Adenocarcinoma of the ovary is an aggressive neoplasm which often metastasizes to the lung or liver. Metastases rarely occur to the pancreas, but a tissue diagnosis is required to confirm this event. Although most tumors of the pancreas are primary pancreatic neoplasms, metastatic lesions have been reported most commonly as arising from renal cell carcinoma.We report the case of a 51-year-old Caucasian woman with ovarian mucinous adenocarcinoma with metastasis to the head of the pancreas that was originally misdiagnosed as a pancreatic primary tumor.Mucinous ovarian adenocarcinomas rarely metastasize to the pancreas. New pancreatic lesions should be investigated through tissue biopsy and tumor markers, while keeping an open-minded differential diagnosis to avoid a misdiagnosis or a delay in treatment. Although most malignant tumors of the pancreas are primary pancreatic neoplasms, metastatic lesions have been reported most commonly as arising from renal cell carcinoma. Here we present a case of mucinous adenocarcinoma of the ovary that metastasized to the pancreas. The tumor was first diagnosed as a primary pancreatic tumor. Ovarian adenocarcinoma can have distant metastases, but these are most often to the liver or lung. Metastasis to the pancreas is quite rare, and a delay in its diagnosis may occur if the pancreatic tumor is not identified as a metastatic disease.A 51-year-old Caucasian woman complained of fatigue, epigastric discomfort, a left neck mass, and a 10-pound weight loss over the previous six months. Her physical examination revealed supraclavicular lymphadenopathy.A cervical lymph biopsy revealed moderately well-differentiated adenocarcinoma, possibly of pancreatic origin. A metastatic workup including positron emission tomography (PET) scan, computed tomography (CT), bone scan, and breast and pelvic ultrasounds was done. Significant cervical, retrosternal and retroperitoneal lymphadenopathy were seen. A 3.5 × 5-cm pancreatic head mass which blended into the porta hepatis was also noted. The mass encased the left gastric artery and involved the portal vein margins. Multiple liver lesions were also seen. Except for a fibroid uterus, her pelvic CT and ultrasound were unremarkable.A percutaneous liver biopsy of our patient revealed a moderately well-differentiated adenocarcinoma consistent with pancreatic origin. Her CA-19-9 level was 171.5.Our patient was then entered into a trial for advanced pancreatic adenocarcinoma using the tyrosine kinase inhibitor Dasatinib. Her response to the trial, however, was poor and a CT scan two months later showed no reduction in her tumor burden. She was removed from the Dasatinib trial and was started on Gemzar (gemcitabine) and Tarceva (erlotinib).Over the next seven months, our patient developed a moderate response to chemotherapy. However, she developed abdominal fullness and shortness of breath. In just a four-month interval between scans, her CT revealed a new 19 × 18 × 9 cm pelvic mass, ascites, and a large right-sided pleural effusion. A CT-guided biopsy revealed poorly differentiated adenocarcinoma. Metastatic malignant cells were also found in her pleural fluid.Because our patient experienced significant discomfort due to the effects of her pelvic mass, a palliative resection was performed. She had a transient response to chemotherapy, but her disease continued to progress with worsening ascites and pleural effusion.Pathology revealed that her pelvic mass measured 19.0 × 18.0 × 9.0 cm. Sectioning revealed a multi-loculated cystic mass involving her entire ovary Figure . The tumIn view of the presence of metastatic adenocarcinoma to the liver and a cervical lymph node Figure , as wellWhen her ovarian mass stained positive for CK7 but negative for CK20 and estrogen receptor, we repeated the stains on her liver and lymph node biospies, which proved to be a match to the ovarian tumor.The histologic and immunohistochemical findings of our patient's ovarian mass are consistent with the results of her lymph node and liver biopsies. We concluded that the mass was most likely from the ovary and not the pancreas, with the pancreatic mass representing another metastasis of her ovarian adenocarcinoma.Mucinous ovarian adenocarcinomas are uncommon and account for only 10% to 15% of reported cases of ovarian neoplasms . Tumors Often characterized by multiseptated cystic lesions of the adenexa, mucinous adenocarcinomas are filled with a gelatinous material that may freely rupture into the peritoneal cavity, thus causing a dissemination called "pseudomyxoma peritoni" . PseudomCarcinoma metastatic to the pancreas is uncommon and arises by direct extension from retroperitoneal or mesenteric lymph nodes or from isolated metastases to the pancreatic parenchyma . The mosDue to the low incidence of pancreatic metastasis, most masses of the pancreas are assumed to be primary pancreatic neoplasms. However, a tissue biopsy is required to truly differentiate between primary and secondary tumors . A delayDifferentiating primary from secondary pancreatic tumors is important in directing a patient's therapy, both in terms of chemotherapy and surgery. Whenever possible, the resection of pancreatic metastasis can be a reasonably safe palliative procedure . Distal Although most lesions of the pancreas are primary pancreatic neoplasms, a tissue biopsy should be obtained whenever possible to differentiate between primary and secondary tumors. Metastases of ovarian mucinous adenocarcinomas to the pancreas are rare, but have been reported in the literature. Confirmatory tissue biopsies, tumor markers, and being mindful of the possibility of metastatic disease can avoid misdiagnosis and delay in treatment for newly discovered pancreatic masses.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.DS researched the case and was a major contributor in writing the manuscript, particularly the case discussion. DC performed research, contributed in writing the case report, and edited the manuscript for its final version. MF performed the histological examination described in the case report and was a major contributor in writing the manuscript. GB was our patient's attending surgeon and provided information on our patient and contributed to the writing of the manuscript. JA was the primary care provider involved in the case, and similarly provided patient information and contributed in writing the manuscript. All authors read and approved the final manuscript.


The artery of Percheron is a solitary trunk representing an uncommon anatomic variant that provides bilateral arterial supply to the paramedian thalami and the rostral midbrain. Occlusion of this artery results in bilateral thalamic and mesencephalic infarctions. The clinical diagnosis is difficult because the complex anatomy causes large clinical variability. We report a case of a comatose patient with normal early head-computed tomography and magnetic resonance imaging. A bilateral paramedian thalamic infarct due to an occlusion of the artery of Percheron was revealed two days later by a new head computed tomography. To our knowledge, this is the first report in the literature of a symptomatic patient presenting an acute Percheron stroke with normal early brain magnetic resonance imaging. Our case indicates that a normal initial magnetic resonance imaging cannot formally eliminate the diagnosis of acute stroke of the artery of Percheron. We discuss the causes of noncontributive brain magnetic resonance imaging at the onset of this acute Percheron stroke and the alternative diagnosis and therapy methods. Percheron described four normal variations of the neurovascular anatomy of the thalami and midbrain . The medWe report a case of a patient admitted for coma without evident cause and normal early imaging by head computed tomography (CT) and MRI. A new head CT two days later revealed a bilateral paramedian thalamic infarct at the origin of the initial symptoms.-weighted fluid-attenuated inversion recovery (FLAIR) images , but with poor therapeutic observance. He was found comatose by the prehospital team. On examination, the patient had the following initial vital signs: temperature 36.4°C, pulse 78 beats/min, respiratory rate 16 breaths/min, and blood pressure 148/87 mmHg. On neurological examination, the Glasgow coma-scale score was 7 with localizing pain being the best motor response and no opening of eyes or verbal response. Pupillary light reflex in the right eye was 4 mm nonreactive and that in the left eye was 3 mm reactive. The deep tendon reflexes were present and symmetric. Babinski sign was found on both feet. Capillary blood glucose was found to be 123 mg/dL. The electrocardiogram showed atrial fibrillation, with no abnormal repolarization. He was intubated and mechanically ventilated and then brought to the emergency department. The initial head CT, performed 65 minutes after loss of consciousness, showed no acute hemorrhage or other abnormality. To eliminate an acute ischemic infarction, a brain MRI was then performed 95 minutes after the onset symptoms. It did not reveal significant acute infarction signals: no lesion was found on diffusion-weighted (DW) images , on appa) images . The T2*A new head CT was performed 48 hours later . It reveBithalamic paramedian infarcts are rare, and it is painful to suspect them because of the complex anatomy causing large clinical variability –7. The fSeveral reasons can explain the MRI results. The imaging was achieved with a head and neck antenna, which is probably less precise on this part of the brain compared with a head antenna. In addition, the brain MRI, performed 95 minutes after the onset of symptoms, was probably done earlier than in other reported cases. This demonstrates that modern neuroimaging is extremely valuable in making a positive diagnosis but normal imaging studies alone, however, do not exclude a vascular etiology. Small lesion size, brainstem lesions, small cortical lesions, and DW-imaging performed within a few hours after stroke onset are features associated with a particular risk of false-negative DW-images , 11. In In conclusion, our case indicates that a normal initial MRI cannot formally eliminate the diagnosis of acute stroke of the artery of Percheron, especially if performed very early or if the technical conditions are not optimal. These findings suggest the presentation of acute rostral brain stem stroke accompanied by a nonconclusive brain MRI should encourage to perform a new brain imaging by MRI within therapeutic times or should consider doing interventional explorations focused on the vertebrobasilar territory. On that basis, intravenous or in situ thrombolytic therapy should be performed. Interventional explorations focused on the vertebrobasilar territory have not been evaluated but may allow to confirm the diagnosis of Percheron artery lesion. However, only one successful case has been described. Anytime the initial imaging is normal, new brain imaging by MRI or CT scan should be performed within the first 48 hours.


