Small bowel adenocarcinoma is a rare malignancy associated with a poor prognosis and there is little evidence of effective treatment. Recurrent small bowel adenocarcinoma is an intractable disease for which there is little information available regarding its treatment by palliative therapy. We present a case of recurrent small bowel adenocarcinoma successfully treated by cytoreductive surgery and palliative chemotherapy.We report the case of a 72-year-old Japanese female who developed a peritoneal metastasis from recurrent small bowel adenocarcinoma after curative resection and adjuvant chemotherapy with S-1 and polysaccharide K. She underwent cytoreductive surgery followed by chemotherapy with folinic acid/fluorouracil/oxaliplatin and folinic acid/fluorouracil/irinotecan with polysaccharide K. Subsequently, no sign of a recurrence was observed 42 months after the second operation.To the best of our knowledge, this is the first case report of the successful treatment of peritoneal metastasis from small bowel adenocarcinoma by cytoreductive surgery and combination chemotherapy . Primary malignancies of the small bowel represent only 2.4% of all gastrointestinal malignancies . AdenocaA 70-year-old Japanese female was referred to our hospital for the evaluation of a small bowel obstruction. She presented with nausea and loss of weight two months before she consulted her family doctor. An upper gastrointestinal barium study and stoppage of ileus tube movement demonstrated a tumor in the jejunum near the ligament of Treitz. Abdominal computed tomography (CT) revealed a huge uterine myoma. During surgery, we found the jejunal tumor located 20 cm away from the ligament of Treitz. We performed an enterectomy, including the regional mesentery and a hysterectomy.The jejunal tumor was a moderately differentiated adenocarcinoma, penetrating the small bowel wall. None of the 10 resected lymph nodes were positive for metastasis. The tumor was diagnosed as stage II according to the American Joint Committee on Cancer staging system. Although the usefulness of adjuvant chemotherapy for SBA has not been confirmed due to the lack of RCTs, recurrence after curative resection of SBA is very high ,4,8. We Nine months after the surgery, the serum tumor marker carcinoembryonic antigen (CEA) was elevated to 6.1 ng/mL . Although body CT and contrast magnetic resonance imaging (MRI) displayed no sign of recurrence, the CEA level was elevated to 8.9 ng/mL the following month. A careful evaluation of her medical history disclosed a hematuria. We consulted a urologist and a bladder tumor was discovered using a cystoscope. Positron emission tomography (PET) revealed fludeoxyglucose accumulation in the ascending colon, sigmoid colon, rectum and bladder. The histology of the bladder tumor resected transurethrally revealed a moderately differentiated adenocarcinoma invading from outside the bladder. A colonoscopy showed tumors in the sigmoid colon and rectum but not in the ascending colon. A histological analysis revealed that these tumors were well-differentiated adenocarcinomas, indicating peritoneal dissemination of SBA. We performed a laparotomy to confirm peritoneal dissemination and to reduce the number of disseminated tumors as cytoreductive surgery has been shown to be useful in some cases of peritoneal carcinomatosis from SBA .During surgery, we found two sigmoid colon tumors, one rectal tumor and one bladder tumor, identical to the preoperative diagnosis Figure . BesidesThe effectiveness of palliative chemotherapy for advanced SBA has been described in a number of retrospective studies ,15-17. WSBA is rarer than CRC or gastric cancer . This raet al. showed that the primary tumor site, performance status, resection status and development of complications predicted outcomes [et al. and Jacks et al., we resected all visible tumors. This patient survived for an extended period with good performance status (0), good resection status and no complications. The pattern of peritoneal carcinomatosis from SBA resembles that from CRC than from gastric cancer. An extrapolation of CRC treatment to SBA treatment seems necessary before confirming a standard treatment.Cytoreductive surgery including parietal peritonectomy with intraperitoneal hyperthermic chemotherapy has demonstrated the effectiveness of peritoneal dissemination for abdominal malignancies including advanced SBA ,10,18. Loutcomes . AlthougCytoreductive surgery and chemotherapy with FOLFOX/FOLFIRI plus PSK seemed useful for recurrent small bowel adenocarcinoma.CEA: carcinoembryonic antigen; CK: cytokeratin; CRC: colorectal cancer; CT: computed tomography; FOLFOX: folinic acid/fluorouracil/oxaliplatin; FOLFIRI: folinic acid/fluorouracil/irinotecan; MR: magnetic resonance; PET: positon emission tomography; PSK: polysaccharide K; RCT: randomized control trial; SBA: small bowel adenocarcinoma.Written informed consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.TY was the primary physician and surgeon, conceived the original study, organized and analyzed the data and prepared the draft of the manuscript. EM and KA were the pathologists and carried out the histological examinations. AI and TT carried out surgery, evaluated laboratory and imaging data and assisted with manuscript editing. All authors read and approved the final manuscript.


Myoepitheliomas of the extremity are rare and usually benign, while a minority display malignant features. This case demonstrates the diagnosis and management of myoepithelioma within the carpal tunnel. Clinical and radiological tumour features were evaluated. Hematoxylin and eosin stained tumour sections were examined, and immunohistochemistry was performed. Histology revealed a nodular mass of epithelioid cells in clusters within a myxoid/chondroid stroma. No mitoses were noted. Cytokeratins, neuron-specific enolase, synaptophysin, glial fibrillary acidic protein, and S100 were positive on immunohistochemistry. A literature review revealed very few prior reports of myoepithelioma in the wrist, and limited data concerning any relationship between recurrence and quality of surgical margins. In this case, wide local excision would have significantly compromised dominant hand function, and therefore a marginal excision was deemed appropriate in the context of bland histological features. Surgical margins noted in future case reports will aid clinical decision making. Myoepithelioma, also known as parachordoma, is a rare soft-tissue tumour more commonly associated with the salivary gland . It is aA 36 year old Caucasian male presented with a mass in the right wrist and paraesthesia in the distribution of the median nerve. The mass had been noticed four years prior to presentation and over this time had increased in size. On examination the mass projected from the volar aspect of the wrist but was poorly demarcated. It was firm to palpate and non-tender. Hand examination revealed attachment of the mass to the flexor digitorum profundus tendon of the index finger. Paraesthesia was exacerbated by repetitive use of all the long flexor tendons. No thenar eminence wasting was noted but some mild clinical weakness was evident in this muscle group.An initial MRI scan performed outside our institution demonstrated a T2 hyperintense lesion, 50 mm long by 14 mm wide, arising from the index finger profundus tendon within the palm and displacing the median nerve. Thallium-201 scintigraphy , neuron-specific enolase (NSE) , glial fibrillary acid protein (GFAP) , S100 protein , smooth muscle actin (SMA) , and synaptophysin . Diaminobenzidine was used as the chromogen.Histological examination revealed a nodular lesion composed of large epithelioid cells, singly and in clusters floating in a loose myxoid/chondroid stroma Fig. . The celPeripheral tumours with chondroid stroma include chondrosarcoma, ESMC, and ossifying fibromyxoid tumour (OFT). Chondrosarcoma was excluded by positive keratin markers and OFT was excluded by the clinical appearance, soft consistency and lack of ossifying and firm fibrous areas. ESMC demonstrates similar clinical and histological features to the reported lesion, but myoepithelioma was favoured, because of positive cytokeratins, GFAP staining Fig. , and S10Myoepithelial tumours are a class of primary soft tissue tumours that are becoming more frequently recognised, probably as a result of the increasing number and range of immunoperoxidase stains. The World Health Organisation (WHO) classification of tumours gives mixed tumour and parachordoma as synonyms of myoepithelial tumours . ApproxiMyoepitheliomas are found in the deep tissue planes in the extremities. Tumours in cutaneous planes are considered tumours of skin appendages rather than primary soft tissue tumours. A rare case, reported by Alberghini and co-workers , was fourd and 5th decades.In the myoepithelioma series of Hornick and Fletcher  the gendThe defining histological characteristics of these tumours are a lobular pattern on low power microscopy, a myxoid or chondroid matrix and an epithelial component which may have plasmacytoid, spindle and clear cell features Fig. . ImmunopThere are a number of important differential diagnoses for myoepithelioma arising in the carpal tunnel. Extraskeletal myxoid chondrosarcoma (ESMC), which has already been mentioned, is often associated with tendons supplying the hand, and can show very similar myxoid and chondroid matrix features to myoepithelioma. In our patient it was excluded mainly by positive cytokeratin markers . We wereSynovial sarcoma arises in similar peripheral locations to myoepithelioma and can also demonstrate myxoid areas and positive cytokeratin markers. It is differentiated from myoepithelioma on the basis of negative SMA and GFAP staining , and alsMalignant peripheral nerve sheath tumour (MPNST) is also an important diagnosis to exclude given the proximity to the median nerve, and its usual epithelioid morphology. MPNST also shares S100 expression and a myxoid stroma  with myoEpithelioid sarcoma, again a key differential for a mass in the wrist or hand, while cytokeratin positive , is easiOssifying fibromyxoid tumour (OFT) is a benign, although locally recurring lesion, with distinct similarities to myoepithelioma such as a lobular architecture, epithelioid component, myxoid matrix and focal bone formation . FurtherIn regard to clinicopathological correlation, Hornick and Fletcher  studied Mitotic rate in malignant salivary myoepitheliomas has been thought to be associated with an increased risk of death from disease although this is only based on a very small number of cases . DarvishSpace-occupying lesions are an uncommon cause of carpal tunnel syndrome, accounting for around 5% of cases, and strongly linked to a unilateral presentation with 35% of these patients having a space-occupying lesion . This emIn the carpal tunnel, the major dilemma associated with the resection of lesions with variable malignant potential concerns the option of curative surgery with wide margins which will permanently compromise hand function, versus a marginal/intralesional excision, which requires accepting a degree of recurrence risk and the potential for re-excision. As discussed, myoepithelial tumours can display subtle features of malignancy, and there is a reported 18% risk of local recurrence in lesions with low-grade cytology . HornickThis case demonstrates the challenge of diagnosing myoepithelioma, and differentiating it from other benign and malignant tumours. It shows that even when using CT guided core biopsy, a widely respected diagnostic technique, tissue samples may not adequately represent the true diagnosis. The difficulty in diagnosing myoepithelioma is also related to its rarity and similarity to ESMC. Differentiation relies on meticulous scrutiny of cell morphology and immunohistochemistry. While distal upper limb myoepithelioma is likely to be low grade or benign, there are still relatively few cases reported in this location, and so careful follow-up is advised.The authors declare that they have no competing interests.All listed authors have made a significant intellectual contribution to the preparation and drafting of this manuscript.Written informed consent was obtained from the patient for publication of this case report and the accompanying images.


