Giant cell tumors of the bone (GCTB) account for 5% of all primary skeletal tumors. Although the tumors are normally benign, recurrence and metastasis of GCTB does occur. The most usual sites of a primary GCTB lesion are the distal femur and proximal tibia, and ~3% of these metastasize to the lung. Primary GCTB lesions in the spine are rare, and there have been few cases reporting the pulmonary metastasis of GCTB in the spine. The present study reports two cases of thoracic and sacral spinal GCTB lesions with pulmonary metastasis. One of the patients was a 45-year-old male who presented to hospital with gradually worsening pain in the left buttock during the last two years and was diagnosed with GCTB of the sacrum. The other patient was a 30-year-old female who complained of persistent back pain for a year and was also diagnosed with GCTB of the sacrum. Arterial embolization was performed prior to surgery and computer navigation was used during the surgery, resulting in the two patients achieving en bloc resection of their respective tumors, with satisfactory rehabilitation to follow. Giant cell tumors of the bone (GCTB) are intramedullary bone tumors with benign and locally aggressive pathological and clinical characteristics ,2. GCTBsA 45-year-old male presented to a local hospital with pain in the left buttock that had persisted for 2 years. The pain was particularly bad when the patient was tired, however, the patient did not initially seek medical attention, as the pain was bearable. As the pain gradually worsened over the two-year period, the patient finally attended a clinic at a local hospital. Magnetic resonance imaging showed an irregularly-shaped mass, 7.5×9.1×9.3 cm in size . SurgeonAt the follow-up examination four months after the first surgery, a chest CT scan revealed a nodule with clear borders in the anterior upper left lobe of the lung . SubsequNine months after the first surgery, a follow-up magnetic resonance imaging (MRI) scan showed a recurrent mass at the site of the original GCTB lesion . The patA chest CT scan performed at a follow-up examination 21 months after the first surgery, showed numerous metastatic nodules in diffuse and random distributions in each lung . The patA 30-year-old female presented to a local hospital due to back pain that had persisted for one year. MRI revealed a 3.2×3.8×3.3-cm lesion in the twelfth thoracic vertebral body, with evident compression of the adjacent spinal canal and foramen. A 3.0×3.4×5.6-cm mass was also present in the spinal canal and posterior column of the T12 vertebra . A fine-The patient was transferred to the Department of Orthopedic Oncology Surgery, Beijing Ji Shui Tan Hospital and computer-guided surgery was subsequently performed to resect the primary tumor . The spiGiant cell tumors of the bone within the vertebrae are rare, accounting for just 2.7–6.5% of all GCTB . AccordiThe ideal treatment for GCTB consists of an en-bloc excision at the early stages of the development of the lesion . HoweverAlthough radiotherapy is recommended in cases of unresectable GCTB , it is n


Extraskeletal myxoid chondrosarcoma (ESMC) of the vulva is an extremely rare tumor and currently, there is little available information on its biological behavior and treatment strategy. The present study reports a case of recurrent ESMC of the vulva in a 32-year-old female. The patient presented with an increasingly painful mass of the right vulva, at the site of an exision which had been performed 7-months previously. The tumor mass was histopathologically diagnosed as primary ESMC of the vulva and subsequently, vulvectomy was performed. Cytological examination showed negative surgical margins. Intraoperative radiation therapy at a single dose of 10 Gy was administered to the bed of the removed tumor. The patient refused chemotherapy and five months after surgery, a new lesion was identified in the inguinal region. Bilateral inguinal-femoral lymph node dissection was performed and external beam radiation therapy at a dose of 40 Gy was administered to the inguinal region. Follow-up examination seven months after surgery revealed no evidence of disease progression and at present, the patient remains alive. This study highlights the importance of analyzing each clinical case of ESMC as this may lead to the development of guidelines for the optimal treatment of this rare tumor. Approximately 90% of all malignant tumors of the vulva are squamous cell carcinomas. Sarcomas of the vulva constitute a variety of malignant neoplasms that account for 1–2% of vulvar cancers .In accordance with the 2013 World Health Organization (WHO) histological classification of tumors , sarcomaStout and Verner first described ESMC in 1953 . In 1994ESMC has a male to female ratio of 2:1, with the peak occurrence in the sixth decade of life . ESMC isLight microscopic examination reveals the presence of rather monomeric, small, round or oval cells with centrally located hyperchromic or vesicular nucleus, with evenly distributed chromatin, scant eosinophilic cytoplasm and often no evident nucleolus. Cells form straight or bent linear chains anastomosing between each other and forming a lacy arcade pattern. Perivascular condensation of the tumor cells, with formation of pseudorosettes, also occurs. Tumor structures are located in the prominent basophilic myxoid slightly vascular matrix. The tumor is divided into small lobules by narrow fibrous septa of different thicknesses. The peripheral region of the tumor is characterized by high cellularity, often with the evidence of rhabdoid and epithelioid cells. Cysts, hemorrhages and geographic necroses can occur in the tumor tissues ,19–23.Irrespective of ESMC localization, pre-operative diagnosis may be difficult, and thus verification is carried out based on the results of core-biopsy, followed by the evaluation of immunohistochemical and histological changes in the tumor tissue .In accordance with the minimum clinical recommendations of the European Society of Medical Oncology (2014) , wide exet al defined high cellularity, large tumor size, presence of anaplasia or rhabdoid features, high mitotic activity (>2 per 10 high-power fields) and proliferative activity  as adverse prognostic factors  and metastatic recurrences are observed in 46% of cases ,32,35.The present study reports a case of recurrent ESMC of the vulva in a 32-year-old female. Written informed consent was obtained from the patient.In June 2011, a 32-year-old female presented to a gynecological clinic  with a painful lesion on the right vulva arising after a trauma. Ultrasound examination revealed a round hypovascular mass, measuring 53×34×37 mm, in the soft tissues of the vulva. The patient underwent surgical excision of the mass. The conclusive diagnosis of the surgical specimen was of an organized hematoma.A mass that was gradually increasingly painful was observed at the site of the excision at 7 months post-surgery. The patient was then referred to the Cancer Research Institute .Gynecological examination revealed a tumor mass in the upper and middle thirds of the right labia majora, with involvement of the pubic area. The lesion consisted of multiple nodules with uneven density and well-defined borders, and was painful when palpated.Histological analysis showed a spindle-like cell sarcoma [G2 ]. ExaminThe ultrasonographic findings revealed a dumbbell-shaped lesion measuring 27×70 mm, with a heterogeneous structure, sharp and smooth contour.Spiral computed tomography scan revealed a well-defined multiple nodular lesion of soft-tissue density, actively accumulating the contrast, which was located in the soft tissues at the level of the symphysis pubis, on the right-hand side. The tumor extended to the right labia majora. Surrounding cellular tissue was thickened, but adjacent bone structures were unchanged. There was no evidence of lymph node involvement and no metastases were revealed.No pathological changes were noted in the abdomen or chest.The patient underwent wide excision of the tumor with reconstructive plastic surgery using local tissue flaps. The tumor was removed through a vertical incision. Cytological examination showed negative surgical margins. Intraoperative radiation therapy (IORT) at a single dose of 10 Gy was delivered to the bed of the removed tumor . The wouMacroscopic imaging of the surgical specimen showed a tumor with multiple nodules, measuring 5 cm at its widest, which was mainly of elastic consistency, with a well-defined fibrotic capsule, and with areas containing gelatinous material and hemorrhages .A pathological study of this case revealed that the tumor was circumscribed by a pseudocapsule and consisted mainly of moderately polymorphic spindle-shaped cells and to a lesser extent, epithelioid cells, with swollen or prolate nuclei, vesicular chromatin, with one hypertrophic hyperchromic nucleolus and with bipolar amphophilic cytoplasmic processes. Cells formed syncytial, clustered and lacy structures immersed in the abundant myxoid matrix, with mild diffuse lymphoid infiltration. Fields of rhabdoid cells and foci of necrosis were observed within the tumor. A few atypical multinucleated giant cells were also present. Moderate mitotic activity with the presence of pathology forms was noted. The tumor tissue, mainly in peripheral areas, was divided into fields by fibrous bands with mild diffuse infiltration . The majThe histological structure and immunophenotype of the tumor cells demonstrated an ESMC, G2 . Soft-tiThe patient refused chemotherapy. A new lesion appeared in the right inguinal region 5 months after the surgery for recurrent vulvar cancer.Magnetic resonance imaging showed heterogeneous enhancement of enlarged lymph nodes, measuring 37×25×34 mm, in the right inguinal region. Cytological examination revealed sarcoma cells. Due to disease progression, a bilateral inguinal-femoral lymph node dissection was performed. Metastatic involvement of ESMC in 1 of 13 lymph nodes was histologically confirmed. The patient subsequently received 40 Gy external beam radiation therapy to the inguinal region. The patient is currently alive with no evidence of disease progression at the 7 month follow-up.ESMC of the vulva is an extremely rare malignant neoplasm. Only 4 clinical cases have been reported in the literature to date –41. The There is little comparative data supporting the superiority of specific treatment regimens due to the low incidence of the ESMC.In the present clinical case, ESMC was diagnosed too late for antitumor treatment to be effective. Improvement of optical imaging techniques, including histochemical methods, can contribute to the early diagnosis of ESMC of the vulva .Due to the absence of a unified approach to the treatment of recurrent tumors, intraoperative radiation therapy to the bed of the removed tumor was selected in the present study, although there were have been no studies regarding the efficiency of IORT for vulvar sarcomas, including ESMC.In spite of the obscure pathogenesis and absence of a pathogenetically proven therapeutic strategy for vulvar sarcomas, including ESMC of the vulva, the analysis of each clinical case has a high value and can contribute to the development of the optimal treatment policy.


