and helped in drafting the manuscript.KN: performed histological examination and provided photographs.YI: shaped the idea for the study, coordinated the study and edited the manuscript.All authors read and approved the final manuscript.


Undifferentiated embryonal sarcoma (UES) of liver is a rare malignant neoplasm, which affects mostly the pediatric population accounting for 13% of pediatric hepatic malignancies, a few cases has been reported in adults.We report a case of undifferentiated embryonal sarcoma of the liver in a 20-year-old Caucasian male. The patient was referred to us for further investigation after a laparotomy in a district hospital for spontaneous abdominal hemorrhage, which was due to a liver mass. After a through evaluation with computed tomography scan and magnetic resonance imaging of the liver and taking into consideration the previous history of the patient, it was decided to surgically explore the patient. Resection of I–IV and VIII hepatic lobe. Patient developed disseminated intravascular coagulation one day after the surgery and died the next day.It is a rare, highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. The prognosis is poor but recent evidence has shown that long-term survival is possible after complete surgical resection with or without postoperative chemotherapy. Physical examination revealed abdominal pain, localized right lower quadrant abdominal tenderness, body temperature slightly elevated 37.7°C), blood pressure 120/90 mmHg and heart rate 110/min. Blood tests showed leukocyte count at 12600/μL, the differential white account showing 75% neutrophils and hematocrit 35.9%. Abdominal and chest radiographs were normal. The primary diagnosis was acute appendicitis and appendectomy was decided. Entering the peritoneal cavity from a McBurney incision, the surgeon found large amount of blood and the surgery was transformed to exploratory laparotomy through a midline incision. The findings were enormous hepatomegaly and bleeding due to spontaneous rupture of the IV lobe of the liver. Active bleeding was managed with direct suture of identifiable vessels and absorbable gauze mesh. Postoperative course was uneventful, except of a continuous elevated body temperature (up to 38.5°C). The patient, five days after surgery, was referred to us in order to undergo further examinations for the nature of the liver abnormality. His medical history was unremarkable. He was previously in good health. His family and past history were not contributory. The patient weighted 65 Kg with 1.75 m height. He was in his right senses and well nourished. Yellow skin, icteric sclera, spider naevi and palmar erythema were not founded. Cardiopulmonary examination was unremarkable. Chest radiograph revealed no pulmonary pathology. The abdomen was swollen with the liver palpable. Laboratory tests showed liver function tests normal  hepatitis markers of HAV, HBV, HCV, and HIV were negative and alpha-fetoprotein (AFP) was 35 ng/ml. The patient underwent computed tomography (CT) of the abdomen that showed hepatomegaly and a large hypodense lesion of the liver with multicystic appearance with septations and solid portions  of the liver was first described as a clinicopathologic entity in 1978. Before that, the nomenclature was variable and included mesenchymoma, primary sarcoma of the liver, fibromyxosarcoma and malignant mesenchymoma .After neuroblastoma and Wilm's tumor, primary hepatic neoplasms are the third most common solid malignant tumors found in children accounting for 2% of the total of childhood cancers. Although rare, malignant mesenchymal hepatic tumors are the fourth most common malignant tumors of the liver in the children, with hepatoblastoma and hepatocellular carcinoma accounting for the majority of primary hepatic malignancies in children .The UES of the liver is typically diagnosed after 6 years of age with a decline in incidence after 10 years of age and 50% of the patients were between the above ages in the longest reported series . In chilThe UES of the liver is a rare tumor that most often presents in childhood but infrequently in adults. Less than 60 cases of UESL in adults have been reported in the pertinent literature ,6-9.There are no specific clinical features. Tumor related symptoms might be regarded as abdominal mass with or without upper abdominal pain or swelling. Fever is probably related to the hemorrhage and necrosis found in the majority of these tumors, as happened in our patient. Of possible significance is the absence of jaundice. Rupture into the tumor or into the peritoneal cavity due to rapid growth of the tumor is not uncommon . Review Radiographs of the abdomen are usually normal. The lesion can be detected by ultrasound, CT and MRI. MRI localizes the lesion more accurately than the other methods, with good resectability correlation. It also can detect vascular invasion, biliary obstruction and hilar adenopathies .UES of the liver is a neoplasm with primitive mesenchymal phenotype. Tumor size often exceeds 10 cm and can be as large as 30 cm. Macroscopic examination shows a single, well-demarcated, soft, globular mass that frequently has cystic, gelatinous, hemorrhagic and necrotic foci. Microscopic examination reveals a pseudocapsule surrounding a neoplasm, composed predominately of spindle, oval, or stellate cells with ill-defined cell borders. The tumor cells are embedded in an abundant myxoid stroma that contains many thin-walled veins. The bile duct-like structures occasionally appear hyperplastic or reactive, although they, too, may show degenerative changes . In our Evaluable treatments included surgery, hepatic arterial ligation, hepatic transplantation, and combinations of surgery and/or chemotherapy and radiation therapy. Radical resection of the tumor is the optimal treatment of choice ,13. AccoThe prognosis for UESL has been poor to until recently and majority of the patients died of tumor recurrence or metastasis within two years after the initial operation . The majThe author(s) declare that they have no competing interests.All authors contributed equally in preparation of the manuscript.