Primary malignant fibrous histiocytoma (MFH) of the lung is extremely rare although it is among the most common soft tissue sarcomas in adults. Surgery is the primary mode of therapy, with high rates of local and distant recurrence, while radiation therapy appears to be a very useful adjunct, decreasing local relapse. We report a case of primary malignant fibrous histiocytoma of the lung. Fourteen years after surgical resection, the patient is still alive although with multiple metastatic lesions throughout his body. Malignant fibrous histiocytoma (MFH) occurs most commonly in the extremities , followed by the retroperitoneum. Tumors typically arise in deep fascia or skeletal muscle. MFH has been reported to occur in the lung, kidney, bladder, scrotum, vas deferens, heart, aorta, stomach, small intestine, orbit, CNS, paraspinal area, dura mater, facial sinuses, nasal cavity, oral cavity, nasopharynx, and soft tissues of the neck. Although metastases to the lung are common, primary lung malignant fibrous histiocytoma is extremely rare. We report a case of a 54-year-old male who in 1995 was histologically diagnosed of having primary lung MFH, reviewing the clinical and radiographic findings. He had a 15-pack-year history of cigarette smoking but had not smoked since 1986. The patient underwent surgical tumor resection but didn't receive any adjuvant therapy. Since then multiple metastatic sites have developed but he is still alive.The patient was admitted to our hospital for the first time in 1986 when he underwent a chest X-ray, as part of routine examination, which revealed a tumor shadow on left lung. Physical examination revealed decreased breath sounds over the left basal lung field. Complete blood count, serum biochemistry and tumor markers , were within normal limits. He denied to undergo bronchoscopy and returned home. The patient was readmitted to our hospital in 25/4/1995 suffering from severe cough, mucous expectoration and shortness of breath over a three-month-period. Radiologic examinations included chest chest X-rays and chest computerized tomography (CT) scan. The spiral chest CT scan revealed a large tumor of soft tissue density replacing most of the left lung and occluding the lumen of the left main bronchus, associated with atelectasis . A technα1-antichymotrypsin and factor Xlla. No adjuvant chemotherapy or irradiation was given, and the patient went home after 18 days of hospitalization and follow up CT scans every six months.Under these circumstances, the patient was consented for surgery and a left pneumonectomy was performed . HistoloIn 1997, the patient presented a nodule on his left shoulder and underwent excision and biopsy. The histological examination demonstrated tumor of mesenchymal origin which presented similar characteristics with the resected lung tumor.In 2008, a chest CT scan showed innumerable, well defined of variable size hypodense nodules which presented inhomogeneous contrast enhancement and scattered in the right lung, the left postpneumonectomy space and the mediastinum. It also revealed lesions of soft tissue density in various parts of the thoracic wall, some of which were associated with rib destruction . FurtherMFH is the most common soft tissue sarcoma in adults, comprising about 10% of all sarcomas, usually arising in the extremities and trunk . It is aWe must take into consideration the fact that primary MFH of the lung is extremely rare  with only about 50 cases been reported in the literature . There mWe must bear in mind that the majority of patients present chest pain, dyspnea, cough, and hemoptysis. Less common symptoms are hypertrophic pulmonary osteoarthropathy, hypoglycaemia, and neutrophilia. In addition, patients may be asymptomatic during their presentation.On the other hand, primary lung MFH of the lung appears on CT as a peripheral tumor of soft tissue density sometimes with areas of low attenuation centrally, bud rarely cavitated. Despite the fact that has been found pleural effusion in 20% of patients calcification has been described in only one adult patient in the literature . AlthougBesides, the final diagnosis is based on microscopic and immunohistochemical studies , histoloHalyard et al. describe in their review eight patients with primary lung MFH alive without evidence of disease more than 5 years after the diagnosis (two of them for 10 years) and one patient who died of the disease after a 6-year period. Regnar et al. reported two patients who survived 11 years after the operation . Rzyman Moreover, survival after complete surgical resection in cases of primary lung MFH has been reported to be better than for other pulmonary sarcomas . Many clLast but not least, the role of adjuvant radiotherapy and chemotherapy has not definitely been defined yet. Le Doussal et al. and Mills et al. clearly concluded that radiotherapy significantly reduces the risk of local relapse. Edbronson et al reported no improvement after systematic chemotherapy , but agaTo put it in a nutshell, we must take into account that primary sarcomas of the lung are rare. Patients with MFH of the lung must be carefully evaluated to rule out a metastatic origin, as MFH is primarily a soft tissue tumor of the extremities. Rates of local and distant recurrence remain high even after radical surgery, but rare cases of long-term survival have been reported. Histologic grade seems to be the most important prognostic factor for disease specific survival. Although the association between metastases and cancer-related deaths is strongly significant, our patient has survived over 14 years developing multiple metastases.


A patient with nevoid basal cell carcinoma syndrome (Gorlin syndrome) presented with two unusual clinical features, i.e. adenocarcinoma of the small bowel and extensive mesenchymal proliferation of the lower gastrointestinal tract.PTCH germ line mutation.We discuss the possibility that these two features are pathogenetically linked to the formerly undescribed patient's  PTCH) gene on chromosome 9q22. NBCCS patients often have a coarse facial appearance with macrocephaly, frontal bossing and prognathism. Falx calcification is frequently found in affected individuals and skeletal anomalies such as bifid ribs, wedge-shaped or fused vertebra and thumb deformities are common. Multiple keratocysts of the jaw can develop between childhood and young adulthood, and most patients get their first basal cell carcinoma (BCC) in their early 20s [Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a rare autosomal dominant disorder caused by mutations in the Patched  that centred at the muscularis mucosa and extended into the submucosa and the lamina propria of the mucosa. The nodules were characterized by unsharp delineation without forming capsules Figure . They weThe composition of smooth muscle cells, Schwann cells and neuronal cells and the multifocal occurrence within the small bowel did not fit into any known histopathological classification. These findings are best described as mesenchymal proliferation with neural and smooth muscle components.These changes were also found in the endoscopic biopsies taken from the terminal ileum and duodenum but not in those from the colon. Microscopic examination of the stomach-biopsies showed chronic gastritis due to H. pylori infection. One biopsy taken in the antrum of the stomach also showed the unusual spindle cell accumulations described above, but only in small areas close to the section border and without confirmation of neuronal components. Thus the diagnosis of mesenchymal proliferation with neural and smooth muscle components was only tentative for the nodules present in the stomach. Consultation of the Department of Pathology of Basel/Switzerland (Dr. E. Bruder) confirmed our findings.PTCH gene were mutated into guanine and adenine, respectively, changing a codon for tyrosine into a stop codon. This mutation has not been described before. It can be expected to completely abolish PTCH function, with serious consequences for the sonic hedgehog signalling pathway. The mutation confirmed the clinical diagnosis of NBCCS in our patient  in exon 8. The adjacent nucleotides adenine and cytosine in position 1136 and 1137 of the t Figure .PTCH germ line mutation exists. The same argument holds true for the mesenchymal proliferation present in the patient's GI tract. It is more likely that this previously not described finding is causally linked to the mutation in the tumor suppressor gene PTCH than that it represents the outcome of an entirely independent pathogenetic chain of events.The NBCCS patient described here displays two unusual clinical features, small bowel adenocarcinoma and extensive mesenchymal proliferation with neural and smooth muscle components of the small bowel. Endoscopic examination and histopathological studies of biopsies provided an educated guess that the stomach was involved too. Although the small bowel represents 75% of the length and 90% of the surface of the GI-tract, tumors of the small bowel account only for 2% of all GI-malignancies. The rarity of the respective conditions strongly argues against an independent occurrence of both, small bowel carcinoma and NBCCS in our patient. Given the odds it is much more likely that a causative relation between the carcinoma and the PTCH germ line mutations in NBCCS patients are either inherited from an affected parent or due to de novo events in a parental germ cell. The truncating germ line mutation found in our patient is likely to abolish the function of one copy of PTCH. However, it can not be completely excluded that a severely truncated form of the protein is produced that might interfere with the normal PTCH gene product, causing some kind of clinical phenotype. Tumorigenesis starts if inactivation of the second, normal PTCH allele by either loss-of-heterozygosity or point mutation occurs by chance in a single cell (two-hit mutagenesis). The resulting reactivation of the hedgehog/PTCH pathway not only causes basal cell carcinoma, but also contributes to the formation of tumors such as medulloblastoma and rhabdomyosarcoma [Helicobacter pylori infection has been identified as the main pathomechanism underlying this observation [PTCH is a second though probably rare pathomechanism in GI malignancies.The PTCH protein acts in a negative feedback pathway as a receptor for different hedgehog proteins . As a keosarcoma . Furtherosarcoma . Reactivosarcoma ., Ligandervation . The coeA highly unusual finding in our patient were the spindle-cell masses in the small bowel that displayed a high degree of cellular inhomogenity. Leiomyomatosis in the GI-tract has been reported occasionally in syndromes such as neurofibromatosis type 1 and tuberous sclerosis, but without the neural cell component found in our patient -11. It iPTCH stop codon mutation? The fact that mesenchymal proliferation of the small bowel has not been previously reported in NBCCS does not necessarily exclude it from the list of possible complications. As long as it remains asymptomatic such feature is likely to remain unrecognized because the small bowel is not included in routine diagnosis of the GI-tract. If the adenocarcinoma in our patient is indeed pathogenetically connected with the mesenchymal proliferation, the malignant potential of the later is probably too low to raise a red flag in a syndrome this rare. More patients with this rare familial tumor syndrome need to be clinically evaluated to see if the mesenchymal proliferation in the small bowel should be added to the phenotypic spectrum of NBCCS.Several questions remain: does our patient just represent an exotic case or is mesenchymal proliferation with neural and smooth muscle components of the small bowel a so far unknown clinical feature of NBCCS? Are both the mesenchymal proliferation and the small bowel carcinoma pathogenetically linked to the Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.PP and OS drafted and wrote the paper. PP, OS and MS designed the figures. JM, CS and GM corrected the paper. PP, GM and MS provided surgical and pathological guidance. All authors were involved in treatment and diagnosis of the patient and finalized and approved the manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/10/360/prepub