Primary sacral tumours are rare, therefore experience ofmanaging their associated complications are very limited.Effective surgical treatment of pelvic chondrosarcomaremains a major challenge for orthopaedic surgeons, due tothe complex anatomic structure of the pelvis, the lack ofdefined compartment borders, the close vicinity to vitalstructures, and the risk of jeopardizing pelvic structuralstability. We report a rare case of a giant sacralchondrosarcoma (100cm x 80cm) in an elderly male whosuccessfully underwent tumour resection with goodfunctional outcome and recovery. Long term follow up isessential in view of the possibility of local tumourrecurrence.Giant Chondrosarcoma, Sacrum, Surgery, Elderly Male It has apredilection for shoulder, pelvis, proximal femur, costalregion and rarely involves sacrum2. Secondarychondrosarcoma can develop from benign lesions such asenchondromatosis or with single or multiple cartilaginousosteochondromas in Maffuci syndrome and Ollier’s disease.Clinical occurrence of chondrosarcoma varies widely. Lowgradechondrosarcoma often grow slowly with a very smallrate of distant metastases. On the other hand, high-grade,mesenchymal and dediffrentiated chondrosarcoma are highlymalignant bone tumours with a poor prognosis. Tumourrelapses are strictly related to histologic grade3. In general,surgery is the most important therapeutic modality becausethese tumours are generally not sensitive to chemotherapyand radiotherapy treatment.Primary tumours of the sacrum are rare. Metastases are themost common malignant tumours of the sacrum and only sixpercent of all malignant bone tumours arise from sacrum,including chordomas (50% of cases), multiple myeloma(10%), lymphoma (9%), osteosarcoma (4%), and in very rarecircumstances chondrosarcoma involve sacrumA 62-year old male, presented with painless gluteal swellingfor the past 8 years. The swelling was gradually enlargingover the years. Due to the size and location of tumour, thepatient was only able to stand with support and sleep in asitting position for the past one year A multidisciplinary intervention by orthopaedic surgeon,plastic surgeon, radiologist, and anaesthetist as well asoncology team was commenced and the patient successfullyunderwent tumour excision. Intra-operative by the tumourmass was noted to have originated from S1 down to thecoccyx and mid buttock and encased the left sciatic nerve onthe left side. Anteriorly, there was presence of amultilobulated mass extending into the pelvis with necroticmaterial not involving the anterior bladder wall or therectum. A total of 27 kg of tumour mass was debulkedleaving a base of tumour of 40 x 30cm which required theplastic surgery team to excise with wound reconstruction andcoverage. The late presentation of this elderly man to doctors isbecause of the insidious onset of pelvic chondrosarcomawith relatively symptom- free presentation at early stage andabsent distant metastasis. Therefore, sacral tumours are oftentoo large for achieving adequate surgical margins duringresection as it remains clinically silent for a long time beforethe patients finally decide to pay a visit to the doctors whenthey start to experience local pain due to its mass effect andcompression. In this case, the huge size of the sacral masswith ulceration and haemoserous discharge, neurologicaldeficit of right leg, CT scan report of massive bonydestruction and intra pelvic involvement of tumour all directing towards the possibility of a highly malignanttumour which is technically demanding to resect, especiallywithout the aid of MRI to delineate the extension of tumourand neurovascular involvement. Due to the enormous size ofthe sacral tumour, we encountered difficulty to fit the patientinto the MRI machine. Wide excision of sacralchondrosarcoma with adequate surgical margin remains theprocedure of choice in chondrosarcoma treatment, since thisis the most effective way of reducing tumour recurrencerate4. As a result, it is important to get early diagnosis andproceed with wide excision aiming to achieve as clearmargin as possible because surgical approaches are oftenlimited by the size of the tumour and the proximity to vitalstructures in advanced cases. The surgeon has to maintain asubtle balance between ensuring adequate resection marginsand the risk of endangering adjacent vital structures as wellas the structural stability of the pelvis. In our case asadequate surgical margins were difficult to achieve, onlytumour debulking surgery was possible because of theextensive involvement of sacral plexus and intra pelvicstructures. Although chondrosarcomas are reported to haverelatively low sensitivity to chemotherapy and radiotherapy,local control is sometimes achieved through chemoradiation.In this patient debulking surgery in a massivesacral chondrosarcoma combined with radiotherapy was anoption for local control of low grade well-differentiatedchondrosarcoma to improve patient quality of life andsurvival rate.


A 52-year-old man with a seven-year history of progressive weakness, gait problems, and pain in his extremities presented with subacute worsening of his symptoms. Examination revealed weakness in all four extremities, increased tone, hyperreflexia, and sensory deficits. MRI of the cervical spine showed an area of signal abnormality and abnormal enhancement within the cervical cord at the C5–C6 level. The patient initially underwent biopsy followed a few days later by a debulking surgery. Postoperatively, the patient showed improvement in strength as well as ambulation. Intraoperatively, the lesion was confirmed to be intramedullary without any dural attachments. Histopathological examination revealed an extensively hyalinized tumor with sparse collections of cells that were immunopositive for both cytokeratin and GFAP, and immunonegative for EMA and progesterone receptor. This is an unusual pattern of expression, with cytokeratin immunopositivity suggesting a meningioma and GFAP immunopositivity suggesting a glioma. Considering the combination of extensive hyalinization with cytokeratin positivity the tumor was thought to be most consistent with a hyalinized meningioma with GFAP positivity. GFAP-positive meningiomas are rare, and these include the recently described ‘whorling-sclerosing’ variant. Only three cases of this tumor have been previously reported, all of which were intracranial. This is the first reported case of an intramedullary whorling-sclerosing meningioma. Meningiomas are generally benign tumors originating from non-neuroepithelial progenitor cells, known as arachnoid cap cells. The WHO classification identifies fifteen distinct histological variants , though cervical (15%) and lumbosacral (5%) tumors are also observed  is specific for neuroglial filaments and has been used as a reliable marker for normal, reactive, and neoplastic astrocytes. It is a structural protein which is the predominant component of glial intermediate filaments. Therefore, a tissue immunopositive for GFAP is assumed to be of glial origin. More recently GFAP immunopositivity has been recognized in other types of neoplastic and normal tissues as well, including epiglottic cartilage, renal carcinoma metastatic to brain, malignant pleomorphic adenoma of the salivary glands, and papillary meningioma Budka .Our case also represents a rare example of cervical intramedullary meningioma. Completely intramedullary meningiomas in the spinal cord are rare, with only six reported cases in the literature Table . Five ofOur report confirms previous reports that spinal meningiomas can be intramedullary and that GFAP positivity is not specific for glial tumors. Fortunately, management is unchanged for these variants and surgical resection carries a good prognosis. Nevertheless, the ‘whorling-sclerosing’ variant of meningioma is a rare type of neoplasm with a specific histopathological and immunological profile that should be recognized.Patient consent has been obtained and is on-file at the University of Toledo Medical Center, Toledo, OH, USA.


Dear Sir,Invasive intestinal amoebiasis presenting as cecal perforation is a rare entity in children. It is associated with high mortality and dismal outcome.[1] We hereby highlight a case of 4-year old child with amoebic colitis causing cecal perforation and peritonitis.A 4-year old boy presented with acute abdomen for 2 days. There was a history of recurrent abdominal pain for 2 weeks. After initial resuscitation, abdominal radiograph was done, which showed multiple air-fluid levels. Ultrasonography showed dilated bowel loops with thickened wall. Blood investigations revealed anemia and markedly raised C-reactive protein. After optimization, laparotomy was performed which revealed gangrenous ileo-cecal region and perforations in cecum and terminal ileum, with foul smelling contents. Ileostomy and ascending colostomy was performed after resecting ileo-cecal region. In the post-operative period, foul-smelling contents kept coming out through the distal stoma for a week. Colonoscopy was done through the distal stoma, which revealed edematous and ulcerated ascending colon. Biopsy showed the presence of flask-shaped ulcers suggestive of amoebic colitis . Metronidazole and diloxanide furoate were started and the child gradually improved. He was discharged after 11 days of admission and a month later distal colostogram was done, following which stoma was closed.Intestinal amoebiasis specifically affects the colon with predilection for right side and 6-11% of patients with invasive form may require surgical intervention.[1] Most of the times, it is asymptomatic. On the other end of the spectrum acute fulminant colitis with perforation, the most dreaded and feared complication associated with very high mortality rate, may result.[1-5] Management is a bit challenging, as there is low threshold of suspicion for amoebiasis as a cause of perforation, and the diagnosis is made intra-operatively or after the biopsy report. Intra-operatively, the diseased colon is extensively friable and disintegrates with simple manipulation, as happened in our case. Eggleston et al and Lubyuski et al advocated diversion and drainage as the main procedure, with resection reserved for gangrenous bowel; mortality associated with resection in these two studies were 71% and 83% respectively. The best management for children with amoebic colitis perforation would be the resection of gangrenous colon with stoma formation, as this will not only remove the diseased segment, but also remove the septic foci and prevent further fecal contamination. Once the patient recovers, intestinal continuity may be restored later on after performing distal colostogram to confirm distal patency. Primary repair should always be discouraged as the chances of anastomotic dehiscence are very high due to inflamed and friable bowel.Source of Support: NilConflict of Interest: None declared


Panniculitis is an inflammatory disorder of subcutaneous adipose tissue, frequently accompanied by vaculitis and systemic involvement. The peak age of patients is 20-30 years, and it is fairly rare in children, especially in infants.Therefore, panniculitis-like lesions in young children require differential diagnostic procedures and sometimes joined efforts of rheumatologists, hematologists and oncologists.to illustrate the difficulties in the diagnosis of subcutaneous panniculitis-like T cell lymphoma in an infant.A 9-month-old child was admitted to our clinic in September 2012 for treatment of his panniculitis. First lesion on the face appeared in June 2012, abscess was suspected and it was surgically drained. Yet, in July 2012 there were new lesions and the boy started having daily spikes of fever. He was started on antibiotics without effect, the number and the size of lesions continued to grow. The biopsy was performed and based on the pathology results and symptoms the diagnosis of Weber panniculitis was made and the boy was referred to our center.9/ml), increased transaminases and lactate dehydrogenase. Chest X-ray, ultrasound of the abdomen, chest and abdomen CT were unremarkable, except for hepatosplenomegaly and obviously enlarged peripheral lymph nodes. Considering the age of the patient the diagnosis of panniculitis seemed questionable and the biopsy was repeated. As a result panniculitis-like T-cell lymphoma of the skin was diagnosed, the child was started on CHOP protocol treatment with dramatic improvement of his general condition and reduction of the lesions.At the initial examination the boy appeared not well, very pale, had daily fevers, and multiple edematous purple skin lesions on his face, legs, arms. He had large peripheral lymph nodes (up to 3 cm), hepatosplenomegaly, anemia (hemoglobin to 88 g/L), mild leucopenia (WBC 3.2 × 10The case demonstrates the importance of critical approach to rheumatological patients that don't fit the general understanding of the pathology and epidemiology of the disorder.None declared.