The purpose of this article is to present a rare case of localized, solitary amyloid tumor of tongue base and emphasize some of the characteristic features of challenging clinical and histopathologic diagnosis. In this paper, we focused on the clinical and pathological specifications of this rare tumor, so any unnecessary examinations or measures may be spared. Negative staining of amyloid material with AAC and osseous metaplasia noted in the histopathologic examination may not be thought as definite criteria for localized amyloidosis, but a supporter of localized, solitary amyloid tumor diagnosis. Amyloid can affect any site in thehead and neck including the orbit, sinuses, oral cavity, salivary glands,pharynx, and larynx. Amyloid involvement of tongue is almost always secondaryto systemic amyloidosis and localized involvement is extremely rare , 2.The etiology, treatment, andoutcome of systemic amyloidosis are totally different from localizedamyloidosis. The mean survival of patients with systemic amyloidosis is between5 to 15 months, whereas patients with localized amyloidosis have excellentprognosis [To rule out a systemic amyloidosisfor these patients is extremely critical because this can markedly change theexpected morbidity and mortality . AbdominThe purpose of this article is topresent a rare case of localized, solitary amyloid tumor of tongue base andemphasize some of the characteristic features of challenging histopathologicdiagnosis.A 61-year-old male patientpresented with pyrosis of tongue and a nodular mass in tongue base. Head andneck examination revealed a 1 × 1 cm well-circumscribed, rubbery, nodular massat the right side of the tongue base. His review of systems was negative forsymptoms including fever and weight loss.A computed tomography was performedand a possible granulomatous or inflammatory mass with calcification wasreported . An inciRough and irregular mass, 2 cm in diameter, was dissected from surrounding normal tissue withminimal tissue loss and enbloc excision could be achieved via transcervical suprahyoid approach.Postoperative microscopic examination of the specimenrevealed a well-circumscribed submucosal lesion characterized withstructureless eosinophilic material deposition. Overlying mucosa was inflamed and focally eroded. The amorphous, more orless homogenous eosinophilic materialresembled amyloid histologically and on Congo red staining, exhibited the classical apple-greenbirefringence under polarizedlight. Deposits of amyloid were also found in and around the walls of smallblood vessels. Immunohistochemical examination of the matrix with monoclonalantibody AA-Congo (AAC) revealed a non-AA-type amyloid deposition . At theDiagnostic workup includingcomplete blood count, liver and renal function tests, urine analysis,esophagography, chest X-ray, electrocardiography, echocardiography, bone-marrowbiopsy, ESR, RF, ANA, and abdominal fat biopsy was carried out.All examinations for exclusion ofsystemic amyloidosis, including bone-marrow and abdominal fat biopsies, werefound to be negative for Congo red staining and amyloid deposition. Bone-marrowaspiration biopsy was found to be free of any type of infiltrations. Thus, thepatient was determined to be free of systemic amyloidosis and diagnosed aslocalized, solitary amyloid tumor of the tongue base.Annual examinations were performed inthe four-year follow-up period.The differential diagnosis of the mass in tongue base includesneoplastic processes and also lingual thyroid. Because the mass was settledunder normal appearing mucosa, a preoperative incisional biopsy was performedin order to rule out thyroid tissue and malignancy. We could not achieve adefinitive pathology with preoperative biopsy so we planned total excision ofthe mass.When amyloidosis is the histopathologic diagnosis in the patientspresenting with a localized mass in head and neck region, the main diagnosticdilemma of the surgeon becomes the extension of the disease. Many diagnosticexaminations were proposed to rule out a systemic disease. Histopathologicexaminations of these lesions may help clinicians about the necessity of thediagnostic examinations. Amyloid tumor of tongue base is a rare condition andmay not be predicted preoperatively, therefore there is little information onthe clinical and histopathologic features of the disease. In this paper, wefocused on the clinical and pathological specifications of this rare tumor, soany unnecessary examinations or measures may be spared and patient may beinformed clearly about the disease.Also, in the literature there is no consensus on terminology of thedisease. We did not prefer to use the term “localized amyloidosis of tonguebase” for this disease because diffuse involvement of tongue with systemicamyloidosis may be mistaken with well-circumscribed amyloid tumor. The terms“localized primary amyloid tumor” and “solitary amyloid tumor” were both usedfor localized amyloid deposits without systemic amyloidosis or multiple myelomain the literature, and better distinguish from the systemic form and betterdefines the disease. So we decided to use the term “solitary amyloid tumor” forthe diagnosis after ruling out systemic involvement of the disease because itbriefly underlines the nature of the disease.The definitive treatment oflocalized amyloidosis was cited to be surgery. Surgery alone may be 100%curative [Although, negative staining withAAC and osseous metaplasia which is noted in histopathological examinationsupports non-AA-type amyloidosis, we performed these examinations to supportour diagnosis. Negative staining of amyloid material with AAC is mostly seen inprimary type of amyloidosis. Also, osseous metaplasia noted in the histopathologicexamination may not be thought as definite criteria for localized amyloidosis, but a supporter oflocalized, solitary amyloid tumor diagnosis. There is data about osseousmetaplasia in the literature regarding the solitary amyloid tumor of other tissues, and to A submucosal, surgically well-defined,well-circumscribed, and rubbery mass in tongue base with calcifications onradiologic studies may not be surgically differentiated from other tumors oftongue. When histopathology reveals amyloid deposits on light microscopy, theseclinical findings may come out as important features in order to differentiatelocal disease from involvement of tongue by a systemic disease.With this little informationregarding the clinical and histopathologic features of localized, solitaryamyloid tumor of tongue base, we may not discuss the necessity of theexaminations in order to rule out systemic disease including abdominal fat andbone-marrow biopsy. Therefore, the publication of more cases or series of thedisease may yield better characterization of the histopathologic, radiologic,and clinical features of solitary amyloid tumor of tongue base.


Primary adenocarcinomas of the seminal vesicle (SVC) are very rare and poorly understood neoplasms with only somewhat more than 50 histologically confirmed cases reported in the literature. We demonstrate a case of SVC and discuss the problems related to diagnosis in this tumor. Primary adenocarcinomas of the seminal vesicle (SVC) are very rare and poorly understood neoplasms with only somewhat more than 50 histologically confirmed cases reported in the literature. We demonA 54-year-old man with dysuria and haematuria was first examined in January 2007. Laboratory parameters including serum prostate specific antigen (PSA) level were normal. Digital rectal examination detected a poorly defined hard tumoral mass on the site of the left seminal vesicle, under the intact rectal mucosa. This finding was confirmed by ultrasonography. Primary rectal tumor was ruled out by colonoscopy. Microscopic analysis of the colon biopsy showed infiltration of poorly differentiated adenocarcinoma restricted to submucosa with no evidence of tumor or dysplastic change in the mucosa.Contrast-enhanced magnetic resonance imaging was performed, which demonstrated a tumor mass in the area of left seminal vesicle, partly affecting the prostate and the anterior wall of the rectum . The patChest X ray and computed tomography (CT) of abdomen were also performed without showing any signs of malignancy. No previous diagnosis of malignant disease was registered in the patient's files. The clinical, radiological, histological, and immunohistological findings were consistent with primary SVC. The expert team of urological oncologists proposed radiotherapy before surgical intervention. However, the patient died of the disease 12 months after the histological diagnosis. Autopsy was not performed because relatives requested it.The tumor should be a microscopically verified carcinoma, localized exclusively or mainly to the seminal vesicle.The presence of other simultaneous primary carcinoma should be excluded.The tumor should preferably resemble the architecture of the non-neoplastic seminal vesicle.In 1956, Dalgaard and Giertson establisThese criteria can be readily applied to surgical resection specimens, but are less suitable in evaluating radiologically guided needle biopsies. Immunostains are helpful in these circumstances but vesicula-specific markers are not available.While primary SVCs are rare, secondary neoplastic involvement of the seminal vesicle is not so infrequent. Moreover, when clinical symptoms occur in a patient with SVC, they often reflect advanced disease with infiltration of adjacent organs obscuring the vesicular origin of the tumor. The most common symptoms are non-specific. These are hematuria and hematospermia, but dysuria; painful defecation and general pelvic pain have also been reported. CT scan,Histological examination is central in diagnosing SVC, despite the lack of organ-specific immunofenotype. Immunohistochemistry is rather helpful to exclude some metastatic tumors than to confirm the origin of the tumor in seminal vesicle . The com33According to the reported data in the literature, the immunohistohemical profile of SVC is not as consistent regarding other tumor markers as for PSA, CK7, and CK20.5 Carcino13In summary, the diagnosis of SVC is based on correlation of clinical, radiological, and histological findings. Immunohistochemistry may be helpful in ruling out some differential diagnostic options but cannot per se distinguish poorly differentiated SVC from metastatic spread of poorly differentiated carcinoma of prostate, certain adenocarcinomas of urinary bladder and from the rare carcinomas originating in müllerian duct cyst.


Lipomas are benign mesenchymal neoplasms composed of mature adipocytes, usually surrounded by a thin fibrous capsule. They are uncommon intra-oral tumors with 1% to 4% occurring in this region. The literature is scanty on lipomas occurring in the buccal soft tissue, especially in our environment.We present a case of a 35-year-old woman of the Tiv ethnic group of Nigeria who presented with a slow growing left cheek swelling that was treated by intra-oral local excision.The purpose of this report is to highlight the existence of this rare but not uncommon disease even in our environment and to emphasize that a high index of suspicion is needed in making a diagnosis. Surgical excision as treatment is associated with an excellent outcome. Lipomas are benign mesenchymal neoplasms composed of mature adipocytes, usually surrounded by a thin fibrous capsule . They arAdequate surgical excision in order to prevent recurrence is the treatment of choice ,5. We reAlthough an isolated case of buccal soft tissue fibrolipoma has been reported in our environment , this paA 35-year-old housewife of the Tiv ethnic group in Nigeria was referred to our Ear, Nose and Throat clinic by family physicians with a 6-year history of a slowly progressive, painless left cheek swelling not preceded by trauma and not associated with fever, weight loss or any other otorhinolaryngological symptoms. Examination revealed a 6 cm by 6 cm non-tender doughy mass in the left cheek with no overlying skin changes. Slipping sign was not demonstrable and there was no bruit over this mass. The intra-oral mucosa over the mass appeared normal. A provisional diagnosis of buccal soft tissue lipoma was made with epidermoid cyst as a differential diagnosis.Imaging using ultrasonography revealed a fairly well circumscribed echogenic mass in the left cheek measuring 1.67 cm by 1.23 cm with no evidence of neovascularization noted within. On this premise, the radiologist made an assessment of lipoma. A computerized tomographic scan was not done because the patient could not afford to pay for it. Other investigations performed included full blood count, serum urea and electrolyte, and urinalysis which were all within normal limits.She was prepared for and had excision under general anesthesia via naso-endotracheal intubation. During surgery, the mass was approached intra-orally by a transverse 5 cm linear incision made in the mucous lining over it Figure . The 4 cMicroscopic examination of the excised soft tissue mass revealed sheets of mature adipocytes containing large clear cytoplasms and eccentric nuclei with inconspicuous vascularity and no evidence of cellular atypia or metaplasia Figure . These fth postoperative day and has remained free of any symptoms for over 36 months of follow-up.Postoperatively, she was placed on ciprofloxacin, ibuprofen and vitamin C tablets with oral saline mouth wash after meals. She was discharged in good condition on the 5Lipomas are adipose mesenchymal neoplasms that rarely occur in the oral cavity with a 1% to 4% reported occurrence in this region ,4. The pIn the oral cavity, the most common sites are the cheek, tongue, palate, mandible and lip where lipomas occur as sessile or encapsulated masses . The etiThe classification for benign lipomas includes the following: classic lipoma; lipoma variants ; hamartomatous lesions; diffuse lipomatous proliferations and hibernoma .Oral lipomas are slow growing tumors and patients commonly present with a well circumscribed mass that has been growing for several years . Our patClinically, they present as soft and compressible masses with doughy consistency which are well defined clinically and radiologically using ultrasonography and computerized tomographic scan  and moreUnlike oral lipomas, lymphoepithelial cysts are found in the floor of the mouth, soft palate and mucosa of the pharyngeal tonsil . AlthougAdequate surgical excision is the treatment for oral lipomas ,5. The sMicroscopically, it is difficult to differentiate between normal adipose tissue and lipomas, therefore, a clinician sending a surgical specimen to the pathologist for microscopic analysis must provide accurate clinical and surgical information in order to make a definitive diagnosis . The micBuccal soft tissue lipomas are rare tumors. A high index of suspicion is required in making a diagnosis. Surgical excision is the ideal treatment with excellent outcome. The importance of histological diagnosis cannot be overemphasized and the features of lipoma are usually straightforward and classical.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.AAA was the principal surgeon, performed the literature search and prepared the manuscript. TLN assisted in the surgery and postoperative management of the patient. ANM interpreted the slides and reviewed the manuscript. GOE prepared the slides and the photomicrographs of the specimen.