Biliary cystadenoma is a rare, benign neoplasm of the bile ducts with malignant potential. Symptoms, predominantly right hypochondrial pain and the feeling of a lump or fullness are usually due to the mass effect. Jaundice is rare. This is the fifth reported patient with an intrahepatic biliary cystadenoma giving rise to episodic biliary obstruction, which is usually caused by choledocholithiasis or periampullary carcinoma. Considering the mean age of previous similar patients , the early age of presentation is very unusual in our patient.A 25-year-old Asian woman presented with right hypochondrial pain and episodic biliary obstruction. Contrast enhanced computed tomography revealed a cystic mass in segment 4B and protruding into and along the left hepatic duct. Laparotomy confirmed the contrast enhanced computed tomography findings and histology revealed an intrahepatic mucinous biliary cystadenoma.Biliary cystadenoma should be considered as a differential diagnosis in patients with cystic liver lesions who present with episodic biliary obstruction. Due to the reported malignant potential, radical surgery such as wide local excision of the lesion or hepatic resection is needed to minimize the risk of local recurrence. Biliary cystadenoma (BCA) is a rare, benign cystic lesion of the liver, arising from the biliary ducts, typically lined with columnar epithelium, and usually with an "ovarian like" cellular stroma. The cyst is usually multilocular. A majority arise from the intrahepatic ducts, predominantly within the left lobe . They ocA 25-year-old Sri Lankan woman was referred to our unit with a preliminary ultrasound scan (USS) suggestive of a liver cyst. She had right hypochondrial pain, and several episodes of intractable itching. Only one of these episodes of itching was associated with clinically and biochemically proven obstructive jaundice.On examination, she was anicteric and did not have any features of liver disease. Abdominal examination was unremarkable.Liver function tests were normal. Serum tumor markers including carcinoembryonic antigen (CEA), α feto protein and CA 19-9 levels were within normal limits. An USS of the liver showed a 5.5 cm × 4 cm multiloculated intrahepatic cyst with several smaller cysts within. The intrahepatic ducts were slightly dilated, with the cyst probably extending into the proximal bile duct. Contrast enhanced computed tomography (CECT) confirmed the presence of the cyst in segment 4B of the liver which had an enhancing thin wall with multiple septa and no intracystic solid component , absence of a mesenchymal stroma is known to be more aggressive, especially in men .et al.[Jaundice is a rare presentation of BCA, and, to the best of our knowledge, episodic jaundice has only been reported in four patients ,5. Thereet al..Pre-operative diagnosis may be difficult. Generally on USS and CECT, they appear as focal lesions with internal septa, hence they may be confused with other cystic hepatic lesions such as a complicated cyst, mesenchymal hamartoma, undifferentiated embryonal sarcoma and cystic metastases in addition to the malignant counterpart of BCA and hydatid cyst . While tTreatment methods for BCA in the past have varied from aspiration, marsupialization, internal drainage, partial excision and enucleation ,9. The mThis article contributes to the medical literature by adding another reported case of episodic biliary obstruction due to an intrahepatic BCA protruding into the common hepatic duct, which may be considered as a very rare cause of intermittent jaundice. Pre-operative diagnosis may be confusing. Hence, a high degree of suspicion and a multidisciplinary approach are needed to plan the surgical procedure and prevent inadequate treatment. The surgical strategy would be to radically excise the lesion. A follow-up protocol is unavailable due to the small number of BCAs. However, we suggest long-term evaluation with clinical examination but more importantly with periodic ultrasound scans at least 3 monthly during the first year, 6 monthly for 2 years and annually subsequently to exclude local recurrence.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.PNS is the principal and corresponding author. PNS actively managed the patient and was the first assistant to the surgeon (AP). PNS contributed to the paper by performing the literature survey, and interpreting and analyzing past cases to decide on management of the patient. PNS wrote the manuscript and edited the successive versions. AP is the senior author and the team surgeon. He was a major contributor in interpreting the cases in the literature and applying them to the management of the patient. AP contributed to the paper by planning the structure and editing successive versions. Both authors read and approved the final manuscript.


Omental cysts are rare abdominal lesions and are difficult to diagnose. Mostly they are detected incidentally during imaging studies performed for unrelated reasons.Presentation can be both acute and chronic. Acute presentations are usually due to complication in cyst. Imaging is helpful in excluding other causes of lump abdomen.We encountered a case of giant lymphatic cyst presenting with abdominal swelling, clinically mimicking huge ovarian cyst.The goal of surgical therapy is complete excision of the cyst, and Omental cysts can be removed without endangering the adjacent bowel. A 42-year-old female presented to us with progressive distension of abdomen of one year duration. There was no history of anorexia or weight loss. It was associated with mild dull aching pain all over the abdomen. Her menstrual history was unremarkable. On examination, her abdomen was hugely distended. A vague soft, cystic lump was palpable involving whole of the abdomen. Routine blood tests were within normal limits. An abdominal Ultrasound examination was performed, and it showed a giant cystic lesion with internal echogenic particles and septations, extending from epigastrium to pelvis. All internal viscera were shifted postero-laterally. The origin of the lump could not be established. Provisional diagnosis of ovarian cyst was made and patient was advised to undergo CT scan. CECT showed the huge cystic lesion filling the whole peritoneal cavity with homogenous density Figure . The intPatient underwent laparotomy and a huge, soft cystic lesion arising from omentum was found. It was filling the abdominal cavity and has displaced all intraabdominal viscera posteriorly Figure . It was Omental cysts are rare and are mostly derived from lymphatic tissue. They are present in the lesser or greater omentum and are lined by endothelium. Omental cyst occurs in all age groups, but most often presents in children and young adults . GairdneWritten informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.AKK and RK: operating surgeons. NA: collected clinical details including photograph, summarized the case history and prepared first draft. SK: conducted a literature search and prepared final draft. All authors read and approved the final manuscript.


A case of a 70 year old man who was found to have an extrahepatic portal vein aneurysm during an evaluation for hematuria is reported. Extrahepatic portal vein aneurysms are rare with only twenty cases reported in the literature. Typically, patients present with hemorrhage requiring surgical exploration or the aneurysm is discovered during evaluation of another abdominal process. Management includes careful follow-up in the asymptomatic patient without underlying liver disease or portal hypertension.


