In male cattle, rupture of the urinary bladder is usually associated with urethral obstruction by uroliths. Less common causes include urethral compression or stricture. This case report describes the findings in a young Limousion breeding bull with rupture of the urinary bladder because of urethral compression by a haematoma after coccygeal fracture.The bull had been introduced into a 40-head Red-Holstein herd one week before being injured. One week after introduction to the herd, the bull had an acute onset of anorexia and he was referred to the clinic. There was marked abdominal distension, reduced skin turgor and enophthalmus. The serum concentration of urea and creatinine was increased. Ultrasonographic examination revealed severe ascites and abdominocentesis yielded clear yellow fluid with high urea and creatinine concentrations, which supported a diagnosis of uroperitoneum. The bull was euthanatized because of a poor prognosis. Postmortem examination revealed a comminuted fracture of the first two coccygeal vertebrae associated with a massive haematoma that obstructed entire pelvic cavity. The haematoma compressed the urethra thereby preventing outflow of urine, which resulted in a 5-cm tear ventrally at the neck of the bladder. It was assumed that the newly-introduced bull had sustained the vertebral fractures when he was mounted by a cow.The present case study serves to expand the differential diagnosis of urinary bladder rupture. Therefore, in addition to obstructive urolithiasis, compression and stricture of the urethra might be considered in male cattle with uroperitoneum. Rupture of the urinary bladder is usually associated with dystocia in cows ,2, and uThe patient was a 1.3-year-old Limousin bull that had been introduced into a 40-head Red-Holstein herd one week before being injured. The herd was housed in a free stall barn with access to a courtyard as well as pasture during the day. One week after introduction to the herd, the bull had an acute onset of anorexia and disturbed general condition. Clinical examination by the referring veterinarian revealed abdominal distension and positive swinging and percussion auscultation of the abdomen on the left side, which was interpreted as left displacement of the abomasum. The bull was referred to the Department of Farm Animals, University of Zurich. On admission, the general condition and behavior of the bull were disturbed and there was reduced skin turgor, enophthalmus and scleral injection. The rectal temperature was normal at 38.7°C, the heart rate was increased at 96 beats per minute and there was marked abdominal distension. The rumen was atonic and distended, and swinging and percussion auscultation were positive on the left side of the abdomen. Intestinal motility was reduced and the abdominal wall was tense. Transrectal examination of the abdomen was not possible because the pelvic cavity was severely constricted. There was a reduced amount of faeces, which were olive coloured and porridge-like in consistency. A urine sample could not be collected.The results of haematological examination showed haemoconcentration with a haematocrit of 54% , leukocytosis with neutrophilia , hyperproteinaemia with a total plasma protein concentration of 106 g/l, normal 60 – 80 g/l) and hyperfibrinogenaemia . The serum concentration of urea  and creatinine  were increased. The serum concentrations of sodium, potassium and chloride were lower than normal and that of inorganic phosphorus was increased . The results of blood gas analysis revealed a compensated metabolic alkalosis with a base excess of +7.7 mmol/l  and a blood pH of 7.41 .Ultrasonographic examination revealed severe ascites involving the entire abdomen. The fluid appeared homogeneous and anechoic, and abdominocentesis yielded clear yellow fluid that smelled of urine. The urea and creatinine concentrations were 57 mmol/l and 7’042 μmol/l, respectively, which supported a diagnosis of uroperitoneum.Based on all the findings, a diagnosis of uroperitoneum caused by urinary bladder rupture was made. The cause was thought to be obstructive urolithiasis. The mass causing pelvic obstruction could not be interpreted, but rule-outs included inflammation, tumour, abscess and haematoma. Because of a poor prognosis, the bull was euthanatized and underwent postmortem examination. This revealed a comminuted fracture of the first two coccygeal vertebrae associated with a massive haematoma that obstructed the entire pelvic cavity. The haematoma compressed the urethra thereby preventing outflow of urine, which resulted in a 5-cm tear ventrally at the neck of the bladder. The final diagnosis was rupture of the urinary bladder because of urethral compression by a haematoma after coccygeal vertebral fracture.Inability to defecate has been reported as a complication of fracture of the proximal coccygeal vertebrae , but to Urine osmolality is two to three times higher than the osmolality of interstitial fluid . The osmThe most common cause of urinary bladder rupture in male cattle is obstructive urolithiasis. In cows, uroperitoneum due to urinary bladder rupture is most often associated with dystocia ,2,11, buThe present case study serves to expand the differential diagnosis of urinary bladder rupture. Therefore, in addition to obstructive urolithiasis, compression and stricture of the urethra might be considered in male cattle with uroperitoneum.The authors declare that they have no competing interests.UB prepared the manuscript and supervised the clinical examination, LT examined the bull, TS performed the postmortem examination. All authors have read and approved the manuscript.


Activating oncogenic mutations of BRAF have been described in patients with gastrointestinal stromal tumor (GIST), but treatment of GIST with BRAF inhibitors and mechanisms of mediating the emergence of resistance in GIST have not been reported. Dabrafenib is a potent ATP-competitive inhibitor of BRAF kinase and is highly selective for mutant BRAF in kinase panel screening, cell lines, and xenografts. We report prolonged antitumor activity in the first patient with V600E BRAF-mutated GIST who was treated with a BRAF inhibitor. Whole exome sequencing performed in tumor tissue obtained at the time of progressive disease demonstrated a somatic gain-of-function PIK3CA mutation (H1047R) as well as a CDKN2A aberration, which may have contributed to eventual resistance to treatment. Gastrointestinal stromal tumor (GIST) is a malignancy of mesenchymal origin that arises in the gastrointestinal tract and is resistant to conventional cytotoxic chemotherapy agents. KIT andClinical trials of tyrosine kinase inhibitors that are highly selective for V600 BRAF mutations have demonstrated high response rates (50-80%) in BRAF-mutant melanoma, as well as improvement in overall survival and progression-free survival-11. ReceHerein, we report antitumor activity in the first patient with BRAF-mutated GIST who was treated with a BRAF inhibitor. Whole exome sequencing of tumor obtained at time of progressive disease did not reveal secondary BRAF or RAS mutations, but did demonstrate a somatic gain-of-function PIK3CA mutation (H1047R) as well as a CDKN2A aberration, which may have been responsible for dabrafenib resistance.A 60 year old man initially presented in September 2007 with abdominal pain and a palpable mass. Computed tomography (CT) revealed a 10 cm heterogeneous mass, and a subsequent biopsy demonstrated GIST, spindled cell histology, positive for CD34 and CD117 by immunohistochemistry with 6 mitoses per 10 high-powered fields. The patient underwent surgical resection revealing a 15 cm mass. DNA was extracted from formalin-fixed paraffin-embedded tumor tissue and subjected to polymerase chain reaction (PCR) amplifications of KIT exons 9, 11, 13, and 17 as well as PDGFRA exons 12 and 18. Sanger sequencing did not identify mutations in either the KIT or PDGFRA genes. The patient presented with a new 14 cm mass at the dome of the bladder after 10 months of adjuvant imatinib therapy (400 mg once daily). The imatinib dose was increased to 800 mg daily, followed by surgical resection of the mass. The patient received adjuvant sunitinib, a multiple tyrosine kinase inhibitor, at a dose of 50 mg on a schedule of once daily for four weeks, then off for two weeks. Nineteen months later, a PET/CT showed recurrent FDG-avid masses in the right internal iliac region and in the right abdomen extending into the rectus abdominis.The patient enrolled on a clinical trial with an investigational KIT/PDGFRA/VEGFR tyrosine kinase inhibitor, but disease progression was noted at his first restaging (two months of treatment). Further testing of the patient’s original tumor revealed a V600E BRAF mutation. The patient was then treated with an investigational MEK inhibitor for three months, during which the tumor initially remained stable but was subsequently found to have enlarged and remained enhancing by CT imaging.The patient was treated on a phase I trial of dabrafenib at a dose of 150 mg twice daily. The patThe patient remained on study for 8 months, after which tumor progression was noted by contrast-enhanced CT imaging. The only treatment-related adverse events were grade 2 rash and acrochrodons (skin tags), as well as grade 1 fatigue and hyperkeratosis of the plantar surface of the feet. After tumor progression was identified, the patient underwent surgical resection of all visible tumors in the abdomen and pelvis. Tissue from this resection was evaluated with whole exome sequencing.To fully account for intratumor heterogeneity, which can be a factor in tumor adaptation and treatment failure, three lBRAF inhibitors have demonstrated antitumor activity in clinical trials of patients with BRAF mutant malignancies-11. We rActivating oncogenic mutations of BRAF have been described in many malignancies, including cutaneous melanoma (67%), colorectal carcinoma (12%), non-small cell lung carcinoma , and KIT wild-type GIST (13%),17. The Dabrafenib is a potent ATP-competitive inhibitor of BRAF kinase and is highly selective for mutant BRAF in kinase panel screening, cell lines, and xenografts. DabrafeKinase inhibitors targeting BRAF have the potential to be an effective therapeutic option for BRAF-mutant GIST patients. The preWhole exome sequencing did not reveal secondary BRAF or RAS mutations but did demonstrate a somatic gain-of-function PIK3CA mutation (H1047R), that has previously been reported in other human cancers. We specIn conclusion, we present the first patient with GIST and a V600E BRAF mutation whose tumor showed regression while receiving treatment with a BRAF inhibitor. To our knowledge, the efficacy of BRAF inhibitors in BRAF-mutant GIST has not been reported, but our case suggests that additional studies and perhaps a global clinical trial are warranted.Whole exome capture was performed with a SeqCap EZ Human Exome v2.0 kit , and sequencing was carried out on a HiSeq 2000 instrument . Sequence alignment and variant calling were performed with DNAnexus software . Tumor-specific variants were identified based on a minimum variant allele ratio of 20%, a minimum read depth of 20, and absence of the variant in a matched normal specimen. Nucleotide variants were translated, and non-synonymous variants were identified using SIFT, PolyPhe