Benign esophageal lesions include a wide variety of rare neoplasms, polyps, and cysts. In general, these lesions are asymptomatic and have little clinical importance. However, on occasion these lesions become symptomatic due to esophageal obstruction, airway obstruction, or compression of mediastinal structures. In these cases, as well as cases when it is unclear if the lesion is malignant or benign, surgical resection is recommended. Resection is most often performed by extramucosal enucleation, a procedure that is oftentimes well-suited for a minimally invasive approach. Here we discuss the general approach and operative techniques used for minimally invasive resection of benign esophageal lesions. Therefore, lesions are most often described as intraluminal, submucosal, intramural, or extraesophageal in origin.2 Intraluminal lesions arising from the mucosa include various polyps, papillomas, and inflammatory pseudotumors. Submucosal and intramural neoplasms include leiomyomas, granular cell tumors, hemangiomas, neurofibromas, lipomas, schwannomas, and rhabdomyomas. Leiomyomas are by far the most common histologic cell type, accounting for more than 80% of all benign esophageal tumors.3 Cysts, including intramural inclusions cysts and extraesophageal congenital duplication cysts, represent the second most common benign esophageal lesion.4 Gastrointestinal stromal tumors (GISTs) may be found anywhere in the gastrointestinal tract, including the esophagus. These neoplasms have malignant potential and management of these lesions is beyond the scope of this discussion.Benign esophageal lesions are a heterogeneous group of rare neoplasms, polyps, and cysts. It is estimated that benign tumors represent less than 1% of all esophageal neoplasms and less than 10% of all resected esophageal neoplasms.4 As a result, dysphagia is the most common complaint on initial presentation. Other reported symptoms include chest pain, dyspnea, chronic cough, wheezing, and upper gastrointestinal bleeding.4 Circumferential growth of benign esophageal tumors can have the typical appearance and presentation of an esophageal stricture or malignant tumor.Most benign esophageal neoplasms are asymptomatic, in part because of their indolent rate of growth and the ability of the esophagus to dilate. Because of this, the true incidence remains unknown. Most symptoms are caused by obstruction of the esophageal lumen or compression of surrounding mediastinal structures. Intraluminal lesions and large submucosal or intramural lesions, typically > 5 cm, are most likely to be symptomatic.2 and the biopsy can create mucosal scarring that may increase the risk of perforation during enucleation.3 Establishing the location of the lesion relative to the cricopharyngeus and gastroesophageal junction is necessary to determine the optimal surgical approach . Endoscopic ultrasound can be useful for assessing which layers of the esophageal wall are involved and evaluate for evidence of invasion into surrounding layers, which would raise suspicion for a malignant lesion.Because dysphagia is the most common initial complaint, many patients undergo a contrast esophagram as part of the initial workup. This study will identify most symptomatic lesions, most often revealing a smooth-surfaced filling defect. Computed tomography scans are not typically necessary, but may be helpful in determining the degree of mass effect caused by the lesion on the esophagus and/or other mediastinal structures, as well as identifying other extraesophageal causes of symptoms. Upper endoscopy is important for confirmation of the diagnosis and for operative planning. Intraluminal lesions can be differentiated from lesions originating from deeper layers of the esophagus, which will have an intact layer of mucosa overlying the lesion. Endoscopic biopsy of intraluminal or submucosal lesions is recommended if there is any suspicion of malignancy. Biopsy is not recommended for intramural lesions, as adequate tissue is rarely obtained to rule out malignant disease (i.e. leiomyoma versus leiomyosarcoma)5 however the safety, feasibility, and improved outcomes associated with minimally invasive approaches (VATS and laparoscopy) are well documented and is preferred over open approaches in the hands of surgeons with expertise in minimally invasive esophageal surgery.3 There are no strict contraindications to a minimally invasive approach for the enucleation of benign lesions. However, lesion size, previous chest or abdominal surgery, prior biopsy, and surgeon experience all contribute to the technical difficulty of the procedure and should be considered when deciding between open or minimally invasive approach. Symptoms suggesting other esophageal pathology, such as reflux disease, hiatal hernia, and motility disorders should be appropriately worked up and addressed at the time of operation.Incidentally encountered lesions with minimal or no symptoms are typically observed. Symptomatic lesions  and lesions that are clearly growing warrant consideration of removal, particularly if there is concern for malignant transformation or to facilitate other esophageal procedures. Smaller intraluminal lesions can typically be resected with endoscopic techniques, such as EMR or polypectomy snaring for pedunculated lesions. Larger lesions may require resection via an esophagotomy. Submucosal, intramural, and extraesophageal lesions are typically amenable to extramucosal enucleation. Resection can be performed with low morbidity using traditional open techniques (thoracotomy and laparotomy);Given that the majority of benign esophageal lesions are submucosal or intramural in origin, the remaining discussion and illustrations will focus on the operative techniques used for minimally invasive extramucosal enucleation of these lesions.The authors typically avoid placing a nasogastric tube at the conclusion of the procedure. A contrast esophagram is obtained on postoperative day 1, assuming the patient is not nauseated and has a good, strong cough. Once esophageal leak has been ruled out, the patient is started on a clear liquid diet. The patient is discharged home with liquid oral pain medication and a bowel regimen once tolerating clear liquids. The diet is advanced gradually as an outpatient, starting with 3 days of clear liquids, followed by 3 days of full liquids, and finally soft solids. The patient is seen 1–2 weeks postoperatively in the clinic, at which time the patient is advanced to a regular diet if doing well.No clear guidelines exist for long-term follow up of patients following enucleation of benign esophageal lesions. Follow up with repeat contrast esophagram in 6 months to a year postoperatively may be considered in cases where repair of mucosal perforation was needed or in cases of postoperative esophageal leak to monitor for esophageal structuring. Patients who develop recurrent symptoms should also be re-evaluated with a contrast esophagram and upper endoscopy to evaluate for late complications and new or recurrent lesions.3 However, meticulous surgical technique and diligent investigation following enucleation should allow the surgeon to identify and repair the injury with minimal risk of postoperative morbidity. As always, conversion to an open operation should be undertaken without hesitation if necessary to fully achieve the goals of the operation and to ensure patient safety and excellent outcomes.In summary, benign esophageal lesion enucleation is a procedure that is ideally suited for minimally invasive surgical techniques. Appropriate patient selection, work-up, approach, and port placement is key to a successful operation. Risk of mortality and morbidity is low, with mucosal perforation being the most feared perioperative complication.