Although diagnostic ureterorenoscopy is a minimally invasive and effective diagnostic procedure, it has the potential for significant postoperative complications. We report the first case in the literature of intrarenal arteriovenous fistulas causing hemodynamic effective anemia 4 days after ureterorenoscopic biopsy.A 63-year-old Caucasian woman presented with hemodynamic effective macrohematuria (hemoglobin 70 g/liter) 4 days after ureterorenoscopy and biopsy of the upper pole collecting system due to recurrent microhematuria. Duplex-sonography and computed tomography angiography revealed multiple arteriovenous fistulas and erosions into the calyceal system. Intra-arterial digital subtraction angiography confirmed this condition. After superselective embolization of the arteriovenous fistulas, the patient had no further episodes of bleeding or microhematuria.If malignancies, urolithiasis or urinary tract infections are ruled out by common diagnostic procedures as the cause of recurrent minor or gross hematuria, the possibility of arteriovenous fistulas should be included in the differential diagnosis and Duplex-Sonography or the more invasive selective renal arteriography should be performed as this is the most definitive method for diagnosing arteriovenous fistula. Although arteriovenous fistulas are rare conditions, they have a considerable clinical impact. In fact they may cause hypertension, local thrombosis, peripheral embolization, high output cardiac failure and hematuria. Although ureterorenoscopy is a minimally invasive and effective diagnostic and therapeutic procedure, it has the potential for significant postoperative complications. We report a case of intrarenal arteriovenous fistulas causing hemodynamic effective anemia 4 days after ureterorenoscopic biopsy.A 63-year-old woman presented with recurrent microhematuria. She had no history of flank pain, macrohematuria, hypertension, renal trauma or percutaneous instrumentation. Physical examination was normal and specifically, there was no abdominal bruit on auscultation. She had a blood pressure of RR 130/80 mmHg. Routine laboratory tests were within normal limits. Urinalysis showed no evidence of infection but was positive for erythrocytes. An initial renal ultrasound revealed a discrete hypoechogeneity of the left upper renal pole. An intravenous pyelogram was performed demonstrating an irregular configuration of the upper pole collecting system, which was also seen in a retrograde ureteropyelography  were then carried out and revealed multiple arteriovenous fistulas (AVFs) and erosions into the calyceal system. Intra-arterial digital subtraction angiography  but may also appear in the midportion or the lower pole of the kidney in equal ratio topographically beneath the calyceal or pelvic mucosa. Congenital AVFs are characterized angiographically, as in our patient, by their cirsoid configuration with multiple communications between arteries  and veins [Based on the angiographic criteria, a second form of congenital AVF exists which is classified as the aneurysmal type and has been mentioned in the literature as a spontaneous or idiopathic fistula . While tIn the pericalyceal renal parenchyma, the small interlobular arteries and their corresponding veins as well as existing AVFs are in close proximity to the collecting system. This explains recurrent hematuria in more than 75% of individuals and possible filling defects or reduced function of the affected kidney in the excretory urography, but these are absent in 50% of cases . In our In contrast to patients presenting with hematuria, we suggest nephrectomy or partial nephrectomy as the treatment of choice in individuals with symptoms of alterations in the cardiovascular system such as renin-mediated hypertension due to fistula-related relative ischemia or high-output cardiac failure caused by increased venous return.Congenital AVFs are rare conditions which may cause cardiovascular complications (in 50% of cases) and recurrent hematuria in more than 75% of individuals.If malignancies, urolithiasis or urinary tract infections are ruled out by common diagnostic procedures as the cause of recurrent minor or gross hematuria, the possibility of AVFs should be included in the differential diagnosis and Duplex-Sonography, or the more invasive selective renal arteriography, as the most definitive method for diagnosing AVF, should be performed. Depending on the general condition of the patient and their symptoms, the treatments of choice include nephrectomy and partial nephrectomy but most urologists aim for superselective embolization.AVF: arteriovenous fistula; CTA: computed tomography angiography; DSA: digital subtraction angiography; i.a. DSA: intra-arterial digital subtraction angiography; RR: blood pressure (measured by the technique of Riva Rocci); URS: ureterorenoscopyThe authors declare that they have no competing interests.MS made substantial contributions to acquisition and interpretation of the data and drafted the manuscript. TW carried out the imaging studies and performed the embolization. WK managed the critically ill patient clinically and also contributed substantially to the interpretation of the literature.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Odontogenic myxoma represents an uncommon benign neoplasm comprising of 3–6% of all odontogenic tumors. This article presents a rare case of odontogenic myxoma occurring in the maxilla of a 7-year-old male patient with a brief review of the pathogenesis, clinical, radiological, histopathological, ultrastructural and immunohistochemical characteristics of odontogenic myxoma. Odontogenic myxoma of the jaws is a rare benign tumor characterized grossly by mucoid or gelatinous grayish-white tissue that replaces the cancellous bone and expands the cortex. It is th2Most frequently, odontogenic myxomas occur in the second or third decades of life.454Children and persons over 50 years of age are seldom affected. Myxomas In this article, we present a case of odontogenic myxoma occurring in the maxilla of a 7-year-old boy followed by a review of the literature.A 7-year-old boy was referred to Modern Dental College and Research Center, Indore, for the evaluation of a swelling on the right side of his face. His medical history revealed that he developed a fever 18 months prior to his admission, which resolved within 6–7 days, but subsequently developed a mild swelling on the right side involving the middle third of the face. The swelling was painless, but the patient noticed intermittent watery nasal discharge from the right nostril. One and a half years later, when the patient reported to our department, he had a bony hard, nontender swelling of approximately 4 × 3 inches, extending superoinferiorly from the infraorbital ridge to 1 inch above the inferior border of the mandible and anteroposteriorly from the right corner of the mouth to 1.5 cm anterior to the tragus. The borders of the swelling were diffused and the skin overlying the swelling was normal in color. The right lower eyelid and the eyeball were pushed upward .Oral examination revealed a nontender, bony hard swelling extending from the maxillary right lateral incisor to the right maxillary tuberosity, thereby obliterating the right buccal vestibule. The associated teeth showed grade-I mobility. However, the adjacent gingiva and oral mucosa appeared normal .The orthopantamograph revealed a unilocular radiolucent lesion extending from 14 to 17. Teeth in the affected region showed displacement and root resorption . ComputeFine needle aspiration cytology showed few stellate and spindle-shaped cells within the myxoid background.On gross examination, the incisional biopsy specimen appeared as a smooth, glistening, gelatinous, lobulated mass. Its color varied from grayish-white to yellow .Histopathological examination of the biopsy specimen revealed the typical features of a myxoma, containing loosely arranged stellate or spindle-shaped cells within a myxoid matrix. Few bony spicules with osteoblastic rimming were also seen at the periphery of the lesion . At placen bloc excision of the maxilla. This mode of treatment was selected because of the high recurrence rate of these tumors after conservative treatment. The course has been uneventful for 6 months after surgical removal of the tumor.Under the histopathologic diagnosis of odontogenic fibromyxoma, the patient underwent removal of the tumor with a partial Odontogenic myxoma (fibromyxoma) is a benign neoplasm of uncertain histogenesis with a characteristic histologic appearance. It often shows infiltration and behaves in a locally aggressive fashion.There has been a great deal of controversy regarding the origin of myxomatous tumors. Virchow, in 1863, coined the term myxoma for a group of tumors that had histologic resemblance to the mucinous substance of the umbilical chord.[Myxomas of the head and neck are rare tumors. Two forms can be identified: (1) facial bone derived, which had been subdivided in the past into true osteogenic myxoma and odontogenic myxoma and (2) “soft tissue”-derived myxoma, derived from the perioral soft tissue, parotid gland, ear and larynx.Traditionally, the myxoma of the maxilla and mandible has been considered to be a neoplasm of odontogenic origin. Although the evidence is mainly circumstantial, support of an odontogenic origin has been perpetuated by its almost exclusive occurrence in the tooth-bearing areas of the jaws, its common association with an unerupted tooth or a developmentally absent tooth, its frequent occurrence in young individuals, its histologic resemblance to dental mesenchyme, especially the dental papilla and the occasional presence of sparse amounts of odontogenic epithelium.et al. supported the notion of odontogenic origin of myxomas by suggesting that fibroblasts that compose the tooth germ undergo modification to give rise to odontogenic myxoma.[In a recent immunohistochemical and ultrastructural study, Moshiri c myxoma.Slootweg and Wittkampf on the other hand showed that the matrix of myxomas of the jaw is entirely different from the matrix seen in the dental pulp and periodontal ligament. In addition, they also argued that myxomas may also develop in the sinonasal tract and other facial bones that originate from the nonodontogenic mesenchyme. According to them, even the presence of odontogenic epithelium is not necessary to make the diagnosis of myxoma of bone.Contrary to the findings of Slootweg and Wittkampf, McClure and Dahlin reviewed more than 600 bone tumors of patients at Mayo Clinic and concluded that there were no true myxomas of the bone except for those found in the mandible and maxilla.et al. reviewed 164 odontogenic myxomas of the jaw and found that 75% occurred between the second and fourth decades .[Kaffe 0 years).et al. differentiated between maxillary and mandibular odontogenic myxomas and suggested that the mean age at the time of diagnosis of maxillary odontogenic myxomas in men was 29.2 years and in women was 35.3 years, while the mandibular odontogenic myxomas in men occur at a mean age of 25.8 years and in women they occur at 29.3 years.[et al.[et al.[Farman .3 years. Gunhan es.[et al. and Regel.[et al. reportedHarder held the view that the odontogenic myxomas only rarely occur before the age of 10 years. However,Mandibular myxomas accounted for 66.4%, with 33.6% in the maxilla. Whereas 65.1% of the mandibular cases were located in the molar and premolar areas, 73.8% cases were seen in the same areas of the maxilla. In the pet Al. found expansion of the jaws in 74% of the cases. When the maxillary sinus is involved, the odontogenic myxomas often fill the entire antrum. In severe cases, nasal obstruction or exopthalmus may be the leading symptoms.[Most odontogenic myxomas are first noticed as a result of a slowly increasing swelling or asymmetry of the affected jaw. Lesions are generally painless and ulceration of the overlying oral mucosa only occurs when the tumor interferes with dental occlusion. Growth may be rapid and infiltration of neighboring soft tissue structure may occur. Both the buccal and the lingual cortical plates of the mandible may expand occasionally. Kaffe etsymptoms. AlthoughOn conventional radiographs, myxomas of the jaws often show multilocular radiolucencies representative of “honey comb,” “soap bubble” or “tennis racquet” appearance, which helps in distinguishing this entity from malignant tumors arising centrally within the jaw bones, because the latter usually cause massive bone destruction without compartments formed by bony trabeculations or bony septa. In the pComputed tomographic images of odontogenic myxomas may show any of the following features:Osteolytic expansile lesions with mild enhancement of the solid portion of the mass in the myxoma of the mandible.Bony expansion and thinning of cortical plates with strong enhancement of the mass lesion in the anterior maxilla.A soft tissue mass with bone destruction and thinning and strands of fine lace-like density representing ossifications in the maxillary sinus.A similar finding was noticed in the computed tomographic image of the present case. Magnetic resonance imaging (MRI) revealed a well-defined, well-enhanced lesion with homogenous signal intensity on every pulse sequence. The lesion showed intermediate signal intensity on the T1-T2-weighted images. UnfortunOn gross examination of the specimen, the gelatinous, loose structure of the myxoma was obvious.Microscopically, the myxoma is made up of loosely arranged spindle-shaped and stellate cells, many of which have long fibrillar processes that tend to intermesh. The loose tissue is not highly cellular, and these cells do not show evidence of significant activity . The intercellular substance is mucoid. The tumor is usually interspersed with a variable number of tiny capillaries and occasionally strands of collagen. In case et al. reviewed the histochemical findings in odontogenic myxomas. The ground substance of odontogenic myxomas has been shown to consist of about 80% hyaluronic acid and 20% chondroitin sulfate. Tumor cells appear to be relatively inactive, with low levels of oxidative enzymes. Tumor cells also show slight alkaline phosphatase activity. The myxoid intercellular matrix stains positively with alcian blue, but PAS staining may be negative.[Farman negative.Odontogenic myxoma tumor cells are mesenchymal in origin and express vimentin and muscle-specific actin. Conflicting description of S-100 and GFAP positivity has been reported. The matren bloc resection and no recurrence was reported even after 6 months of the surgery.An extensive study on the ultrastructure of odontogenic myxomas was published by Goldblat in 1976. Two basic types of tumor cells were described, secretory and nonsecretory. The secretory cell type was considered the principal tumor cell and resembled fibroblasts. While geMyxomas of the head and neck are rare tumors of uncertain histogenesis. Extensive emphasis should be levied on to determine the origin of these locally aggressive myxomas and thereby put to rest various controversies surrounding these lesions.