Uterine sarcomas are relatively rare tumors that account for approximately 1-3% of female genital tract malignancies and between 4-9% of uterine cancers. Less than 8% of all cases are Mullerian adenosarcoma, a distinctive uterine neoplasm characterized by a benign, but occasionally atypical, epithelial and a malignant, usually low-grade, stromal component, both of which should be integral and neoplastic constituents of the tumor. Mullerian adenosarcoma with sarcomatous overgrowth (MASO) is a very aggressive variant, associated with post-operative recurrence, metastases, even when diagnosed in early stage.We present a fourth MASO case derived from uterine cervix in a 72-year-old woman with metrorrhagia and a polypoid mass protruding through the cervical ostium. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, systematic pelvic lymph node dissection, omental biopsy and appendectomy were performed. Surgery treatment was associated with adjuvant whole-pelvis radiation (45 Gy) and adjuvant chemotherapy (cisplatin/ifosfamide). After nine months of follow up, the patient was free of tumor.The rarity of MASO of the cervix involves a management difficult. Most authors recommend total abdominal hysterectomy, usually accompanied by bilateral salpingo-oophorectomy. There is no common agreement on staging by lymphadenectomy during primary surgery and adjuvant chemo-radio therapy. Uterine sarcomas are relatively rare tumors that account for approximately 1-3% of female genital tract malignancies and between 4-9% of uterine cancers has beenParticular interest in this treatment is covered by Mullerian adenosarcoma, whose term was introduced by Clement and Scully  in 1974 MASO of the uterine cervix is extremely rare. In this report we present a MASO case, derived from uterine cervix of a 72-year-old woman. To our knowledge it is the fourth case reported in the English literature for its location and the second MASO case of the cervix with heterologous elements.A 72-year-old Caucasian woman, Para 2012, in menopause from the age of 51 years, who had never taken hormone replacement therapy (HRT), presented with metrorrhagia from about 20 days. In physiological anamnesis the patient reported urinary incontinence and nocturia, she denied taking oral contraceptive in fertile-age. Pelvic examination revealed an elongated fleshy polyp, protruding through the cervical ostium, probably from endocervix. At the transvaginal ultrasonography revealed a polypoid mass subverting completely the uterine echostructure, with a thin residual posterior margin of intact myometrium. Intense neovascularization. The polypoid mass was removed for biopsy. Macroscopically, the lesion was measured as 3 × 1, 5 × 1, 5 cm. Microscopically, it was composed of a mucus-blood material encompassing lymphocytes, histiocytes and atypical elements of neoplastic nature. There were markedly anaplastic focal areas composed of pleomorphic spindle cell proliferation. About the immunophenotype, vimentin was positive and desmin was positive in focal areas, but keratin (MNF116) was negative. These findings deposed for a mesenchymal neoplasm.The patient has performed an abdominal Computed Tomography contrast medium that shows a solid lesion with axial diameter of about 6 cm, with inhomogeneous densitometry, assuming contrast medium, surrounded by a thicker rim, containing gas bubbles, present in the pelvic, in continuity with the uterus, extending to the cranio-caudal about 9 cm.The serum concentrations of cancer markers CEA, AFP, CA 19-9, CA 125, HE4, CA 15-3 are within the norm.Cystoscopic examination revealed an urethra displaced upwards, bladder trigone raised significantly compared to standard as ab-extrinseco compression, but covered with mucosa of normal appearance.Total abdominal hysterectomy, bilateral salpingo-oophorectomy, systematic pelvic lymph node dissection, omental biopsy and appendectomy were performed. On gross examination, a mass of about 8 cm deformed uterine profile. Ovaries and other pelvic organs grossly appeared normal. Gastrointestinal tract, paracolic gutters, liver, spleen, kidneys, and the undersurface of the diaphragm were free of lesions.Surgery treatment was associated with adjuvant whole-pelvis radiation (45 Gy) and adiuvant chemotherapy (cisplatin/ifosfamide). After three month of follow up, the patient was free of tumor.On gross examination, a friable and polypoid lesion arised from uterine cervix and involved the cervical-isthmic mucosa and the endometrium. A focal chronic ulceration of the head of the polyp was noted. The cut surface of the tumor was fleshy and gelatinous. There was focal myometrial invasion. On microscopic examination, the tumor was characterized by intimate admixture of epithelial component and sarcomatous stromal component. The epithelial component was minimal and mostly without epithelial atypia; while the sarcomatous component was clearly predominant (pure sarcoma: 70%), of high grade, with a high mitotic rate (10 MFs/10 HPFS). In addition, heterologous pure sarcomatous areas were noted comprising of rhabdomyosarcoma. There was no lymph node metastasis or lymphatic vascular space invasion, but a non-neoplastic focal thrombosis of small blood vessels in the head of the polyp was noted. Omentum and appendix were negative for tumor localization. The cytoplasm of the stromal cells showed a strong positive reaction for vimentin and desmin, but was negative for smooth muscle actin. Also, tumor cells were negative for β-HCG, estrogen and progesterone receptors. Only the focal areas of stromal tissue were positive for MYF 4 and also glands and rare isolated cells were positive for Keratin (MNF116).  and to a possible exposure to other different predisposing factors, pelvic irradiation and the use of tamoxifen for breast cancer.Although the etiologic factors are still unknown, at least three possible risk factors are currently under discussion:- Pelvic irradiation, exceptional cases have been reported in patients with a history of previous pelvic irradiation; - The hyperestrogenism, such as after prolonged unbalanced estrogen stimulation or long term oral contraceptive use; - Treatment with tamoxifen, a small series has been reported after treatment with tamoxifen for breast cancer.However, these may be coincidental associations and there are no proven etiologic factors. Anyway, our patient had none of these risk factors.Uterine endometrial adenosarcomas were typically found in post-menopausal women with the median age at presentation of 58 years. CompareAbout the immunophenotype, smooth muscle actin and desmin may also be positive. In areas of high-grade sarcoma and of sarcomatous overgrowth, the mesenchymal component exhibits a higher MIB1 proliferation index and may be p53 positive. The immunophenotype is similar to that of an undifferentiated uterine sarcoma with usually loss of expression of the cell differentiation markers estrogen receptor, progesterone receptor and CD10,30. AreaThe differential diagnosis of MASO of the uterine cervix should be made with caution. It includes benign lesions  and malignant lesions . MA can be easily distinguished from adenofibroma  using the criteria defined as unique to adenosarcoma such as, a marked degree of atypia of mesenchymal cells, a histological malignant element, the presence of myometrial invasion, and two or more mitotic figure per 10 HPF,25. HoweThe relative rarity of cervical MASO made the assessment of the most effective means of management difficult,19-23. MIn conclusion, we present an extremely rare case of cervical MASO, our knowledge the fourth case reported in the English literature for its location and the second MASO case of the cervix with heterologous elements. Table In uterus, adenosarcoma with sarcomatous overgrowth are aggressive tumors frequently associated with postoperative recurrence or metastases, and poor prognosis, markedly contrasting with typical mullerian adenosarcoma of the uterus. Myometrial invasion and heterologous elements seem to be principle prognostic factors. AggressWritten informed consent was obtained from the patient for publication of this case report. 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.TSP and SG made substantial contribution to conception and design. SDG and GG have been involved in drafting the manuscript. MR revised it critically for important intellectual content. GBN gave final approval of the published version. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2407/11/236/prepub