The skull base surgery is one of the most demanding surgeries. There are different structures that can be injured easily, by operating in the skull base. It is very important for the neurosurgeon to choose the right approach in order to reach the lesion without harming the other intact structures. Due to the pioneering work of Cushing, Hirsch, Yasargil, Krause, Dandy and other dedicated neurosurgeons, it is possible to address the tumor and other lesions in the anterior, the mid-line and the posterior cranial base. With the transsphenoidal, the frontolateral, the pterional and the lateral suboccipital approach nearly every region of the skull base is exposable.In the current state many different skull base approaches are described for various neurosurgical diseases during the last 20 years. The selection of an approach may differ from country to country, e.g., in the United States orbitozygomaticotomy for special lesions of the anterior skull base or petrosectomy for clivus meningiomas, are found more frequently than in Europe.The reason for writing the review was the question: Are there keyhole approaches with which someone can deal with a vast variety of lesions in the neurosurgical field?In my opinion the different surgical approaches mentioned above cover almost 95% of all skull base tumors and lesions. In the following text these approaches will be described.These approaches are:1) pterional approach2) frontolateral approach3) transsphenoidal approach4) suboccipital lateral approachThese approaches can be extended and combined with each other. In the following we want to enhance this philosophy. In the 1970 s Yasargil  laid to There are many variants for the pterional approach. Mainly it is a trepanation which permits access to the frontal and to the temporal lobe as well as the Sylvian fissure Figure . After aYasargil also described a technique that allows access to the same area: his skin incision is done until the temporal fascia is reached. He then creates a triangular galea flap and seperates the muscle after additional horizontal incision in order to protect the frontal branch of the facial Nerve. In our opinion the rate of nerve injury is minimal also if one is using the first technique. The advantage of the Yasargil Technique can be seen if one wants to combine the pterional approach with the subfrontal approach. The muscle can be retracted much easier downwards with this technique. In my opinion the rate of atrophic changes of the temporal muscle postoperatively is a little bit higher. We are using Palacaos to fill the burr holes. Other authors are using selected bone dust.A 51-year-old man underwent an operation for a craniopharyngioma, through a pterional approach from the right side six months before. He was presented with a pituitary infarction. He has been treated with Testosterone (Testogel), Desmopressin (Minirin) and Hydrocortisone. He suffered from a progressive visual disturbance  for 8 weeks. A bitemporal hemianopsia was diagnosed.We diagnosed a recurrence of the craniopharyngioma Figure . Besides64-year-old woman presented with unknown symptoms of vertigo and diplopia. Further there were no clinical deficits. The MRI showed a left space-consuming lesion latero para- and retrosellar of approximately 2 × 2,5 cm size Figure .There were normal findings post-operatively with occasional diplopia, while the wound was healing per primam. Pathology revealed a meningothelial tumor of WHO grade I.66-year-old patient, presented with a history of six seizures, suffers from a known retrochiasmal lesion with a growth in the MRI examinations.We addressed the tumor via the pterional approach from the right side Figure . The chiThere were no neurological deficits and the healing of the wound was by first intention. In contrast the patient showed fluctuating vigilambulism with nausea, vertigo and electrolyte imbalance. The patient was prescribed a long-term hydrocortisone substitution and Desmopressin (Minirin) for a short period of time.The frontolateral or unilateral subfrontal approach provides exposure of the anterior cranial base. It allows addressing olfactory groove meningiomas (OGMs) by a minimally invasive procedure and it is also one of the traditionally used approaches on OGMs ,10. FranThis approach allows different skin incisions, which depend on the patient's anatomy and physiognomy. The osteoplastic trepanation above the pterion and above the temporal muscle follows a curved skin incision or an eyebrow skin incision  Figure . The treA 47-year-old patient with bitemporal hemianopsia and a 6-weeks history of progredient headache was presented. The patient manifested no further neurological deficits.Neuropathological findings revealed parts of an epithlial cyst inflammatory cell infiltration in connective tissue stroma. The MRI showed a cystic lesion above the pituitary gland Figure . We perfThe further course was without any complications and the wound healing was per primam.Visual field defects were on the decrease. Furthermore, we couldn't recognize any hormonal failure.The transsphenoidal route was first used by the Egyptians in order to remove the brain. The pioneering work of Harvey Cushing  and OskaThe classic technique begins with the patient in supine position. An x-ray machine is positioned laterally to control each step of intervention. In microsurgery the surgeon stands at the tip of the head whereas in pure endoscopic pituitary surgery the neurosurgeon stands at the shoulder of the patient. We would like to concentrate on the microsurgical approach in our review article. After incising the septal mucosa Figure  and reveThe posterior cranial base can be explored through the lateral suboccipital route. Processes in the posterior fossa such as acoustic neurinoma surgery can be carried out through the lateral suboccipital approach. The unilateral suboccipital approach was popularized by Woolsey (1903) and with great contributions by Krause (1905) ,22. AfteWe normally use a semi-sitting position for the suboccipital lateral route Figure . The opeAttention should be paid to the variable emissary veins, which lead to the sigmoid sinus. First the dura should be opened under microscopic magnification in a triangular shape in the region of the pars horizontalis to gain cerebrospinal fluid and in order to relax the cerebellum.Then the dura has to be opened near the sinus via a curved skin incision that connects with the previous dural opening.By applying the transsphenoidal, the unilateral subfrontal, pterional and lateral suboccipital approach, the surgeon can expose almost any skull base involvement. The diameters of exposure can be modified by combining different approaches. The introduction of microsurgical techniques in the 1960 s ,32 is poComplex pathologies at the skull base may need tailored approaches, which mainly have the base on one of the mentioned techniques. Extension of approaches can also be done in an interdisciplinary fashion with colleagues from other faculties like ENT or maxillofacical surgery, which is recommended by the author. While ENT can help accessing the skull base, e.g., in acoustic neuroma surgery  or by thThe authors declare that they have no competing interests.MS obtained clinical photos, coordinated, and drafted the manuscript. RP drafted the manuscript and participated in its coordination and design. JT, CL, AH and KB provided critical review of the manuscript for important intellectual content. All authors read and approved the final manuscript.A written informed consent was obtained from the patients for publication of the case reports and accompanying images. Copies of the written consents are available for review by the Editor-in-Chief of this journal.