Pulmonary giant cell carcinoma is one of the rare histological subtypes with pleomorphic, sarcomatoid or sarcomatous elements. The prognosis of patients with this tumor tends to be poor, because surgery, irradiation and chemotherapy are not usually effective.We herein report a patient with pulmonary giant cell carcinoma with stage IV disease in whom aggressive multi-modality therapy resulted in a long-term survival. A 51-year-old male underwent an emergent operation with a partial resection of small intestinal metastases due to bleeding from the tumor. The patient also underwent a left pneumonectomy due to hemothorax as a result of the rapid growth of the primary tumor. Thereafter, two different regimens of chemotherapy and a partial resection for other site of small intestinal metastases and a splenectomy for splenic metastases were performed. The patient is presently doing well without any evidence of recurrence for 3 years after the initial operation.This is a first report of a rare case with stage IV pulmonary giant cell carcinoma who has survived long-term after undergoing aggressive surgical treatment and chemotherapy. The recent World Health Organization (WHO) classification of lung tumors has unified the heterogeneous group of non-small cell lung carcinomas that contains sarcoma or sarcoma-like components under the designation of "carcinomas with pleomorphic, sarcomatoid or sarcomatous elements" . This gr1+2) without any invasion of the surrounding tissue such as the vessels, plexus or thoracic wall and with no mediastinal lymph node swelling. Abdominal CT revealed a huge mass, measuring 12.7 × 7.5 cm in size in the small intestine. Prior to performing any treatment for the presumed lung cancer, we tried to stop the continuous bleeding from tumor in the small intestine. As a result, we performed an emergency operation. The tumor was observed in the jejunum at a location about 30 cm from the ligament of Treitz on the anal side and a 25 cm length of the jejunum, including the tumor, was thus resected. Six days later, the patient experienced sudden chest pain, dyspnoea and hemoptysis. The patient's chest X ray showed the left lung mass shadow to have rapidly increased in size, while the broncho-fiberscopy findings showed bleeding from the left upper bronchus and an obstruction of the left lower bronchus due to coagulation. Hemothorax due to a rupture of the lung induced by the rapid growth of the tumor was found after an emergency thoracotmy. The tumor was so large that it was difficult to approach the interlobular pulmonary artery. Therefore, a left pneumonectomy with mediastinal lymph nodal dissection was performed. Thereafter, intraoperative intrapleural hypotonic cisplatin treatment [2 + gemcitabine 800 mg/m2+ vinorelbine 20 mg/m2), at days 1 and 8, and thereafter every 4 weeks). The splenic metastases increased in size while the size of the tumor in the small intestine decreased. At this time, no recurrence site except for those in the small intestine and spleen were found, therefore, to avoid the risk of bleeding either from tumors in the small intestine or a rupture of spleen in the future, surgical treatment consisting of a partial resection of the small intestine and a splenectomy was performed. The intestinal tumor was found in the jejunum at a location about 10 cm from the ligament of Treitz on the anal side and a total 20 cm length of the jejunum, including the tumor, was resected. A pathological examination revealed a proliferation of pure giant cell carcinoma with extensive necrosis both in the small intestine and the spleen, thus suggesting the chemotherapy to be effective in the both organs. Thereafter, the patient received 2 additional cycles of this triplet chemotherapy. The patient experienced neither any hematological nor severe non-hematological adverse events. About 6 months later, metastases in multiple abdominal lymph nodes were found . After receiving a total of 10 cycles of chemotherapy on an outpatient basis, abdominal CT showed the chemotherapeutic effect to be a complete response  and hematocrit of 16.0 % . The patient's chest X-ray demonstrated a huge mass lesion in the left upper lung field , chemothet al, reported the overall survival of total 78 patients with pleomorphic (spindle/ giant cell) carcinoma (stage I-IV), among whom 57 patients received a surgical resection, to be poor with a median survival time of 10 months and a survival rate of 10% at 5 years [et al, the mean survival time of resected pleomorphic carcinoma patients was 5 months while the median survival time of pleomorphic carcinoma patients treated with concurrent or sequential chemo-radiotherapy was 2.7 months [2, respectively once every 3 weeks [2, on days 1 and 8, and thereafter every 3 weeks) in this patient was unusual and the dose intensity was relatively small. However, this regimen nevertheless effectively treated his disease and he was also able to work normally during the treatment process. At present, the patient has survived for 3 years since the first operation and has remained healthy without any signs of recurrence for 15 months after the last treatment.Fishback 7 months . To our  3 weeks , the admThis is a first report of a rare case with stage IV pulmonary giant cell carcinoma who has survived long-term after undergoing aggressive surgical treatment and chemotherapy.The author(s) declare that they have no competing interests.FS: Conceived the study, participated in its design and coordination and drafted the manuscript.RM and TO: carried out the literature search and helped in drafting the manuscriptJI, TN and HW: participated in the study design and helped with preparation of the manuscriptYI: Shaped the idea for the manuscript, coordinated the study and edited the manuscript.All authors conceived of the study, and participated in its design and coordination. All authors read and approved the final manuscript.