Acute appendicitis is a common surgical presentation for which surgical intervention, an appendicectomy, has remained a largely unchallenged primary treatment modality. Traditionally, it has been felt that the pathophysiological progressive nature of appendicitis ultimately leads to perforation. A number of recent studies, however, suggest that the process of appendiceal inflammation may follow a more remitting nature with evidence indicating spontaneous resolution. It is hypothesised that the treatment of uncomplicated appendicitis may therefore be amenable to conservative management with antibiotics. This article aims to highlight some of the issues and challenges relating to the conservative management of acute appendicitis and further demonstrates potential diagnostic and treatment difficulties involved in managing the more unfamiliar condition of recurrent appendicitis. In 2008 a 65-year-old man was admitted via the accident and emergency department to the general surgery team with a short history of right iliac fossa pain. He gave a good history of appendicitis with central abdominal pain localising to the right iliac fossa, some nausea, anorexia and a feeling of being generally unwell. His observations remained within normal limits throughout his admission and within 24 hours of conservative management, analgesia and antibiotics his pain had settled completely.Ultrasonography was performed during his admission and reported a blind-ended tube in the right iliac fossa, with a diameter of 10mm and appearances consistent with a slightly distended and oedematous appendix. In addition, superior and slightly medial to this was a poorly defined irregular echogenic region measuring up to 50mm across. This was thought to be merely bowel but the possibility of a fluid collection could not be excluded. Given the obvious improvement in his clinical symptoms, he was discharged home with oral antibiotics and an appendicectomy was not performed.The patient was seen in the outpatient clinic fours weeks after discharge. He remained well, there was no further evidence of a collection or appendix mass on examination and he was discharged from clinic.The patient re-presented six months later with further generalised abdominal pain, again localising to his right iliac fossa. Routine blood tests were all within normal limits and, once more, his pain settled conservatively over 48 hours and he was discharged home. As a result of his ongoing symp toms, a gastroenterology opinion was sought to exclude the possibility of irritable bowel disease. Computed tomography colonography was reporting as normal with typical appearances of the terminal ileum, caecum and ileocaecal valve. No comment was offered with respect to the appendix and no obvious cause for his pain was identified. He was discharged back to the care of his general practitioner.In 2011 the patient presented for a third time. He described the same symptoms as previously and furthermore reported that he had been suffering from recurrent, self-limiting episodes on a two-monthly basis for the last three years. There had been no red flag symptoms during this time and he had remained well in the interim period. He had sought medical attention on this occasion due to his pain being more severe, increasingly persistent and unresponsive to analgesia.Repeat ultrasonography demonstrated an ill defined, fluid filled area in the right iliac fossa, measuring 54mm x 19mm x 22mm. Adjacent to this was a poorly defined, thick walled tubular structure, 52mm long and 18mm wide. A differential diagnosis was proffered suggestive of a collection relating to an inflamed section of bowel, possibly the appendix. On this occasion, the patient deteriorated clinically with high temperatures, tachycardia and localised peritonism. He was taken to theatre and an emergency open appendicectomy performed. The surgical findings included a perforated gangrenous appendix in a pus filled abscess. He made an uneventful recovery and was discharged two days later. Histological examination confirmed the presence of an acute appendicitis and, in addition, reported evidence of an underlying carcinoid in the tip of the appendix with complete resection margins.In 2010–20H acute appendicitis accounted for over 57,000 acute hospital admissions in England.The diagnosis of appendicitis is primarily a clinical one based on a consistent history and clinical presentation. However, radiology has become increasingly used to support the diagnosis. Ultrasonography is considered the first-line radiological investigation for the assessment of complicated appendicitis in which there is a question of an appendix mass or abscess. It is quick, cheap, non-invasive and does not expose the patient to a radiation load. Computed tomography (CT), however, is more sensitive and specific than ultrasonography when diagnosing acute appendicitis and related complications (ultrasonography sensitivity of 86% and specificity of 81% compared with CT sensitivity of 94% and specificity of 95%).Traditionally, appendicitis is recognised as an acute progressive disease. The cause is usually an obstruction of the lumen of the appendix by a faecalith, lymphoid hyperplasia (such as in inflammatory bowel disease), parasites or, more rarely, a foreign body or tumour.A faecalith is the most common of these aetiologies, accounting for simple appendicitis in 40%, gangrenous nonperforated appendicitis in 65% and perforated appendicitis in 90% of cases.While initially a localised condition, ongoing pathology may lead to thrombosis of the appendicular artery and veins, ultimately resulting in a perforation, gangrene and necrosis of the appendix.–––For over 120 years, since McBurney defined the surgical appendectomy in 1894, it has remained the gold standard for the management of appendicitis. In recent years there has been increasing debate on the methodology of this surgical management, with the advent and development of laparoscopic surgery resulting in the ‘laparoscopic ap pendicectomy’ becoming more widespread. The evidence regarding the choice of surgical intervention remains divided as a number of studies report a reduction in overall length of hospital stay, post-operative pain and morbidity with the laparoscopic approach–Over the last few years there has been an increasing interest in the idea of conservative management for acute appendicitis. Conservative non-operative antibiotic management of other intra-abdominal inflammations such as uncomplicated diverticulitis is a well established alternative to operative management. Recently, there have been a number of studies including several large randomised trials comparing conservative management of uncomplicated appendicitis with surgery.et al performed a multicentre RCT on 252 men presenting with acute appendicitis.In 2008 Styrud –Similarly, a French study in 2011 randomised 245 patients diagnosed with uncomplicated appendicitis on CT to an antibiotic alone (amoxicillin and clavulanic acid) and a surgical arm.A meta-analysis in 2010 of the five RCTs comparing surgical and conservative management of acute appendicitis also concluded that although antibiotics may be used as a primary treatment alternative for selected patients with suspected uncomplicated appendicitis, it was unlikely to supersede appendectomy based on the currently available evidence.The quality of the studies was low to moderate, a subgroup analysis could not be made and therefore it was also recommended that appendicectomy remains the standard treatment for acute appendicitis.Despite best available evidence, there continue to be certain situations where, anecdotally, conservative management may play a primary role. In those patients who are considered ‘high risk’ with significant co-morbidities and anaesthetic risk, in those at the extremes of age and in those where surgical intervention is simply unavailable (submariners), conservative management may well be considered the treatment modality of choice. Similarly, patients who are diagnosed with appendicitis early in the disease process (as our patient was) may respond favourably to initial antibiotic therapy.12 months. The case presented in this report demonstrates symptomatic recurrence over the extended period of three years and highlights the significant potential for the longer term impact of not performing definitive operative management. Indeed, there are no similar data reporting recurrence rates in conservatively managed patients after 12 months and future studies must incorporate a longer follow-up period.The evidence, however, remains very limited and is largely case-based in nature. In these cases, the incidence of recurrence following the conservative management of appendicitis must remain a concern. Cases of recurrent appendicitis are well documented in the literature and involve repeated admissions and associated patient morbidity. Studies clearly outline a significant potential for recurrence in those patients treated conservatively, even within an initial limited follow-up period of There remains a limited body of evidence for the conservative management of appendicitis. While the risk of recurrence is well documented, the authors believe that there may remain a potential role for the use of conservative management in selected patient groups, using antibiotic therapy as a bridge prior to performing definitive elective surgery. In some cases this may be to allow for a period of optimisation, reducing the risks of immediate operative management or, alternatively, it may be used in those patients where surgical intervention is unavailable. Patient selection remains crucial and it is not our opinion that this should be the treatment option of choice for all patients. Further research needs to incorporate long-term follow-up before definitive best practice guidelines can be determined. The question still remains that by managing patients conservatively are we simply just delaying the inevitable?


A 60-year-old male reported to us with an atypical case of giant steatocystoma multiplex in the scrotum with calcification. There was no family history of similar lesions. Yellowish, creamy material was expressed from a nodule during punch biopsy. The diagnosis was based on clinical as well as histological findings. Successful surgical excision was done to cure the case without any complications. Steatocystoma multiplex occurs as numerous, epithelial-lined, sebum-filled dermal cysts with characteristic sebaceous glands in the cyst walls . UsuallyA 60-year-old male presented to us with asymptomatic multiple cystic papule and nodules of various size and shape in group with firm adhesion on the scrotum for a period of 15 years. To start with the investigation we noticed a small, firm, elevated lesion on the scrotum which remained stationary for 10 years. With the passage of time, and since the past year, the lesion increased in size and number. Large-sized nodules grouped in large quantities in scrotum are rarely found. Similar lesions with extensive calcification with firm adhesion seem to be extremely rare. On examination, it was evidenced that there were multiple large- to very large-sized, discrete, mobile, firm, yellowish to skin-colored, dermal cystic papules and nodules of various atypical shape, with adhesion to the skin distributed only in scrotum. Pricking the lesion expressed oily fluid from some of the lesions. There were no signs of inflammation in any of the lesions. Other systems were normal, and routine laboratory examination revealed no abnormality.Skin biopsy of the cystic nodule revealed histologic features suggestive of steatocystoma multiplex. The cyst was located in the dermis with a folded cyst wall. The cyst wall had a basal layer in palisade arrangement of the cells towards the cavity. There were few flattened sebaceous gland lobules close to the cyst wall.The lesions were surgically excised after giving spinal anesthesia to the patient.  Steatoma or sebaceous cyst is due to an overproduction of sebum by one or more sebaceous glands while the usual outlet is closed. When this collection remains in situ for years, the peripheral pressure destroys the epithelial lining of the cyst, and the sebum may undergo calcareous degeneration.∼2 cm, but the nodules may be larger. The overlying epidermis is usually normal with no central punctum present. The lesions are typically located on the upper trunk, neck, axillae, scrotum, and proximal extremities. A few isolated steatomas scattered in various parts of the body are of frequent occurrence. Occasionally, one sees large numbers of the pinhead-sized or the pea-sized lesions. Although there are reports of involvement of various sites, such as scalp, forehead, chest, trunk, groin, axilla, scrotum, and so forth, an increase in size and number with extensive calcification and adhesion tightly to the scrotum for many years are unlikely and an asymptomatic presentation. Steatocystoma multiplex is an uncommon disorder that is characterized by multiple soft, movable, small, cystic nodules. The diameter of lesions typically varies from 0.2The colour of the lesions varies from yellowish to skin colour and this variation has been attributed to the depth of the lesions—the superficial lesions being yellowish, and the deeper lesions skin coloured . In our The causative factors of steatocystoma multiplex still remaine unclear, yet Setoyama et al.  suggest The pathogenesis of these lesions has been extensively studied. Steatocystomas were initially thought to be sebaceous or retention cysts . RegardiEruptive vellus hair cysts can mimic steatocystoma multiplex clinically. Epidermal inclusion cysts also have to be excluded . Biopsy The definite treatment of individual lesions is excision. However, the sheer number of cysts and calcification usually precludes this type of treatment. In such instances, incision and complete expression of cysts contents or aspiration may be effective temporally in reducing the lesion . Laser i


A 60-year-old male with increasing abdominal distension was admitted to The Fifth Affiliated Hospital of Guangzhou Medical University . The abdominal sonogram exhibited a huge abdominal cystic-based mass with solid components in the left upper quadrant. A contrast-enhanced computed tomography scan of the abdomen revealed a large heterogeneous cystic solid tumor, but the source of the tumor could not be determined. The laparotomy demonstrated a huge cystic-based tumor with an integrated cystic wall, arising from the posterior wall of the gastric body. Multiple septa and ~3,500 ml of yellowish fluid were found in the cystic cavity. Pathological analysis showed that the tumor contained a mixture of polygonal and spindle cells. Immunohistochemical study indicated that the tumor cells were positive for CD117 and CD34. The final diagnosis was gastric gastrointestinal stromal tumor. The patient recovered well and no recurrence or metastasis was identified following a 12-month follow-up. Gastrointestinal stromal tumors (GISTs) are the most common type of mesenchymal tumors of the gastrointestinal (GI) tract. Different from other mesenchymal tumors, such as GI smooth muscle and nerve sheath tumors, GISTs are a spontaneous differentiation of stromal tumors . GISTs m9/l) and elevated blood (250 U/l) and urinary  amylase levels. The serum levels of specific tumor markers  were within the normal range. However, the level of CA125 (142.3 U/ml) was significantly higher. The patient provided written informed consent.A 60-year-old male, who exhibited increasing abdominal distension for one month, with a huge congenital right inguinal hernia, was admitted to The Fifth Affiliated Hospital of Guangzhou Medical University . The patient denied any abdominal pain, vomiting or constipation. Physical examination revealed a large firm mass that occupied almost the entire abdomen; the mass extended from the bottom of the xiphoid process to 2.5 cm over the pubic symphysis. In addition, part of the bowel was apparent in the right side of the scrotum. Laboratory results indicated that the patient was anemic , with an increased peripheral blood platelet count  scan of the abdomen revealed a 28.5×22.8×19.2-cm3 heterogeneous cystic solid tumor. Partial septum was found in the cyst cavity. The mass, with an unclear boundary between the stomach and spleen, compressed the left lobe of the liver and the stomach, pancreas and kidneys, leading to narrowing of the gastric lumen and bilateral hydronephrosis. No evidence of swelling of the regional lymph nodes or involvement of the major vessels was identified (The abdominal sonogram showed a huge abdominal cystic-based mass with solid components in the left upper quadrant. The cystic portion was irregular in shape with a wall of uneven thickness and an unsmooth inner surface. The size of the solid portion was ~8.3×4.6×9.0 cmentified . An endoentified . AccordiThe patient underwent an exploratory laparotomy with L-shaped incision in the left upper quadrant. The laparotomy revealed a huge cystic-based tumor with an integrated cystic wall, arising from the posterior wall of the gastric body. Multiple septa and ~3,500 ml of yellowish fluid were found in the cystic cavity. No clear boundary was identified between the tumor and splenic hilum. The patient’s tumor and spleen were completely resected. The resected tumor weighed 122 g and exhibited a fish-flesh cut surface . PatholoGISTs are the most common type of mesenchymal tumors. Without a myogenic or neurogenic nature, GISTs are considered to originate from interstitial Cajal cells of the wall of the GI tract . MicroscThe clinical manifestation of GISTs, such as abdominal distension, lower abdominal pain, GI bleeding and abdominal mass, varies with the size and location of tumors . The medDue to the wide range of symptoms and its rarity, the diagnosis of GISTs require a high degree of suspicion. The primary mode of diagnosis and assessment of the severity of the disease is by contrast-enhanced CT scan of the abdomen and pelvis ,9. In thTo date, complete surgical resection is the most effective treatment for GISTs and has a major impact on the prognosis of patients and tumor recurrence . In the In conclusion, large cystic-based GISTs are rare and GISTs must be considered as one possibility when cystic tumors of unknown origin are identified in the abdomen.