Perivascular epithelioid cell tumor (PEComa), other than angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangioleiomyomatosis (LAM), is a very rare mesenchymal tumor with an unpredictable natural history. The uterus is the most prevalent reported site of involvement of PEComa-not otherwise specified (PEComa-NOS). To the best of our knowledge, about 100 PEComa-NOS have been reported in the English Language medical literature, of which 38 were uterine PEComa-NOS. These reported cases of uterine PEComa-NOS have usually shown clinically benign behavior, but 13 tumors, three of them associated with tuberous sclerosis complex (TSC), exhibited local aggressive behavior and four of them showed distant metastases.We report the case of a 59-year-old woman, who presented with renal and pulmonary lesions seven years after the initial diagnosis of uterine leiomyosarcoma. Left nephrectomy and right middle lobe wedge resection were performed. Histological and immunohistochemical analysis of the renal and pulmonary lesions, in addition to retrospective re-evaluation of the previous uterine tumor, led to the final diagnosis of malignant uterine PEComa with late renal and pulmonary metastases. All three lesions had the typical histological appearance of PEComa-NOS showing a biphasic growth pattern with continuous transition between spindle cells and epithelioid cells, often arranged around vascular spaces. Immunohistochemically, the tumor cells of both phenotypes in all three lesions stained for melanocytic (HMB-45 and Melan-A/MART-1) and myoid  markers.The findings indicate that despite the small number of reported cases, PEComas-NOS should be considered tumors of uncertain malignant potential, and metastases to other organs might become evident even several years after the primary diagnosis. The World Health Organization defines perivascular epithelioid cell tumors (PEComas) as "mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells (PECs)" . In 1991To the best of our knowledge, about 100 PEComas-NOS have been reported in the English Language medical literature, of which 38 were uterine PEComas-NOS -17. ThesA 59-year-old woman with a past medical history of hysterectomy and bilateral salpingo-ophrectomy for uterine leiomyosarcoma  seven years prior presented to our institution for her regular periodic computed tomography (CT) scans of the abdomen, chest and pelvis at 6-month intervals as tumor follow-up investigations. These CT scans revealed several coalescing rim-enhancing nodules  in the upper and middle poles of her left kidney and a solitary rim-enhancing nodule (measuring 1.5 cm) in her right middle lobe of lung, which were absent on her previous CT scans six months earlier. The clinical impression was metastasis from her previous uterine leiomyosarcoma. No additional nodules were identified in the brain, gastrointestinal (GI) tract, liver, spleen, pancreas, bladder and bones on subsequent staging magnetic resonance imaging (MRI) of the brain, abdomen, chest and pelvis, and positron emission tomography (PET) scan. The patient did not have any stigmata or family history of TSC, and had no history of melanoma. General physical examination was unremarkable. Her hemogram, urine and blood biochemical analyses were within normal ranges.A left radical nephrectomy and video-assisted thoracoscopic surgical wedge resection of the right middle lobe solitary nodule were performed. Grossly, her resected left kidney revealed a sharply demarcated and lobulated firm gray-tan nodular tumor (7.5 × 6.2 × 5.4 cm) occupying the upper and middle poles, and showed focally hemorrhagic areas on cut surface Figure . The intHistologically, the renal tumor was biphasic and composed mainly of medium to large epithelioid cells and focal areas of spindle/elongated cells around numerous small blood vessels Figures . The medMicroscopic examination of the pulmonary tumor (not shown) exhibited the same morphological features as the renal tumor described above Figures . The intImmunohistochemically, both the epithelioid and spindle neoplastic cells of the renal tumor were strong and diffusely (>80% of the neoplastic cells) positive for desmin (cytoplasmic and membranous) {Ventana, Tucson, Ariz} Figure , HMB-45 On the basis of the above histopathological and immunohistochemical features, a definitive diagnosis of malignant uterine PEComa with late renal and pulmonary metastases was rendered. The patient elected not to have any adjuvant chemotherapy or radiotherapy. Five follow-up CT scans of the brain, abdomen, chest and pelvis, and PET scans performed at 3-month intervals after surgery revealed no nodules. She is alive with no evidence of local recurrence or distant metastasis after 15 months of follow-up, and scheduled to have regular periodic CT scans of the abdomen, chest and pelvis at 3-month intervals as her tumor follow-up plan.In this report, we describe a case of unusual epithelioid tumors in the kidney and the lung seven years after the initial diagnosis of uterine leiomyosarcoma, with pathological and immunohistochemical features entirely compatible with those of a PEComa. PEComas are characterized by epithelioid to spindle cells with eosinophilic to clear cytoplasm, an intimate relationship with blood vessels, and demonstrate positive immunostaining for markers of both melanocytic  and myoid  differentiation . PEComasPEComas are of interest primarily because of their immunoreactivity with melanocytic and myoid markers. They are also almost always negative for S-100 protein and cytokeratins. Folpe and colleagues  recentlyNot unexpectedly, the uterine tumor of the case herein presented was initially misdiagnosed as a uterine leiomyosarcoma at an outside institution seven years prior. Myomelanocytic marker expression was found to be prominent in both components of all the three lesions in the case herein presented. Controversy exists regarding the minimum criteria for the diagnosis of malignant PEComa ,19. In sIn addition to epithelioid smooth muscle tumors (epithelioid leiomyosarcoma and epithelioid leiomyoma), the other important differential diagnosis of PEComa include malignant melanoma, clear cell sarcoma of tendon and aponeuroeses (melanoma of the soft parts), alveolar soft part sarcoma, endometrial stromal sarcoma with clear cell features, uterine tumor resembling sex cord tumor, carcinoma , paraganglioma, angiomyolipoma, and any other tumor with focal or prominent clear cell change. Malignant melanoma and clear cell sarcoma of tendon and aponeuroeses can be differentiated from PEComas based on S-100 positivity; however, up to 11% of PEComas express S-100 as well . The addClinically, most PEComas follow a benign course . MalignaOptimal treatment for PEComas is not well established at this time. Currently, surgery is the mainstay of treatment for primary PEComa at presentation as well as for local recurrences and metastases, with the aim of obtaining clear resection margins. The role of adjuvant therapy remains unclear. Metastases have been successfully managed by resection alone . PrimaryWe have described a case of malignant PEComa-NOS of the uterus with late renal and pulmonary metastases seven years after hysterectomy. Malignant PEComa should be considered in the light microscopic differential diagnosis for all pleomorphic sarcomas. PEComas-NOS show a marked female predominance, are rare but anatomically ubiquitous mesenchymal tumors that are composed of nests and sheets of usually epithelioid but occasionally spindled cells with clear to granular eosinophilic cytoplasm and a focal association with vascular spaces. They usually show immunoreactivity for both melanocytic (HMB-45 and/or Melan-A/Mart-1) and myoid/muscle-specific (actin and/or desmin) markers, which are useful for confirming the diagnosis. The mainstay of treatment is wide excision. Though most PEComas are benign, a subset behaves in a malignant fashion; size >8 cm, high mitotic index, presence of necrosis, marked cytological atypia, and/or infiltrative growth pattern may be associated with malignant behavior. However, since relatively few malignant PEComas have been reported, firm criteria for malignancy have yet to be established and the identity of the normal PEC remains elusive. Further studies on additional cases with longer clinical follow-up would be necessary to accurately predict the biologic behavior of these distinctive tumors. Finally, because PEComas can behave in an aggressive manner, careful follow up is warranted.AML, angiomyolipoma;CCMMT, clear cell myomelanocytic tumour;CCST, clear cell sugar tumour;LAM, lymphangiomyomatosis;PEC, perivascular epithelioid cell;PEComa, perivascular epithelioid cell tumor;TSC, tuberous sclerosis complexThe author(s) declare that they have no competing interests.HBA participated in the histopathological evaluation, performed the literature review, acquired the photomicrographs and drafted the manuscript. AVP conceived and designed the study, gave the final histopathological diagnosis and revised the manuscript for important intellectual content. Both authors read and approved the final manuscript.


Diverticular disease of the colon is a common benign condition. The majority of patients with diverticular disease are asymptomatic and are managed non-operatively, however complications such as perforation, bleeding, fistulation and stricture formation can necessitate surgical intervention. A giant colonic diverticulum is defined as a diverticulum larger than 4 cm in diameter. Despite the increasing incidence of colonic diverticular disease, giant colonic diverticula remain a rare clinical entity.This is the first reported case of laparoscopic-assisted resection of a giant colonic diverticulum. We discuss the symptoms and signs of this rare complication of diverticular disease and suggest investigations and management. Reflecting on this case and those reported in the literature to date, we highlight potential diagnostic difficulties and consider the differential diagnosis of intra-abdominal gas-filled cysts.The presence of a giant colonic diverticulum carries substantial risk of complications. Diagnosis is based on history and examination supported by abdominal X-ray and computed tomography findings. In view of the chronic course of symptoms and potential for complications, elective surgical removal is recommended. Colonic resection is the treatment of choice for this condition and, where possible, should be performed laparoscopically. Diverticular disease of the colon is a common benign condition that occurs in excess of 60% in those aged over 70 years ,2. It isAbout 5% of patients who have symptomatic diverticula experience complications such as perforation, bleeding, fistulation and stricture formation which can necessitate surgical intervention.A giant colonic diverticulum (GCD) is defined as a diverticulum larger than 4 cm in diameter . The meaThe presentation of GCD is variable, ranging from the asymptomatic patient 4%) to a host of non-specific gastrointestinal (GI) symptoms with only 10% of patients presenting with an abdominal mass % to a ho.Despite the increasing incidence of colonic diverticular disease, GCD remains a rare clinical entity . We repoA 53-year-old white Italian man initially presented to gastroenterologists with a 5-week history of dyspepsia, epigastric pain and a palpable mass in the left hypochrondrium. There was no history of anorexia, dysphagia, weight loss, change in bowel habit or gastrointestinal blood loss. His past medical history included early Alzheimer’s disease and discoid lupus.Examination revealed a well circumscribed, mobile mass in the left hypochrondrium extending above the level of the ribs raising the possibility of an enlarged spleen. There was no palpable lymphadenopathy.A blood film showed atypical myelomonocytic cells but a subsequent bone marrow aspiration was normal. All other routine blood tests were within normal limits. An abdominal ultrasound scan demonstrated a normal spleen and a separate gas-filled cyst in the left hypochondrium.Over the next few weeks, the patient developed diarrhoea and lost 3 kg in weight. He reported that the mass appeared to be fluctuating in size.An abdomen computed tomography (CT) scan  demonstrHe was referred to colorectal surgeons and a barium enema was performed to further assess the extent of the diverticular disease. This confirmed moderate sigmoid diverticulosis but did not demonstrate direct communication between the colon and the giant cyst . The diaA laparoscopic-assisted sigmoid colectomy was performed 6 weeks later. Four 12 mm ports were inserted and pneumoperitoneum achieved. Three of the ports were positioned along the lateral edge of the right rectus abdominus muscle and triangulated to provide optimum access to the left colon. The fourth port was in the left iliac fossa. The large cystic structure was clearly visible in the left hypochondrium at the apex of a long mobile loop of sigmoid colon on the anti-mesenteric border . The remThe patient made an excellent postoperative recovery and was discharged on the fourth postoperative day.et al. [Macroscopic assessment of the segmental colonic resection confirmed the presence of diverticular disease with an associated giant cyst measuring 11 cm in maximal diameter. The wall of the cyst measured 0.6 to 1 cm in thickness. Microscopically, it did not contain any elements of bowel wall and instead was composed of reactive scar tissue with foreign body type giant cell reaction. The presence of plant material admixed with inflammatory debris was thought to be indicative of faecal matter and suggested a direct communication between the cyst and bowel lumen. However, this was not identified histologically. There was no evidence of dysplasia or malignancy. In accordance with the classification suggested by Steenvoorde et al. , the histh century [Diverticular disease of the colon is a significant cause of morbidity and mortality in the western world and its frequency increased throughout the whole of the 20 century ,8. Since century ,8.GCDs are defined as those that are larger than 4 cm in diameter  and withAs in our patient, it is not unusual for these patients to undergo multiple investigations before making the correct diagnosis. Plain supine abdominal X-ray is the simplest and most readily available investigation and should be used as the first line in suspected cases. If a large air filled structure with or without fluid levels is visualised then an abdominal CT scan would be indicated. Barium enema failed to demonstrate a communication between the giant diverticulum and the colon in approximately one-third of reported cases ,4. It isThe use of abdominal ultrasound has been reported to be helpful in only 25% of cases . Early cThe role of colonoscopy in diagnosing GCD is limited. The ostium between the diverticulum and the colon is frequently too small to be detected ,2,4 and et al. suggested a histological classification of GCD based on three subtypes  to a host of gastrointestinal symptoms including abdominal pain (68%), constipation (18%), rectal bleeding (13%), vomiting (12%), abdominal distension (11%), diarrhoea (11%) and abdominal mass (10%) [% to a hoThe presence of a GCD carries substantial risk of complications 12% to 19%) including inflammation, perforation, abscess formation, fistula formation, urinary obstruction , volvulu2% to 19%In view of the chronic course of symptoms and potential for complications, elective surgical removal is recommended . Colonic