Cotyledonoid dissecting leiomyoma of the uterus is a recently described rare variant of benign uterine leiomyoma. We report a case of cotyledonoid dissecting leiomyoma in a 52 year old woman who presented with menorrhagia and abdominal pain. An ultrasound scan showed a bulky uterus and a cystic heterogenous mass near the left ovary. At hysterectomy, the left broad ligament mass was removed. This was continuous with an ill-defined nodular area in the myometrial fundus. Microscopy revealed a benign smooth muscle proliferation in the myometrium that extended beyond the uterus and into the broad ligament. The lesion appeared to be dissecting the myometrial fibres and showed areas of oedema, hyalinisation and perinodular hydropic change. Cellular atypia, mitoses and coagulative necrosis were absent. The patient is alive and well 18 months after surgery. It is important to recognize this benign and unusual appearing variant of leiomyoma in order to prevent inappropriate treatment. Uterine leiomyomas can present in a variety of unusual growth patterns. One of the most unusual variants is called cotyledonoid dissecting leiomyoma or Sternberg tumour . These tA 52 year old woman, gravida 5, para 5, presented with a 5 months history of menorrhagia, abdominal pain and tiredness. A transvaginal ultrasound scan showed a bulky uterus with an irregular and thickened endometrium of 14 mm. A 10 mm well rounded and echo poor area was also noted in the endometrium. In the region of the left ovary there was a cystic lesion that had thick irregular borders and solid components measuring 40 mm in maximum dimension. The right ovary was unremarkable. The CA 125 level was 12 units/ml. Since the left ovarian lesion was reported as suspicious the patient underwent surgery. At the time of laparotomy, a bulky uterus was seen and a spongy lesion was noted in the region of the left ovary. A total abdominal hysterectomy with left salpingo-oophorectomy was performed and the patient is alive and well 18 months after the operation.Tissue sections were fixed in 10% formalin, embedded in paraffin and stained with haematoxylin-eosin. Immunohistochemical staining with the streptavidin-biotin peroxidase detection system was performed using a Ventana automated immunostainer . The antibodies smooth muscle actin , desmin , CD10 , Ventana, Tucson, Arizona), HMB45  and CD31  were used.Macroscopic examination of the specimen revealed a corpus and cervix 110 × 60 × 50 mm with an attached left fallopian tube 30 mm in length and left ovary 20 × 15 × 10 mm. A large mass was present in the left broad ligament in the region of the left ovary measuring 60 × 40 × 30 mm. This mass had a red, spongy, cystic and gelatinous cut surface reminiscent of placental tissue and was distinct from the ovary and fallopian tube but apparently continuous with an ill-defined nodular area in the myometrium of the uterine fundus Fig. . This noHistological examination showed that the submucosal leiomyoma was composed of bland smooth muscle cells growing in broad, sweeping fascicles with no cellular atypia, abnormal cellularity or increased mitotic activity (up to 1 mitosis per 10 high power fields). Immunohistochemically, the constituent cells were positive with smooth muscle actin and desmin Fig.  while CDUterine smooth muscle tumours are known to exhibit a wide variety of growth patterns. One unusual variant is the cotyledonoid dissecting leiomyoma of the uterus or "Sternberg tumour" first described in 1996 by Roth et al. [Description of new variants of any lesion comes with attendant issues regarding nomenclature. Apart from the cotyledonoid dissecting leiomyoma described by Roth et al.  there arTherefore, care should be taken not to confuse these new variants of smooth muscle tumours with unusual growth patterns. This is especially important for tumours with different prognostic implications like the proposed "cotyledonoid hydropic intravenous leiomyomatosis" . CarefulIn summary, cotyledonoid dissecting leiomyoma is a rare variant of benign leiomyoma that is closely related to and shares some features with other and similarly named leiomyoma variants. Not only can its bizarre gross appearance lead to a mistaken diagnosis of a malignant neoplasm, but it should also be distinguished from other unusual leiomyoma variants, especially to exclude intravascular spread, for appropriate classification and potential prognostic implications.The author(s) declare that they have no competing interests.AW drafted the manuscript. RG and TR helped to draft the manuscript. MM and GD contributed the clinical background.All authors read and approved the final manuscript.