Lingual mandibular bone defects, also known as Stafne bone cavity (SC), are unilateral asymptomatic radiolucencies, generally seen in the mandibular angle, below the inferior alveolar canal. Although panoramic radiographies normally offer enough information to make a correct diagnosis, additional studies are often required, especially in atypical cases. The present report describes an atypical presentation of a Stafne’s bone cavity in a 78 years-old male patient. In this particular case, an asymptomatic and radiolucid lesion was observed during a routine dental examination. The computed tomography (CT) showed an involvement of both lingual and buccal mandibular plates producing a tunnel-like lesion. No history of mandibular trauma or surgery was refered. An additional magnetic resonance imaging (MRI) was made to discard submandibular gland pathology and to confirm the diagnosis. Since SC is asymptomatic and nonprogressive, a conservative approach based in clinical and radiological follow-ups was considered to be the most suitable treatment option. Key words:Stafne bone cavity, lingual mandibular bone defect, case report. The Stafne bone cavity (SC) was first described in 1942 by Edward Stafne, which reported 35 cases of unilateral, radiolucent and asymptomatic cavities located between the lower first molar and the mandibular angle  of the mandible was performed. Axial sections showed a defect, with discontinuity of the lingual plate, as well as, a significant erosion of the buccal cortex Fig. . The 3D-Although both mandibular plates were affected, a diagnosis of SC was made and no further therapy was institu-ted. A new CT was made one year after the initial diagnosis and no changes regarding the size and features of the lesion were observed Fig. .The typical presentation of a SC is an elliptical, homogeneous radiotransparency, with a well-defined border located below the inferior alveolar canal, often involving the lower border of the mandible . This leThe pathogenesis of the SC is not yet fully understood. The first theory, originally supported by Stafne , suggestThis evolution pattern might be explained by the pressure exerted by the neighboring structures like the salivary glands ,5,7. It The surgical removal of the lesion and the posterior histological study are not recommended in the great majority of cases ,5,8. A pOn the other hand, several papers consider the MRI an essential tool in these type of defects, providing multiple imaging planes and different echo sequences ,11. T1- To our knowledge, this is the first published case with involvement of both mandibular plates. In fact, in the classification proposed by Arriji et al.  this feaThe differential diagnosis in this particular case was more complex and included entities such as the keratocystic odontogenic tumour, giant cells granuloma, hemangioma, arteriovenous malformation, and others malignant pathologies like the ossifying fibrossarcoma or the malignant ameloblastoma, although it must be said that these malignant lesions usually present irregular margins and a fast growth ,14. IntrMost of the studies published to date indicate that SC is asymptomatic and nonprogressive, and therefore a conservative approach based in radiological follow-ups is the most suitable approach. A surgical procedure should only be considered in patients with symptoms or when the findings are not consistent with these defects ,5,7.


Osteoid osteoma is a benign bone tumor of undetermined etiology, composed of a central zone named nidus which is an atypical bone completely enclosed within a wellvascularized stroma and a peripheral sclerotic reaction zone. There are three types of radiographic features: cortical, medullary and subperiosteal. Forty-four patients with osteoid osteoma were studied retrospectively. In plain films, 35 patients presented as the cortical type, six cases were located in the medullary zone and three had subperiosteal osteoid osteoma. In all the cases, the nidus was visualized on computed tomography (CT) scan. The nidus was visible in four out of five patients who had also undergone magnetic resonance imaging (MRI). Double-density sign, seen on radionuclide bone scans was positive in all patients. MRI is more sensitive in the diagnosis of bone marrow and soft tissue abnormalities adjacent to the lesion, and in the nidus that is located closer to the medullary zone. On the other hand, CT is more specific when it comes to detecting the lesion’s nidus. Osteoid osteoma was first described by Dr. Jaffe in 1935 as a benign bone tumor .s) compared to the extra-reticular lesions . Spo. Spo1]. Since the aim of this assessment in which 45 patients have been reviewed is to assess and introduce different radiological features of osteoid osteoma, according to its cortical, subperiosteal or medullary bone location, we considered it sufficient to introduce five patients who had special radiological manifestations.A 20-year-old woman presented with right hip pain radiating to the knee from one year ago. The pain tended to worsen at night, woke her up and was relieved by asprin, but after a while the pain increased again. The plain radiograph of the hip revealed no abnormalities but CT scan showed a sclerotic lesion with a central nidus in the cortex. In radionuclide scan, an increased uptake was detected in the same region. This type of osteoid osteoma is the most common type regarding the location of the lesion  and the involved bone  .A 28-year-old man who had a history of tumor removal surgery about one year before presented with reswelling and relapse of the lesion. One year ago the tumor had involved the proximal phalanx of the second finger of the right hand. The pathologic evaluation had been compatible with osteoid osteoma. This time the patient complained of reswelling of the same place from 3 months before. The lesion relapsed in the form of an osteolytic lesion (medullary type) accompanied by tenderness and limitation of motion. The tumor resection confirmed relapse of the tumor .A 20-year-old man presented with a 2 year history of left ankle pain accompanied by swelling of the region. The pain occurred typically at night. Left ankle radiograph  revealed features of subperiosteal osteoid osteoma along with a soft tissue mass around the lesion. .A 22-year-old man presented with continuous low back pain for 5 months. The pain was not affected by motion and activity. Radiography revealed a sclerotic region in the left pedicle of the L1 vertebra along with scoliosis in the thoracolumbar region. CT scan also showed an osteolytic and osteoblastic lesion in the same place. Moreover, T1W MRI demonstrated focal low signal intensity surrounded by soft tissue component that replaced the pedicle of the L1 vertebra on the left side in the axial sections. Diffuse low signal intensity due to edema was seen in the adjacent bone. Radionuclide scan revealed an increased uptake in the same place . All theA 9-year-old boy presented with a one-year pain in the right shin more dominant at night. In physical exam, a hard mass with bone consistency was detected in the mid part of the shin.In radiography, a solid periosteal reaction in the medial part of the tibia without an obvious nidus was noticed. CT scan revealed a nidus along with a remarkable periosteal reaction around the lesion . TherefoWe retrospectively studied the patients’ files with the pathological diagnosis of osteoid osteoma in the department of radiology at Imam Reza Hospital of Mashhad, University of Medical Sciences, Iran, within one year. Forty-four patients with osteoid osteoma were included. Plain films, CT images and isotope scans were available for all patients. Only five of these cases had also undergone MRI.In this case series, there were 31 male and 14 female patients, making the diagnosis twice as common in men as in women. The mean age of the patients with osteoid osteoma was 17 years . The distribution of the disease in different decades of life is shown in In most cases, radiological manifestations were seen simultaneously with clinical symptoms. In others, the pain preceded radiographic signs.Pain was the only symptom in these patients which is initially mild and intermittent, but later becomes persistent and severe. The pain occurs generally at night. Only in one patient night pain was not reported. Favorable response to aspirin and NSAID was seen in 41 cases and only three of the patients showed a relative response. Ten patients noted radiating referral pain.Additional clinical symptoms were swelling over the affected area seen in clinical examinations of nine patients and restricted movement in five. Pain increased with activity in six patients, while it was relieved by activity in 38 cases .The average duration of pain reported by patients was approximately 17 months, within a range of 3 weeks to7 years from the onset of the disease. Recurrence of the disease after surgical resection was diagnosed in three patients in the same region. In one case the lesion was initially diagnosed in the femur, but after resection a second lesion was discovered in the pubic symphysis. Regarding the anatomic location of the tumor, the femoral neck and proximal tibia were commonly involved with 19 and 9 cases, respectively. Other affected sites, in order of prevalence included the humerus, talus, spine, phalanx, acetabulum and pubic symphysis .3.2.1. Plain FilmIn our series, 35 patients presented with the cortical type, six cases were located in the medullary zone and three had subperiosteal osteoid osteoma .3.2.2. CT ScanIn all the cases, the nidus was visualized. In one case where the lesion was located at T8 vertebra, CT scan only showed an increased density in the vertebra pedicle with a suspected nidus. Calcification and ossification within the nidus was either mild or absent in 15 cases. In two cases where the osteoid osteoma was located within the vertebra, scoliosis was present and the lesion was visualized as an increase of density in the pedicle (one thoracic and one lumbar vertebra). In the case with L1 lesion, increased density was seen in the body as well as the pedicle of the vertebra.3.2.3. MRIThe nidus was visible in four out of five patients, who had also undergone MRI. In the fifth patient the nidus was only visible on CT. However, in all five cases signal changes related to bone marrow and soft tissue edema adjacent to the lesion were clearly detected in MRI.3.2.4. Radionuclide Bone ScanWe used this sign together with a pathological report to verify the diagnosis of osteoid osteoma which was positive in all patients .Finally, osteoid osteoma is known as a benign bone tumor, twice as common in males as in females. The highest incidence occurs in the second decade of life. Night pain relieved by aspirin is the most common symptom. Concerning the site of origin there are three types of osteoid osteoma; namely, cortical (classic type), medullary and subperiosteal.The most common sites of involvement are the femoral neck and proximal tibia. This bone disease is detectable by plain film, CT, MRI and radionuclide bone scan, each bearing different characteristics in the diagnosis of this lesion. MRI is more sensitive in the diagnosis of bone marrow and soft tissue abnormalities adjacent to the lesion. The closer the nidus is to the medullary zone, the greater the role of MRI in detecting the nidus compared to CT. On the other hand, CT is more specific when it comes to detecting the lesion’s nidus.