Testicular tumors can be classified as seminomatous and non-seminomatous germ-cell tumor (NSGCT) types. Mixed germ cell tumors contain more than one germ cell component and are much more common than any of the pure histologic forms representing 32%-60% of all germ cell tumors. The composition of these tumors varies. Here we present a rare case of a mixed germ cell tumor composed of seminoma, Yolk sack tumor and teratoma containing a sarcoma component of somatic type malignancy.A 32-year-old Caucasian male presented with history of right-sided scrotal swelling since 6 months. Backache was present since 2 months and a history of right epididimitis was also present since 8 months. Alpha-Fetoprotein, beta-HCG and LDH values were found abmormal. USG of the scrotum revealed a large right testis swelling characterized by scarce cystic elements and calcifications. CT scan of the abdomen showed nodular metastasis involving the interaortocaval, precaval, and right para-aortic lymph nodes. The block of enlarged lymph nodes infiltrated the psoas muscle. The patient underwent right-sided high orchidectomy and was given chemotherapy of the BEP regimen. After the 2nd cycle the patient discontinued the chemotherapy and when he came for follow-up after a gap of 3 months, despite the normalisation in tumor markers values, the retroperitoneal mass was relapsed. CT scan of the chest showed multiple lung metastases.More than 50% of germ-cell tumors include more than 2 basic germ-cell tumor types, with the exception of spermatocytic seminoma. About 90% of the patients with nonseminomatous tumors can achieve complete cure with aggressive chemotherapy and most of them can be cured. Although prognosis of testicular tumors depends largely on clinical stage, histological type and adhesion to the treatment influence the prognosis as well. Testicular cancer is a respectively rare neoplasm; It make up approximately two percent of all malignant cancers in men and account for up to ten percent of all malignant disease occurring within the male genitourinary system. Most of these tumors occur in three age groups - infancy, late adolescence and early adulthood. More importantly, testis tumors are the most common malignant disease, developing in men between 20 and 40 years of age and are the third leading cause of death among men of this age group .Pathologically, testis cancers are divided into two classes; germ cell tumors which are derived from germinal epithelium and non-germinal tumors which are of gonadal stroma origin. Tumors of germ cell origin comprise about 95% of all testis cancer. Germ cell tumors are divided into two basic groups: seminomas which occur in approximately 40% of the population and non-seminomatous tumors (NSGC) which may be seen in pure or mixed form .NSGCs are further divided into the following five groups:1) embryonal carcinoma with or without seminoma, which occurs in about 25% of the group;2) teratoma with or without seminoma, which occurs in about 7% of the group;3) teratocarcinoma including teratoma with embryonal carcinoma, choriocarcinoma, or both with or without seminoma occurring in about 25% of the group;4) choriocarcinoma with or without seminoma or embryonal carcinoma or both account for the remaining 1-3%.Mixed germ cell tumors contain more than one germ cell component and are much more common than any of the pure histologic forms representing 32%-60% of all germ cell tumors. Essentially, any admixture of the germ cell tumors as seen in pure form may be seen, one of the most common admixtures being embryonal carcinoma and teratoma [A 32-year-old Caucasian male presented with history of right-sided scrotal swelling since 6 months. According to the patient, it was a right -sided painless swelling which had progressively increased in size to its present dimensions. There was heaviness in the right side of the scrotum. There was no skin involvement. Backache was present since 2 months, more on walking and on straining. A history of right epididimitis since 8 months was also present.The patient was averagely built and nourished. Vital parameters were stable. The abdomen was soft on palpation with no obvious organomegaly. The chest was clear on auscultation. On local examination, a large right -sided scrotal swelling was found. The scrotal skin was normal. No scar, sinuses or dilated veins were seen. On palpation, local temperature was normal and testicular sensations were absent on the affected side. It was a painless, non tender, non transilluminant swelling with variegated consistency. Per-abdominal examination did not reveal any abnormality. Virchow's nodes were negative.Hematocrit was 42,5%, WBC:7100, PLT:213000, Glu:92, Ur:41 mg/dL, Cr:0,9 mg/dL, CPK:132.Tumor markers: Alpha-Fetoprotein (AFP):2628 ng/ml, Beta-HCG:6,96 IU/ml, LDH:979USG of the scrotum revealed a large right testis swelling of 45 × 37 × 53 mm. The eco architecture of the affected testis was promiscuous characterized by scarce cystic elements and calcifications. No dilated veins were seen.USG of the abdomen showed a large right retroperitoneal mass corresponding to para-aortic lymph node block.CT scan of the abdomen showed nodular metastasis involving the interaortocaval, precaval, and right paraaortic lymph nodes. The block of enlarged lymph node was filling almost the entire right retroperitoneal space infiltrating the psoas muscle figures  and 2. LBoth Chest x-Ray and CT scan of the chest showed no lymphadenopathy.The patient underwent right-sided high orchidectomy.On gross pathological examination, there was a, solitary, non encapsulated tumor measuring 4 × 3 × 26 cm located mainly to the originating area of the spermatic cord. The tumor was hard in palpation. On cut section, it had a grating feel and hoary colour. There were areas of hemorrhage and necrosis with yellowish discoloration. Histopathology showed a mixed germ-cell tumor with predominant yolk sac tumor 45%) containing areas of necrosis  having an 80% possibility of complete cure. However, the histologic subtype appears to critically influence the prognosis: It is the choriocarcinoma component which is the most aggressive as it spreads rapidly through the hematogenous route, thus leading to a poorer prognosis of patients carrying mixed NSGCs as compared to seminomas . The preIn our case, this unusual combination not commonly reported in the literature (yolk sac tumor and immature teratomatous element consisted of rhabdomyosarcoma), appears to have influenced the prognosis.Another important factor significantly contributing to the definite cure is completion of treatment and regular follow-up of the patient. As demonstrated in our case, any break in the treatment can and will lead to flaring up of the complications and carries very poor prognosis.More than 50% of germ-cell tumors include more than 2 basic germ-cell tumor types, with the exception of spermatocytic seminoma. About 90% of the patients with nonseminomatous tumors can achieve complete cure with aggressive chemotherapy and most of them can be cured. Although prognosis of testicular tumors depends largely on clinical stage, histological type and adhesion to the treatment influence the prognosis as well.The authors would like to thank the patient's relatives for providing informed consent for the publication of this case report.The authors declare that they have no competing interests.SK, PP and GP were involved in the case directly. SK drafted the manuscript. NG and MO took part in the care of the patient PP contributed in the preparation of the manuscript. TV contributed in carrying out the medical literature search. SK was involved in conception of the article and revising it critically for important intellectual data before final approval. All authors reviewed the final drafting of this manuscript.


Intramuscular hemangiomas are uncommon neoplasm's arising most frequently in the masseter and trapezius muscle. Due to it's location it is often mistaken for a parotid swelling and rarely is an accurate pre-operative diagnosis achieved clinically. The intra masseteric location also poses special problem in terms of proximity to the facial nerve and the post operative flattening following excision of the masseter muscle. A case of intramuscular hemangioma in a 17 year old girl is presented. Inadequacy of computed tomography scan and cytology in achieving a pre-operative diagnosis and also the treatment modalities are reviewed here. An estrogen receptor and progesterone receptor study has been done to verify the hormonal basis of this tumour. A 17 year old girl from India, Asia presented with a swelling in the right cheek of five months duration. She first noticed the swelling while washing her face. Swelling gradually increased in size, becoming more pronounced during mastication and while waking up in morning. Over the last one month she developed pain over the swelling. She gave no history of trauma or oral contraceptive pill usage. On physical examination there was a swelling in the region of the right parotid measuring 3 x 2 cm which was non-tender. Swelling was 3 cm in front of the tragus and 2 cm below the zygoma. There was no compressibility and the overlying skin was normal. On clenching the masseter the swelling diminished in size. There was no facial nerve involvement and parotid duct orifice was normal.FNAC revealed greenish color aspirate, cytology of which did not reveal any cellular material, this probably was extravasated blood and the greenish tinge, due to breakdown products of hemoglobin. This was evident as areas of hemorrhage on histology. A contrast CT was done  which shHemangiomas of skeletal muscle represent 0.8% of all benign vascular neoplasm . Of thesAllen & Enzinger classified them as large vessel [>140 mm in diameter] small vessel [<140 mm in diameter] and mixed vessel types . They coThese tumours present as gradually enlarging mass lesions with duration often less than a year . AccuratA variety of tumours can be confused clinically with an IMH. Most of them are often mistaken for salivary neoplasms & the differential diagnosis include cysts, lymphangiomas, rhabdomyosarcomas, masseteric hypertrophy, and schwannomas.FNAC is inconclusive in arriving at a diagnosis as it yields only a bloody aspirate . SuperseThough contrast CT may demonstrate the vascular nature of the tumour MRI has shown superiority in the exquisite delineation and contrast of the lesion from it's surrounding due to its multiplanar capability.The management of IMH should be individualized based on such factors as tumour location, age, depth of invasion, Cosmesis. Many treatment modalities like cryotherapy, radiation therapy, steroid administration and embolization have been advocated but the treatment of choice at present remains surgical excision ,2,9. LocThe preauricular incision combined with a superficial parotidectomy allows for a complete excision with preservation of branches of the facial nerve with very little cosmetic and functional disability. Intraoral approaches give limited exposure to the facial nerve branches and often result in nerve injury .Any lesion in the region of the parotid must be evaluated thoroughly prior to surgery. If the FNAC shows a bloody aspirate on repeat sessions, the possibility of a vascular lesion must be thought of. MRI should be the next line of investigation. Arteriography is usually not necessary. As proximal vascular control can be achieved through the same incision, total masseteric excision has very little cosmetic or functional disability and offers a better chance of clearance.