Sarcoma development has been associated with genetics, irradiation, viral infections, and immunodeficiency. Reports of sarcomas arising in the setting of prior trauma, as in burn scars or fracture sites, are rare. We report a case of a leiomyosarcoma arising in an arm that had previously been replanted at the level of the elbow joint following traumatic amputation when the patient was eight years old. He presented twenty-four years later with a 10.8 cm mass in the replanted arm located on the volar forearm. The tumor was completely resected and pathology examination showed a high-grade, subfascial spindle cell sarcoma diagnosed as a grade 3 leiomyosarcoma with stage pT2bNxMx. The patient underwent treatment with brachytherapy, reconstruction with a free flap, and subsequently chemotherapy. To the best of our knowledge, this is the first case report of leiomyosarcoma developing in a replanted extremity. Development of leiomyosarcoma in this case could be related to revascularization, scar formation, or chronic injury after replantation. The patient remains healthy without signs of recurrence at three-year follow-up. Soft tissue leiomyosarcoma is a relatively rare tumor, arising from smooth muscle and comprising approximately 5–10% of all soft tissue sarcomas .Few reports exist in the literature of sarcoma occurring in the setting of prior musculoskeletal trauma. Case reports exist in the non-English literature that describe posttraumatic osteosarcoma , osteosaWe present the case of a patient who sustained a traumatic amputation around the level of the elbow joint as a child and subsequently developed leiomyosarcoma in the replanted limb 24 years later.At the age of 8, the patient was involved in a tractor accident, and his right arm was severed just distal to the elbow joint. He underwent multiple surgical procedures, resulting in successful replantation of the limb. Of note, immunosuppression was not required given that he had undergone a replantation and not a transplant. Despite suboptimal function, he was working full time in construction at the time of presentation.24 years subsequent to the accident, the patient returned to the hand surgeon who had performed his replantation as a child when he noticed a mass approximately the size of an orange in his right volar medial forearm. MRI was obtained that demonstrated 10 × 10 × 10 cm heterogenous, subfascial mass, ostensibly originating from the area of the interosseous membrane . His surHe was subsequently referred to our tertiary sarcoma center for evaluation by a musculoskeletal oncologist. At that time, he had essentially normal neurologic hand function with a composite grasp and an elbow ankylosed at approximately 30° of flexion. The arm had enumerable scars with evidence of multiple prior soft tissue transfers, but no draining sinuses or evidence of infection. The most recent incision had been made along a previous incision line and extended proximally through the antecubital fossa. A large mass was palpable in this region. Repeat imaging studies 6 weeks after the incomplete excision revealed that the lesion had essentially completely regrown to its preoperative size and shape.A lengthy discussion was held with the patient, whose primary concern was to salvage the limb if at all possible. Due largely to his extreme physical and emotional investments to save the arm as a child, he was unwilling to accept the possibility of an amputation. An angiogram was performed, identifying the ulnar artery as the only major vessel feeding the distal arm, though there was an extensive collateral tree. It was determined at this time that excision with negative margins would be technically challenging but feasible.The patient was taken for surgical resection with a limb-sparing procedure and brachytherapy. The median nerve was adhered to the tumor, but the musculoskeletal oncologist was able to salvage it by sacrificing some of the branches. The ulnar artery, however, was encased by tumor. Vascular surgery evaluated the patient intraoperatively and was able to identify a Doppler signal from the brachial artery proximally but could not find a good vessel for a bypass. Therefore, the decision was made to ligate the ulnar artery despite it being the predominant vascular supply to the distal limb. Surprisingly, the radial pulse was dopplerable and the hand remained warm and soft. The tumor was removed en bloc and brachytherapy catheters were placed under a vacuum-assisted-closure (VAC) dressing as described .Postoperatively, the radial pulse remained dopplerable, and the hand remained well perfused. Pathology revealed a 10.8 cm residual high-grade leiomyosarcoma with 5–10% necrosis and 28 mitoses per 10 high-powered fields . MarginsAfter recovering from these operations, the patient had 4 cycles of ifosfamide and doxorubicin adjuvant chemotherapy. At his most recent follow-up, three years postoperatively, the patient had no evidence of local or systemic recurrence. He was back to work full time as a laborer and his wounds were completely healed Figures  and 3.Significant trauma has been reported to be a rare risk factor for development of neoplasia, particularly in the case of burns. A review of 80 years of burn literature found 412 case reports of neoplasm arising in burn scars. While squamous cell carcinoma was by far the most common at 71%, 5% of the cases were sarcoma, including one report of leiomyosarcoma . The meaThe pathogenesis of burn scar malignancies is not precisely understood. Initially, it was hypothesized that the damaged tissue released toxins that induced cellular mutations . AnotherLeiomyosarcoma is typically an intra-abdominal or retroperitoneal tumor, though, in a study that spanned 7 years, 12% were located in the extremities and all but one was subfascial. While a vascular origin was suggested by the relationship of the tumor to the vessels in the majority of cases, definitive diagnosis could only be established in 5 of the cases. Tumor size >10 cm at presentation, mitotic rate >19 per 10 HPF, marginal or intralesional excision, and lack of adjuvant radiotherapy were found to be significant risk factors for progression of leiomyosarcomas located in the extremities. In their series, 2-year and 5-year disease-free survival rates were 42.3% and 32.6%, respectively [3, MTS stage III, presence of metastases, and axial anatomic location. Radiation and chemotherapy did not appear to directly affect outcome, but patients who underwent surgery with adjuvant therapy trended towards longer survival compared with those treated with surgery alone [Another series of 66 patients over 22 years with leiomyosarcoma arising from the soft tissues of the extremities found that only 16% were located in the upper extremities. Mortality in this series was similar with 50% survival at a mean of 3 years after diagnosis. Identified risk factors for mortality included tumor size >5 cmTo our knowledge, this is the first case reported in the literature of sarcoma development in a replanted limb and joins a very small number of cases of posttraumatic sarcoma in the extremities. The development of leiomyosarcoma in the upper extremity is unusual and even more unique in the setting of a previously replanted arm. While the precise etiology of posttraumatic sarcoma development has not been well established, analogous progression of burn scars into malignancies is proposed to result from development of an immune privileged site that hinders the body's ability to recognize and eliminate cancerous cells. This explanation is also plausible in the setting of severe trauma such as an amputation with multiple procedures required for successful replantation due to disruption of local lymphatic systems in both situations, which prevent tumor specific antigens from being carried to regional lymph nodes and allow for relatively unrestricted tumor growth , 16.In this case, similar to the average latency period seen in burn scar neoplasms, the patient developed a mass 24 years after his initial trauma. Although he presented with several poor prognostic indicators including a tumor size of >10 cm and a high number of mitoses per HPF, he was aggressively treated with wide resection and brachytherapy followed by chemotherapy in accordance with his determination to salvage the limb, minimize the chance of local recurrence, and eradicate micrometastatic disease. He has maintained baseline function of his replanted arm and remains free of local recurrence and evidence of metastases at three-year follow-up.


Pyometra is the accumulation of pus in the uterine cavity, and spontaneous perforation of pyometra resulting in generalized diffuse peritonitis is extremely uncommon. We report a rare case of diffuse peritonitis caused by spontaneous perforation of pyometra. A 66-year-old postmenopausal woman with diffuse abdominal pain and vomiting was admitted to our institution. She had a history of mixed connective-tissue disease and had been taking steroids for 20 years. Under a diagnosis of generalized peritonitis secondary to perforation of the gastrointestinal tract or uterus, supravaginal hysterectomy and bilateral salpingo-oophorectomy were performed. Unfortunately, wound dehiscence and infection occurred during the postoperative course, which were exacerbated by her immunocompromised state. Despite intensive care and a course of antibiotics, the patient died of multiple organ failure resulting from sepsis on the 36th postoperative day. Although correct diagnosis, early intervention, and proper treatment can reduce morbidity and mortality of spontaneous perforation of pyometra, if severe infection occurs, this disease can be life threatening for immunocompromised hosts. Pyometra is the accumulation of pus in the uterine cavity resulting from interference with its natural drainage , which m3/μL, hemoglobin 11.3 g/dL, and C-reactive protein (CRP) 7.32 mg/dL. Progression to sepsis was suspected because the WBC count documented at the previous institution was 13.0 × 103/μL, a drastic decline. A contrast-enhanced computed tomography (CT) scan showed the presence of fluid within the abdominal cavity and a significantly distended fluid-filled uterus. In addition, free air was detected in the abdominal and uterine cavities . Her abdomen was distended, with tenderness in the lower portion. Vaginal examination showed no cervical and vaginal anomalies, vaginal discharge, or detectable pelvic mass. Results of laboratory investigations on admission were as follows: white blood cell (WBC) count 3.0 × 10cavities .A preoperative diagnosis of generalized peritonitis secondary to perforation of the gastrointestinal tract or uterus was established. Consequently, emergent laparotomy was performed. At laparotomy about 300 mL of seropurulent, fluid was encountered in the peritoneal cavity. No abnormal findings were found in the gastrointestinal tract; however, a perforation with a diameter of 1 cm was found in the uterine fundus. The fallopian tubes and ovaries were normal. A supravaginal hysterectomy and bilateral salpingo-oophorectomy were performed . Klebsiella pneumoniae, Escherichia coli, and Bacteroides fragilis. Histologic examination revealed pyometra with no evidence of malignancy. Cervical cytology performed after surgery was negative for intraepithelial lesion or malignancy.Culture of the pus grew Candida albicans and β-d-glucan was increased in the blood. Subsequently, wound dehiscence occurred, and secondary wound closure was performed on POD 11. She was transferred to the intensive care unit again after surgery, and vancomycin  was added to antibiotic therapy because methicillin-resistant Staphylococcus aureus was detected from wound culture. From POD 11, high fever continued and platelet count declined once again. Although hepatic and renal dysfunctions were not revealed, sepsis and multiple organ failure were suspected. Despite the readministration of doripenem, thromboses of the inferior vena cava caused by infection and cerebral infarction were found on POD 26. Although antibiotics were changed from doripenem to linezolid  or vancomycin, sepsis grew progressively worse. Despite exhaustive clinical efforts, the patient died on POD 36.Pyometra is rare in the general population but more common in elderly women. It is caused as a result of occlusion of the cervical canal by malignant or benign tumors, surgery, radiotherapy, or senile cervicitis . Pyometr Escherichia coli  and Bacteroides species . The other bacteria were Klebsiella, Streptococcus, Staphylococcus, Acinetobacter, Porphyromonas, Enterococcus, and Actinomyces species. No bacteria were detected from the culture in four cases (16.0%). Data regarding complicated diseases were available in 29 cases. Nine cases (31.0%) involved an immunocompromised state, such as renal failure, diabetes mellitus, and steroid administration. The prognoses were documented in 33 cases. The total number of patients who died was five (15.2%). Of those in an immunocompromised state, four patients died, or the mortality rate was 44.4%. This may suggest that immunocompromised hosts represent a group at high risk for spontaneous perforation of pyometra. It is hoped that further cases are accumulated and that statistical analysis is performed.A PubMed literature search using key words such as “pyometra,” “perforation,” and “rupture” was performed, and only English-language articles were collected. Although correct diagnosis, early intervention, and proper treatment can reduce morbidity and mortality of spontaneous perforation of pyometra, if severe infection occurs, this disease can be life threatening for immunocompromised hosts.


Osseous choristoma is a normal bone tissue in an ectopic position. In the oral region lingual localization occurs more frequently and the mass is generally localized on the dorsum of the tongue. Definitive diagnosis is obtained only after histopathologic examination. The etiology remains already debatable. The treatment of choice is surgical excision. In this paper we present a case of tongue osseous choristoma and a review of the literature. The term choristoma is used to describe the growth of normal tissue in an abnormal position. Choristoma of the mouth may be composed of several different tissue types. These include bone, cartilage, gastric mucosa, glial tissue, and tumor-like masses of sebaceous glands. Osseous choristoma is a well circumscribed benign growth of normal, mature osseous tissue in ectopic sites.Osseous choristoma of the tongue is an extremely rare condition, of which only 66 cases have been reported in the literature till now. The osseous histotype is the most frequently described among choristomas. In the oral region lingual localization occurs more frequently and the mass is generally localized on the dorsum of the tongue. The etiology remains already debatable. The treatment of choice is surgical excision.A 10-year-old girl was referred to our ENT unit for a whitish 1 cm sessile swelling of the paramedian dorsum of the tongue, near foramen caecum . Upon paAfter 1-year follow-up there is no evidence of recurrence.The term osseous choristoma was introduced by Krolls et al. in 1971 , 3.Osseous choristoma of the tongue is an extremely rare condition. In the literature 66 cases have been described  1–45]. –45. 1–45The most frequent affected region is the posterior third of the tongue dorsum near to the foramen caecum and circumvallate papillae. Pathogenesis of choristoma is still unknown and remains already debatable. Several theories tried to explain the pathogenesis of this disease. Some authors suggested that remnants of the undescended thyroid tissue might produce an osseous lesion but in some rare case choristoma is localized not in midline but on the border of the tongue . In thesIn our opinion embryologic theory sounds true. Embryologically the tongue is a very complex structure. First and third branchial arches give rise, respectively, to the anterior two-thirds and posterior third of the tongue. It was suggested that pluripotential cells from these arches give origin to the osseous choristoma , 12.Choristoma appears as a sessile or pedunculated mass usually covered by normal mucosa. The sizes of the lesions vary from 3 mm to 50 mm at their largest diameter.Most of osseous choristoma of the tongue presents as a frequently asymptomatic swelling. The most frequent symptom is lump (46% of cases). Rarely patient complains of dysphagia (5 cases), gagging (4 cases), pain (4 cases), and nausea (1 case). Symptoms are correlated to lesion size, tumour localization, and surrounding tissues flogosis. The differential clinical diagnosis can be also based on the tumor location. When the lesion is located on the dorsal tongue near the foramen caecum we should consider in differential diagnosis benign tumours , thyroglossal duct cyst, lingual thyroid, mucocele, pyogenic granuloma, and malignant tumours  . TraumatThe treatment of choice is surgical excision. Recurrence or malignant evolution has not been described.