Oncogenous osteomalacia (OOM), which is also known as tumour-induced osteomalacia, is a rare condition associated with a neoplasm and a related systemic bone demineralization caused by renal phosphate wasting. OOM usually occurs in association with a variety of different mesenchymal tumours, and they were categorized into four distinct morphological patterns which they termed "phosphaturic mesenchymal tumour". Of its 4 histopathological subtypes, the mixed connective tissue variant is most commonly observed. Only 10% of cases appear in the head and neck regions and moreover, only 5 previously published tumors were localized in the sinonasal area. The authors describe a case of a man with a PMT originating from the frontoethmoidal region.A 53-year-old man was referred to our ORL clinic due to a presence of a mass at the nasal root having been growing asymptomatically for 1 year. CT scans demonstrated a large (25 × 20 × 35 mm) bilateral frontoethmoidal mass with destruction of nasal bones. The tumor did not appear to invade to the anterior skull base. A selective angiography revealed a moderate hypervascularization of the tumour during early and late arterial phases. The tumour was removed from the external approach and the definitive histopathological diagnosis was a phospaturic mesenchymal tumor. Dual energy X-ray absorptiometry revealed a slight osteopenia of the first and second lumbar vertebrae and neck of the thigh bone. The serum and urinary levels of both calcium and anorganic phosphate were within normal limits. The patient is doing well three years after the operation, and the serum and urine levels of calcium and phosphate remain well within normal limits.PMT is rare in the sinonasal region, it can be rarely observed without the signs of osteomalacia. Oncogenous osteomalacia (OOM), which is also known as tumour-induced osteomalacia, is a rare condition associated with a neoplasm and a related systemic bone demineralization caused by renal phosphate wasting ,2. In 19Because of its scarcity, most ENT surgeons remain oblivious to the existence of PMTMCT. Here, the case of a 53-year-old man with a PMTMCT, originating bilaterally from the frontoethmoidal region, is described.A 53-year-old man was referred to our ORL clinic due to a presence of a mass at the nasal root having been growing asymptomatically for 1 year. The patient's past medical history was unremarkable. The root of the nose was enlarged by a non-tender, firm, ovoid mass, the overlaying skin was intact. Nasal endoscopy revealed a smooth bulge in the superior turbinate apparent on both sides. An endoscopic biopsy was performed, and was accompanied by profuse bleeding. The tentative histopathological diagnosis was a juvenile angiofibroma. A computed tomography (CT) scan demonstrated a large (25 × 20 × 35 mm) bilateral frontoethmoidal, strongly enhancing (90 HU) mass with concomitant destruction of nasal bones, the frontal processes of the maxilla, inferior wall of the frontal sinuses and medial orbital wall. MRI scans demonstrated that the tumour did not appear to invade the anterior skull base [Figure The tumour was removed under general anesthesia. After an H-shaped skin incision was made, we encountered a grayish-white mass invested in a thin capsule. The tumour was easily extirpated from the intact posterior wall of the frontal sinuses, anterior ethmoids, and nasal cavity, and the posterosuperior part of the nasal septum was also removed. The peroperative bleeding was moderate, and it ceased immediately after the tumour resection. The definitive histopathological diagnosis was a PMTMCT variant Figure . It consThe course, thereafter, was uneventful, and two years after the operation the patient is doing well; there is no evidence of the disease in either endoscopic or CT examinations, and the serum and urine levels of calcium and phosphate remain well within normal limits.Phosphaturic mesenchymal tumour (PMT) is representative of a very rare group of neoplasms, usually benign ,5,6. PMTThese tumours may occur in almost any location; sinonasal area is affected very rarely ,2,5,7. Iin vitro and in vivo [The mechanism of the tumour-induced osteomalacia remained unclear for many years but all the evidence pointed to a circulating phosphaturic agent ,3,7,11. et al in their study revealed 3 cases of PMCMCT without a known history of phosphaturia, which they had considered a non-phosphaturic variant of this histopathologic entity [There were neither clinical nor laboratory signs of osteomalacia in our patient with the tumour, which met unambiguously all histopathological criteria for a PMTMCT. Similarly, Folpe c entity . They spc entity . UnfortuPMTMCT is basically a benign lesion, histologically malignant, metastasizing variants of which happen to occur extremely rarely ,7. NonetIn general, a reasonable treatment protocol for PMT MTC is a complete surgical removal, which dramatically resolves the tumour-associated osteomalacia (known for its resistance to conservative therapy). Owing to its local invasiveness, the lesion should be removed using wide margins of resection ,5-7. RemIn most patients with oncogenous osteomalacia, the causative tumour is a PMTMCT. About 5% of all these lesions originate in the frontoethmoidal area. Here, the tumour may easily go unrecognized until a thorough battery of CT scans is performed. Local invasion is a characteristic feature of benign PMTMCT, requiring wide-margin resection. Given the spatial limitations imposed by surrounding anatomical structures, residual tumour may be left behind in that area, making careful follow-ups an absolute necessity.There is no type of financial interest that is related to the manuscrip, including stock or ownership of a business entity connected to a product described in the paper, paid consulting for the company or competing companies, or patent rights to a drug or piece of equipment.PK performed the operation and is the main author and supervisor of the manuscript.IS performed preoperative diagnostics, did follow-up, drafted the manuscript, cooperated during the manuscript preparing,.MG carried out histological analysis and wrote histological part of the manuscript.PM did searching and analysis of the literature, prepared the table and participated in the design of the paper.KZ was the opponent of the manuscript, did critical revision of the manuscript and participated on the design of the paper, was responsible for the picture preparing.All authors read and approved the final manuscript.


A high index of suspicion is required to make the diagnosis of constrictive pericarditis (CP) in patients presenting with cirrhosis and volume overload, as they can otherwise go misdiagnosed for years.Case report.A 51 year-old man with a history of presumed alcoholic cirrhosis presented to the emergency department with anasarca. Abdominal ultrasound with Doppler demonstrated a nodular cirrhotic liver, but no evidence of portal hypertension or ascites. The chest x-ray, however, was significant for a right-sided pleural effusion and pericardial calcification, suggestive of (CP). Transthoracic echocardiogram and ECG-gated computerized tomography scan of the chest without IV contrast confirmed the diagnosis. The patient was referred to thoracic surgery for definitive pericardiectomy.The diagnosis of CP is often neglected by admitting physicians, who usually attribute the symptoms to another disease process. Although a multimodality approach is necessary for the diagnosis of CP, this case highlights the utility of chest x-ray, a relatively non-invasive and inexpensive test, in expediting the diagnosis. A 51-year-old man presented to the emergency department with anasarca. The patient had a history of presumed alcoholic cirrhosis 15 years prior, but denied any alcohol use since that time. Physical examination was remarkable for massive anasarca, with edema prominent up to his chest. Laboratory studies revealed new elevated creatinine and hyponatremia. Liver function tests and urinalysis were unremarkable. Abdominal ultrasound with Doppler demonstrated a nodular cirrhotic liver, but no evidence of portal hypertension or ascites. The chest x-ray was significant for a right-sided pleural effusion and pericardial calcification (FigureBoth authors declare that they have no competing interests.Dr. Toledano made substantive contributions to the design of the study and drafting of the manuscript. Dr. Bhagra made substantive contributions to the drafting and revision of the manuscript. Both authors gave final approval to the version to be published.


Esophageal schwannoma is a rare diagnosis and historically has been a tumor of middle-aged females. We report a case of a 22-year-old male presenting initially with dyspnea secondary to tracheal compression from an 8 × 6 × 3.0 cm esophageal schwannoma. The tumor was surgically resected, and diagnosis was confirmed with immunohistochemical and pathological studies. We report the youngest case of esophageal schwannoma in an otherwise healthy individual. Schwannomas of the esophagus are peripheral nerve sheath tumors characterized by peripheral lymphoid cuffing, benign nuclear atypia, and spindle-shaped cells . We repoA 22-year-old Asian American male presented for symptoms of dyspnea for six months that were being treated as asthma. This progressed to solid food dysphagia and a 20-pound weight loss over two years. The remainder of his history was noncontributory, and the physical exam was only positive for audible wheezing. A chest X-ray revealed a large mediastinal mass, with subsequent computed tomography (CT) scan demonstrating an upper esophageal mass that adhered to the posterior wall of the esophagus. The trachea, significantly narrowed, was seen to be bowing anteriorly Figures  and 2. Flexible esophagoscopy identified the mass at 19 cm from the incisors and with attachment to the left anterolateral esophageal wall. The following week, he was taken to the operating room for excision of the mass.A right posterolateral thoracotomy was performed. The tumor was found in the submucosal layer along the wall. The azygos vein was visualized inferior to the mass and doubly ligated. The vagus nerve was free from involvement. The esophagus was opened from below the thoracic inlet to the level of the azygos vein. The mass was dissected and easily shelled out. Areas of the posterior wall were resected for tumor margins, and the esophagotomy was closed in two layers.Gross examination of the esophageal mass revealed a smooth, multilobulated, but well-circumscribed mass measuring 8.5 cm in greatest dimension . On cut The patient tolerated the procedure well. His postoperative course was complicated by a contained leak at the esophagotomy repair site that was managed conservatively and postoperative pneumonia. He remained afebrile and hemodynamically stable. He was discharged postoperative day eight with a regular diet. At the three month followup visit, he was eating normally and had no symptoms.Esophageal schwannomas are rare with only 30 reported cases in the English literature with many coming from Asia 18/27) [7 2–4]. . 4]. PriSchwannomas are generally treated via surgical resection, usually through a thoracotomy with enucleation. With complete excision, the prognosis of schwannomas is generally excellent with recurrence being rare . Our case is the youngest in an otherwise healthy individual and one of the largest to present with an esophageal schwannoma. His Asian decent underscores the predominance of this disease in this population. Though rare, or perhaps underreported in the medical literature, it should be on the differential for unexplained dyspnea or dysphagia in any age group when radiographic text reveals a large mediastinal mass.