There is very small occurrence of adenocarcinoma in the small bowel. We present a case of primary duodenal adenocarcinoma and discuss the findings of the case diagnostic modalities, current knowledge on the molecular biology behind small bowel neoplasms and treatment options.The patient had a history of iron deficiency anemia and occult bleeding with extensive workup consisting of upper endoscopy, colonoscopy, capsule endoscopy, upper gastrointestinal series with small bowel follow through and push enteroscopy. Due to persistent abdominal pain and iron deficiency anemia the patient underwent push enteroscopy which revealed adenocarcinoma of the duodenum. The patient underwent en-bloc duodenectomy which revealed T3N1M0 adenocarcinoma of the 4th portion of the duodenum.Primary duodenal carcinoma, although rare should be considered in the differential diagnosis of occult gastrointestinal bleeding when evaluation of the lower and upper GI tract is unremarkable. We discuss the current evaluation and management of this small bowel neoplasm. DietPrimary SBNs are much rarer than those that arise from a secondary neoplastic process . MetastaThe mean age of presentation of SBN is 64 with a range of 47-87 years ,19. Obscrd and 4th portions of the organ are often missed on barium x-ray examination [SBN are usually discovered during the evaluation of OGIB, anemia, and abdominal pain. Abdominal X-ray may help in showing obstruction, however duodenal carcinomas especially those in the 3mination  yieldingmination . Colonosmination . Likewismination .The utilization of CT enterocolysis (CTE) in the detection of SBN overcomes the individual short comings of both barium enterocolysis and conventional CT and utilizes the advantages of both into a single technique and has begun to substitute enterocolysis in clinical practice . Contraset al. found that upper GI endoscopy had a 36% false-negative result rate in identifying duodenal tumors due to depth of insertion. Push enteroscopy (PE) provides many benefits including direct visualization of lesions in the proximal duodenum and jejunum, allowing the ability to biopsy and provide therapeutic measures in cases of bleeding. The investigation of obscure bleeding by PE may find a diagnostic cause in 25-28% of cases [Tocchi of cases . PE and of cases . Howeverof cases .Capsule endoscopy (CE) has been shown to be a safe and effective non invasive method of diagnosis for small bowel abnormalities ,33 and aIt has been shown that CE diagnosed SBN in 9% of patients who underwent the procedure for investigation of OGIB and in 8.3% of those who were investigated for non bleeding causes . HoweverBalloon assisted enteroscopy (BAE) utilizing either single balloon enteroscopy (SBE) or double balloon enteroscopy (DBE) offers a number of advantages when compared to other small bowel imaging studies. The advantages include visualization of the entire small bowel with the ability to provide tissue diagnosis and provide therapeutic modalities such as control of bleeding and dilation of strictures ,43. OptiThe failure of BAE to show superiority over CE in the detection of lesions may be due to complete evaluation of the entire small bowel in only 60-70% of cases ,49. A diDuodenal AC has a shorter median overall survival rate compared with patients with tumors located in the jejunum or ileum . SBNs arHistorically treatment of SBN has relied solely upon surgery as the only curative treatment and has been divided between two techniques which are pancreatoduodenectomy (PD) and duodenal segmentectomy (DS). PD is considered to be the procedure of choice. DS, is used for more palliative measures . StudiesChemotherapy is mainly utilized as a palliative measure and has not been well studied due to the low prevalence of AC in the small bowel. The largest published study investigating chemotherapeutic measures for small bowel AC involved 14 subjects with metastatic small bowel AC and involved a chemotherapeutic regiment containing 5-fluorouracil (5-FU) . Patientras gene [ras and p53 gene mutations are similar in both [Due to the rarity of SBN, little has been published about oncogenesis as well as clinicopathologic features . An analras gene  similar ras gene , and tha in both .In terms of the APC gene, SBN have a lower rate of mutations involving the APC gene compared to its involvement in CRC . DuodenaA 66 year old African-American female presented with complaints of 10 pound weight loss and a four week history of intermittent abdominal pain, nausea, and non bloody emesis. Her medical history was significant for type 2 diabetes mellitus, hypertension, coronary heart disease, and peptic ulcer disease. The patient denied any significant alcohol or tobacco use. Her family history was positive for colon cancer. Physical exam and laboratory tests were unremarkable. A colonoscopy and an esophagogastroduodenosopy (EGD) to the second portion of the duodenum were performed revealing three small tubular adenomas of the colon and helicobacter pylori gastritis. She was treated with two weeks of amoxicillin 1 gram and clarithromycin 500 mg orally twice daily for two weeks.H. pylori. A CT scan of the abdomen and pelvis with oral contrast was unremarkable. An outpatient wireless capsule endoscopy was ordered; however, it was cancelled due to the reluctances of the patient to swallow the capsule.She did well after the antibiotic therapy and was not seen again until two years later when she was hospitalized for severe, symptomatic anemia. For eight months prior to admission, she noted recurrent intermittent abdominal pain and nausea with non-bloody emesis and progressive fatigue. She denied melena or hematochezia. On admission, her hemoglobin was 5.4 gm/dl and hematocrit was 17.3%, with a normal MCV. Her stool was hemoccult positive. She required a transfusion of four units of packed red blood cells. An EGD to the second portion of the duodenum revealed mild gastritis, negative for Patient again required hospitalization for severe symptomatic anemia with hemoglobin of 5 gm/dl. Her indices and iron studies at this time were consistent with iron deficiency anemia. She denied melena, hematochezia or bloody emesis. She required another 4 unit blood transfusion. An EGD was performed and again it was unremarkable. Due to her inability to swallow the wireless capsule, the endoscope was used to deliver the capsule into the stomach. The study however was limited due to retained debris in the mid duodenum, significantly limiting visualization of the small bowel.Patient presented three months later with symptomatic anemia, hemoglobin of 4.6 gm/dl and hematocrit of 14.2%. She required four units of packed red blood cells. Two days prior to admission, she noted black tarry stools. Physical exam was unremarkable with the exception of palpable tenderness in the epigastric and left upper quadrant.Three way abdominal x-ray of the abdomen was performed and was unremarkable. MRA with and without contrast and CT scan of abdomen and pelvis with oral contrast showed no evidence of localized abnormality in the abdomen or pelvis in terms of solid organs or vasculature. An upper GI series with KUB was performed revealing eccentric broadband defect along the inner or medial wall of the 2nd portion of the duodenum, with the other portions being unremarkable.Push enteroscopy was performed which revealed a circumferential fungating mass in the 4th portion of the duodenum, which was actively oozing blood and upon further investigation it appeared the mass extended to the ligament of Treitz Figure . The arePathology from surgical specimen revealed T3N1M0 AC, with the tumor being 4.5 centimeters in greatest dimension and showing invasion through the muscularis propria and into the sub-serosa but not through it. The resected margins were clear. Of the two lymph nodes collected one was positive for metastatic carcinoma, with the tumor nodule measuring 1.5 centimeters in diameter and showing invasion through the lymphatic capsule.Patient was referred to oncology for consultation however did not follow up as scheduled, and has been lost to follow up care.In patients presenting with OGIB, iron deficiency anemia or other warning signs and symptoms SBN, should be considered in the differential due to its insidious presentation. In terms of oncogenesis more research is needed in order to better understand its development, but evidence suggests a multi-factorial genetic cause. Options in the evaluation of small bowel pathology may require CE, BAE, and/or CTE. An initial approach may be with CE or CTE due to the fact it is non invasive with subsequent utilization of BAE if the evaluation is unrevealing or if lesions are detected that require tissue confirmation. Surgery is the best curative option in terms of treatment of these types of malignancies with PD being better for advanced diseases and DS for disease of the distal duodenum. Chemotherapeutic options are improving and providing longer survival rates and palliative benefits.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.GI: Gastrointestinal; SBN: Small bowel neoplasms; NCDB: National Cancer Data Base; SEER: Surveillance Epidemiology End Results; AC: Adenocarcinoma; HNPCC: hereditary nonpolyposis colorectal cancer; FAP: familial adenomatous polyposis; OGIB: Obscure GI bleeding; CRC: Colorectal cancer; CTE: CT enterocolysis; PE: Push enteroscopy; CE: Capsule endoscopy; BAE: Balloon assisted enteroscopy; SBE: Single Balloon enteroscopy; DBE: Double Balloon enteroscopy; PD: pancreatoduodenectomy; DS: duodenal segmentectomy; 5-FU: 5-fluorouracil; MMR: Mismatch repair; MCR: mutation cluster region; EGD: esophagogastroduodenosopyThe authors declare that they have no competing interests.FAM, JEP, TD, and AD contributed directly and equally to patient care; PTK, SG, AGG, and JEP contributed to literature research and analysis of the data; All authors contributed in the writing and critical development of the manuscript. All authors have read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/10/109/prepub


Gall bladder carcinoma is rare, and metastatic gall bladder carcinoma from hepatocellular carcinoma has been reported in only a few patients.We present a 73-year-old man with a history of hepatitis B virus-related liver cirrhosis and hepatocellular carcinoma. He received transcatheter arterial chemoembolization, and was diagnosed to have an alpha-fetoprotein producing gall bladder tumor with intraluminal growth. Open cholecystectomy was performed. Pathologic examination of the lesion revealed a well-differentiated hepatoid carcinoma. The lesion was thought most likely to be a metastatic lesion from previous hepatocellular carcinoma. His alpha-fetoprotein level dropped to normal levels five months after the surgery.This unusual intraluminal growing tumor proved to be a well-differentiated hepatoid carcinoma, most likely a metastatic lesion from previous hepatocellular carcinoma. This case reminds clinicians that in looking for likely hepatocellular carcinoma recurrence, when no detectable hepatic lesion can account for an elevated alpha-fetoprotein level, the gall bladder should be included in the search for the site of metastasis. Gall bladder carcinoma is a rare disease, whether primary or metastatic. It is usually diagnosed at a late stage due to asymptomatic properties when small. Sometimes carcinoma from the gall bladder also produces alpha-fetoprotein (α-FP) because the gall bladder shares the same embryologic origin as the liver. Only a handful of cases of α-FP producing gall bladder carcinoma had been reported in the literature, and many of them have been from Japan. Cell types of these reported cases include clear cell carcinoma, pleomorphic carcinoma or undifferentiated carcinoma. Here we present a rare case of a well-differentiated gall bladder hepatoid carcinoma producing α-FP after treatment of primary hepatocellular carcinoma (HCC).A 73-year-old man visited our emergency room (ER) in October 2004 with right upper abdominal pain and fever for the previous two days. Vital signs on arrival at the ER were blood pressure (BP): 161/107 mmHg, pulse rate (PR): 90/minute, respiratory rate (RR): 20/minute, and body temperature (BT): 38.9ºC. His consciousness was unaffected but he had an acutely ill appearance. Tracing back his medical history, he had hepatitis B virus (HBV)-related liver cirrhosis (Child-Pugh Classification Grade A), and in August 1999, a small HCC  was diagnosed. He received transcatheter arterial chemoembolization (TACE) at a medical center and a second TACE again in May 2001 for recurrent HCC. He declined further TACE for residual recurrent lesions. Therefore oral chemotherapy with the regimen tegafur/uracil (100 mg/224 mg) had been prescribed to him from December 2001 to November 2003, and was ceased due to loss at follow-up.3 (Neu/Lym: 83.5/6.4%), platelets: 169 k/mm3, total and direct (T/D) bilirubin: 1.4/0.6 mg/dL, serum glutamic-oxaloacetic transaminase/serum glutamic-pyruvic transaminase (sGOT/sGPT): 38/32 U/L, albumin (Alb): 3.9 gm/dL, C-reactive protein: 11.9 mg/dL, NH3: 98 mcg/dL, and α-FP level: 231.1 ng/mL. Abdominal echogram revealed a thickened gall bladder wall, gall stones and a suspected tumor mass in the distended gall bladder. An abdominal computed tomography (CT) scan showed liver cirrhosis, gall stones, diffusely thickened gall bladder wall with increased contrast enhancement compatible with acute cholecystitis, and a hypodense mass lesion without contrast enhancement in the gall bladder  abdominal pain. Murphy's sign was positive. Bowel sounds were normal and his abdomen was soft, with no rebounding pain. There was no shifting dullness. Lab data included: white blood cell count (WBC): 14,520/mmAn open cholecystectomy via a right subcostal incision was performed on the next day. A distended gall bladder with multiple pigmented gall stones and a tumor mass, 6.0 × 4.0 × 2.0 cm in size, were found in the gall bladder lumen. When seen, the mass had already detached from the mucosa at the opening of the sac and no stalk could be identified  outpatient department and his α-FP levels went down to 3.1 ng/mL in March 2005, and to 3.08 ng/mL in August 2005, five and 10 months after cholecystectomy, respectively. He died in July 2006 in another hospital due to upper gastrointestinal bleeding from a hemorrhagic duodenal ulcer complicated by hepatorenal syndrome.