Pheochromocytoma is a rare and usually benign neuroendocrine neoplasm. Only 10% of all these tumors are malignant and there are no definitive histological or cytological criteria of malignancy. Single malignancy criteria are the presence of advanced locoregional disease or metastases. We report a case, with a giant retroperitoneal tumor having multiple metastases including palpable rib metastases, who was diagnosed as a malignant pheochromocytoma. The patient was treated with surgery. The literature was reviewed to evaluate tumor features and current diagnostic and therapeutic approaches for patients with metastatic or potentially malignant pheochromocytoma. Pheochromocytoma is a rare neuroendocrine neoplasm that synthesizes, stores, metabolizes, and also secretes catecholamines. Pheochromocytoma has a prevalence rate of around 300,000 adults/year . Having A case of 63-year-old man with complaints of severe chest pain, sweating, and nausea was investigated. Acute coronary syndrome was considered as the differential diagnosis, but the coronary angiography was normal. Abdominal ultrasound demonstrated a 15 × 14 × 13 cm mass in the left adrenal gland , and the123 metaiodobenzylguanidine (MIBG) or F18-fluorodeoxyglucose positron emission tomography (FDG-PET) scans are also recommended because they may help to detect metastases that cannot be detected by CT or MRI [Pheochromocytomas are rare catecholamine-secreting tumors derived from the chromaffin cells of the embryonic neural crest. The triad of headache, sweating, and palpitations in patients with hypertension is diagnostic, with 94% specificity and 91% sensitivity , 5. MetaSurgical resection is the only curative treatment for pheochromocytomas and sympathetic paragangliomas. Because there has been currently no effective cure for malignant pheochromocytoma, most treatments are palliative; however, in some cases, tumor and metastasis resection, if resectable, improve survival and quality of life and reduce exposure of the cardiovascular system . In patiOur case is a patient with a large-sized tumor (1750 gr) with multiple metastases in multiple organs which is denominated as malignancy. There were a large number of mitoses in the pathology evaluation and a metastatic rib masses which confirmed the diagnosis.The giant symptomatic, secreting pheochromocytoma with multiple metastasis in multiple organs is rare. We would like to emphasize the importance of preoperative diagnosis and using imaging modalities that can establish the right diagnosis of malignant giant pheochromocytoma. The treatment in these forms requires a multidisciplinary management. In this manner, we can reduce the mortality and the complications with increased survival rates and improved quality of life of patients.


Acquired abdominal intercostal hernia (AAIH) is a rare disease phenomenon where intra-abdominal contents reach the intercostal space directly from the peritoneal cavity through an acquired defect in the abdominal wall musculature and fascia. We discuss a case of a 51-year-old obese female who arrived to the emergency room with a painful swelling between her left 10th rib and 11th rib. She gave a history of a stab wound to the area 15 years earlier. A CT scan revealed a fat containing intercostal hernia with no diaphragmatic defect. An open operative approach with a hernia patch was used to repair this hernia. These hernias are difficult to diagnose, so a high clinical suspicion and thorough history and physical exam are important. This review discusses pathogenesis, clinical presentation, complications, and appropriate treatment strategies of AAIH. Intercostal hernias are rare phenomena caused by a disruption or weakness in the thoracoabdominal wall musculature resulting in herniation of fascia layers between adjacent ribs. Historically, these hernias have been characterized by their contents. They may only be an empty sac comprised solely of fascia elements  or may cThis is a fifty-one-year-old obese and hypertensive female who presented with a painful mass at her left upper abdominal quadrant and lower chest for about 24 hours. In addition to pain, she complained of nausea but denied vomiting or changes in bowel habits. She reported a history of stab injury to her left chest about fifteen years ago. She has had this mass for several years but had remained asymptomatic. Workup by her primary care in the past including a computed tomography (CT) scan concluded that the mass was most likely a lipoma.On physical examination, the patient was noted to be obese with a tender, firm, and nonreducible mass at the left upper quadrant and lower chest measuring about 8 × 8 cm. A new CT scan was obtained showing an abdominal intercostal hernia between the 11th rib and 10th rib. The hernia content was comprised of omentum, and no evidence of a diaphragmatic defect was seen on CT Figures .The patient was taken to the operative room where she was placed in a right lateral position. Under general anesthesia an incision was made over the hernia along the intercostal space. The hernia sac was identified and dissected clean of the surrounding subcutaneous tissue Figures  and 4.The hernia sac was opened and found to contain omentum, which was reduced back into the peritoneal cavity. The sac was subsequently excised, exposing a clear defect between the tenth rib and eleventh rib .A self-expanding polypropylene and ePTFE hernia patch (VENTRALEX Hernia Patch)  was thenAcquired abdominal intercostal hernia (AAIH) is an extremely rare phenomenon having only 19 cases reported in the literature worldwide [Abdominal intercostal hernias (AIH) are due to weakened or torn muscular layers of the thoracoabdominal wall, which is unable to provide adequate resistance to the outward forces of visceral contents pressing against it during variations in internal pressure . The outWhile disruption of the thoracoabdominal wall seems to be the only pathogenesis for the occurrence of abdominal intercostal hernias, it appears that it is not sufficient for all cases. It is likely that a combination of weakened tissues in the event of sudden increases in intra-abdominal pressure results in intercostal hernias or incarceration of previously reducible ones. This may explain why some patients with a distant history of anterior abdominal wall trauma, like in the case presented here, suddenly develop complications after years of being asymptomatic. The time interval between trauma and hospitalization for abdominal intercostal hernia, spontaneous or acquired, is highly variable. Some authors report hospitalization within the same day after trauma , while oSpecific areas of the chest wall are more vulnerable to herniation than others due to inherent weakness in certain anatomical zones . The cheThe diagnosis of any type of intercostal hernia can be difficult to make due to edema, hematoma, or obesity, which obscure the protruding abdominal wall contents . For thiSurgical management is necessary in nearly all cases due to risk of incarceration and strangulation of organs . In factBecause there are so few reports of acquired abdominal intercostal hernias, determining the efficacy of various surgical techniques employed is difficult. The surgeon must account for many factors about the patient and the injury before deciding on a repair technique. Closure of the defect can be achieved by the direct approach, as in the present case, which consists of a thoracotomy  performed along the intercostal space. It can also be done by an indirect approach, which consists of laparoscopy or open abdominal incision (laparotomy) , 13, 26.In emergency situations, the open abdominal approach is the most prudent operative choice as it allows the surgeon easy access to other intra-abdominal injuries often associated with blunt or penetrating injuries to the abdomen and thorax . LaparosIn nonemergent settings, as in our case, the direct intercostal approach has been shown to be effective and safe , 27. TheWhile Losanoff et al. found success using cable loops to approximate the ribs , such anRegardless of approach, the most recent comprehensive literature review on AAIH by Erdas et al. reports that recurrences occurred in 28.6%  of casesIn conclusion, physicians must maintain a high index of suspicion for both abdominal and transdiaphragmatic intercostal hernias in patients who present with palpable bulges over the chest wall, especially in those with a history of penetrating or blunt trauma to the abdomen and thorax. The CT is the diagnostic instrument of choice. Because there are so few reports of acquired abdominal intercostal hernias, determining the efficacy of various surgical techniques employed is difficult. The surgeon's experience and patient factors should be considered before deciding on a repair technique. Although the rates of recurrences and complications for AAIH have limited statistical credence, the cases reported in the literature do lend support for their potential in causing significant morbidities. Therefore, swift surgical management should be pursued in symptomatic patients with AAIH.