Liposarcoma occurs very rarely in the mediastinum. Patients often remain asymptomatic until it grows large enough to cause direct invasion or compression of adjacent organs. We report a case of a 77-year-old male presented with dyspnea of exertion and was found to have a large mediastinal mass which was eventually diagnosed as primary mediastinal well-differentiated liposarcoma. The limited respiratory function at the initial presentation prompted phrenic nerve preserving incomplete resection rather than radical removal of the adjacent mediastinal structures. After surgical removal, the recurrence for well-differentiated mediastinal liposarcomas in the mediastinum is unknown; therefore, close follow-up is crucial. Liposarcoma is the most commonly diagnosed soft tissue sarcoma in adults but occurs very rarely in the mediastinum . The tumA 77-year-old Hispanic male presented with a six months history of exertional dyspnea and recent onset of chest pain. He denied fevers or weight loss. His vital signs were normal. Physical examination demonstrated dullness on percussion and decreased breath sounds at the bases bilaterally. Forced expiratory volume in 1 second (FEV1) was 1.78 L and forced vital capacity (FVC) was 2.29 L. Laboratory data, electrocardiogram and arterial blood gas analyses were normal. Chest radiograph demonstrated a widened mediastinum and bilateral lower lobe haziness Figure . MacroscLiposarcoma most commonly occurs in the lower extremities and retroperitoneum and rarely in the subcutaneous tissue, paratesticular fat tissue and the mediastinum . Most paFindings in plain chest radiography are nonspecific, and offer little except confirming the presence of a soft-tissue mass. CT provides valuable morphologic information and helps in the differentiation of various types of lipomatous tumors -6,10-12.The pathological evaluation is the most important step to differentiate between mediastinal liposarcoma and all other mediastinal fat-containing tumors: lipoma, thymolipoma, teratoma, lymphoma, germ cell tumor, or even herniated peritoneal fat. The World Health Organization currently recognized four subtypes of liposarcoma: well-differentiated, myxoid, pleomorphic and dedifferentiated Table  13]. We. We13]. While many of the principles governing the evaluation and diagnosis of soft tissue sarcomas certainly apply to liposarcoma, there are features unique for the treatment of anterior mediastinal liposarcoma that deserve special consideration. Particularly important is the tumor proximity to the vital mediastinal organs: heart, aorta, lungs, superior vena cava, phrenic nerves and diaphragm. The current standard of treatment is surgical removal and perioperative adjuvant radiation and chemotherapy -9. SurgiWe reported a case with a large mediastinal mass. Our patient had many of the radiological signs for higher grade malignancy: enormous size, multinodularity with thick calcified septation, but his final histocytological diagnosis was well-differentiated liposarcoma, which has a more benign clinical course. After surgical removal, the recurrence for well-differentiated mediastinal liposarcomas in the mediastinum is unknown; therefore, close follow-up is mandatory.Written informed consent was obtained from the patient for publication of this case report. A copy of written consent is available for review by the Editors-in Chief of this journal.The authors declare that they have no competing interests.SF and KD wrote the manuscript. KD, CG, DH and RT performed surgery. WK and RT supervised manuscript and entire treatment. All authors read and approved the final manuscript.


Hydatid disease is a major health problem worldwide. Primary hydatid disease of the pancreas is very rare and acute pancreatitis secondary to hydatid cyst has rarely been reported. We report the case of a 38-year-old man who presented acute pancreatitis. A diagnosis of hydatid cyst of the pancreas, measuring 10 cm, was established by abdominal computed tomography before surgery. The treatment consisted of a distal pancreatectomy. The postoperative period was uneventful. Additionally, a review of the literature regarding case reports of acute pancreatitis due to pancreatic hydatid cyst is presented. Hydatid disease caused by the larval stage of the Echinococcus parasite is a public health problem in endemic countries, especially in Tunisia. Hydatid disease can involve any organ. The liver is the most common organ involved and, together with the lungs, account for 90% of cases. Other involved sites (less than 10% of cases) are muscles, bones, kidneys, brain, and spleen. Pancreatic hydatid cysts are rare, accounting for less than 1% of cases . IsolateA 38-year-old man was admitted to our clinic with complaints of diffuse abdominal pain, nausea, vomiting for 7 days. The patient did not have any fever or jaundice. Moreover, he did not have any significant medical antecedents. On physical examination, vital signs were normal. Tenderness in the epigastrium was detected while examination of other systems was normal. Laboratory analyses were as follows: white blood cells were 13 000/mmc; hemoglobin was 14 g/dl; platelets were 142 000/mmc; amylase was 2100 U/l ; alanine aminotransferase (ALT) was 300 U/l ; aspartate transaminase (AST) was 120 U/l ; alkaline phosphatase (ALP) was 270 U/l ; gamma-glutamyl transpeptidase (GGT) was 130 U/l ; total bilirubin was 9 mg/l ; direct bilirubin was 3 mg/l ; C-reactive protein was 20 mg/l ; and erythrocyte sedimentation rate was 70 mm/h. Serological tests including HBsAg, anti-HBc IgM and anti-HCV were negative. Hydatid serology, which was based on an enzyme-linked immunosorbent assay (ELISA) test for echinococcal antigens, was positive . Lung radiography and hepatic ultrasound were normal. Abdominal computed tomography (CT) revealed a multi-loculated 100 × 90 mm cystic lesion in both the corpus and the tail of the pancreas, which was also associated with an enlargement of the pancreas and with a peripancreatic edema, indicating an acute pancreatitis. Abdominal CT-scan showed also daughter cysts, some peripheral calcifications and a detachment of the hydatid membrane in the pancreatic cyst. This is evidenced by a pressure drop inside the cyst and thus, an opening of the cyst in the pancreatic duct which is dilated  compared to the other sites of hydatid disease . The modThe mean age of the patients was 28 years, with a range of 18-38 years. The ratio of men to women was 3. The cyst was found in the body (n = 4), tail (n = 2) or head (n = 2). The location was solitarily in the pancreas (n = 7), and associated with a liver hydatid cyst (n = 1) . No specUltrasonography will typically demonstrate a multivesicular cyst, limited by a clean wall, containing daughter cysts and some peripheral calcifications . ComputeSurgical treatment of hydatid pancreatic cysts may be challenging. Furthermore, depending on the cyst's location, several procedures have been suggested, ranging from cyst fenestration, internal derivation, to central or distal pancreatectomy -7,15-17.Hydatid cyst of the pancreas is an extremely rare pathology but it may be a causal factor in acute pancreatitis, especially in endemic areas. Radiological examinations may help clinicians in diagnosing cystic masses in the pancreas. In the case of acute pancreatitis due to pancreatic hydatid cyst, there is definitely a closed relationship between the main pancreatic duct and the pancreatic cyst, imposing a derivative or a resective procedure.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.AM prepared the manuscript and performed the literature review. MJ formulated and assisted in the preparation of the manuscript. AM and MK conceived and performed the technique described in this manuscript. ZBS had given final approval of the version to be published. All authors have read and approved the final manuscript.