α-FP is a serum glycoprotein elevating in pregnancy and pathologic tumor growth such as HCC and germ cell tumors. It is used to evaluate treatment and to detect recurrence of these tumors. Sometimes, gall bladder tumors also produce α-FP because the gall bladder and liver have identical embryologic origin. Recurrence or metastasis of these tumors can be detected by a relapsing α-FP level .Gall bladder tumors, either primary or metastasis, are rare and often diagnosed at a late stage because of asymptomatic properties when small and without biliary tract obstruction. Primary α-FP producing gall bladder tumors consist of several cell types, including undifferentiated carcinoma, papillary clear cell carcinoma and hepatoid carcinoma. It is possible that these α-FP producing gall bladder tumors may have cell types within the two extremes of the spectrum, undifferentiated carcinoma and well differentiated hepatoid carcinoma . We regaet al. also reported two α-FP producing gall bladder tumors with direct invasion to liver showing cell types of primary undifferentiated carcinoma and a poorly differentiated tumor with a wide range of polymorphism containing hepatoid differentiation, respectively [et al. reported an autopsy result showing HCC with gall bladder metastasis presenting with massive intraluminal growth [et al. reported another autopsy case of HCC with gall bladder metastasis also presenting with intraluminal tumor growth [Undifferentiated gall gladder carcinoma has been reported in an autopsy specimen by Ng and Ng . Brown aectively . As for ectively  and withectively . HCC metl growth . Chiba er growth . Both ofr growth .et al. reported ectopic liver tissue with hepatocarcinogenesis [In our patient, an intraluminal growing gall bladder tumor with intact gall bladder wall was found and microscopic examination revealed uniformly well differentiated hepatoid carcinoma without tumor emboli in vessels. The patient recovered from the operation without major or minor complications and a normal α-FP level was noted 10 months after the surgery, and 20 months after cessation of palliative oral chemotherapy. We believe that primary α-FP producing gall bladder tumors have undifferentiated cell type or polymorphic pattern, and that the α-FP production may be the result of dedifferentiation. Since our case appeared to be a uniformly well differentiated hepatoid carcinoma after treatment of HCC, it is most likely a metastatic lesion. Arakawa ogenesis  which isIn summary, this unusual intraluminal growing tumor proved to be a well-differentiated hepatoid carcinoma and is most likely a metastatic lesion from previous HCC. This case reminds clinicians that, in looking for likely HCC recurrence, when no detectable hepatic lesion can account for elevated α-FP levels, the gall bladder should be included in the search for the site of metastasis.Alb: albumin; BP: blood pressure; BT: body temperature; CT: computed tomography; ER: emergency room; HBV: hepatitis B virus; HCC: hepatocellular carcinoma; PR: pulse rate; RR: respiratory rate; RUQ: right upper quadrant; sGOT/sGPT: serum glutamic-oxaloacetic transaminase/serum glutamic-pyruvic transaminase; TACE: transcatheter arterial chemoembolization; T/D: total and direct; α-FP: alpha-fetoprotein.Written informed consent was obtained from the patient's family for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.CY was the major contributor in writing the manuscript. CR performed the operation (open cholecystectomy) and initiated the idea for this case report. CH performed the histologic examination as well as the special staining (α-FP stain) of the specimen. HT treated and followed-up this patient. SM reviewed related articles and provided information on the nature and course of HCC and its setting in Taiwan. CY interpreted the image study (CT scan) and provided radiologic information about this case. All authors read and approved the final manuscript.


Osteosarcoma most commonly arises in the long bones of the skeleton, and rarely develops in the bones of the foot. We describe a patient who presented with left foot pain, whose radiographic evaluation revealed a lytic destructive mass in the cuboid bone. A biopsy showed an osteoblastoma-like variant of osteosarcoma and the patient was treated with preoperative chemotherapy and amputation. Osteosarcoma of the foot is uncommon and the literature reveals that it is often associated with a delay in diagnosis. Osteosarcoma is a malignant neoplasm of bone in which tumor cells produce neoplastic bone matrix . It is tA 32 year old man presented to an outside hospital with a one month history of left foot pain. He recalled that the pain started after he had twisted his ankle during a round of golf. Since that time, the pain had persisted and was most severe along the lateral aspect of his left foot. On physical examination, there was moderate swelling over the lateral aspect of the hindfoot, with point tenderness directly over the cuboid bone. The remainder of the foot exam was normal. A plain radiograph of the left foot showed a large oval, cystic mass within the cuboid bone that had relatively well-defined margins but with obfuscation of the lateral cortex figure . There wMagnetic resonance imaging (MRI) demonstrated a gadolinium-enhancing lesion, with increased signal intensity on the T2 weighted image figure . The lesThe patient's pain became progressively more severe and he no longer was able to weight bear. An open biopsy was performed and a frozen section revealed a giant cell rich lesion with atypical mononuclear stromal cells and areas of extracellular eosinophilic matrix. The differential diagnosis was an atypical giant cell tumor versus a variant of osteosarcoma. A discussion regarding immediate management concluded that it would be best to thoroughly curette the lesion and pack the defect with cement, which would stabilize the lateral column and be adequate therapy for a benign lesion. If, however, the histologic analysis of the additional tissue demonstrated an osteosarcoma, then an ampution would be performed at a later date and the curettage would not have compromised treatment for this type of tumor.Evaluation of the entire tissue specimen revealed a neoplasm that varied in morphology. The majority of the tumor was solid and in regions consisted of randomly interconnecting trabeculae of woven bone rimmed prominently by neoplastic osteoblasts. The plump osteoblasts had abundant eosinophilic cytoplasm and round or oval nuclei with fine chromatin. The intertrabecular spaces were filled with loose vascular connective tissue containing congested capillaries, extravasated red blood cells, and scattered osteoclast-type giant cells. The morphologic features were reminiscent of osteoblastoma figure . In otheA staging computed tomography (CT) scan of the chest showed no evidence of metastatic disease. The patient was started on an appropriate chemotherapy regimen and subsequently underwent a below the knee amputation (BKA). Dissection of the cuboid revealed residual small foci of osteosarcoma in the bone neighbouring the cement plug, as well as in the soft tissues immediately adjacent to the bone. No vascular invasion was identified and all margins of resection were widely negative.nd half of the 2nd decade of life [Osteosarcoma is a malignant neoplasm with a spectrum of biological potential although most are high grade and behave in an aggressive fashion. This form of cancer usually affects patients in the 2 of life . When os of life . Osteosa of life . The disIt is exceedingly uncommon for osteosarcoma to originate in the bones of the foot. In a review of all cases of osteosarcoma from 1911-1992 at the Rizzoli Orthopedic Institute in Italy, the incidence of osteosarcoma in the foot was 0.6%, with 12 identified cases of the foot out of a total of 1,929 osteosarcomas . The calClassically, osteosarcoma appears as a mixed lytic and blastic mass with poorly defined margins, cortical destruction, and a soft tissue mass ,6. HowevOsteosarcoma of the foot has some clinical features that differ from those associated with osteosarcoma of long bone. In terms of age of presentation, patients with osteosarcoma of the foot are older, with a mean age of diagnosis at 32 years old [Osteosarcoma is classified histologically into a variety of different subtypes with the most common being chondroblastic . Fox et Important distinctions exist between osteoblastoma-like osteosarcoma and conventional osteosarcoma. Bertoni et al. reviewed a series of 11 patients with osteoblastoma-like osteosarcoma, and found that the average age of presentation was older at 29 years of age . The lesThis case report, in summary, presents the rare diagnosis of osteoblastoma-like variant of osteosarcoma arising in the cuboid bone which, to the best of our knowledge, is the first published case. This report highlights the importance of tissue biopsy in the evaluation of suspicious foot lesions, the limitations of imaging studies, and the challenges of pathologic analysis in establishing a diagnosis when elements of both benign and malignant etiologies co-exist.The authors declare that they have no competing interests.NLK conceived the idea and wrote the paper.AER was responsible for editing and approving the final manuscriptKAR was responsible for editing and approving the final manuscript.All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Malignant eccrine spiradenomas are exceedingly rare tumors. They can arise from a preexisting eccrine spiradenoma or occur as a primary malignant tumor. Clinical features of these tumors may include a history of enlargement in a previously stable lesion. Tumor can be of low or high grade. Low-grade malignant eccrine spiradenoma has a better prognosis. We present a malignant eccrine spiradenoma arising from a preexisting eccrine spiradenoma, which has an infrequent localization between lateral edge of nose and medial canthus. Malignant eccrine spiradenomas are exceedingly rare tumors. They can arise from a preexisting eccrine spiradenoma or occur as a primary malignant tumor. ClinicalA 64-year-old female patient was admitted to our department, with a 1 × 1 cm swelling on her upper lateral aspect of her nose, which had been present for 6 months. She had noticed a sudden enlargment of the painless, dark pink-blue noduler lesion on her nose for the past two months . There wAt higher magnifications, two distinct populations of neoplastic epithelial cells  and tubule formations were characteristic features of the tumor. Tubules lined by two rows of epithelial cells were found within the tumor. The presence of basaloid secretion material, some surrounded by neoplastic cells, throughout the neoplasm attracted attention .The resulting appereance resembles an eccrine spiradenoma. However in some areas, the tumor showed malignant change. These malignant components were composed of solid aggregates of tumor cells with mild to modarate nuclear atypia, high mitotic figures (12/10 high-power field) and focal tumor cell necrosis Figures  and 4. Vde novo and those develop from a benign eccrine spiradenoma.[Eccrine spiradenomas are benign adnexal tumors with slow growth and long clinical history. Malignant eccrine spiradenomas (MES) are rare but have been divided into two categories: Those arise radenoma. The firsradenoma. Typicallradenoma. Clinicalradenoma. There isradenoma. Large maradenoma. In our cet al., in their study of 12 MES patients, reported equal sex incidence and an average age of occurrence at 62 years.[et al., reported 57% local recurrance and 39% metastasis and related death in MES.[et al., reported 8 of 9 cases of MES in literature are high grade criteria with nuclear pleomorphism, high mitotic count and abnormal mitoses.[et al.,[Granter 62 years. Seven tu62 years. Tay et a.[et al.,7 MES is The clinical course of MES varies in the reported cases. Metastasis of bones, lungs, brain, and lymph nodes have been described.10 Close Rapid changes of appearence of the benign skin lesions, rapid growth, color change, and ulcerations are clues to the malignant transformation. Therefore, they must be followed up carefully. In conclusion, we need more information about MES, its biological behavior, and treatment modalities.