Renal cell carcinoma is well known for its frequency to metastasise, particularly to lungs, liver, bones and brain. Metastasis to the skin is much less common. Presentation as a result of the skin lesion is even more unusual, with only 14 previously reported cases in the English literature. The majority of these cases have been reported in patients with recurrent disease or with other metastases.We present only the second case of non-recurrent renal cell carcinoma with a solitary cutaneous facial metastasis reported in the English literature.Clinicians should conduct a careful inspection of the skin in patients with renal cell carcinoma and also have a high index of suspicion of primary internal organ malignancy in patients presenting with a skin lesion. Renal cell carcinoma (RCC) accounts for 3% of all adult malignancies . PresentMetastasis of RCC to organs such as the lungs, liver, bones and brain is well known, but there are far fewer reports of metastases to the skin. Cutaneous lesions as the primary presentation of renal cell carcinoma are extremely uncommon with only a handful of reports in the literature. We report a case of RCC presenting as a solitary cutaneous facial metastasis with no other organ involvement and no past history of RCC. This is only the second such case to be reported in the English literature  and may be the first case presenting as a facial abscess.A 36 year old male was referred by his general practitioner to the accident and emergency department with what was thought to be an abscess on his chin Figures  and 2. TDuring the two weeks before presentation the patient had been feeling generally unwell with fatigue and lethargy. He also reported weight loss of about 5 kg over the last month.On further questioning he described some left sided dull, 'dragging' abdominal pain and associated back pain. He denied any change in his bowel habit and had no haematuria or other urinary symptoms. He had no past medical history and was not on any medications. He was a non-smoker.On examination the patient was lethargic with a prominent lesion on his chin 2 cm in diameter. The lesion was erythematous, tender, tense and was similar to the appearance of an abscess. He was afebrile.On examination of the abdomen he was found to have a number of purpuric spots over the upper torso and hepatosplenomegaly.Investigations showed normal full blood count, urea and electrolytes, liver function tests and chest X-ray. His C-reactive protein was elevated at 155.In view of his weight loss and hepatosplenomegaly he was admitted under the medical team for further investigation with a suspicion of lymphoreticular malignancy.The surgical team were asked to review regarding the "abscess" on the patient's chin. However, due to the pending investigations a decision was made to wait for the results before contemplating incision and drainage.The patient had a computed tomography (CT) scan of the abdomen and pelvis which diagnosed a left renal carcinoma 12 cm in diameter arising from the anterior cortex, but no thrombus or tumour was identified in the left renal vein. The patient was then referred to the urology team.The urology team arranged a chest CT scan and a bone scan which were normal and subsequently performed left radical nephrectomy and biopsy of his chin lesion.The histology of his left kidney showed clear cell renal carcinoma, Stage 3, with tumour invading the left renal vein at the kidney hilum (G3 pT3b N0 M1).Malignant cells were found in the chin biopsy histology, which were consistent with metastatic carcinoma.The patient was referred to the oncologists for further treatment. He underwent subcutaneous interferon injections at a dose of 5 mega units three times per week for a total of 4 months. He also underwent radiotherapy to the site of the lesion on his chin, followed by resection of the lump. One year later the patient developed some pain in his iliac crest and right posterior ribs for which he underwent palliative radiotherapy although bone scan and X-rays did not show any evidence of metastasis.It is now two and a half years since diagnosis and the patient remains disease free, having had a recent follow up CT scan which showed no evidence of recurrence.th most common site of metastasis [Renal cell carcinoma (RCC) has been well described for its frequency to metastasise, occurring in approximately one third of patients at the time of diagnosis . Howevertastasis . RCC is tastasis ,6.There have been several previously reported cases of RCC metastasising to the skin although there are only a handful of reports of RCC presenting with skin metastasis.In our search through the literature  we found only 14 other cases of non-recurrent renal cell carcinoma presenting with skin metastasis reported in English -12 and 1The only previously reported case in the English literature of a patient with non-recurrent RCC presenting with a solitary facial metastasis is of 63 year old male with a solitary chin metastasis reported in 1978. No details of other organ involvement or outcome were provided in the article [A Japanese literature review reported 75 cases of RCC associated with skin metastasis. The commonest site of metastasis was to the trunk (40%), followed by the scalp (25%). Only 8% had metastases to the face. 24% had cutaneous metastases at the time of diagnosis .A large Indian study, reviewing a total of 306 patients with RCC seen over a 12 year period found only 10 cases (3.3%) with skin metastases. Of these, half of the patients presented with skin metastases during follow-up after nephrectomy. In only one case the skin nodule was actually the presenting symptom. This patient, a 32 year old male had a nodule located on the chest and also had lung metastases at the time of diagnosis (outcome not documented). 