Both giant synovial osteochondroma and parosteal osteosarcoma are rare musculo-skeletal tumors, often localized in the vicinity of the knee. Misdiagnosis of a malignant bone tumor can entail fatal consequences. Etiology of giant synovial osteochondroma is widely unsolved but is believed to originate from synovial chondromatosis, a mostly benign metaplasia of the synovial membrane. Parosteal osteosarcoma is a low-grade surface osteosarcoma with a propensity of local recurrence and the potential of distant metastasis and therefore requiring a different therapeutical approach. We report the case of a popliteal giant osteochondroma mimicking a parosteal osteosarcoma. Relevant facts of this rare entity regarding pathogenesis, treatment, and differential diagnoses will be discussed. For benign lesions of the bone, no reliable data regarding incidence rates are available. A large amount of benign bone tumors remains undiscovered due to an asymptomatic course. Many diagnoses of benign bone lesions are incidental findings. Malignant primary bone tumors are even less frequent, accounting for only 0.2% of all neoplasms . ClassicGiant synovial osteochondroma is a very rare form of an osteochondromatous tumor and not to be confused with classic osteochondroma (osteo-cartilaginous exostosis), the most common bone tumor. Pathogenesis of this lesion is related to a synovial metaplasia in most cases. Descriptions are limited to case studies although its vicinity to large joints and tendons implies a high rate of mechanical impairment. Predilection site is the intra-articular, infrapatellar region of the knee. Besides an intra-articular form, which is often named giant synovial osteochondroma, an equally rare extra-synovial form does exist. It preferentially appears in the tendonsheath-rich areas of hand and foot or the knee and is also referred to as extraskeletal chondroma.Differentiation between parosteal osteosarcoma and giant synovial osteochondroma proves to be difficult as localization, clinical course, and imaging can show remarkable similarities.A 39-year-old Caucasian man presented with a painful swelling of the right popliteal space, which he first noticed 6 months ago. Patient’s history indicated no injury to the leg.Physical examination revealed an approximately 6×4 cm hard and slightly tender mass palpable at the medial aspect of the popliteal space adjacent to the pes anserine. The mass was firm on palpation and immobile to the deep tissue. There were no inflammatory signs whatsoever. The functional testing of the knee joint showed a limited range of motion with a moderate flexion deficit of 120-0-0 °F/E. There was no pain during rotation and compression and the ligamentous structures showed sufficient stability. No signs of an intra-articular effusion were observed. Muscle strength and neurovascular examination of the leg were normal. No further, similar masses were noted during the general examination as well as no lymphadenopathy. The remainder of the physical examination and routine blood investigations, including uric acid levels, alkaline phosphatase, and inflammatory parameters  were unremarkable.Plain radiographs with anterior-posterior Figure  and lateOn magnetic resonance (MR) images a homogeneous and lobulated yet relatively well-circumscribed juxtacortical mass was visualized. The lesion was located close to the bone and between the medial head of the gastrocnemicus muscle, which was slightly displaced dorsally, and the pes anserine.T1-weighted sagittal images Figure A showed The lesion showed only peripheral contrast enhancement after gadolinium contrast medium administration Figure . On wholRadiographically, maligancy could not be ruled out. Surgical biopsy was indicated to obtain a histologically verified diagnosis. Surgery was accomplished in a prone position and excisional biopsy with marginal resection of the lesion was performed. Macroscopically, a thin synovium-like membrane covering the bone-like lesion was discovered and resected. A second, smaller nodule attached to the cranial pole of the primary lesion was also excised in the process. Both specimens were sent for histopathologic analysis.Histopathological examination of the first surgical specimen showed a spindle-shaped 7×3.5×3.5 cm large, ossified piece of tissue Figure . A smallThe second specimen consisted of tendon-like tissue with irregular shape and appended fat-tissue. The size was 4×2×2 cm; the sawcut Figure , arrows The critical report noted an extensive formation of osseous tissue with tiny hyalinous caps for the primary lesion. These caps showed benign cartilage with cells arranged in a vertical pattern Figure . For theIn this case, the patient remained free of symptoms and recurrence 2.5 years after surgery. Clinical examination and repeated imaging of the patient’s right knee is continuously performed every 6 months for a period of 5 years.After clinical examination and imaging, a number of differential diagnoses for extra-osseous, heavily mineralized masses had to be taken into consideration. These included  osteochondroma, myositis ossificans, tumoral calcinosis, and parosteal osteosarcoma .Giant synovial osteochondroma is a rare benign tumor that can be found mostly intra-articulary in the vicinity of the knee and hip joint. Besides trauma , an extrThe macroscopical and radiologic appearance of the lesion, either lobulated or smooth, depends on the potential genesis. A conglomerate of multiple small synovial chondromas is supposedly able to create one single coherent, lobulated cartilaginous giant osteochondroma. Otherwise, the continuous growth of a singular synovial chondroma is discussed to lead to a smooth and unruffled circumscribed tumor. In both scenarios, the cartilaginous structure of the synovial giant osteochondroma can be replaced completely by bone through enchondral ossification . NutritiA review of the literature shows that studies to this entity are limited to case reports ,5,7-12. The differentiation between an extra-/para-articular or intra-articular giant synovial osteochondroma has to be established by the localization seen on preoperative imaging and encountered during surgery. It has to be noted that synovial covering of a chondromatous mass may derive from the tendon-sheaths or synovium of the popliteal bursae as well.vice versa and presents a reverse zoning phenomenon. Surgical trauma might cause recurrence and marginal excision is only indicated after the lesion has matured.Myositis ossificans is a benign, self-limiting, solitary, ossifying soft-tissue disease typically occurring within the muscles of the upper and lower extremities . It is cTumoral calcinosis is a very rare benign soft tissue calcification. Lesions consist of massive calcium deposits mostly occurring at the para-articular soft tissue . The disParosteal osteosarcoma originates at the surface of the bone. Sites of predilection are the distal femur (planum popliteum), and less frequently, the proximal tibia and proximal humerus . ParosteThis is why parosteal osteosarcoma remains hard to differentiate from benign entities -27. Howevice versa. For a histological clarification of uncertain lesions, a biopsy is indicated, even if the symptoms are minor. In differential diagnostics between osteochondroma and parosteal osteosarcoma, histological analysis is not yet routinely able to provide diagnostic certainty in all cases. Therefore, surgical therapy with complete excision and narrow follow-ups are strongly recommended until further developments in immuno-histochemical analyses give absolute certainty.Assessing dignity of extra-osseous, mineralized masses by imaging alone can provide some difficulty. Benignant lesions in the vicinity of the knee can show remarkable similarities to malignancies and 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. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article.AT carried out the surgery, performed the study, and prepared and revised the manuscript. UL and FP helped to draft the manuscript, examined the patient for various check-ups, and helped with an up-to-date literature review. FL helped to draft the document and handled the illustrations and picture-work. RE revised the manuscript critically for important intellectual content and helped to draft the manuscript. HM was crucial in the revision process of the reviewed manuscript and helped to check the quality of written English. All authors read and approved the final manuscript and its revision.


Angiomyolipoma is a benign neoplasm composed of varying admixtures of blood vessels, smooth muscle cells, and adipose tissue. Because of an increased risk of spontaneous haemorrhage, surgical approach is needed greater than 4–8 cm size. We here report our partial nephrectomy experience in the 24 cm size giant angiomyolipoma. 26-year-old woman referred to our clinic with a 24 cm size angiomyolipoma in her lower pole of right kidney. The inferior vena cava was deviated to the left by the mass. All the blood tests were normal and we offered her the choices of partial nephrectomy or nephrectomy. Right subcostal approach was used. The patient underwent resection of the mass with a safety region of 1 cm. Frozen section evaluation was consistent with angiomyolipoma and free for surgical margin. Warm ischemia time was 35 min. and intraoperative bleeding volume was 200 cc. Postoperative 2nd day the drain was taken and hospital stay was 4 days. In literature we observed very rare angiomyolipoma cases with such a large dimension treated by partial nephrectomy without arterial embolization. If technically suitable partial nephrectomy is the main chioce in this kind of benign lesions in young patients. Angiomyolipoma (AML) is a rare benign neoplasm, typically composed of varying admixtures of blood vessels, smooth muscle cells, and adipose tissue 1). The average size of these tumors is 2 to 8 cm, with cases involving tumors of up to 20 cm reported in literature [. The aveBecause of an increased risk of spontaneous haemorrhage, selective transcatheter arterial embolization (TAE) or surgical approach are needed greater than 4 to 8 cm size. We here report our partial nephrectomy experience in the 24 cm size giant angiomyolipoma.A 26-year-old woman without the tuberous sclerosis complex (TSC) presented with abdominal fullness, right upper quadrant pain and constipation. On physical examination, a right upper quadrant mass was palpated bimanually. Computerized tomography of abdomen demonstrated a 24 cm size angiomyolipoma that was arising from the lower pole of the right kidney . The infHistopathology showed an encapsulated soft tissue mass which was measured 24 cm at its greatest dimension and weighed 3425 g. The lesion was characterized microscopically by a proliferation of cells, including those demonstrating adipose and smooth muscle differentiation, with the formation of thick-walled vascular structures .Warm ischemia time was 35 min and intraoperative bleeding volume was 200 cc. The patient's postoperative course was uneventful. Postoperative 2nd day the drain was taken and she was discharged home on postoperative day 4.6 months later we evaluated the right kidney as a normal one except postoperative differences .Renal AML is a benign mesenchymal tumor, identified in less than 0.2% of the general population and a prevalence of 0.3% to 3% of all surgical resected renal tumors . Twenty In general asymptomatic AMLs with a diameter of 4 cm or smaller can be followed by imaging studies. Active surveillance can be performed even for large AMLs which do not uniformly become symptomatic until reaching more than 8 cm . For larTAE is a safe and tolerable procedure it can be done to decrease the risk of bleeding without loss of renal function. After TAE, the recuurence is infrequent but additional treatment may be necessary , 8. The Recently several cases have been reported for whom nephron-sparing surgery was performed. Especially in sporadic renal AML, this technique offers preservation of renal function with acceptable complication and recurrence rates. Preoperative embolization of the large tumors is recommended to avoid excess blood loss during surgery . But theIn our case report we performed a partial nephrectomy procedure in a young female patient without tuberous sclerosis complex. We met very rare AML cases with such big dimensions that were treated by partial nephrectomy without arterial embolization in our literature survey.Patient's age, strong diabetes history in her family, the anatomical localisation of the tumor in the kidney with the adjacent organs were the main reasons for the decision of the nephron-sparing surgery. We did not try preoperative embolization because of its modest effect on tumor size and to avoid additional burden for our patient. Although our intraoperative bleeding volume was acceptable, we believe that preoperative embolization may reduce this risk.The management of AML is a complex procedure especially in large or multiple ones. TAE is effective at controlling acute haemorhage but has limited value in the long term management. It may help us before surgical intervention to prevent hemorrhage. New treatment modalities such as focused ablation and the use of antiangiogenic molecules should be further explored. Although life-threatening hemorrhage or fistulas of the collecting system are the main complications partial nephrectomy may be the best choice if the patient's anatomy is available and your surgical experience is enough about nephron-sparing surgery.


Lipoid proteinosis is a rare autosomal recessive disorder which may be seen within a family very occasionally. Herein, we report lipoid proteinosis in two sisters characterized by verrucous lesions and hoarseness of voice, dysphagia and multiple beaded papules along the margins of their eyelids, fissured lips and thick ferenulum. Lipoid proteinosis is a rare autosomal recessive metabolic disturbance characterized by various cutaneous manifestations including hoarseness from early childhood and mucosal manifestations attributed to infiltrative deposits of oral cavity and uvula and some parts of upper respiratory tract. Extra-cutaneous features may include epilepsy, mental retardation and other neurologic and psychiatric manifestations.Some cases have been reported from different sites of the world but lipoid proteinosis is very rare in siblings. Herein, we report two siblings with interesting verrucous lesions on limbs.A 31 year-old Iranian woman presented with a long history of non-pruritic verrucous lesions involving the extensor of her limbs. She had a previous history of developing scars at sites of minor trauma and suffered from hoarseness. She had a history of several episodes of respiratory tract obstruction and dysphagia for two years and aphonia for four months. The patient had no history of epilepsy or photosensitivity.Her older sister, aged 32, had similar but milder dermatological manifestations.On physical examination of these sisters, numerous flesh colored verrucous symmetric papules were observed on the elbows, knees, feet, toes and fingers. Yellow papules were found on soft palate  and multHistological examination of the skin lesions in the elbow showed deposition of a periodic acid-Schiffpositive (PAS +), pink amorphous material in the papillary dermis and around blood vessels and appendages . All theLipoid proteinosis was first described by Urbach and Weithe in 1929, characteThe first clinical sign is often hoarseness of voice, which presents at birth, or early childhood4]5] an[5] an4][ an[5] anThe eyelid lesions, which are pathognomnic for the disease, are described in 50% of cases.8] These These8] The exact pathogenesis of this disease is not clear but has been postulated to be as a result of either a lysosomal storage disorder involving multiple enzyme defects or from a disturbance in collagen synthesis. 11]12] [12] 11][ [12] 11]Histologically, lipoid proteinosis is characterized by deposition of a PAS-positive, diastase resistant material at the level of the basement membrane, papillary dermis, surrounding blood vessels, and around adnexal epithelia, especially sweat coils.16] Ultr Ultr16] Although more than 250 cases have been reported so far, occurrence of the disease in siblings is very rare. Interestingly, most familial cases have been reported from South Asia, including India,18]19] [19] 18][ [19] 18]Treatment of this condition is usually unsatisfactory. Reported approaches include oral steroids, dimethyl sulphoxide, intrales