Purpose: Glomus tumours are characteristically benign solitary tumours. At our knowledge, about 23 reports are present inliterature regarding the malignant counterpart, but only a minority developed metastases. We describe a locally aggressiveglomus tumour with lymphnode metastasis.            Patient: The patient was a 40 year-old man presenting a 1.5-cm lesion on the right wrist incompletely excised and a recurrenttumour, 4 × 2 cm in size, removed after 9 months, for which he received radiotherapy. After 2 years he developed an axillarylymphnode metastasis.            Results: Histologically, both tumours (primary and metastasis) were similar. There were sheets and nests of uniform smallcells with scant eosinophilic cytoplasm and round to polygonal nuclei; there was some degree of pleomorphism and themitotic index was high (up to 18 m/10 HPF). The tumour cells were positive for vimentin and smooth muscle actin, butnegative for desmin, NSE, Factor VIII, chromogranin, cytokeratin. Remarkably, in the primary, the cells strongly expressedp53 (70%) and MIB-1 (35%).            Discussions: In many reported malignant cases, the histology of the tumour cells suggested that they were malignant, yet theclinical course has been benign. Carefully reviewing the literature, it seems that actually we have enough histological criteriato identify the cases with biological adverse outcome. Those unfortunate cases behave as high grade sarcomas and thereforemay deserve an aggressive therapeutic treatment.


Mucinous adenocarcinoma is a rare neoplasm of the gastrointestinal tract and one of the three major histological subtypes of the primary appendiceal adenocarcinoma. The most common type of presentation is that of acute appendicitis and the diagnosis is usually occurred after appendectomy. The accurate preoperative diagnosis and management of the above condition represents a real challenge when uncommon anatomic anomalies such intestinal malrotation and situs inversus take place. Situs inversus totalis with an incidence of 0.01% is an uncommon condition caused by a single autosomal recessive gene of incomplete penetration in which the major visceral organs are mirrored from their normal positions.We present an unusual case of a 59 years old, previously healthy man presented with a left lower quadrant abdominal pain, accompanied with low fever, leukocytosis, anorexia and constipation. A chest radiograph demonstrated dextrocardia with a right side positioned stomach bubble. Both preoperative US and CT scan of the abdomen and pelvis declared situs inversus, with a characteristic thickening in its wall, appendix situated in the left lower quadrant of the abdomen. These findings reached to the diagnosis of acute appendicitis with situs inversus and a standard appendicectomy was performed. Pathologic evaluation established primary mucinous adenocarcinoma of the appendix and three months afterwards the patient underwent a subsequent extended left hemicolectomy.In conclusion, the occurrence of primary appendiceal mucinous adenocarcinoma along with situs inversus, definitely accounts as a unique clinical case. Even synchronous manifestation of primary mucinous adenocarcinoma of the appendix and situs inversus totalis represents an unusual anatomo-pathological entity, all physicians should be familiar having the knowledge to make an appropriate and accurate diagnosis that will lead to prompt and correct treatment. Appendiceal carcinomas represent a relatively uncommon clinical entity. A recently published population-based study, concentrated in the period 1973-1998, from The National Cancer Institute's (NCI) Surveillance, Epidemiology, and End Results (SEER) program concluded that the age-adjusted incidence of appendiceal malignancies is approximately 0.12 cases per 1,000,000 people per year. The same study suggested the mucinous adenocarcinoma as the most frequent histologic type among the appendiceal identified carcinomas, while carcinoid was the second most frequent histologic diagnosis [Mucinous adenocarcinoma represents an uncommon neoplasm of the gastrointestinal tract and one of the three major histological subtypes of the primary appendiceal adenocarcinoma ,2 occupy2 and 240 mg/m2 respectively, either 200 mg/M2 of oxaliplatin for a 2-h chemoperfusion [The formal right hemicolectomy is considered as the recommended treatment for all patients with nonmetastatic adenocarcinoma of the appendix. Though mucinous' adenocarcinoma spread to adjacent organs is detectable at presentation in a percentage 63%, the approximate overall 5-year survival rate at about 20.5%, arising to 55-60% after right hemicolectomy for nonmetastatic disease  with a mild suffering secondary to point tenderness on palpation of his left lower quadrant. Left-sided "Rebound" and "McBurney's" signs were also noted. Laboratory tests showed mild leukocytosis (11.6 × 109/L), accompanied with moderate polymorphonuclear predominance (89%) and an elevated CRP concentration (1 mg 55/L) and normal blood biochemistry analysis.A standard preoperative chest radiograph demonstrated dextrocardia, with the stomach bubble situated on the right as well. Therewithal both Ultrasound (US) and Computed tomography (CT) abdominal scanning revealed situs inversus, with the appendix in the left lower quadrant of the abdomen, with remarkable thickening in its wall. It should be underlined that until then the patient had been unsuspicious of having situs inversus. These findings reached to the diagnosis of acute appendicitis with situs inversus and a standard appendicectomy with a McBurney - like (oblique) left lower quadrant muscle-splitting incision was performed. The patient recovered uneventfully and two days after was exited the hospital in a fine condition.The appendix was submitted for histopathological examination. On gross examination accumulation of mucus within the lumen and focal thickening of the wall were observed. Histological examination revealed a mucinous adenocarcinoma of the appendix Figure , 2 with Three months after the pathologic evaluation, the patient underwent a subsequent extended left hemicolectomy, in the pattern of the formal extended right hemicolectomy, with resection as well of 19 lymph nodes. On histology, no malignancy was indentified, only three lesions of tubular adenomas, while the lymph nodes presented plainly reactionary inflammation. No evidence of local recurrence or metastatic disease is confirmed by the standard follow-up, including yearly CT scanning of the abdomen Figure , 4, and Primary mucinous adenocarcinoma of the appendix constitutes a scarce malignancy of the appendix and often associated with a second GI malignancy of the gastrointestinal tract. Usually the appendiceal malignancies are mistaken for acute appendicitis and therefore their diagnosis follows appendectomy. Even the coexistence of primary appendiceal mucinous adenocarcinoma along with situs inversus totalis, definitely accounts as a unique clinical case, all physicians should be familiar having the knowledge to make an appropriate and accurate diagnosis that will lead to prompt and correct treatment.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in Chief of this journal.The authors declare that they have no competing interests.AtP has edited the manuscript. AlP has operated and managed the patient. NK has edited the manuscript. KB has operated and managed the patient. KE has diagnosed and edited the manuscript and EF has supervised the whole attempt. All authors read and approved the final manuscript.


Primary malignant neoplasms of the liver are some ofthe most uncommon malignancies in many parts of the world. Theyinclude hepatocellular carcinoma and stromal tumors such ashepatic angiosarcoma. It is a lethal tumor with life expectancyof less than six months. Once discovered, it is often too latefor surgical intervention. Like other vascular tumors of the liverand spleen, intraperitoneal hemorrhage is a well-documentedfinding of angiosarcoma which can be lethal if not diagnosed andtreated immediately. As in our case, intraperitoneal hemorrhagefrom primary tumor rupture was the only clinical presentation ofthis neoplasm. Approximately 15% of patients present withacute hemoperitoneum from either tumor rupture or peritonealmetastasis. Although several therapeutic options are available,we describe apalliative therapy for hepatic angiosarcoma utilizingtranscatheter arterial chemoembolization (TACE) techniquesincorporating the newer embolization agent Embospheres to locallytarget and treat this aggressive tumor. A 54-year-old male with a past medical history significant for hemachromatosis, coronary bypass graftsurgery, with an ejection fraction of 20%, presented to an outside institutionwith clinical and computed tomography findings of acute intraperitoneal hemorrhage. At that time, an emergentmesenteric angiogram was obtained demonstrating a multicentricpathological vascular mass involving the right hepatic lobe  and mixture of Doxorubicin and Mitomycin C.During embolization, stasis of flow wasidentified within the tumor vascularity consistent with occlusion of the neovascularfeeding vessels to the mass. Successful embolization was noted fluoroscopically with diffuseuptake of the ethiodol solution within the tumor resulting in a “snow storm” uptakepattern  was advanced into the right hepatic artery and aselective hepatic arteriogram was obtained with particular attention to pertinenthepatic arterial anatomy. A diffuselyvascular enhancing mass consistent with thediagnosis of hepatic angiosarcoma was confirmed along with other satellite massesin the superior surface of the right lobe. Again, no arterial contrastextravasation was noted on this exam to suggest intraperitoneal hemorrhage. The portal vein was patent. TACE wasperformed with a combination of ethiodol (lipidol) , Embospheres 500–700 Hepatic malignancies include hepatocellular carcinoma, gastrointestinal and nongastrointestinalmetastases, and primary or metastatic sarcomas . A primaRecently,transarterial placement of yttrium-90 glass microspheres , SIR-sphere , and LCbeads  for the treatment of unresectable hepatocellularcarcinoma and colorectal metastasis respectfully are available in the United States . Also, d