90% of the patients also had metastases to other organs at the time of diagnosis [A Greek study reviewed 6 patients with RCC presenting with skin metastases, although none were to the face. 3 of them had solitary cutaneous metastases, one 76 year old male also had lung metastases, other organ involvement was not documented in the other two cases . The 3 patients with multiple cutaneous metastases all had other organ involvement at the time of diagnosis. Sites of cutaneous metastasis were scalp, thorax and abdominal wall .A large retrospective study of 130 patients with metastatic renal cancer revealed cutaneous metastases in 6 patients. 2 of these had only local metastases in the previous nephrectomy scar, the other 4 had only distal metastases, 3 of which were to the scalp and one patient had multiple facial, neck, chest and upper extremity metastases. No details regarding other organ involvement or previous RCC were provided in the article .Other authors have reported single cases. A 55 year old male presented with a bleeding metastasis on the tip of the nose, the patient also had liver and lung metastases and died within weeks from a brain haemorrhage . A 74 yeThere have also been 4 further reports of recurrent RCC presenting with cutaneous metastases after nephrectomy:-▪ 55 year old male with a solitary metastasis on the chin presented during follow-up, the patient declined further treatment and died 6 months later .▪ 61 year old male with a scalp metastasis and also had bone and chest metastases .▪ 75 year old female presented with a skin metastasis on the back 4 years post nephrectomy. The patient also had lung and bone metastases .▪ 61 year old male with 3 skin lesions in front of left ear. The patient also had liver metastases at time of diagnosis and developed recurrence of the skin lesions post operatively. He died 1 year post-nephrectomy .Several of the above studies have noted the poor prognosis of patients with skin metastases at the time of diagnosis of RCC, often heralding advanced disease. However, in our case the patient remains disease free two and a half years further on. It is likely that the improved outcome is a result of having only a solitary cutaneous metastasis, along with the prompt diagnosis and treatment of the underlying primary malignancy.Metastasis at the time of diagnosis frequently occurs in RCC, with the skin not infrequently involved. RCC may present as a result of symptoms from metastases, including the skin, although solitary cutaneous metastases as a primary presentation are uncommon.Clinicians should be aware of the possibility of primary internal organ malignancy in patients presenting with cutaneous lesions and therefore conduct a careful examination of the skin in patients with RCC. Prompt diagnosis and treatment may affect the eventual outcome.The author(s) declare that they have no competing interests.NP carried out the literature search and drafted the manuscript. HA summarised the case notes and assisted with the literature search and drafting of the manuscript. SA-D conceived of writing the case report, obtained the notes and photographs and assisted with drafting the manuscript. All authors read and approved the final manuscript.


Hepatoid adenocarcinoma (HAC) is a peculiar type of extrahepatic adenocarcinoma generally characterized by adenocarcinomatous and hepatocellular carcinoma (HCC)-like foci. Stomach is the most frequent site where hepatoid adenocarcinoma occurs, although it has been described in many other organs. On the other side, liposarcoma is a rare, malignant tumor that develops from fat cells.We describe here a case of hepatoid carcinoma in collision with a liposarcoma of the left colon serosa in a 71-year-old man. It presented as an abdominal mass involving several organs, falsely mimicking metastatic colonic adenocarcinoma. Recognition of this entity was evident on microscopic evaluation following surgery. The patient had an objective response following liposomal antracycline chemotherapy, with a 3-year overall survival.To our knowledge, this is the first case of a hepatoid tumor colliding with a liposarcoma of the left colon serosa reported to date. Hepatoid adenocarcinoma (HAC) is a type of extrahepatic adenocarcinoma, which shows a striking morphologic similarity to hepatocellular carcinoma. It is generally characterized by adenocarcinomatous and hepatocellular carcinoma (HCC)-like foci. This variant of adenocarcinoma has been demonstrated to be an alpha-fetoprotein (AFP)-producing carcinoma arising in extra hepatic organs, and it mimics hepatocellular carcinoma in morphological and functional terms.In most instances, hepatoid carcinoma has been found in the stomach ,2, but iA prompt and accurate diagnosis of hepatoid adenocarcinoma is important because prognosis is very poor compared with that of both common types of adenocarcinoma  and AFP-Liposarcoma is a rare, malignant tumor that develops from fat cells. It is the most common soft tissue sarcoma and accounts for 20% of all malignant mesenchymal tumors. This tumor typically appears in the deep fat tissues of the thigh or abdomen in people aged 50 to 70. Prognosis varies depending on site of origin, type of cancer cell, tumor size, depth, and proximity to lymph nodes. Metastases are common and the 5-year survival rate for a deep and high-grade liposarcoma is less than 50%. This paper describes the unusual occurrence of a hepatoid carcinoma colliding with a liposarcoma.A 71-year-old man was admitted because of abdominal pain in January 2003. His past medical history was significant for a monoclonal gammopathy and surgery for hip prosthesis 2 years before the current admission. At another hospital an abdominal computed tomography (CT) scan had revealed a left-sided mass (10 × 9 