Pseudomyogenic hemangioendothelioma is a rare soft tissue tumor usually found in young adults, predominantly males. Fibrous dysplasia is a common benign bone tumor, which accounts for 5~7 % of all the primary benign bone tumors. However, pseudomyogenic hemangioendothelioma secondary to fibrous dysplasia is extremely rare. To the best of our knowledge, this is the first case of pseudomyogenic hemangioendothelioma secondary to fibrous dysplasia.This study describes a case of a 14-year-old female who suffered from pseudomyogenic hemangioendothelioma secondary to fibrous dysplasia of the left lower extremity. The patient underwent two operations successively due to pathological fractures in the left femur and tibia in a local hospital. She was diagnosed with fibrous dysplasia according to the postoperative pathological examinations. However, less than 1 year later, she was diagnosed with a recurrence of fibrous dysplasia in her left femur during a follow-up in our hospital. She underwent a curettage and grafting in the left femur. Postoperative pathological examinations demonstrated the diagnosis of fibrous dysplasia. Nevertheless, she presented to our clinic with a chief complaint of pain and swelling in her left tibia and calcaneus 4 months later. The patient underwent fine-needle aspiration in her left tibia. According to the histological and immunohistochemical findings, the diagnosis of pseudomyogenic hemangioendothelioma was confirmed by an expert pathology consultant. Finally, the patient had to undergo an amputation of the left thigh. Postoperative pathological examinations confirmed the diagnosis of pseudomyogenic hemangioendothelioma. Postoperative follow-up at 3 months disclosed no evidence of recurrent disease and no residual side effects from therapy.Pseudomyogenic hemangioendothelioma is a rare endothelial neoplasm which often mimics myoid and epithelioid tumors morphologically. For the diagnosis, the immunostaining is very important but not decisive and enough. Analysis based on any single factor or incomplete information may easily lead to arbitrary conclusion. Clinical information including age, gender, tumor location, disease course, and recurrence is important for appropriate diagnosis, and full understanding of the tumor is indispensable. Pseudomyogenic hemangioendothelioma (PMH), also known as epithelioid sarcoma-like hemangioendothelioma, occurs more frequently in young adult males and usually arises in the extremities, especially on the lower limb, and often involves multiple tissue planes –5. In thA 14-year-old female attended the Orthopedic Oncology Clinic at The First Affiliated Hospital of Nanchang University  for a follow-up. She underwent two operations successively due to pathological fractures in the left femur and tibia in a local hospital about 1 year ago. Then, she was diagnosed with FD according to the postoperative pathological examinations. Recently, she had no obvious symptoms and discomfort. Physical examinations showed nothing remarkable, but a little swelling in her left distal thigh. She had a negative family history of bone tumor. There was no fever or respiratory embarrassment accompanying the swelling. No history of weight loss or exposure to tuberculosis was mentioned.In a further examination, physical examination showed no palpable head, neck, supraclavicular, axillary, or epitrochlear lymph nodes. Inflammatory markers were within the normal limits. Plain radiographs indicated an expansile osteolytic lesion with marginal sclerosis and a matrix with a ground glass appearance and without periosteal reaction of the left distal femur, which was consistent with FD . Then, aBased on the exclusion of surgical contraindications, professional surgeons, who specialized in treating bone tumors, performed the surgery of curettage and grafting in the left femur. Histologic analysis of hematoxylin and eosin-stained specimens showed irregularly shaped spicules of immature bone without osteoblastic rimming and fibrous stroma with few mitotic activities . ImmunohNevertheless, she presented to our clinic again with a chief complaint of pain and swelling in her left tibia and calcaneus 4 months later. Radiographic review showed a prominent osteolytic lesion located in the distal portion of the tibia and calcaneus with cortical destruction . The patFinally, the patient had to undergo an amputation of the left thigh. The tumor consisted of densely distributed pleomorphic cells, ranging in configuration from small spindled elements to large plump epithelioid variants with prominent pink cytoplasm. Scattered cells with vacuolated cytoplasm were also present. Prominent pleomorphic nuclei contained optically empty centers with peripheral marginalization of the chromatin, in addition to conspicuous nucleoli. Multinucleated cells were also present. Vasoformative elements, such as multi-cellular vascular channels or intracytoplasmic vacuoles, were identified . ImmunohThe patient was discharged without any complications 1 week after the amputation. At the time of the 3-month follow-up, the patient reported no pain or discomfort in her left lower extremity. No evidence of recurrence or distal metastasis was noted during the 3 months after surgery. However, it is necessary to perform continuous observations of the patient because of a high rate of recurrence and metastasis.Pseudomyogenic hemangioendothelioma is a rare soft tissue tumor—distinct from epithelioid hemangioendothelioma —originalBy contrast, fibrous dysplasia (FD) is a common tumor-like lesion characterized by solitary or multifocal polyostotic intramedullary lesions . It has However, malignant transformation of FD is rare and occurs in less than 1 % of the cases , 14. TheFD can be divided into two major types: monostotic and polyostotic . AccordiPMH is extremely difficult to diagnose because of no morphological evidence suggestive of endothelial differentiation is present to confirm a radiological pattern of vascular neoplasm (multiple well-limited purely lytic lesions) . The tumBased on previous reports in other locations, PMH has a more indolent clinical course with a small risk of metastasis . TherefoIn conclusion, we present an extremely rare case of pseudomyogenic hemangioendothelioma secondary to fibrous dysplasia. Pseudomyogenic hemangioendothelioma is a rare endothelial neoplasm which often mimics myoid and epithelioid tumors morphologically. For the diagnosis, the immunostaining is very important but not decisive and enough. Clinical information including age, gender, tumor location, disease course, and recurrence is important for appropriate diagnosis, and full understanding of the tumor is indispensable. Analysis based on any single factor or incomplete information may easily lead to arbitrary conclusion. However, the reports of this tumor are limited. Further studies on more cases are in need for the full understanding and appropriate diagnosis of it. An awareness of the potential for PMH to present in this way is helpful for both radiologists and pathologists when confronted with this rare tumor. With regards to the present case, the patient remained symptom-free 3 months after surgery. Due to the risk of recurrence, and additional risk of metastasis, long-term follow-ups should be continued.FD, fibrous dysplasia; PMH, pseudomyogenic hemangioendothelioma


Ganglion and synovial cysts occur mainly, but not necessarily, in association with osteoarthritis. Presentation varies widely, ranging from small, incidentally detected, asymptomatic lesions to giant ones that might be the source of symptoms, either due to their compressive effect on adjacent structures or due to complications, such as rupture. On magnetic resonance imaging they are typically presented as smooth, well-circumscribed, thin-walled, unilocular, and homogeneously T2-hyperintense lesions. An identifiable thin stalk communicating to the joint space is not infrequent. Nevertheless, depending on their age, anatomic location, and eventual complication, they might have many distinct appearances, including septae and internal debris, which the radiologist must be familiar with in order to accurately differentiate them from worrisome cystic-like lesions. With regard to this diversity, some illustrative cases are presented. Ganglion cysts (GC) and synovial cysts (SC) are among the most frequently occuring benign cystic lesions in the joints. Degenerative joint disease is the main predisposing factor –6, but tDue to their strong similarities and their unclear pathogenesis, the scientific nomenclature associated with these lesions, labeled interchangeably in the literature, remains controversial. However, according to current evidence they are distinct, not only from an anatomopathological point of view, but also in their potential therapeutic approach . By defiContrary to SCs, GCs lack a synovial cell lining and are constituted by a dense collagenous capsule surrounding a mucopolysaccharide-rich gelatinous fluid –3, 6, siExcept for the spine, where zygoapophyseal or facet joint cysts frequently cause radiculopathy, neurogenic claudication, sensory deficits and, to a lesser extent, motor deficits , 6, mostSurgical excision of symptomatic, soft-tissue cystic lesions of this type, arthroscopic when possible, has been the advocated treatment so far, with satisfactory results. However, percutaneous image-guided procedures, including aspiration, with or without cyst rupture and/or steroid injection, are also effective alternatives that, despite the higher recurrence and failure rates, may avoid surgery without precluding it if warranted , 5–8. ReUltrasound (US), as a low-cost, widely available modality, is the initial imaging method of choice for any palpable soft-tissue mass in the extremities, usually differentiating cystic from non-cystic ones , 8. TherMRI should be performed after US in doubtful cases. It has become the gold-standard modality in the characterization of periarticular cystic lesions, mainly due to its excellent soft-tissue contrast and extremely high diagnostic power . AccuratRegarding the optimal MRI quality in the study of this kind of lesion in the extremities, the smallest surface coil that covers the entire lesion should be chosen and an initial large field of view, including the contralateral side, followed by a smaller field of view targeted to the lesion, should be used. Imaging acquisition of at least two perpendicular planes is mandatory, usually including the following weighted-sequences: T1, proton-density (PD) or T2, with and without fat suppression (FS), or short inversion time inversion-recovery (STIR) .The MRI protocol that better demonstrates facet joint SCs and GCs in the spine should include at least T2-weighted sequences acquired in both axial and sagittal planes , 6.Delayed arthrography, either by means of radiography, computed tomography, or MRI, 1–2 h after intra-articular injection of water-soluble contrast agent, improves sensitivity of imaging in the demonstration of a cyst-joint communication, detectable in less than 50 % on standard US or MRI , 12.As pointed out above, the radiologic distinction between an SC and a GC is frequently impossible, location being the most helpful criterion. As an example, while SC are very likely to occur around the knee and the hip, GC are most commonly found in the distal extremities, particularly in the wrist . The disRegardless of their distinction, most SCs and GCs on MRI look like smooth, well-circumscribed, and homogeneous cystic masses of variable size, with giant ones mainly occurring in large joints such as the knee and the shoulder  and beinTo summarize, the main features that any radiologist should be able to accurately describe are the precise location of the cyst and its relationship with the adjacent structures, so as to recognize signs of complication and rule out potentially worrisome solid components.The great majority of SCs arising from facet joints occur in the lumbar spine, L4/L5 being the most affected level. These lesions are seldom reported in the cervical spine and are even rarer in the thoracic spine. The advent of imaging techniques has led to an increase in the detection of lumbar facet SCs, whose incidence is approximately 0.65 % [Most patients are women in their sixties, usually presenting with chronic painful unilateral lumbar radiculopathy. Acute presentation of cauda equina syndrome secondary to intracystic hemorrhage has also been described .The MRI features of both symptomatic facet SCs presented in Figs. Besides bursitis, most periarticular cysts in the shoulder are associated with labrocapsular or rotator cuff tears resulting in the passage of fluid from the joint to the pericapsular soft tissues. The main example, paralabral cysts, usually occurs in the setting of a superior or a posterosuperior labral tear, the cysts tending to extend into the suprascapular and the spinoglenoid notches, respectively, with resultant compression of the suprascapular nerve and subsequent denervation of the supra and infraspinatus muscles, or the infraspinatus muscle alone, if the site of compression is the spinoglenoid notch, distal to the branch to the supraspinatus , 14.Far less common, acromioclavicular and intramuscular cysts are mainly but not necessarily associated with full- or partial-thickness rotator cuff tears, their presence improving the sensitivity and specificity of MRI detection of partial-thickness tears . IntramuRegardless of the type of cyst depicted on MRI, considering the strong association, labral or rotator cuff tears must always be ruled out, as well as muscle atrophy.With an estimated prevalence of 19 % in symptomatic patients having a MRI examination of the wrist and of 51 % in non-symptomatic ones , GCs areMost GCs in the wrist are asymptomatic and easily diagnosed by physical examination, seen as a smooth nodular tumefaction of firm consistency on palpation of 1–2 cm that transilluminates, imaging remaining reserved for less obvious cases, especially those presenting with sensory and/or motor symptoms due to nerve compression. Nevertheless, spontaneous resolution occurs in up to 50 % of cases, and the main reason that patients seek medical evaluation is cosmetic concern, as symptoms are rarely significant .As previously mentioned, US is the first-line imaging modality . The GC US-guided aspiration is an effective procedure when treatment is required, reducing patient discomfort and the risk of damage to adjacent structures when compared to the non-guided technique , as wellCystic lesions around the hip are incidentally found in up to 26 % of asymptomatic patients during imaging studies . After tSome other particular entities should be included in the differential diagnosis of a cystic lesion around the hip. Given their high prevalence, paralabral cysts deserve special mention. The mechanism of cyst formation is similar to that of paralabral cysts in the shoulder, with the passage of synovial tissue and/or fluid to the adjacent soft tissues through a labral tear, the majority occurring in the anterosuperior part of the acetabular labrum , 19. As Besides its strong diagnostic power for the lesions described, US-guided drainage and steroid injection is extremely convenient for symptomatic relief of bursitis , 17, 18.The joint most commonly affected by SCs is the knee. Although popliteal or Baker’s cysts are not true SCs, in practical terms, they are considered similar for the same reason described above with regard to an iliopsoas bursa. Present in up to 38 % of knees imaged by MR, they consist of an enlarged gastrocnemius-semimembranosus bursa, which in more than 50 % of the general population normally communicates with the joint space through a synovial protrusion that follows the path of least resistance in the posteromedial aspect of the joint capsule . LocatedAlthough far less common than a Baker’s cyst, SCs may arise from other locations around the knee, such as the tibiofibular joint, which communicates with the knee joint in 10 % of adults . An impoBesides the general risk factors for the development of intra- and periarticular cysts described for other joints, such as osteoarthritis, some other knee-specific disorders include meniscal and cruciate ligament lesions . In factAlthough MRI is the gold-standard technique in characterizing cystic lesions in the knee , US is aSCs and GCs occur frequently but not necessarily in association with osteoarthritis. The knee and the wrist are the most commonly involved joints, but their occurrence in other sites such as the facet joints, the shoulder, and the hip is not as rare as traditionally believed. Their typical appearance on MRI consists of a smooth, well-circumscribed, thin-walled, homogeneous cystic lesion, not infrequently with an identifiable pedicle connecting to the joint. Nevertheless, a more complex appearance with thin septae and internal T2-hypointense debris should not be misinterpreted, neither as complication signs nor as malignant-like ones. Wall thickening and irregularity, internal heterogeneous T1-hyperintense serohematic content, and surrounding edema suggest acute complication.