Objective: Desmoid tumors are rare benign neoplasms of fibroblastic origin, frequently presenting in the abdomen of adult patients. Little is reported about clinical features and outcome of desmoid tumors appearing in infants and children, especially when they are located in the face. Methods: We report a girl with a desmoid tumor of her cheek, which was present at birth. Results: The tumor was treated by surgical excision, with no additional treatment, other than follow-up. No recurrence of the tumor was witnessed 15 months following surgical excision. Summary: Radical surgical treatment with tumor excision, sometimes accompanied by radiotherapy, is the current standard therapy for infantile desmoid tumors. Pharmacological treatment may be added in selected cases. These modalities may be challenging when desmoid tumors appear in the face because of risks of facial distortion and associated growth problems. Desmoid tumors are benign fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. This overgrowth of fibrous tissue is typically infiltrative, well-defined, and firm. Desmoid tumors are locally aggressive but lack the potential to metastasize.1Common anatomic sites for appearance of desmoid tumors include extremities, abdominal wall, and mesentery. Desmoid tumors in the head and neck are uncommon in all age groups.Infantile aggressive fibromatosis represents the childhood form of the musculoaponeurotic fibromatosis in adults.In this article, we report an infant girl with a desmoid tumor in the deep layers of her cheek. The clinical and imaging findings are followed by a histopathological description of the tumor.A 3-month-old girl with a history of a solid, immobile mass in her right cheek presented to our clinic. The lesion was present at birth and did not change dramatically in the first 3 months of life. The mass was firm on palpation, well-defined, and mobile Fig .Magnetic resonance imaging (MRI) study performed at this age demonstrated a hyperintense fibrotic mass in the subcutaneous layers of the right cheek, penetrating to the upper lip Fig .An incisional biopsy from the mass was performed. The histopathologic examination was nondiagnostic but was suspicious for a rhabdomyomatous mesenchymal hamartoma. With this working diagnosis, the patient was followed closely with a view to excision at a later age.An additional MRI study performed, when the girl was 17 months old, demonstrated the same mass, which did not change in size or appearance from the previous MRI.At this stage, a wide surgical excision of the mass was performed using the Weber-Fergusson approach. The skin above the mass was spared.3. Microscopically, the lesion consisted of subcutaneous fat and muscle with an infiltrative process composed of haphazardly arranged bland spindle cells on a collagenous background, invading adjacent muscle fibers, and focally reaching the cauterized surgical margin. There was no necrosis, and no mitotic figures were observed. The immunohistochemical stain for β-catenin was positive in several nuclei. Focally, cells were also positive for α-actin. A desmin immunostain was negative. The findings were compatible with desmoid-type fibromatosis.The gross pathological examination of the specimen revealed a solid, gray, ill-defined mass measuring 0.8 × 0.9 × 1.3 cmThe previous biopsy was reexamined, and the histopathological analysis was found to be compatible with an invading edge of fibromatosis Fig .The patient had no family history of Gardner syndrome or familial adenomatous polyposis. The patient was presented to the pediatric oncology tumor board. Because of the young age and the problematic location of the tumor, close clinical and radiological follow-up was recommended. A decision was made to avoid postoperative radiotherapy in this young girl because of the potential side effects of irradiation in this site and age.No recurrence of the tumor was witnessed 15 months after the surgical excision Fig .Desmoid tumors are histologically benign, fibrous neoplasms originating from the musculoaponeurotic structures of the body. They often appear as infiltrative, usually well-differentiated, firm overgrowths of fibrous tissue and tend to be aggressive locally.APC (adenomatous polyposis coli) tumor suppressor gene, which is known to be a tumor suppressor gene. Desmoid tumors are associated with the bi-allelic APC mutation. One of the 2 known mutations occurs distal to the second β-catenin binding/degradation repeat of the gene.Desmoid tumors are common in patients with familial adenomatous polyposis, an autosomal inherited disease caused by a genetic mutation in the Desmoid tumors are reported to account for 0.03% of all neoplasms.Although desmoid tumors are more common in the second to fifth decades of life, they can appear in younger children and older adults.Infantile aggressive fibromatosis represents the childhood version of desmoid tumors seen in adults.Polyposis coli and Gardner syndrome and tend to be related to abdominal surgery.In the adult form, desmoid tumors most commonly develop in the anterior abdominal wall (arising from the rectus abdominis muscle) and in the shoulder girdle but may arise in any skeletal muscle. Retroperitoneal desmoids are common in familial In their subcutaneous locations, desmoid tumors are firm, smooth, and mobile. They often adhere to surrounding structures but usually spare the overlying skin. Desmoids were reported in intra-abdominal locations such as the urinary system. However, desmoid tumors are extremely rare in the head and neck, especially in infants and young children.APC gene. Proliferative response of fibroblasts to estrogen was established.3The pathogenesis of desmoid tumors is uncertain and may be related to trauma, hormonal factors, or mutation in the Computed tomographic (CT) scan and MRI are used for diagnosis and follow-up of desmoid tumors. MRI is better than CT scan in defining the pattern and the extent of involvement as well as recurrence after surgery.Histologically, desmoid tumors are composed of abundant collagen surrounding poorly circumscribed bundles of spindle cells. These eosinophilic spindle cells contain regular nuclei and a pale cytoplasm. No mitoses or giant cells are present, a finding that helps differentiate these tumors from fibrosarcoma.1Treatment of desmoid tumors consists of aggressive surgical resection with clear surgical margins. Positive margins after surgery can be associated with a high risk of recurrence (˜70%).Radiation therapy can be considered for treatment of recurrent disease or as a primary modality used to avoid a mutilating surgical resection. Radiotherapy yields local control rates of approximately 75% to 80%.Pharmacologic therapy with antiestrogen and prostaglandin inhibitors. The rationale behind this treatment is based on the possible endocrine etiology of desmoid tumors in which fibroblast proliferation is seen in response to estrogen. This hypothesis is supported by the fact that desmoid tumors most commonly appear in young women during or after pregnancy; the tumors regress during menopause, after tamoxifen treatment, and after exposure to oral contraceptives.Chemotherapy  is reserved mostly for recurrent extra-abdominal desmoid tumors.In patients who refuse surgery or represent poor surgical candidates, the following alternative modalities of treatment may be considered:Clinical features associated with high rate of recurrence include presentation at greater than 5 years of age, tumor location at the extremities, and an incomplete surgical resection.Histological features associated with high rate of recurrence include microscopic evidence of tumor at the surgical margins, a mitotic index of 5 or more per 10 high-power fields, and evidence of necrosis and inflammation within the tumor.4Infantile aggressive fibromatosis is a rare entity. It arises as a solitary mass in the skeletal muscle, adjacent fascia, aponeurosis, or periosteum. Microscopically it can present in 2 forms: the diffuse  type and the desmoid type. The less common desmoid form is rarely seen in infancy. Several case reports have described an aggressive infantile fibromatosis involving the mandible.12In most of these cases, radical surgical excision of the tumor and reconstruction of the mandible was the treatment modality, with no evidence of recurrence. However, in some cases, conservative surgical resection of the tumor was conducted with adjuvant chemotherapy and radiotherapy to minimize the recurrence rate and limit the surgical mutilation.9Other case reports described desmoid tumor in the submandibular region of a 3-year-old girl20A single-case report describing an extra-abdominal fibromatosis of the cheekA retrospective review dealing with treatment of desmoid tumors in the head and neck was recently published. Tostevin et al22The use of chemotherapy in infantile desmoid tumors was suggested in one study. The authors treated 3 children with progressive unresectable fibromatosis in unfavorable locations with pegylated liposomal doxorubicin . Tumor response was obtained on MRI in all patients. All children have retained a normal cardiac function after the completion of chemotherapy as evaluated by left-ventricular shortening fraction. Severe neutropenia was not observed.23APC gene. Desmoid tumors of the head and neck are uncommon in all age populations. Infantile aggressive fibromatosis is the childhood version of fibromatosis in adults. Complete surgical resection followed by radiation therapy is the current standard therapy. Pharmacological treatment may be added in selected cases.Desmoid tumors are benign, rare, firm fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. These tumors are infiltrative and locally aggressive and thus tend to recur but lack the potential to metastasize. The pathogenesis of desmoid tumors is uncertain and may be related to trauma, hormonal factors, or a mutation in the Our report of an infant with a desmoid tumor on her cheek represents an example of the desmoid type of infantile aggressive fibromatosis arising in an exceedingly rare location. Surgical excision resulted in a complete removal of the tumor without evidence of local recurrence in 15 months' follow-up period.