cm) involving the diaphragm, spleen, and, from the outside, the left colon flexure. Tumor marker (CEA and CA19-9) serum levels were normal, with the exception of serum AFP, which was markedly elevated (44074.6 ng/ml). However, no CT evidence of primary hepatocellular carcinoma was found. Endoscopy of the upper and lower gastrointestinal tract did not show any primary tumor. A FNAB was performed and cytologic analysis was consistent with a moderately differentiated adenocarcinoma.Since surgery was deemed not to be feasible, 5-fluorouracil and oxaliplatin-based chemotherapy was administered for a total of 6 cycles, resulting in stable disease.As the abdominal mass was shown to have slightly shrunk Figure , a laparThe gross appearance of the operated specimen was that of a lobulated, compact, yellow-greenish mass immersed in an adipose-like stromal tissue. Included in the pathological evaluation were also the spleen (12.5 × 6.0 cm), a 7-cm intestinal tract, and tissue samples from colon serosa (6.0 cm), omentum (19 × 18 cm), diaphragm 4.5 × 3.0 cm), peritoneum (8.5 × 3.5 cm), transverse mesocolon (2.5 × 2.0 cm), and pancreatic tail lymph nodes. The spleen had an irregular surface due to the presence of multiple, small gray-whitish nodules, which were also observed in the context of the other sampled tissues. The maximum diameter of the nodules was 2.5 cm. Microscopically, each nodule appeared to be made of single or multiple layers of cells, sometimes with branchlike divisions or in islet-like configuration. Cells in the nodules had polygonal appearance, acidophilic cytoplasm, and large, single or multiple hyperchromatic nuclei, often with vacuolization. These cells stained positive for AFP on immunohistochemistry .The serum AFP level decreased after surgery (9000 ng/ml). The patient received first line chemotherapy with liposomal doxorubicin for 8 cycles resulting in disease control for 2 years. The patient then had hepatic metastases and died 3 years after the first diagnosis.The unusual presentation of two entirely different primary malignancies in close proximity to each other is defined as a "collision tumor". To our knowledge, this is the first case of a hepatoid tumor colliding with a liposarcoma of the left colon serosa reported to date. This collision tumor presented as an abdominal mass with extensive involvement of abdominal organs, thus falsely mimicking metastatic dedifferentiated colonic adenocarcinoma. In the absence of a primary identifiable liver disease, this was consistent with a hepatoid carcinoma arising from the peritoneum. The dual nature of the mass was revealed on histologic analysis; it has to be stressed that the two tumors collided at a clear boundary with no evidence of transition both grossly and microscopically; in addition, the immunohistochemical staining pattern for AFP was quite different between the two tumors ,9 15]. I 15. I9] To data, this is the first case of hepatoid carcinoma colliding with a liposarcoma of the left colon serosa. The unusually good response of this tumor to chemotherapy is worth reporting, especially because systemic chemotherapy is considered little or non effective. Although this is a single case report, patient's response shows that liposomial antracycline therapy is possible for this type of tumor.The author(s) declare that they have no competing interests.MO: Primary author in producing and revising the manuscript. TT, GC, FDV: carried out second line chemotherapy and provided data about follow-up of the patient; Helped to draft the manuscriptFC: helped in scrutiny of manuscript. EL, PC, GG: carried out surgical procedure and provided data about surgical procedure. GDC: carried out the first line of chemotherapy. All authors read and approved the final manuscript.


Extraskeletal osteosarcoma is a rare malignant mesenchymal tumor, with a predominant occurrence in the extremities. Only two cases of mesenteric extraskeletal osteosarcoma have been documented. We describe an unusual case of extraskeletal osteosarcoma with telangiectatic features occurring in the mesentery.A 67-year-old male presented with blood-tinged stool of 1-month's duration. On colonoscopy, a solid mass was detected protruding from the colon wall. Computed tomography showed a 15 × 9.7 cm heterogeneously enhancing mass, with mottled calcification and a cystic portion, occupying the left upper quadrant of the abdominal cavity. Curative resection of the tumor was performed, and the excised tumor was composed of large multilocular cysts containing old hematomas and necrotic debris. The histology revealed an osteosarcoma showing osteoid formation and blood-filled spaces lined with atypical cells. Despite postoperative chemotherapy, he developed a recurrent peritoneal mass and multiple lung metastases 3 months postoperatively.Given the rarity of cases of mesenteric extraskeletal osteosarcoma, its biologic behavior at this location remains to be determined. However, extraskeletal osteosarcoma with telangiectatic features is an uncommon entity to be recognized because of the possible fatal outcome related to the tumors. Extraskeletal osteosarcoma (EOS) is a rare malignant mesenchymal tumor characterized by the production of neoplastic osseous tissues, without attachment to bone or the periosteum . It occuA 67-year-old male presented with abdominal pain and blood-tinged stool of 1-month's duration. His past medical history revealed hypertension well controlled by medication for 8 years and that he had been a smoker for 20 pack years. His family history was noncontributory. Physical examination revealed a protuberant abdomen with a huge tender intra-abdominal mass. Laboratory findings including blood analysis, serum electrolytes, and hepatic and renal functions were within normal limits, as was the