We present a rare finding of a ‘corkscrew appearance’ of the distal cerebral vessels in a young Asian woman who presented with acute stroke.A 32-year-old Asian woman presented with a 3-month history of recurrent right-sided transient ischemic attacks. Her clinical workup and brain imaging results were normal. A digital subtraction angiogram revealed an abnormal corkscrew appearance of all intracranial distal vessels. She was discharged on a single antiplatelet drug. She had no further transient ischemic attacks on clinical follow-up. A digital subtraction angiogram performed 1 year later revealed no changes in the appearance of these vessels.To the best of our knowledge no similar previous reports exist in the literature. The present report describes a unique case of an unusual corkscrew appearance of the distal intracranial vessels. However, the underlying etiology in the present case remains unknown. We present a case of a young Asian woman who presented with recurrent right-sided transient ischemic attacks (TIAs) and no neurological deficits. A full screening workup was performed for ‘young stroke’, and all investigation results were within normal limits. Brain imaging revealed no abnormal findings. A digital subtraction angiogram (DSA) showed a diffuse corkscrew appearance of all distal vessels of the cerebral vasculature. We found no clinical associations for the same appearance. To the best of our knowledge this is the first reported case with this appearance of the cerebral vessels.A 32-year-old right-handed Asian woman presented with recurrent 15- to 20-minute episodes of right upper limb weakness and paresthesia (recurrent TIAs) over a period of 3 months. On clinical examination, all her peripheral pulses were normal, and there were no focal neurological deficits. The patient underwent full urine and blood screening for vasculitis, and the results were negative or within normal limits. Two-dimensional echocardiography results were normal, and no other associated clinical conditions were present. No significant family history was noted. There was no history of any drug intake or exposure to toxins prior to the onset of the symptoms. The results of magnetic resonance imaging (MRI) of the brain were normal. Cerebral DSA showed an abnormal corkscrew appearance of all intracranial distal vessels (Figureset al.[et al.[In a review of the literature, we found no previous reports of an abnormal corkscrew appearance involving only the intracranial small vessels presenting with stroke in young patients. Osborn and Anderson reportedet al. reported[et al., and a c[et al.. To the Dissection as a cause of the present radiological findings was considered because it is an often underdiagnosed cause of stroke in young patients. However, diffuse involvement with a corkscrew appearance of almost all small intracranial vessels makes dissection a very unlikely etiology. Moreover, the typical angiographic findings of intracranial vessel dissection, such as alternating stenosis and dilatation, delayed arterial emptying, and anastomoses, a beaded appearance, or pseudoaneurysm, were noet al.[It is likely that the DSA findings were incidental and unrelated to the TIAs in the present case. However, in the absence of any other possible cause of the TIAs, a causative association cannot be ruled out. Coutts et al. recentlyWe report a possibly unique case of an unusual corkscrew appearance of the small vessels of the intracranial circulation in a young woman who presented with stroke and without any underlying known clinical conditions or associations.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.AA analyzed and interpreted the patient data. LSPK also analyzed the data and performed the literature review on these findings and contributed to preparation of the manuscript. SPO assisted in the clinical workup and follow-up of the patient as well as the editing and review of the manuscript. All authors read and approved the final manuscript.


Aggressive fibromatosis is a rare soft tissue tumor. Although it lacks metastatic potential, it can grow aggressively in a locally infiltrating pattern. The tumors frequently recur after surgical excision, which remains the treatment of choice. Optional combinations of radiotherapy and/or chemotherapy have been used postoperatively for recurrent disease and/or inoperable cases. A palpable mass was detected in the popliteal fossa of the right lower extremity in a 48-year-old man. Magnetic resonance imaging showed a contrast-enhancing noncalcified lesion initially felt to represent a vascular tumor. An invasive mass adherent to the surrounding tissue was visualized intraoperatively and extensively debulked. The patient's postoperative course was uneventful. Histologic examination of the surgical specimen was consistent with an extra-abdominal desmoid tumor. After appropriate recognition, wide local excision may be the most appropriate treatment for fibromatosis of the extremity. However, the rarity of this tumor and the difficulty inherent in distinguishing it from similar-appearing tumors are necessitating histologic confirmation of the diagnosis. Desmoid tumors, also known as aggressive fibromatosis, are an extremely rare entity. They are slow growing and histologically benign, but tend to be locally invasive at various anatomic sites. Desmoid tumors originate most frequently from abdominal fascial or musculoaponeurotic structures, although they may appear at extra-abdominal sites. The most common extra-abdominal locations include shoulder, chest wall, back, thigh, and head/neck , 2. ExtrDesmoid tumors pose a clinically challenging problem because of their tendency to mimic vascular neoplasms. Generally, histopathological examination is necessary for definitive diagnosis, as in our case. Radical resection is necessary for successful excision since desmoid tumors tend to recur locally. However, surgery, radiotherapy, or both are regarded as the treatment(s) of choice for lesions . This caA 48-year-old man presented with a tender swelling in his right lower extremity of 3 months' duration. Physical examination revealed a visually obvious 8 × 9 cm pulsatile mass on the lateral margin of the popliteal fossa. The distal extremity was stiff and tender to palpation. There was no previous history of trauma or surgical intervention. Lower extremity Doppler ultrasonography showed an 8 × 9 × 10-cm solid heterogeneous mass in the lateral popliteal fossa. Magnetic resonance imagining (MRI) demonstrated a large mass that closely approximated the muscular structures and surrounding connective tissue . The lesIntraoperatively, the patient was placed in the supine position under epidural anesthesia. The lesion was adherent to surrounding tissues including muscle and nerve. It originated from the muscular fascia of the deep muscle within the popliteal fossa. The lesion itself was large, gray-white, fibrotic, and irregular. Its appearance was not typical of a vascular lesion. No infiltration of the surrounding large vessels was identified, but the tumor invested nerves in the popliteal fossa. As this mass was thought to be suspicious for malignancy, enbloc dissection of the tumor was then carried out, including dissection of its attachments to the deep popliteal region, accomplished with the assistance of orthopedic and neurological surgeons. Total excision was attempted, but was unsuccessful as a tumor segment 0.5 × 0.5 cm in diameter which heavily infiltrated the tibial nerve could not be excised. Macroscopic surgical margins were free from all aspects of the tumor mass . PostopeThe patient's early postoperative course was uneventful. The patient refused any subsequent radiotherapy or chemotherapy, and he was discharged on the 9th postoperative day. Eighteen months later, he is free from disease without evidence of local recurrence or distant metastasis.The incidence of desmoid tumors has been reported as 2–4 cases per 1 million . Extra-aPossible risk factors for the development of desmoid tumors include female sex, or a previous history of surgery, trauma, or pregnancy. Genetic susceptibility is believed to play a role in the development of the disease, but genetic risk factors have not been identified . In our Considering their rarity, desmoid tumors are often misdiagnosed. Prior studies, such as that of McKinnon et al.  noted thThe histologic appearance of the tumor is usually consistent in various microscopic fields within a given case as well as from case to case. Desmoid tumors are described as dense, collagenous lesions with intertwining bundles of spindle cells without epithelial components. The tumor cells are uniform and lack mitotic activity. No necrosis or pleomorphism is present . GrosslyIn conclusion, desmoids arising in the lower extremity are extremely rare. They can mimic vascular tumors given their typical clinical and radiographic appearance. The possibility of an extremity desmoid tumor should be kept in mind when evaluating an extremity mass, but the diagnosis should be made only on the basis of a detailed histological examination. It is difficult to achieve complete resection because of their propensity for local invasion. For this reason, tumors should be removed as soon as possible after identification in order to achieve the most optimal resection possible.


Chondrosarcoma is well-known to be primarily resistant to conventional radiation and chemotherapy.We present the case of a 32-year-old Caucasian man with clear cell chondrosarcoma who presented with symptomatic recurrence in his pelvis and metastases to his skull and lungs. Our patient underwent systemic therapy with sunitinib and then consolidation with proton beam radiation to his symptomatic site. He achieved complete symptomatic relief with a significantly improved performance status and had an almost complete and durable metabolic response on fluorine-18-fluorodeoxyglucose positron emission tomography.Our findings have important clinical implications and suggest novel clinical trials for this difficult to treat disease. Chondrosarcomas are malignant tumors that arise from cartilaginous tissue and comprise four major subtypes: mesenchymal, clear cell, conventional and dedifferentiated ,2. In abRecent advances in the molecular understanding of sarcomas and the development of targeted therapy for sarcoma treatment have led to interest in the possibility of testing targeted agents in chondrosarcomas . Gene exWe present the case of a 32-year-old Caucasian man who initially presented five years previously with right hip pain and was found at that time to have a large multilobulated mass arising from his right ilium and extending to his right sacrum with involvement of his gluteal musculature Figure . A biopsApproximately two-and-a-half years later, our patient presented with significant pain in his right pelvis, difficulty in ambulating and a decreased performance status due to pain. A bone scan showed uptake in his left temporal bone and an excisional biopsy confirmed recurrence of the clear cell chondrosarcoma Figure . A positSunitinib is a multitargeted receptor tyrosine kinase inhibitor that exhibits inhibitory activity against multiple targets including c-KIT, vascular endothelial growth factor receptors , PDGFR-α and -β, fms-like tyrosine kinase receptor-3, colony stimulating factor 1 receptor, and RET ,18. InhiIn a murine model, sunitinib was shown to be synergistic with radiation in increasing apoptosis in endothelial cells, enhancing destruction of the tumor vasculature and increasing time to tumor growth . While tet al. published results of a phase I study employing sunitinib concurrent with stereotactic radiation for patients with oligometastatic disease [Kao  disease . In the Our study is the first to show that sunitinib has beneficial activity in chondrosarcoma and may be safely combined with proton beam radiation therapy. Randomized phase II or III clinical trials are needed to assess the role of sunitinib in this disease and as a radiosensitizer.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 contributed to the care of the patient and analyzed the data. JDR performed the histological examination of the tumor sections. JD and LHD drafted the manuscript. All authors read and approved the final manuscript.