Leiomyosarcoma of the right atrium is a very rare cardiac tumor. Various combinations of treatments including resection or transplant surgery and Chemotherapy have been advocated. We report a case of a man who presented with pulmonary embolism secondary to right atrial leiomyosarcoma. He was managed by excision of the tumor and reconstruction of the right atrium with autologous pericardium. Postoperatively tumor dissemination was controlled with adjuvant chemotherapy.A vigorous attempt aiming at tumor clearance followed by adjuvant multimodality therapy along with a tumor surveillance program may improve survival. Primary cardiac malignancies (PCM) are rare. The prevalence of primary cardiac malignancies has been estimated at only 0.001% - 0.28% . MetastaWe present a case of a 36 year old male who was admitted with recent onset of shortness of breath. CT pulmonary angiogram demonstrated large right sided pulmonary emboli Figure . MoreoveAlthough resection margins were clear the margin width was deemed to be inadequate. After recovery from surgery the patient was submitted to chemotherapy with Doxirubicin and Isofomaide. A tumor surveillance plan involving serial echocardiograms is planned.Of the few hundred cases of malignant tumors of the heart reported, most have been based on autopsy series. Moreover, throughout the last 30 years literature from a 100 plus articles on cardiac neoplasms, only few publications are reporting on primary cardiac malignancies .Cardiac sarcomas are the second most common type of primary cardiac neoplasms with Leiomyosarcomas to consist of 8% of cardiac sarcomas ,7. As peLeiomyosarcoma favors the left atrium and most likely originates from pulmonary vein smooth muscles and present as a left atrial tumor. Affected patients typically present in the 4th decade of life which is slightly younger than the average age at presentation for cardiac sarcoma patients. Unlike angiosarcomas hemorrhage is unusual. Those tumors are likely to involve the mitral valve and extend into the pulmonary veins and therefore present with pulmonary congestion. Macroscopically the tumor appears as gelatinous mass and maybe multiple in up to 30% of the cases .In our case report the site of origin of the tumor was the right atrium which is rare. The presentation was consistent with thromboembolic phenomenon commonly associated with this tumor site. Other possible presentations include progressive or sudden right sided cardiac failure on the account of tricuspid valve blockage by the tumor or paroxysmal atrial arrhythmias. Broadly speaking, cardiac tumors produce a large variety of symptoms through any of 4 mechanisms. Their mass can obstruct intracardiac blood flow or interfere with valve function. Local invasion can lead to arrhythmias or pericardial effusions with tamponade. Bits of tumor can embolize, causing systemic deficits when the tumors are on the left side of the heart. Finally, the tumors may cause systemic or constitutional symptoms.Echocardiographic imaging is the most sensitive imaging technique with ability to identify tumors as small as 3 mm. However, soft-tissue characterization remains limited compared with that achieved with computed tomography (CT) and magnetic resonance (MR) imaging, and myocardial disease such as tumor infiltration is not clearly depicted . On the In this case CT scan raised the suspicion of intracardiac tumor by depicting a low attenuation filling defect and echocardiography confirmed the diagnosis. A preoperative tissue diagnosis was not attempted due to the emergent presentation. However atypical appearance of the right atrium and of the tumor raised the suspicion of malignancy and frozen section examination was confirmatory. It has been recommended that all atrial tumors should be subjected to frozen section examination in order to ensure optimum surgical resection.According to Mayer et al half theOperative mortality has been reported to be high at 8.3% with an overall actuarial survival of 14% at 24 months after resection . LikewisAs per Burke et al , the surThe optimum treatment of Leiomyosarcoma is not known. Of the several reports in the literature, patients subjected to multimodality treatment including heart transplantation  have loWe adopted a strategy that would ensure local control of the tumor by surgical resection and address systemic spread by adjuvant chemotherapy. Tumor shrinkage can be achieved by chemotherapy prior to surgery in non emergency setting. Given the high risk of tumor recurrence we plan to follow-up the patient with serial echocardiographic scans with the view to further surgical or chemotherapeutic intervention aimed at early treatment of recurrence.In conclusion PCM are rare and will always pose a diagnostic dilemma. Nevertheless, atypical presentation of suspected "atrial myxoma" should raise the possibility of rare atrial tumors. Unfortunately, almost half of those tumors have metastasized at presentation, up to 30% could be multifocal and the rest may be amenable to surgery. All such tumors should be subjected to frozen section examination intraoperatively.Surgery carries a high mortality and the overall long survival was only achieved in patients who survived the initial surgery well.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for the review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.HP conceived of the study and wrote the manuscript with the help of MTA. VY overlooked the progress of the manuscript and advised on valuable points. All authors read and approved the final manuscript.


Intramural dissection of the esophagus is a rare disorder characterized by a lesion between the submucosa and mucosa dividing the esophagus into a false and true lumen. The etiology of esophageal dissection remains uncertain but it affects predominantly women in their seventies and eighties. Symptoms may include uncharacteristic ones such as retrosternal pain, odynophagia or dysphagia. Conservative management is thought to be adequate and surgery should only be performed if complications such as abscess formation or perforation appear. Here we report the case and surgical management of a combined esophageal perforation and dissection.We report the case of a combined esophageal perforation and dissection in a 45-year-old Caucasian woman with a history of relapsing periods of dysphagia since her childhood. The clinical course in this patient was complicated by progression to a second perforation, which made a definitive surgical management by esophagectomy necessary.To the best of our knowledge, this is the first reported case of a combined esophageal perforation and dissection complicated by progression to a second perforation. This emphasizes that cautious and intensive observation is necessary in patients with esophageal dissection. According to their extent, esophageal injuries are classified into (i) transmural, short and predominantly distally localized perforations, (ii) mucosal, short lesions in the distal esophagus, and (iii) intramural dissections -3]. Int. Int3]. Here we report a very rare case of transmural esophageal dissection with complete transmural perforation after endoscopic recovery of an impacted pearl onion  in a patient suffering from chronic dysphagia since her childhood. To the best of our knowledge, this is the first reported case of esophageal dissection progressing to complete perforation.A 45-year-old German Caucasian woman was transferred to our department of general surgery with a suspected esophageal perforation after endoscopic recovery of a pearl onion which was impacted in the middle third of the esophagus. After recovery of the pearl onion, upper gastrointestinal endoscopy revealed slightly bleeding mucosa at the site of impaction as well as an impassable stenosis. Her medical history, included relapsing periods of dysphagia since her childhood that were never examined by endoscopy or gastrografin swallow. At admission, she presented in a stable condition with normal laboratory findings. Clinical investigation revealed no abnormalities other than retrosternal pain as well as emphysema of the skin. Chest and abdominal radiography showed free intra-abdominal air as well as a pneumomediastinum. A gastrografin swallow was performed revealing leakage at the distal esophagus and pneumoperitoneum Figure . ExploraBecause of pathological drainage continuing on the tenth postoperative day as well as increasing white cell counts, we performed a gastrografin swallow that revealed mucosal irregularities and a double-barreled esophagus Figure . Upper gThese findings left us without any feasible conservative management options. The presence of mediastinitis due to a suspected transmural perforation prompted us to perform a transhiatal esophagectomy with cervical esophagostoma and blind closure of the stomach.The pathology report showed the true esophageal lumen and a transmural perforation as well as a second lumen that was focally covered by a flat squamous epithelium with multiple ulcerations within the submucosal layer Figure . The posThe etiology of esophageal dissection still remains unclear. Two pathogenetic theories have been postulated . The firBecause symptoms such as severe central chest pain might be uncharacteristic, more frequent diseases such as myocardial infarction, aortic dissection, or gastrointestinal ulcer are often initially suspected .Diagnostic principles in esophageal intramural dissection include radiological contrast swallow, CT of the chest and/or endoscopy of the upper gastrointestinal tract ,6. TypicIntramural dissection of the esophagus has a good prognosis and can be managed conservatively with initial intravenous fluid and nutrition . If, durIn our patient, it is speculative whether esophageal dissection originated from abnormal swallowing after impaction of a pearl onion or due to the endoscopic procedure. We hypothesize that the initial trauma permitted esophageal dissection as well as distal perforation. The false lumen probably remained initially invisible because of intramural formation of a hematoma that prevented contrast media from entering it. This theory is supported by the histological examination of the resected esophagus which identified a partial epithelial lining in the false lumen and which may occur after several days.To the best of our knowledge, we have described the first case of a combined esophageal dissection and perforation complicated by progression to a second perforation which underlines that cautious and intensive observation is necessary in patients with esophageal dissection.CT: computed tomography.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.All authors analyzed and interpreted the patient data. UR performed the histological examination of the organs. AK, EB and MP were major contributors in writing the manuscript. All authors read and approved the final manuscript.


Schwannoma originating from the vagus nerve within the mediastinum is a rare, usually benign tumor. A 44-year old male was presented with chest pain. Chest radiography, CT scan and MRI showed a well circumscribed mass, 5 × 4 cm located in the aortopulmonary window. The mass was found at surgery to be in close proximity with the aortic arch and the left pulmonary hilum, alongside the left vagus nerve. The encapsulated tumor was completely resected through a left thoracotomy incision and it was found to be a benign schwannoma in pathology. The patient is free of recurrence 6 years after surgery. Neurogenic tumors represent approximately 20% of all adult and 25% of all pediatric primary mediastinal neoplasms. They are divided into nerve sheath, ganglion cell and paraganglionic cell neoplasms . Neuroge2-weighted sequences [Scwannomas are lobulated, encapsulated spherical masses, different from neurofibromas in that matter. Men and women are equally affected in their third and fourth decades . Usuallyequences ,7.A 44-years old male presented with a sense of heaviness and pain in the left anterior chest wall. Preoperative chest radiography showed a sharply demarcated extrapulmonary mass withour calcifications in the middle mediastinal compartment, between aortic arch and left hilum. Contrast-enhanced CT scan of the chest showed a 5.1 × 4 cm mass in the aortopulmonary window, with smooth and clear margins, low densities (HU: 9-15) and light enrichment after administration of contrast material HU: 22-37), without calcifications and fat (Figure 2-37, witth intercostal space preserving the latissimus dorsi instead of VATS procedure because of the proximity of the tumor to the aortic arch and the left main pulmonary artery. Complete resection, as is the case for benign schwannomas, was performed of this 5 × 4 cm tumor, located in close proximity to the vascular structures of the aortopulmonary window, alongside the left vagus nerve which was left intact. The tumor was removed with its entire capsule and was found to be benign at histology, because there were no atypia, mitoses, increased cellularity and necrosis. (Figure He underwent a left anterolateral thoracotomy in the 4The patient had no postoperative complications and he is free of recurrence 73 months later.Benign schwannomas of the vagus nerve are very rare middle mediastinal neurogenic tumors of nerve sheath origin ,2. SchwaThe tumor was confirmed to be a schwannoma because microscopically it showed spindle cells in fascicles in a loose stroma. Strong positivity of the tumor cells for S-100 protein confirmed the diagnosis of schwannoma -7. MaligThe long-term survival after complete resection of the extremely rare malignant scwhannomas of the vagus nerve seems to be satisfactory in sporadically reported cases ,9.Written informed consent was obtained from the patient for publication of this case-report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.All authors have read and approved the final manuscript. KSR performed the operation, has been involved in drafting the manuscript and has given the final approval to publish the manuscript. SKR has been involved in critically revising the manuscript. CNF has made contribution to design of the manuscript and has been involved in drafting and critically revising the manuscript. TKZ did the histology and immunohistochemistry of the tumor and has been involved in drafting the manuscript