Acrokeratoelastoidosis is a rare condition characterized by bilateral multiple hyperkeratotic papules on the palms, soles, and dorsum of the hands and feet. To the best of our knowledge, only around 40 cases of acrokeratoelastoidosis have been reported worldwide, which are mostly familial.We report the first case from Saudi Arabia in a 5-year-old Saudi girl of Arabian origin, who presented symptoms of acrokeratoelastoidosis with unilateral sporadic keratosis on her left hand and left foot. We also review the clinical and histopathologic features, etiology, differential diagnosis and its treatment.Given the rarity of acrokeratoelastoidosis, little is known about the disease. Further studies are required to understand the pathogenesis of the disease for better treatment options. Additional case reports of acrokeratoelastoidosis will help in recognizing risk factors, patient characteristics, environmental influences and possibly new etiological factors. Acrokeratoelastoidosis (AKE) is a rare type of palmoplantar keratosis that manifests as round or oval papules distributed along the marginal borders of the palms and soles. Reported cases are either sporadic or familial where the familial cases are mostly autosomal dominant. Several treatments have been tried with variable success. The most common histopathologic features of AKE are hyperkeratosis, acanthosis and elastorrhexis. Here, we present a case of AKE in a 5-year-old Saudi girl and a review of the literature for the clinical presentation, histopathologic features, etiology, differential diagnosis, and treatment of the disease.AKE was first described by the Brazilian dermatologist, Oswaldo Costa in 1953 . It is oFor the differential diagnosis of AKE, focal acral hyperkeratosis has a similar clinical appearance, but during histopathological examination, elastorrhexis is not seen and alterations are confined to the epidermis (hyperkeratosis and acanthosis) . AnotherA 5-year-old Saudi girl of Arabian origin presented at King Abdulaziz Medical City, King Fahad National Guard Hospital’s dermatology clinic with a history of asymptomatic skin lesions on her left hand and left foot Figure . Her parClinical examination showed multiple yellowish hyperkeratotic papules, coalescing into verrucous plaques that were distributed in a line along the marginal surfaces of her left hand and foot. On her left hand, the lesions were on the palmer surface extending from the thenar eminence to the proximal part of her fourth finger. On her left foot, they were on the lateral side of her foot and fifth toe distally.Histological examination of a 4mm punch biopsy specimen taken from a papule on her left foot revealed hyperkeratosis and acanthosis Figure A. The spAKE is a rare disease, and while its pathogenesis remains unclear, the onset of the disease is frequently seen in infancy or adolescence . Most caFor sporadic forms of the disease, chronic trauma and excessive sun exposure have been proposed as possible causal factors. For familial forms of the disease, the most common inheritance mode is autosomal dominant, most probably related to chromosome 2 , but recThe clinical characteristic of AKE is multiple, yellowish papules that are sometimes shiny and keratotic. The papules can be located symmetrically on the lateral surfaces of the palms and soles; the space between the thumb and the forefinger; the anterior surface of the lower legs; and over the knuckles and nail folds . Hyperhyet al. suggested that erbium:YAG laser surgery may be considered a possible therapy. However, patients should be informed of the limited clinical improvement obtained with this treatment [Typical histopathological findings in AKE include hyperkeratosis with hypergranulosis, acanthosis, and epidermal hyperplasia. Van Gieson stain shows reduced number of elastic fibers in the dermis with a high proportion of fragmented fibers. There is an abnormal deposition or repair of connective tissue within the affected area. Usually, no treatment is required because lesions are asymptomatic with no adverse effects , but thereatment .Given the rarity of AKE, little is known about its incidence, etiology, and pathophysiology. Further studies are needed to address its associations with other diseases including autoimmune diseases. Genetic studies may reveal more about the disease and, therefore, may be helpful in identifying better treatment options. Additional case reports of AKE will help in recognizing the risk factors, patient characteristics, environmental influences, and possibly new etiological factors.Written informed consent was obtained from the patient’s legal guardian 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.AKE: Acrokeratoelastoidosis.The authors declare that they have no competing interests.HSAlK was the principal author and major contributor in writing the manuscript. AAAlH analyzed and interpreted the patient data. AHAlH reviewed the literature and AAAlS read and corrected the manuscript. All authors read and approved the final manuscript.


Primary cardiac sarcomas are rare tumors with a median survival of 6–12 months. Data suggest that an aggressive multidisciplinary approach may improve patient outcome. We present the case of a male who underwent resection of cardiac sarcoma three times from the age of 32 to 34. This report discusses the malignant nature of cardiac sarcoma and the importance of postoperative multidisciplinary care. Primary cardiac sarcomas comprise less than 1% of sarcomas, 75% being benign and fatal with location . Delay iIn March of 2009, a 32-year-old male presented with shortness of breath and chest pain. Cardiac enzymes were negative and a CT scan revealed a large left atrial mass. A transthoracic echocardiogram (TTE) confirmed the left atrial mass with severely hypoechoic perimeters attached to the posterior aspect of the left atrium. The patient was hemodynamically unstable and complete resection was performed via sternotomy with transseptal exposure of the left atrium. The tumor was attached to the free wall of the left atrium and the undersurface of the mitral valve. The entire epicardium was excised with the tumor over the left atrium, exposing the muscle of the left atrium to the inner atrium. Pathology revealed a 5.7 × 4.7 cm high grade sarcoma with evidence of muscle differentiation and immunohistochemical studies revealed strongly positive vimentin. The patient was discharged on postoperative day seven with instructions to follow up with the surgeon and an oncologist.In September of 2009 the patient presented with shortness of breath, chest pain, and palpitations. He had not followed up with an oncologist due to financial hardship. CT and MRI scans revealed three lobular enhancing masses within the left atrium. The largest, measuring 3.5 × 2.6 cm, was attached to the lateral wall of the atrium and on the posterolateral leaflet of the mitral valve, protruding into the left ventricle with extension into the left inferior pulmonary vein. The second mass was broad based posteriorly near the origin of the left inferior pulmonary vein. The third mass was in the interatrial septum . Liver aThe patient underwent a redo-sternotomy with cardiopulmonary bypass. A transseptal en bloc resection was performed including the posterior aspect of the pericardium, the entire left atrium up to the left pulmonary veins, and 1 cm from the annulus of the mitral valve . The mitPathology revealed malignant fibrous histiocytoma, lobulated and invasive into the myocardial tissue, with mitosis band, signifying the differentiation of the tumors, and strongly positive vimentin and CD68 stain . The patA CT scan in March of 2011 revealed a 2.8 × 3.7 cm left atrial recurrence 10 weeks after completion of chemotherapy. TTE revealed an echogenic density in the left atrium originating in the interatrial septum . Redo-stThe patient received adjuvant chemotherapy. The follow-up scans were negative for regrowth in the heart, but the tumor had spread in the pulmonary cavity with extrathoracic manifestations in the brain and liver. Chemotherapy and radiation (Gamma Knife radiosurgery) were utilized in 2012, with limited regrowth and partial response. Early 2013, the patient was admitted into acute care there upon progressed to multiorgan failure and expired on March 2013. The patient's survival after primary resection was 48 months.This case demonstrates the aggressive nature of cardiac sarcoma. The completeness of resection is considered a primary determinant to long-term survival. The goal is to achieve an R0 resection with adequate margins if possible when there is no evidence of extracardiac disease, or surgical debulking if deemed unresectable . Most leThe malignant nature of these tumors is well established and even after adjuvant therapy mortality is high . IncomplThis case is notable for the short interval to recurrence: 6 months between the first and second presentation and 4 months between completion of chemotherapy and the diagnosed second recurrence. Completeness of resection at first presentation is a positive feature in disease-free survival. Without adjuvant chemotherapy recurrence of cardiac sarcomas increases, as demonstrated in this case. Recurrence of primary cardiac sarcomas is common and close surveillance with an oncologist is essential.Video 1: MRI heart morphology at Presentation 2, depicting the mass attached to the lateral wall of the atrium and on the posterolateral leaflet of the mitral valve, prolapsing into the ventricle. The three masses can be seen in the left atrium interfering with blood flow and presentation of symptoms.Video 2: Echocardiogram at Presentation 2 showing the mobile, echogenic mass in the left heart prolapsing through the mitral valve into the ventricle in various cross sectional views.