serum alkaline phosphatase.en bloc mass excision with segmental colon resection and splenectomy was performed. During the operation, the surgeon described the tumor as being located in the mesentery and involving the stomach, greater omentum, pancreas, and transverse colon. The resected tumor measured 18.5 × 13 × 9.5 cm. It was located in the mesentery and perforated the abutting colon wall. The external surface of the mass was smooth. The cut surface consisted of a gray-white to tan-yellow solid area with a gritty sensation and a large multicystic area  and adriamycin (25 mg/m2). However, he developed a recurrent peritoneal mass measuring 11 × 9.5 cm and multiple lung and liver metastases 3 months postoperatively. He died 4 months later.After the uneventful surgery, the patient underwent the first cycle of chemotherapy consisting of intravenous ifosfamide  [The criteria for the diagnosis of primary extraskeletal osteosarcoma by Allan s origin . The seces (25%) . The fires (25%) . Before es (25%) ,11.A diligent search for osteoid is very important for the diagnosis of extraosseous osteosarcoma. In our case, the limited amount of tumor tissue in the colonoscopic biopsy concealed the presence of osteoid, so that we were unable to reach a definite diagnosis. In addition, the seemingly contradictory results of the immunohistochemistry were not helpful. Both osseous and extraosseous osteosarcomas show notoriously varied immunophenotypes by reacting to such reagents as factor-XIII related antigen, S100, desmin, alpha-smooth muscle actin, cytokeratin, and epithelial membrane antigen ,13.The differential diagnosis includes various benign and malignant soft tissue osteogenic lesions. Previous reports have indicated that many EOS or telangiectatic EOS cases were initially misinterpreted as different entities. Occasionally, EOS has been mistaken for myositis ossificans, which is a benign ossifying process usually occurring in young adults as a solitary, well circumscribed mass within skeletal muscle. The typical lesion of myositis ossificans shows a zoning effect with peripheral differentiation into well formed bone and a lack of cytologic atypia . Tumor oThe diagnosis of malignant mesenchymoma can be made by showing two or more identifiable malignant elements, exclusive of fibrosarcoma, and chondrosarcoma even when osteogenic sarcoma is identified ,10. AmonPatients with extraskeletal osteosarcoma generally have a poor prognosis and the majority develops metastatic disease within 3 years of diagnosis . The repThe author(s) declare that they have no competing interests.KHL performed the review of the literature and participated in the draft of the manuscript.JKJ and DYK performed the surgery and provided clinical information of the patient.CC participated in the histopathological analysis, and in the coordination of the study.JSL participated in the draft of the study, and in the conception of the study.JHL conceived the study, was the coordinator of the study, and drafted the manuscript.All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Appendicectomy specimens removed from patients with suspected acute appendicitis often appear macroscopically normal but histopathological analysis of these cases may reveal a more sinister underlying pathology. We evaluated histopathological reports of 1225 appendicectomy specimens at the Norfolk and Norwich University Hospital (NNUH) over the past three years.Histopathology reports for all appendices analysed at the NNUH between March 2003 and March 2006 were reviewed by examination of the case notes. The analysis focussed on the confirmation of acute appendicitis, incidental unexpected incidental findings other than inflammation, whether these abnormalities were suspected on gross examination at the time of surgery, and the effect on patient management and prognosis.The histopathology reports disclosed a variety of abnormal incidental lesions. Of the 1225 specimens, 46 (3.75%) revealed abnormal diagnoses other than inflammatory changes. Twenty-four (1.96%) of these were clinically significant and affected further patient management. Only two of these (0.16%) were suspected on macroscopic examination intra-operatively.Twenty-four of the 1225 specimens (1.96%) had an impact on patient management or outcome and were not suspected on macroscopic examination at the time of surgery. These would have been missed had the specimens not been examined microscopically. The intra-operative diagnosis of the surgeon is therefore unreliable in detecting abnormalities of the appendix. This study supports the sending of all appendicectomy specimens for routine histopathological examination. Acute appendicitis is the most common general surgical emergency . In Englet al suggest that appendices should not routinely be sent unless there is an obvious macroscopic abnormality at surgery [Clinical findings form the basis for diagnosis which may be consolidated by blood tests such as C-reactive protein and white cell count, and management is early appendicectomy . The pra surgery . They ar surgery -16.Less than 50% of appendiceal tumours are identified intraoperatively . FurtherWe report on the incidence of unexpected pathology in resected appendix specimens at the Norfolk and Norwich University Hospital.Between March 2003 and March 2006, 1225 patients underwent appendicectomy for a clinical presentation consistent with acute appendicitis. There were 642 males (52%) and 583 females (48%), with a mean age of 32 years (range 6 months to 90 years). Three-hundred and thirty-nine were removed laparoscopically (28%), 886 (72%) open, with an increasing trend towards laparoscopic removal over the past twelve months.Operative records stored electronically on the ORSOS (Operating Room Scheduling Office System) database