Synovial hemangioma is a rare condition and is frequently misdiagnosed, leading to a diagnostic delay of many years.We present a case of an atypical synovial hemangioma in a 12-year-old Caucasian boy with a diagnostic delay of 3 years.It is important to know that synovial hemangioma mostly affects the knee joint, showing recurrent bloody effusions without a history of trauma. If there are no intermittent effusions, the diagnosis will be even more difficult. In cases of nonspecific symptoms and longstanding knee pain the diagnosis of a synovial hemangioma should also be considered in order to avoid diagnostic delay. Magnetic resonance imaging is the main diagnostic tool to evaluate patients with synovial hemangioma, showing characteristic lace-like or linear patterns.Angiography can identify feeder vessels and offers the possibility of embolisation in the same setting. Surgical excision, either done per arthroscopy or per arthrotomy, is recommended as soon as possible to avoid the risk of damage to the cartilage. Hemangiomas of bone constitute 1% of all primary bone tumours. The soft tissue types are even less common and often arise in the skin and subcutaneous tissue. Muscle and synovial linings are less frequent sites of origin. Since the first case was described by Bouchut in 1856, fewer than 200 cases have been reported. Most cases have been the intra-articular and intermediate type of hemangio-hamartoma, another form of vascular tumour of the leg representing an arteriovenous malformation which involves the synovia and causes intra-articular bleeding. Only a few of these have been true synovial hemangioma [Usually the patient presents with a history of recurrent atraumatic bloody effusions -4.Nonspecific presentations are also common and may lead to a diagnostic delay of many years . We presIn the absence of specific vessels to embolise, surgical excision, either done per arthroscopy or per arthrotomy, is the treatment of choice.A 12-year-old Caucasian boy presented with a history of pain and swelling in his left knee joint for 3 years for which he had received no previous treatment. His physical examination revealed a soft, non-tender, palpable 3 × 4 cm mass on the medial aspect of his left knee. In full flexion the mass appeared more pronounced. He denied any history of trauma and there was no effusion in his knee, with the joint not showing any signs of instability; he also had a full range of knee motion and normal strength in the lower extremities. McMurray and Apley tests were negative. There was no difference in leg length and there were no cutaneous lesions. Laboratory tests, including a complete coagulation profile, were all within normal range and his medical, developmental and family histories were unremarkable.Plain radiographs and magnetic resonance imaging (MRI) scans were obtained Figures , 2 and 3Due to the differential diagnosis, an incisional biopsy was performed first, strictly according to the guidelines of orthopaedic tumour surgery. The biopsy specimen was 4 cm in diameter, measuring synovial tissue. An intraoperative frozen section showed a hemangioma with huge, cavernous spaces but also containing capillary vessels. Because of the diffuse extension of the hemangioma, angiography was done some days later in order to find some feeding arteries and to embolise them preoperatively in the same session. Angiographically, neither the hemangioma nor any feeding arteries could be visualized. Arthrotomy, through an anteromedial longitudinal skin incision, followed due to the diffuse extension of the tumour. The extra-articular and intra-articular masses were excised and the postoperative course was uneventful. The final histological evaluation confirmed a cavernous synovial hemangioma (Figure Synovial hemangiomas are frequently misdiagnosed leading to a diagnostic delay of many years; there are even reports of delays of up to 20 and 40 years .Usually, a patient presents in childhood with a history of recurrent atraumatic painful bloody knee effusions -5. TheseAngiography should be part of the diagnosis; it can define the size and location of the lesion and can identify feeder vessels or an associated arteriovenous malformation ,2.It must be performed early in cases of associated cutaneous hemangioma or abnormal varicosity, because these findings are indicative of a more general vascular abnormality .In those instances selective embolisation of feeder vessels is an interesting alternative to surgery . AngiogrTreatment methods have varied in the past and include radiotherapy, open surgical resection, arthroscopic excision, arthroscopic ablation with a holmium, YAG laser, embolisation, and the use of sclerosing agents, cautery and freezing ,15. SomeSynovial hemangioma is a rare condition and mostly affects the knee joints. Recurrent bloody effusions without a history of trauma should alert the surgeon to this diagnosis. If there are no intermittent bloody effusions there may be a diagnostic delay of up to many years. For this reason a synovial hemangioma should also be considered in cases with nonspecific presentations and longstanding knee pain. If a synovial hemangioma is assumed, plain films are often of poor diagnostic value and magnetic resonance imaging is the main diagnostic tool to evaluate patients with a suspected synovial hemangioma. Angiography should also be part of the diagnostic approach as it can identify feeder vessels and offers the possibility of embolisation in the same setting. Several treatment methods have been proposed but in our opinion the treatment of choice is surgical excision; if the tumour is pedunculated and intra-articular, arthroscopy is the treatment of choice. If the synovial hemangioma is an intermediate type then arthrotomy should be performed. In any event, treatment should be initiated as early as possible to reduce the risk of damage to the cartilage.Written informed consent was obtained from the patient's parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SZ: study concept and design, patient care, drafting the manuscript AVA: study concept and design, patient care, data analysis, literature review, drafting and revising the manuscript JH: study concept and design, patient care, drafting the manuscript MH: data analysis, literature review and drafting the manuscript PR: study concept and design, patient care, drafting the manuscript. All authors read and approved the final manuscript and all participated in this work.


Scar or incisional endometriosis is a rare, often misdiagnosed, pathologic condition of the abdominal wall. Two cases of incisional endometriosis are presented. Both patients presented with atypical cyclic pain and palpable nodules on scars of previous cesarean sections. In both cases, the mass was totally excised, after accurate preoperative evaluation with 2-D ultrasound, power Doppler and MRI. Microscopic examination confirmed the preoperatively presumed diagnosis of cutaneous endometriosis. In cases of suspected scar endometriosis, preoperative diagnostic imaging is valuable in determining the extent of disease, thus enhancing accurate and total excision. Extraperitoneal endometriosis, i.e. the presence of ectopic, functional endometrium outside the peritoneal cavity is exceedingly rare. Cutaneous endometriosis is a form of extraperitoneal endometriosis, sometimes associated with previous laparoscopic or open abdominal operative procedures . DiagnosA 28 year-old white woman, G2P1, presented with atypical cyclic pain on a small, firm lump at the outer margins of the scar of a previous cesarean section, performed five years earlier. Patient history details were as follows: Occupation: housewife; Ethnicity: Greek; Weight 75 Kg; Height: 165 cm; Medical history: one previous cesarean section, otherwise unremarkable; Family history: unremarkable; Patient habits and medications: non-smoker, no alcohol consumption, no current medications. Sonographic examination of the abdominal scar showed a hypoechoic mass, with internal echoes, infiltrating the subcutaneous fat, extending up to the sheath of the rectus abdominis muscle Figure . Power DA 29 year-old white woman, G2P1, presented complaining of atypical cyclic pain on the scar of a previous cesarean section, performed six months earlier. Patient history details were as follows: Occupation: Secretary; Ethnicity: Greek; Weight: 65 Kg; Height: 167 cm; Medical history: one previous cesarean section, otherwise unremarkable; Family history: unremarkable; Patient habits and medications: non-smoker, no alcohol consumption, no current medications. On clinical examination, a firm lump was found on the abdominal scar. Ultrasound scan showed a large hypoechoic mass with internal echoes, measuring approximately 10 mm, located at the lateral margin of the scar Figure . Power DCutaneous endometriosis developing on scars of previous operations is a rare condition. Scar or incisional endometriosis is usually confined in superficial layers of the abdominal wall, but it may sometimes infiltrate deeper layers and present in exceptional cases even as a uterocateneous fistula . VariousIncisional endometriosis presents typically as a firm, palpable lump at the site of a surgical scar, usually accompanied by cyclic pain and swelling during menses. Cyclic bleeding may also occur. Cyclic symptoms and signs should alert to clinical diagnosis of endometriosis. Macroscopically, the mass is usually non-discrete, rubbery and often multiloculated, containing chocolate cysts. Differential diagnosis includes hernias, lipomas, hematomas, abscesses, cheloids, suture granulomas, sebaceous cysts, as well as malignant tumors, including desmoid tumors, sarcomas, lymphomas or primary malignancies of the skin and metastatic tumors .Due to the rarity of incisional endometriosis, there is no data available concerning cost-effectiveness of different diagnostic methods. At a first glance, the simplest and less costly approach would be excisional biopsy followed by histological examination of the lesion, without prior imaging evaluation. However, this may lead to inadequate excision, and subsequently disease recurrence, necessitating re-excision. On the other hand, preoperative evaluation with imaging techniques can facilitate total surgical excision. On 2-D sonography, scar endometriosis lesions may appear as cystic or multicystic, mixed or solid masses, with internal vascularity on power Doppler. Though these findings are not specific , 2-D sonCombined oral contraceptives, progestogen-only therapy and GnRH-analogues have been used in the therapeutic management of cutaneous endometriosis, but recurrence is common after discontinuation of treatment. On the other hand, wide excision of the whole lesion, even if this necessitates fascial excision, leads to permanent cure. Recurrence is rare following surgical treatment, and is usually attributed to inadequate excision .In conclusion, use of diagnostic imaging, including 2-D ultrasound, power Doppler sonography and MRI, in the preoperative assessment of suspected scar endometriosis lesions is very helpful for accurate determination of the extent of disease. This approach enhances total surgical excision, which is crucial for definitive diagnosis and avoidance of disease recurrence.2-D: Two-dimensional; MR(I): Magnetic Resonance (Imaging); G: Gravida; P: para; GnRH: Gonadotropin Releasing Hormone.The authors declare that they have no competing interests.GP conceived the study and participated in patient management acquisition of data, interpretation of data, and was a major contributor in writing the manuscript. JT participated in patient management, acquisition of data, and drafting of the manuscript. MZ revised critically the manuscript adding substantial intellectual content. DA participated in patient management, acquisition of data, and drafting of the manuscript. JB coordinated the study and patient management and revised critically the manuscript. All authors have read and approved the final manuscript.Written informed consent was obtained from both patients – in their native language – for publication of this case report and accompanying images. Copies of the written consent are available for review by the Editor-in-Chief of this journal