Hemangiomas of the small bowel are rare tumors that often present with gastrointestinal bleeding. Diagnosis can be difficult and exploratory laparotomy has often proved to be the only method with which to determine the presence and location of these tumors. We report two cases of small bowel hemangioma in children aged 10 and 7 years, in which the diagnosis was made by laparoscopy. Laparoscopy identifies the affected segment of bowel and allows delivery to a minimally extended umbilical port site. The avoidance of an open laparotomy helps to reduce post-operative analgesic requirement and achieves an early return of bowel function. Hemangiomas of the gastrointestinal tract are rare and account for only 0.05% of all intestinal neoplasms. They are1Hemangiomas of the small bowel usually present with intestinal bleeding. This may be acute with massive blood loss which can be life threatening or chronic with anemia as in our two cases. Other forms of presentation include intussusception, small bo11The first patient was a 10-year-old girl who was under investigation for recurrent abdominal pain and iron deficiency anemia requiring blood transfusions. Physical examination was unremarkable. Blood count revealed hemoglobin of 6.8 g/dL, mean cell volume (MCV) of 72. Double contrast barium follows through and Technetium 99 meter pertechnetate (Meckel's) scan were normal. In addition, anti-endomysial and autoimmune antibody screens were normal. However, fecal occult blood tests were positive on a number of occasions. Upper and lower gastrointestinal endoscopies were arranged, however, before these investigations could be performed, the patient presented acutely with collapse and melaena. Following fluid resuscitation, she underwent urgent oesophago-gastro-duodenoscopy (OGD) and colonoscopy, both of which revealed no abnormality. Laparoscopy was undertaken at the same time and revealed a 2 cm vascular malformation of the jejunum [Figures The second patient was a 7-year-old girl who initially presented with a three weeks history of lethargy, anorexia, and abdominal pain. Physical examination was normal. On admission, her hemoglobin was 4 g/dL with an MCV of 62 and required blood transfusion. Fecal occult blood tests were negative. A Meckel's scan, abdominal ultrasound scan, OGD and colonoscopy were normal. She underwent laparoscopy, which revealed a 2 cm hemangioma in the distal jejunum. A mini laparotomy was performed via the umbilicus to allow resection of the affected bowel segment and primary end-to-end anastomosis. She made an uneventful recovery and was found to have normal hemoglobin and hematinics at follow up. Histology confirmed a cavernous hemangioma extending through the full thickness of the bowel wall.Hemangiomas of the small bowel are rare tumors that often present with gastrointestinal bleeding. They are commonly found in the small bowel and hence often make diagnosis difficult. Initial investigations in the patient with gastrointestinal bleeding often include upper and lower gastrointestinal endoscopy, barium contrast study, and a Meckel's scan. These investigations are often normal and while useful in excluding other diagnoses, do not provide clear indication of a hemangioma. Other investigations such as red blood cell scan and angiography have a rather low yield when the vascular malformation is not actively bleeding and one may have to resort to exploratory laparotomy to determine the presence and location these tumors.3Laparoscopy as employed in our two cases allows a diagnosis to be made without performing a laparotomy. The laparoscopy identifies the affected segment of bowel and if open excision is subsequently required, allows delivery to a minimally extended umbilical port site. Resection and anastomosis is then performed in the usual manner. A port site is positioned at the umbilicus for the laparoscope, with two lower abdominal ports for instruments. The small bowel is examined from the ileocecal junction proximally. Laparoscopy can be performed in conjunction with upper and lower gastrointestinal endoscopy under the same anesthetic, as in our first case. Prior insufflation of the gastrointestinal tract during endoscopy did not create any difficulties for the laparoscopy or identification of the pathology in this case. The avoidance of an open laparotomy helps to reduce post-operative analgesic requirement and achieves an early return of bowel function. Furthermore, there is the added cosmetic benefit of the small umbilical scar in girls.Laparoscopy in these two cases also allowed a thorough examination of the whole length of small bowel using the ‘walking’ maneuver. The good light source and magnification enabled easy viewing of any mesenteric or seromuscular abnormalities. We did not proceed to intra-operative small bowel endoscopy as we felt that additional large hemangiomas were unlikely. We were also confident that the resected specimen had had a recent bleed because of the eroded appearance of the mucosal surface of the hemangioma when examined intra-operatively. We believe laparoscopy compliments other modalities in the management of occult gastrointestinal bleeding and the use of various modalities or in combination can be considered depending on the circumstances.


We describe an autopsy case of non-functioning pancreatic neuroendocrine carcinoma metastasizing to the myocardium. A 63-year-old Japanese man was admitted to the hospital presenting with dyspnea. Echocardiography revealed marked left ventricular hypertrophy and diffuse myocardial thickening with pericardial effusion. The patient died of heart failure. An autopsy revealed that the whole pancreas, weighing 400 g, was occupied by tumor cells with neuroendocrine differentiation. The heart, weighing 780 g, showed numerous metastatic nodules and diffuse myocardial thickening. Histopathologically, the tumor was diagnosed as non-functioning pancreatic neuroendocrine carcinoma. Immunohistochemical analysis for D2-40 disclosed severe lymphatic infiltration of tumor cells, characterized by diffuse thickening of the myocardium. Pancreatic neuroendocrine tumors are rare neoplasms accounting for less than 5% of all primary pancreatic malignancies . Non-funA 63-year-old Japanese man was admitted to our hospital complaining of dyspnea. He had a history of smoking (30 cigarettes a day for 40 years) and had been diagnosed as hypertensive in the past. Echocardiography revealed marked left ventricular hypertrophy and diffuse myocardial thickening with pericardial effusion, simulating hypertrophic cardiomyopathy. Computed tomography (CT) revealed multiple lymphadenosis of the superior mediastinum, the neck and the hilum of the lung. Biopsies of cervical lymph nodes and the pericardium revealed metastatic neuroendocrine tumor suggestive of pancreatic, digestive tract, or lung origin. A cytologic examination from pericardial effusion was negative for malignancy. Although the primary site was not identified, the tumor was diagnosed as metastatic atypical carcinoid. After two courses of anticancer chemotherapy , the patient underwent radiation therapy. In spite of the intensive treatment, severe heart failure and anemia developed gradually. The patient died of heart failure, and an autopsy was conducted to disclose the origin of the cancer and the nature of the systemic disease, including cardiac muscle lesions.Grossly, the whole pancreas, weighing 400 g, was occupied by a tumor Fig.  of cellsNon-functioning pancreatic endocrine carcinomas are hormonally silent by definition. Growing large, they display symptoms attributable to local disease or distant metastases. In autopsy series, the heart is a relatively common site for metastatic tumors, which are at least 100 times more common than primary malignant cardiac tumors ,3. The iIn conclusion, a rare autopsy case of non-functioning pancreatic neuroendocrine carcinoma metastasizing to myocardium and mimicking hypertrophic cardiomyopathy is reported.Written informed consent was obtained from the patient for the publication of this case report with accompanying images. A copy of the written consent is available for reviewing by the Editor-in-Chief of the journal.The authors declare that they have no competing interests.All authors analyzed and interpreted the autopsy data. RK and SK performed autopsy and histological examinations. EK and KM assisted with the histological examinations. TK is the major contributor to the writing of the manuscript. SK supported the case analysis and the writing of the manuscript. All authors read and approved the final manuscript.