We describe a 58-year-old lady who presented initially to her general practitioner with a palpable warty urethral nodule. She was subsequently referred to the urology department for further investigations. She underwent flexible cystoscopy and imaging, followed by rigid cystoscopy and excision of the nodule. Histological analysis was consistent with renal cell carcinoma (RCC). CT imaging confirmed the presence of an invading metastatic left renal cell carcinoma with bilateral metastatic deposits to the lungs and adrenal glands. The patient was enlisted on the Panther Trial and received a course of Pazopanib before undergoing radical nephrectomy. Two years later she is still alive with metastases remaining reduced in size and numbers. During this study we have performed a literature review of similar cases with this unusual presentation of RCC. Renal cell carcinoma metastasising to the ureters and bladder is uncommon but metastasis to the urethra is a rare entity indeed. This case is notable in two ways; firstly, it is only the second available report ever to be published regarding detection of a metastatic urethral nodule from an undiagnosed RCC primary. Secondly, it is the sole of available cases to present directly with a palpable nodule and not indirectly as urinary retention or isolated haematuria.We describe this very rare presentation of RCC adding to the multitude of symptoms associated with this malignancy. This study also comes to add to the current evidence that urethral metastatic deposits may be best treated with local excision rather than proceeding with more radical interventions. Furthermore we have recorded a positive result of neoadjuvant Pazopanib for metastatic RCC prior to nephrectomy.A 58-year-old lady with multiple comorbidities presented to her general practitioner with a palpable warty nodule at her urethral meatus. She had a prior 2-year history of malaise and microcytic anaemia. These were attributed to her known history of gastritis, systemic lupus erythematosus, and hypothyroidism.The urethral nodule had gradually appeared over a course of a few months. There was no history of weight loss or abdominal pain. On examination the abdomen was soft and nontender with no palpable masses. She was thus referred to the urology clinic for further investigations. Five days prior to her appointment she suffered from a single episode of frank haematuria. In view of the haematuria a renal tract ultrasound scan and flexible cystoscopy were organised.The ultrasound scan of the renal tract demonstrated a single large mass arising from the left kidney, reported at the time as a possible angiolipoma with no other possible findings. A week later flexible cystoscopy confirmed the presence of a warty nodule near the urethral meatus ; the resIn view of the findings the patient was put on the waiting list for a rigid cystoscopy and excision of the nodule. In the interim period a CT urogram  was requThe patient underwent excision of the nodule the following week. Histological examination of the nodule confirmed the presence of a Fuhrman Grade 3 invading clear cell renal carcinoma .The results of the CT scan and of the histological analysis were discussed in the urology MDT. It was decided that the patient should be started on the Panther Trial and treated with neoadjuvant Pazopanib, with a view of reducing the tumour size prior to considering surgical resection.A follow-up CT scan  four monThe patient is now two and a half years after the initial diagnosis; she has been followed closely with regular CT scans and flexible cystoscopies. There has been no evidence of local tumour recurrence. Based on imaging, some of the adrenal and pulmonary metastases have disappeared whilst the persisting ones have reduced in size and have remained stable for over a year. There is no current evidence of any new lymph node involvement.Renal cell carcinomas (RCCs) are a relatively common neoplasm, representing 3% of all adult malignancies. Contrary to popular belief, the triad of haematuria, flank pain, and palpable abdominal mass rarely occur simultaneously. In the majority of cases only one of these symptoms occurs, while being not uncommon to present with a manifestation of metastases or of a paraneoplastic syndrome.RCC is well known for its tendency to metastasise to other organs. The commonest recipients for metastases are the lungs, liver, bone, and brain. Metastatic deposits to the urethra from a RCC are a remarkably rare occurrence with only seven cases reported in the international literature –7. The rAlmost all the reported cases (except one) involve late metachronous metastases in patients with a known diagnosis that had already received treatment. This is indeed the first ever documented case to present with a palpable urethral nodule along with visible haematuria, with the only other analogous case having manifested as isolated acute urinary retention before the metastatic deposit being identified on cystoscopy .Prior published cases have reported poor outcomes following the detection of the urethral metastasis, despite transurethral resection or more radical therapeutic interventions such as phalangectomy . In our Urethral outgrowths should always undergo excisional biopsy, and although rare, a possibility of a metastatic deposit needs to be considered.Metastatic urethral deposits from a RCC primary may be best treated with local excision with regular cystoscopic surveillance rather than with a more radical resection.


We report an example of dysplastic intestinal-type metaplasia involving endometriosis of the appendix in a 45 year old woman. One other example of this phenomenon has been reported. As it occurs within the muscular wall of the appendix, confusion with low grade appendiceal mucinous neoplasm (LAMN) may occur. Evidence supporting the metaplastic nature of the intestinal epithelium is offered. As the initial pathological diagnosis was of invasive cancer with perforation of the appendix treatment consisted of peritonectomy and hyperthermic intraperitoneal chemotherapy (HIPEC).http://www.diagnosticpathology.diagnomx.eu/vs/1068246472111756.The virtual slides for this article can be found here:  Endometriosis of the appendix may be an incidental finding or the cause of appendicitis, intussusception or perforation . VariousA 45 year old woman underwent surgery at another hospital following a diagnosis of acute appendicitis. The appendix appeared enlarged and perforated. Multiple “peritoneal mucinous implants” were observed in the right pelvis. Following a pathologic diagnosis of “invasive low-grade carcinoma of the appendix colonizing appendicial endometriosis”, the patient was referred to our hospital for consideration of second look laparotomy and HIPEC. An extensive evaluation revealed no evidence of metastatic disease.2) was administered. Pathologic examination showed foci of endometriosis on the ileal surface of the right hemicolectomy specimen, the left Fallopian tube and in one of the fragments of peritoneum. The mucin was acellular. Both ovaries had functional cysts.Without recourse to pathological review of the appendectomy specimen, second look laparotomy was performed exactly one year later. There was no macroscopic evidence of neoplasia, but mucin was seen localized to the right peritoneal surface. Free intrabdominal mucin was absent. Right hemicolectomy, resection of two segments of small intestine, omentectomy, bilateral ovariectomy, and peritoneal resections were carried out. HIPEC  stained sections of the appendectomy specimen from the referring hospital were reviewed. In turn, 4- micron thick recuts of all the formalin-fixed, paraffin-embedded tissue blocks were stained with HPS in our laboratory. Unstained sections from all blocks were submitted for histochemical and immunohistochemical studies: periodic acid-Schiff stain with diastase (PAS-D) and without (PAS), and monoclonal antibodies directed against pancytokeratin AE/AE3, cytokeratin 7, cytokeratin 20, CD10, estrogen (ER) and progesterone (PR) receptors, and Ki-67 (MIB-1).The pathology report from the referring hospital described an appendix 6 cm in length and varying from 1 to 2,5 cm in diameter with presence of a mucocele and perforation. The wall varied from 0,5 to 1 cm in thickness with “brownish zones”. Microscopic examination showed acute appendicitis, acellular mucin dissecting the wall with extension to the serosa compatible with perforation , and intussusception of the appendix .Endometriosis can display metaplastic changes. In the ovary 12-68% of endometriosis lesions show metaplasia in the form of ciliated, eosinophilic, hobnail, squamous or mucinous change, with endocervical-type metaplasia encountered more commonly than intestinal-type .One other case of dysplastic intestinal-type metaplasia involving appendicial endometriosis has been reported . A 39 yeSimilarly, we believe our case represents intestinal metaplasia rather than colonization, as: 1) no connection between the overlying native epithelium and the foci of endometriosis was found, 2) multiple foci of direct transition between endometriotic epithelium and intestinal-type epithelium were present, 3) ER-positive mucinous cells at sites of transition from endometriotic to mucinous epithelium were identified, and 4) the known potential of endometrium to undergo intestinal metaplasia ,11.The differential diagnosis in the present case is with low-grade appendiceal mucinous neoplasm (LAMN). LAMN is characterized by invasive intestinal-type epithelium with abundant mucin production, minimal architectural complexity and low-grade cytologic atypia . Two feaThe decision to perform peritonectomy and HIPEC was based on an initial pathological diagnosis of invasive cancer. Given the uniqueness of our case it is impossible to know what further treatment, if any, would have been warranted had the correct diagnosis initially been made. We therefore suggest that in any appendectomy specimen with endometriosis and suspicion of neoplasia that the possibility of intestinal metaplasia be considered in the differential diagnosis.Written informed consent was obtained from the patient for the publication of this report and any accompanying images.The authors declare that they have no competing interests.AM provided the pathologic diagnosis and drafted the manuscript. PD and LS provided clinical information, wrote the case details and contributed to the nonpathological aspects of the discussion. All authors read and approved the final manuscript.