were searched for "appendicectomy, emergency" from March 2003 to March 2006 inclusive. The histopathology reports of this cohort of patients were examined by searching the case notes. Findings were recorded as (a) evidence of acute appendicitis (including perforation and gangrene) and (b) incidental, abnormal findings. The case notes of the abnormal cases were further examined for subsequent investigations, follow-up and outcomes.Appendicectomy specimens are prepared according to a hospital-defined protocol, involving immediate fixing in formalin prior to transport to the pathology laboratory. Specimens are sectioned at the tip, body and base and are examined by a consultant or senior pathologist. Details of macroscopic and microscopic findings are issued in the final report.The pathology request forms submitted with the specimen and operative notes of the unexpected abnormal cases were reviewed for evidence that the pathologies were suspected on gross examination of the appendix by the surgeon intra-operatively.The patient notes of abnormal findings were also reviewed to determine the clinical significance of the findings. A result was defined as being clinically significant if further follow up investigations  or further surgical management was required, or if the result affected patient prognosis.Enterobius and 1 Schistosoma), 3 showed endometriosis and 6 showed Crohn's disease. Twenty-three showed benign tumours or tumour-like conditions . Three cases of malignant tumours were identified, 2 of which were primary adenocarcinomas and one of which was a metastatic ovarian adenocarcinoma.  reported changes consistent with acute inflammation . Two-hundred and eighty four (23%) appendices were within normal limits. Forty-six specimens revealed incidental abnormal diagnoses. Twenty-four of these were clinically significant and two were suspected by the operating surgeon (carcinoids). Eleven of these revealed intraluminal parasites  meeting. One patient went on to have colonoscopy and caecal biopsies, which were negative for tumour. The other underwent CT abdomen and pelvis, which revealed no abnormalities. Both patients are currently being monitored as out-patients with regular colonoscopies in line with the colon cancer protocol.Of the 13 carcinoids, 8 were completely resected. Five were not completely resected and required follow-up. All 5 were clinically significant. Only 2 of these were suspected by the surgeon intra-operatively on the basis of finding a firm yellow material at the tip. Both were large tumours (2.5 cm and 1.6 cm) with focal invasion of the mesoappendix. Although the resection margins were tumour-free in both cases, their large diameter was predictive of a high risk of recurrence and metastasis. Following MDT discussion, right hemicolectomy was performed on one of these cases. The other was also discussed at MDT and further resection deemed unnecessary due to the negligible mitotic rate. This patient as had follow-up CT scans due to unrelated renal pathology, which have revealed no further problems with respect to the bowel.Of the remaining three carcinoids, all were less than 1 cm and diagnosed microscopically and all underwent right hemicolectomy.The 3 cases of adenocarcinoma were all microscopic findings. Two were primary tumours and underwent right hemicolectomy. The pathologist's report subsequently confirmed complete clearance. The third was a metastatic ovarian carcinoma and received palliative care.The histopathological examination of the appendix serves two purposes. First, it allows the diagnosis of acute appendicitis to be confirmed, especially where this is not evident intra-operatively. Second, histopathological examination may disclose additional pathologies that may not be evident on gross examination intra-operatively but may affect subsequent clinical management of the patient. Specimens reported as negative for acute appendicitis are useful in eliminating acute appendicitis as a cause of symptoms and allowing further investigations to be performed should symptoms persist. Even in these negative appendicitis cases, patients' symptoms frequently disappear post-operatively. It has been suggested that in these cases there may be an early sub-clinical appendicitis .et al report an intra-operative detection rate of less than 50% for all types of appendiceal tumour [et al, suggested that surgeons missed abnormal pathological findings in 10 out of 13 patients, the majority of which required further investigation or treatment [The examination of certain surgical specimens, such as hernia sacs, and "doughnuts" following rectal surgery, does not yield further useful information and is unnecessary ,10. Howel tumour . Deans ereatment .Enterobius infection is often associated with acute appendicitis by causing intraluminal obstruction, and may be effectively eradicated by anti-helminthic treatment [In this study histopathological examination disclosed a variety of lesions. reatment . Hyperplreatment . Mucinoureatment . Carcinoreatment ,19. Adenreatment . Howeverreatment . These ireatment . For CT reatment . All patreatment .Table This study illustrates that intra-operative detection of abnormal appendices by the surgeon is unreliable and supports the sending of all appendicectomy specimens for routine histopathological analysis.The author(s) declare that they have no competing interests.AEJ and AWP conceived the study, collected and analysed the data and drafted and referenced the article. JRJ and KS reviewed the draft and made corrections and suggestions for improvement. AEJ revised the article. All authors read and approved the final draft.The study was funded by the authors. There are no additional acknowledgements.The pre-publication history for this paper can be accessed here: