Nerve sheath tumours of the kidney are particularly rare and, in the few reported cases, are all situated in the hilar region.We describe the case of a tumour presenting towards the lateral border of the ventral aspect of the mid-zone of the kidney. This was a spindle cell lesion in which the cells strongly and diffusely expressed cytokeratins, but were negative for epithelial membrane antigen. The cells also expressed S-100 protein and glial fibrillary acidic protein, confirming the diagnosis of a cellular schwannoma.To the best of our knowledge, this is the first case of a cellular schwannoma presenting towards the lateral border of the kidney. The case also highlights the importance of using a panel of antibodies in diagnosing spindle cell neoplasms in the kidney. Schwannomas are encapsulated nerve sheath tumours that occur at all ages, but are most common between the ages of 20 and 50 years and affect both genders equally. The tumours have a predilection for the head, neck and flexor surfaces of the upper and lower extremities. Deeply situated tumours predominate in the posterior mediastinum and the retroperitoneum . A schwaA 55-year-old man presented with lower urinary tract symptoms in the form of hesitancy, poor stream and urgency. The patient did not have any flank and/or colicky pain. He had no history of urinary tract-related diseases or previous related illnesses and was constitutionally well with good appetite and normal body mass index. No abnormalities were detected on clinical examination.Laboratory investigations revealed that serum prostate specific antigen was 5.1 ng/ml and serum creatinine was 86 μmol/l. Urine microscopy revealed no atypical cells and no haematuria. Prostate core biopsies showed benign prostatic hyperplasia.An incidental exophytic lesion measuring 3 × 3.2 × 4.2 cm was discovered in the right kidney on abdominal ultrasound. This was a homogeneous hypoechoic structure with a well-defined margin seen lying towards the lateral border of the ventral aspect of the mid-zone of the kidney. The lesion appeared as a soft tissue abnormality on contrast renal computed tomography Figure . There wThe lesion was removed by laparoscopic partial nephrectomy with a small amount of renal parenchyma and surrounding fat with clear surgical margins as a curative approach. The specimen comprised a nodule surrounded by fat and attached with a small pedicle to a piece of renal tissue. The nodule was well-circumscribed, seemingly encapsulated, firm in consistency and measured 2.5 × 1.4 × 2.5 cm. The cut surface was grey-white with microcystic areas.Microscopic examination showed a well-circumscribed, partially encapsulated spindle cell lesion. The spindle cells were arranged in whorls and intersecting fascicles with focal intervening sclerosis. The tumour displayed relatively uniform cellularity. No cytological atypia, necrosis or mitoses were present. Thick-walled, hyalinised blood vessels and aggregates of foamy macrophages were present Figure . A cuff Immunostaining for a panel of cytokeratins was performed including broad range cytokeratins (AE1/AE3) and CK 7. The cells strongly and diffusely expressed cytokeratins on using a broad range cytokeratin cocktail, but were negative for CK7. The cells were also negative for epithelial membrane antigen (EMA). The tumour cells expressed S-100 protein and glial fibrillary acidic protein (GFAP) and were negative for HMB45, melan A, smooth muscle actin (SMA), desmin, CD34 and CD117. Immunostaining for Ki67 showed a very low index of less than 1% Figure .The morphology and immunoprofile were characteristic of a cellular schwannoma.A schwannoma is a benign nerve sheath tumour consisting of a highly ordered cellular component (Antoni A areas) and a loose myxoid component (Antoni B areas). Antoni A areas are composed of compact spindle cells that usually have wavy nuclei and indistinct cytoplasmic borders. The cells are arranged in interlacing fascicles and short bundles. In highly differentiated Antoni A areas, there may be nuclear palisading, whorling of the cells and Verocay bodies, formed by two compact rows of well-aligned nuclei separated by fibrillary cell processes. In Antoni B areas, the cells are arranged haphazardly in a loose matrix.Cellular schwannoma is a rare variant of schwannomas, composed almost entirely of Antoni A areas that lack Verocay bodies. Small amounts of Antoni B may be present, usually not exceeding 10% of the lesion . UnderneNerve sheath tumours of the kidney seem to be particularly rare, with only eight cases reported in the literature -10. The We have reported the case of a renal schwannoma. To the best of the authors' knowledge, this is the first case of a schwannoma reported towards the lateral border of the kidney rather than in the hilar region. On microscopic examination, this was a cellular schwannoma and hence did not show the typical features of a classical schwannoma, including the Antoni A and Antoni B areas. On immunostaining, the cells showed diffuse and strong positivity for cytokeratins. Focal cytokeratin expression has been reported in schwannomas , but in S-100 protein has been shown to have a broad distribution in human tissues, including the renal tubules. Lin et al.  studied Different markers were used to exclude other spindle cell lesions. The markers for smooth muscle differentiation, desmin and SMA, were negative. Melan A and HMB45 were negative excluding a melanocytic lesion or an angiomyolipoma. CD34 and CD117 were used to exclude a gastrointestinal stromal tumour, whereas GFAP expression confirmed neural differentiation.The patient presented with lower urinary tract symptoms, which were caused by benign prostatic hyperplasia, and the symptoms were not related to the renal neoplasm. The renal tumour was an incidental finding during routine investigations. There were no symptoms attributable to the lesion, which was completely asymptomatic. No flank pain and/or colic was ever experienced by the patient, and urine analysis did not show haematuria or atypical cells. The patient was constitutionally well, with a good appetite and normal body mass index and did not report any recent weight loss.The differential diagnosis included RCC, therefore a curative approach was taken with laparoscopic partial nephrectomy including renal parenchyma with clear surgical margins.A dimercaptosuccinic acid renal scan follow-up showed a differential renal function of 49% in the right kidney. Three years after the removal of the tumour the patient is well.To the best of our knowledge, this is the first case of a renal schwannoma presenting towards the lateral border of the kidney. This was a cellular schwannoma, which showed, in addition to the expression of S-100 protein and GFAP, strong and diffuse expression of cytokeratins.Renal schwannomas, although rare, must be considered in the differential diagnosis of spindle cell lesions of the kidney. We report a case of an incidentally identified perirenal schwannoma, which was treated by partial nephrectomy. In 1988, Somers et al. [EMA: epithelial membrane antigen; GFAP: glial fibrillary acidic protein; MPNST: malignant peripheral nerve sheath tumour; RCC: renal cell carcinoma; SMA: smooth muscle actin.The authors declare that they have no competing interests.MEB carried out the histopathological diagnosis and verification by immunohistochemistry, and drafted the manuscript. BK participated in the management of the patient and in writing the manuscript. DH performed the surgical procedure and revised the 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.


We report a case of this rare tumor and describe the surgical approach that we adopted and the clinical outcome; we also report his condition at 24 months' follow-up. Although treatment was with radical surgery, sufficient shoulder function could be obtained in our patient. We also describe the interesting MR findings of this tumor which correlated well with the histopathologic findings. Dermatofibrosarcoma protuberans (DFSP) is a relatively rare, slow-growing, malignant mesenchymal tumor of the dermis. The tumor accounts for less than 0.01% of all malignancies. The neop3DFSP usually begins as a polypoid protuberance or an indurated plaque on the skin. With time the tumor grows in size and ulcerations may appear on nodular projections on its surface. The tumor is mostly located on the trunk, with the other common locations being the head and neck. The size of the tumor is generally under 5 cm.56 In the5We report a case of this rare tumor and describe the surgical approach that we adopted and the clinical outcome; we also report his condition at 24 months' follow-up. Although treatment was with radical surgery, sufficient shoulder function could be obtained in our patient. We also describe the interesting MR findings of this tumor which correlated well with the histopathologic findings.The patient  was admitted to the Department of Orthopedics and Traumatology Service of Adana Numune Education and Research Hospital as 8562 protocol number on April 2006. He complained of a sizable, bulky tumor on the proximal part of his right arm, partially abutting on the shoulder; there was an ulceration present on the surface of the mass. According to the patient, he had first noticed a 1-cm nodule on his arm 5 years ago, which had grown gradually since then to attain its present size. He also explained that his dread of injections had prevented him from seeking medical aid earlier.The physical examination revealed a huge mass on the proximal, anterolateral aspect of the right arm and shoulder; there were potato-like projections arising from the mass. The tumor was 13 × 18 cm in size; the overlying skin was of normal color, with hemorrhagic ulcerations on it . The tumThe scout x-rays of the shoulder and proximal arm revealed a huge bulky soft tissue mass without involvement of the bony structures. The lung fields were normal on chest x-ray. MRI was done to evaluate the communications of tumor with neighboring structures and showed a multilobulated, relatively homogenous, high-signal-intensity solid mass with focal invasions at the deltoid muscle and deep extensions into the biceps, brachialis, and triceps muscles inferiorly; there was also infiltration of the cutaneus and subcutaneous tissues on fat-suppression images . The masIn view of the possibility of peripheral extension of the tumor tissue, a wide resection was planned so as to include more than a 3-cm margin of normal cutaneus tissue, at least 2 cm of the underlying normal tissue of the deltoid muscle, and also approximately 0.5 cm of noninfiltrated superficial layers of the other arm muscles . We alsoThe surgical procedure was carried out as planned and the surgical defect was covered. Macroscopic examination of the excised tumor revealed potato-like bulbous extensions on its surface. The cut surfaces were pink-white in color. Microscopic examination, which included histopathologic and immunohistochemical analysis with CD-34, confirmed the diagnosis of DFSP . The surThree weeks after repair of the surgical defect, there was limitation of the range of movement of the shoulder, with only 20° abduction and minimal medial-lateral rotation. A physical therapy and rehabilitation program was prescribed, and after 3 months of an intensive exercise program, the performance of the shoulder had improved, with 90° abduction, 70° external rotation, and 25° internal rotation Figures .We followed up the patient every 3 months. The patient's general condition was very good at the last follow-up visit, 24 months after the surgery. No evidence of local relapse was seen, though there was a residual deformity at the operation site. No enlargement of cervical and axillary lymph nodes was present. Hematologic tests and radiographs of the shoulder, arm, and chest were normal. Follow-up MR examination was normal. Bone scintigraphy also showed no abnormality. At the last follow-up, the patient was actively using his arm and reported that he was carrying out his routine daily activities easily.DFSP was first described clinically in 1890. Darrier and Ferran described the tumor as 'progressive and recurrent dermatofibrosarcoma or skin fibrosarcoma.' The term 'dermatofibrosarcoma protuberans' was first used in 1925 by Hoffman.7 The entThe cause of DFSP is not known. However, cytogenetic investigations have shown tumor cells with chromosomal anomalies together with mononuclear cellular origin. Some studies also describe the occurrence of DSFP at vaccination sites, in the scars of surgical incisions or of dermal burns, and in areas affected by leishmaniasis. Histologically DSFP is composed of monomorphic, benign appearing spindle cells. These cells are organized in a storiform or rush mat pattern and align perpendicular around the vessels in central locations.‐11 A rarRoughly 50% of DFSP lesions are found on the trunk. The rest are found on the proximal extremities and the head and neck region. Involvement of hands and feet is rare.5‐911 The93Multiple finger-like projections grow within the nodules at the periphery of the tumor mass. The DFSP with its finger-like projections is frequently disseminated to beyond to the level of vision. Insufficient excisions of these projections were thought to be responsible for the high relapse rate. The tumo3The metastases rate is reported to be under 5% in DFSP. Metastases may occur many years after the onset of the disease and are mostly to lungs, followed by the regional lymph nodes; the visceral organs and bones are rarely affected. Generally, distant metastases is seen after recurrent local relapses.5‐9 Howev5The tumor size and mobility is evaluated by physical examination. Blood tests are not useful for diagnosis. The scout x-rays reveal only a soft tissue mass without any other additional findings. MRI is useful to demonstrate deep tissue invasion, especially in the advanced stages. Nuclear study is useful for ruling out bone invasion. Though x-rays must be taken in all cases, computed tomography is not indicated if there is no bone invasion.Histologically, DFSPs should be differentiated from fibrohistiocytic neoplasms and lesions such as malignant fibrous histiocytoma, atypical fibrosarcoma, dermatofibrosarcoma, infantile myofibromatosis, nodular fasciitis, keloid, and hypertrophic cicatrix, all of which may have similar pathologic findings. In the immunohistochemical investigations, the CD-34 marker is positive in all the cut slices. Other fibrohistiocytic lesions usually show negative results in CD-34 expression.47813147813The preferred therapy of DFSP is radical (wide) surgical excision. The technique should include resection of a 3-cm margin of skin beyond the borders of the tumor and should include the fascia and even muscle tissue if necessary to attain negative intact borders histopathologically.‐681516 L68153578Radiotherapy is of limited value in the treatment of DFSP. However, it might have some role to play when the resection border is positive or when extensive excision is not possible due to cosmetic or functional difficulties.578 Imati57It has been reported that some cases of DFSP relapse as late as 5 years after surgery. A major weakness of our study is that the follow-up time is only 2 years. However, literature reports do suggest that recurrences mostly occur within the first 1 or 2 years.We performed a wide resection in our case, excising a 3-cm margin of intact tissue, which also included normal underlying muscle. Radiotherapy and chemotherapy were not used because the incision borders were negative on histopathology. Today our patient lives a normal life, without evidence of relapse or metastases. The history and clinical situation of our patient corresponds with that of patients in previous reports. In the literature, the average size of these tumors is reported as being under 5 cm. In our patient the tumor was 18 × 13 cm in size, which is exceptional; despite the large size there were no metastases.To conclude, wide radical excision is the preferred surgical method for therapy of DFSP.


A 39-year-old male presented with recurrent attacks of painless haematuria. The patient has a history of partial cystectomy for bladder paraganglioma 10 years prior to the presentation. Imaging study and cystoscopic examination revealed a small anterior wall bladder tumor. The histological examination of the lesion confirmed that it was a urinary paraganglioma. Partial cystectomy was performed to this recurrent lesion. This case report stresses the importance of life-long follow-up of these lesions. Extra-adrenal paragangliomas account for approximately 15% of all pheochromocytomas. It arises from chromaffin cells of the paraganglionic system, and is located anywhere from the bladder to the base of the skull in association with the sympathetic chain [In the urinary tract, paraganglia can persist around and within the bladder wall as well as along the ureter. Bladder paraganglioma constitutes less than 0.06% of all bladder tumors and less than 1% of pheochromocytoma . The comSurgical removal, either through partial cystectomy or transurethral resection, is still the gold standard of treatment. The current case report stresses the importance of the life-long regular follow-up after the surgical management to detect possible remote recurrence.A 39-year-old male presented with recurrent attacks of gross painless haematuria for 5 months. The patient has a history of partial cystectomy for paraganglioma 10 years prior to the presentation. The pathological report stated that the surgical margin was negative. The urine catecholamine level postresection was normal. He was followed for one year postoperatively with cystoscopic examination without any sign of recurrence. The patient does not have similar history in the family. Regarding his second presentation, the patient had no attacks of headache, sweating, palpitation, anxiety, or postmicturition syncope. His blood pressure was within normal range. Urine analysis revealed red blood cells in large quantity. Plasma metanephrines and urine vanillylmandelic acid (VMA) levels were within normal limits. Abdominal CT scan showed a 3 × 2 cm oval-shaped homogenous anterior bladder wall mass. The mass showed intense uniform enhancement with contrast study. There was no associated lymphadenopathy or invasion to the surrounding pelvic structures . α adrenergic blockade. Cystoscopic examination under spinal anesthesia showed a small exophytic yellowish submucosal mass in the left anterior wall near the bladder neck. Histological analysis revealed a highly vascular neoplasm covered by normal urothelium. Tumor cells were backed into small nests surrounded by a fibrovascular network. The cytoplasm was eosinophilic with indistinct borders  scintigraphy and abdominal CT scan 12 months after the procedure showed no recurrence.Postoperative hormone levels, including plasma metanephrines, and urinary VMA, were normal. Iodine-131-Bladder pheochromocytoma occurs more frequently in women than in men, and mainly during the third decade of life . The tum123I-MIBG, 18F-FDA, 18F-FDOPA, 18F-FDG, 11C-epinephrine, and 11C-hydroxyephedrine. 123I-MIBG is preferred over 131I-MIBG because of higher sensitivity, lower radiation exposure, and improved imaging quality [18F-FDG in patients with metastatic paraganglioma [At present, the best imaging method to detect and follow patients with paragangliomas is uncertain. Anatomical imaging with CT scan and MRI can usually detect most of these lesions. MRI using various sequences has been proven to be superior to CT scan . In addi quality . Timmersanglioma . The patient had his first paraganglioma removed at age of 29 which raise the suspicion of familial form of the disease. Recent data have suggested that between 25%–30% of these tumors might be familial in nature . GermlinAssessments of plasma and/or urine catecholamine levels are very important in these patients even if they are asymptomatic. It is crucial in the initial assessment and in the follow up afterward. Lenders et al. reported that plasma metanephrines are more sensitive and specific than urinary metanephrines for these lesions . On the Cystoscopic appearance of a yellow, submucosal tumor should raise the suspicion of a bladder pheochromocytoma. Precautions should be taken to avoid potentially lethal transient hypertensive crises during tumor manipulation. Preoperative alpha blockade is used to counteract the effects of the elevated catecholamines during surgical manipulation. Before tumor removal, beta blockers can be initiated to protect against cardiovascular abnormalities like tachycardia or arrhythmias.Treatment of bladder paraganglioma requires total excision of the tumor by partial cystectomy. Transurethral resection is usually inadequate because the majority of tumors extend in the deep layers of the detrusor muscle. Total cystectomy is reserved for large lesions when bladder preservation is impossible or in the presence of lymph node metastasis. Pelvic lymph node dissection has been recommended in order to exclude metastatic disease in all cases of bladder paraganglioma . The pre 123I-MIBG scintiscan). There is no consensus about the frequency of these measures; however, we suggested that there should be at least an annual follow-up for these patients if they are asymptomatic or whenever clinically indicated.Life-long follow-up is necessary to detect late recurrences . Follow


Port-site herniation is a rare but potentially dangerous complication after laparoscopic surgery. Closure of port sites, especially those measuring 10 mm or more, has been recommended to avoid such an event.We herein report the only case of a port site hernia among a series 52 consecutive cases of laparoscopy-assisted distal gastrectomy (LADG) carried out by our unit between July 2002 and March 2007. In this case the small bowel herniated and incarcerated through the port site on day 4 after LADG despite closure of the fascia. Initial manifestations experienced by the patient, possibly due to obstruction, and including mild abdominal pain and nausea, occurred on the third day postoperatively. The definitive diagnosis was made on day 4 based on symptoms related to leakage from the duodenal stump, which was considered to have developed after severe obstruction of the bowel. Re-operation for reduction of the incarcerated bowel and tube duodenostomy with peritoneal drainage were required to manage this complication.We present this case report and review of literature to discuss further regarding methods of fascial closure after laparoscopic surgery. Bowel herniation through the fascial defect created by the entry of trocars is now recognized as a rare but potentially serious complication of laparoscopic surgery . AlthougAn 80-year-old man was found to have early gastric cancer during his yearly check-up by gastrointestinal endoscopy. He was 158 cm in height and weighed 62 kg. Gastrointestinal endoscopy showed a depressed lesion that was diagnosed as early gastric cancer by pathological examination of biopsy specimens. He underwent LADG with regional lymph node dissection (D1 including the nodes surrounding the origin of left gastric artery). A 12-mm trocar for the laparoscope was placed in the umbilicus. Pneumoperitoneum was then established with carbon dioxide and the intraperitoneal pressure was maintained at 10 mm Hg. Two more 12-mm trocars were inserted in the midclavicular line below the costal margin and 2 cm above the umbilicus on each of both flanks and were used for active surgical instruments. All trocars used were the non-bladed type. The specimen was removed through a small medial incision which was 55 mm in length placed after resection of the stomach, and then Roux-en-Y reconstruction (RY) was carried out . Complete obstruction of the small bowel and leakage of contrast media were demonstrated by Gastrografin swallow and subsequent abdominal computed tomography (CT). CT also showed a mass lesion at the trocar insertion site on the upper left flank, suggesting herniation through the port site Fig. . Marked Port-site herniation, which is one of the major complications after laparoscopic procedures , sometimSymptoms of trocar-site herniation vary depending on the severity of bowel obstruction. Mild symptoms such as slight nausea and vague abdominal pain, both of which are most frequently seen in the early normal postoperative course after abdominal surgery, could be the first and only complaints at the early stage of this complication. Thus, the diagnosis may be delayed. In our case, mild abdominal pain with general malaise might have been symptoms related to the early stage of the onset. Abdominal CT showing the enlarged duodenum also suggested that leakage from the duodenal stump occurred due to the obstruction of the distal bowel. Thus, severe complication might have been avoided, if early diagnosis had been made. Although the benefit of Roux-en-Y is apparent , the duoPort-site herniation is a potentially dangerous complication after laparoscopic procedures. Careful management of the postoperative course is recommended especially for patients with risk factors such as obesity and extensive manipulation of the trocar during prolonged surgical duration.The author(s) declare that they have no competing interests.TI, NF, HT and TK performed the first and second operation. TI and KK were responsible for the postoperative management. TI, HT, TW, and KN were involved in editing the 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.


Carcinoma ex pleomorphic adenoma is a rare histologic subtype of salivary gland cancer with an overall poor prognosis. Limited histopathologic analyses have shown that some such tumors exhibit significant HER2/neu immunoreactivity, suggesting a potential role for HER2-based therapy. We report here a case of a 58-year old man with metastatic carcinoma ex pleomorphic adenoma who achieved a sustained long term response to combination therapy with trastuzumab and capecitabine.A 58 year old man presented with T1N2bM0 carcinoma ex pleomorphic adenoma and underwent surgery followed by adjuvant radiation therapy. Multiple metastases to bone were documented one year later. Since the original tumor was strongly HER2/neu positive by immunohistochemistry, the patient was treated with trastuzumab, capecitabine, and zoledronic acid. He experienced total resolution of symptoms and repeat FDG-PET scan after three cycles revealed interval disease resolution. Continued treatment has resulted in maintenance of disease control for over 2 years.This case illustrates the successful long term treatment of carcinoma ex pleomorphic adenoma with targeted therapy with trastuzumab in combination with chemotherapy. In the absence of definitive clinical trials which are unlikely to be performed due to the rarity of this tumor, case reports such as this one suggest potential utility for trastuzumab in combination with chemotherapy in the treatment of HER2/neu-overexpressing carcinoma ex pleomorphic adenoma. Malignant salivary tumors represent 3 to 5% of all malignant head and neck tumors, with 2500 new cases diagnosed in the Unites States yearly . CarcinoThere is no current chemotherapeutic standard for treatment of metastatic salivary carcinomas. Due to the infrequency of these tumors, the literature consists primarily of case reports and small phase II studies which combine multiple histologic subtypes. Objective response rates in the 14-30% range and rarely as high as 50% for cisplatin-based combinations have been reported [A 58 year-old man with a 44 pack-year history of smoking initially presented with a 3 year history of a painless right neck mass and new temperomandibular joint pain. Follow-up two months later after treatment with muscle relaxants revealed persistent pain and a new right facial nerve palsy. Physical exam revealed a 2 cm nontender palpable mass proximal to the right temperomandibular joint, while CT and FDG-PET also showed three enlarged hypermetabolic lymph nodes. Fine needle aspiration revealed poorly differentiated carcinoma. The patient underwent right total parotidectomy with sacrifice of cranial nerve VII. Tumor size was 2 cm, 4/18 lymph nodes  were involved by tumor, and the final pathologic diagnosis was carcinoma ex pleomorphic adenoma, high grade. The final stage was T1N2bM0. He received 60 Gy of radiation to the right parotid bed and involved lymph nodes. One year later, FDG-PET revealed multiple areas of increased bony uptake and bone biopsy confirmed disease recurrence Figure .2 days 1-14 of a 21 day cycle [At the time of documented recurrence, immunohistochemical analysis of the original parotid resection revealed 3+ HER2/neu staining. Subsequent FISH analysis  showed that the HER2/neu gene was not amplified. Given the strong immunostaining for HER2/neu and the known benefit of combining HER2-directed therapy with chemotherapy in HER2-overexpressing breast carcinoma, therapy was initiated with trastuzumab 6 mg/kg every 21 days and capecitabine 2000 mg/may cycle . ZoledroOur case demonstrates the potential utility of using targeted therapy in combination with standard chemotherapy in HER2/neu-overexpressing carcinoma ex pleomorphic adenoma. There is a lack of knowledge regarding the role of targeted therapies in the treatment of salivary gland cancers. Recent data indicate that HER2/neu immunohistochemical expression is relatively common in carcinoma ex pleomorphic adenoma, occurring in 30-46% of cases . HoweverHowever, two recent case reports, in addition to our patient, suggest that HER2/neu is a potential molecular target for a subgroup of patients with salivary gland tumors and strong HER2/neu immunoreactivity. Nashed et al. recently reported a durable complete response in a patient with ductal carcinoma arising in a pleomorphic adenoma who was treated with docetaxel and trastuzumab after initial stabilization with trastuzumab alone . This paThe patient has moved to another location and was unable to be contacted for consent.The authors declare that they have no competing interests.ESh provided the clinical case and has participated in the conception, design, and writing of the manuscript. RJK has participated in the manuscript writing. ESz provided the clinical case and has participated in the conception, design, and writing of the manuscript.All authors approved the final manuscript.


A 14-year-old male presented to the neurosurgical clinic with swelling just above the right eye which had been growing slowly for the last eight years. The swelling first appeared following a non-penetrating trauma eight years ago. On examination it was a non-tender, non-erythematous, firm, round swelling causing marked proptosis and diplopia on downward gaze only. The visual acuity was intact. MRI showed an intraorbital, extraconal mass isointense on T1 and hypointense on T2 imaging. A diagnosis of orbital tumor was made. A white, friable mass consistent with meningioma was resected. However histopathology report later showed it to be an Aspergilloma. The patient was successfully treated with anti-fungal medicine and was disease-free at one year follow-up. Orbital masses can be neoplastic, infectious or inflammatory in origin. Owing to the confined nature of the orbital space they present with overlapping clinical manifestations. Yet based on some disease-specific symptoms, signs and radiological imaging a definite diagnosis can usually be reached. An indolent growth of a solitary, discrete mass is usually suggestive of a tumor. Fungal infections of the orbit are almost exclusively seen in immune compromised states. In this report, we present a case of an orbital mass of infectious origin with fungal etiology that grew slowly over a period of eight years and had clinical features suggestive of a neoplasm.A 14-year-old South East Indian male presented to our tertiary care hospital with a painless, non-tender swelling just above the right eye. The patient, a school-going child from a rural coastal village in Pakistan, was accompanied by his father.The swelling had developed over the last eight years following a superficial orbital trauma by a cricket bat with no bone or eye involvement. There was marked proptosis of the right eye but no complaints of visual impairment, pain or discharge. Binocular diplopia was present on downward gaze. He had undergone resection of the mass through the superior palpebrae in a rural out-patient clinic previously. At that time, the mass was apparently partially resected. The tissue was not sent for histopathological evaluation and no medical record was available regarding that procedure. Review of systems was unremarkable. The patient had no constitutional complaints, and reported no change in appetite or weight.Past medical history was remarkable only for the out-patient resection procedure three years ago. No records were available from that time. The patient was delivered at home, at term, after an unremarkable pregnancy, and achieved all mile-stones at appropriate ages. No record of child-hood immunizations was present. There was no evidence of a disease causing immuno-suppression in the patient, and nutritional status was within normal limits. Family history was unremarkable, and he was taking no medications at that time. There was no history of alcohol, tobacco or illicit drug use and the patient was not sexually active.th percentile for height, and 40th for weight, and was in no obvious distress. General physical exam was unremarkable with no lymphadenopathy noted. The neurological exam, including that of the cranial nerves II, III, IV and VI was unremarkable. A non-tender, non-erythematous, moderately hard intraorbital mass was palpable superior-medial to the right eye-ball. The rest of the systemic exams, including head and neck exam with sinuses, were unremarkable.The patient was at the 50The MR imaging demonstrated an extraconal mass in the right orbit. The mass was isointense on T1 weighted imaging and hypointense on T2 weighted imaging. Significant mass effect was noted with the orbital contents pushed inferio-laterally. Infiltration in the surrounding tissue or extra-orbital extension was not observed. Post gadolinium T1 weighted imaging showed intense homogenous contrast enhancement .Based on the history, clinical presentation and radiological imaging, a diagnosis of orbital tumor was made. Surgical excision was carried out using the anterior fossa approach. Intraoperatively, the mass was located under the periorbital fascia. After opening this fascia, the frontal nerve and then its branch the supraorbital nerve was identified leading into the mass but then it could not be followed any further. All the muscles including the superior oblique and levator palpebrae superioris were pushed down by the mass. The mass was pearly white, friable and of cartilaginous consistency with no infiltration into the surrounding tissue, resembling a meningioma or neurofibroma. The mass was discrete and seemed to be arising from the supraorbital nerve. The supraorbital nerve is a terminal branch of the frontal nerve which arises from the ophthalmic division of the trigeminal nerve, the fifth cranial nerve. The tissue was sent for histopathological diagnosis which showed chronic granulomatous inflammation with multi-nucleated giant cells and areas of necrosis. Special stains; PAS (periodic acid Schiff) and tissue culture revealed numerous septate fungal hyphae consistent with Aspergillus Fumigatus . There wAt one year post-surgical follow-up there were no complaints of visual or neurological functions. The examination and work-up was unremarkable.This is a case of a 14-year-old male with an orbital mass, histologically proven to be a Aspergillus infection but clinically suggestive of a tumor.Primary orbital tumors of the pediatric age group are dermoid and epidermoid cyst, capillary hemangioma and rhabdomyosarcoma ; in the Orbital infections occur due to spread of infection from paranasal sinuses or direct inoculation due to trauma, surgery or skin infections . OrbitalMR imaging is preferably used to assess the orbital disease as it gives a detailed picture of soft tissue structures, especially in case of fungal infections which have the propensity to extend into surrounding structures. The MRI findings in orbital fungal infections are very characteristic. These include a mass lesion producing hypo-to-isointense signals on T1 weighted and extremely hypointense on T2 weighted with bright homogenous enhancement on post-gadolinium T1 weighted images . Optic nIn our case, MRI findings were of an extraconal mass, isointense on T1 weighted and hypointense on T2 weighted with intense homogenous contrast enhancement on post-gadolinium T1 weighted. The literature shows that these findings are characteristic of an Aspergillum infection . The lon


We report a neonate with acute appendicitis who was mistakenly diagnosed and treated initially as neonatal necrotizing enterocolitis. The diagnostic dilemma of this rare and life-threatening condition in premature babies and newborns is underlined. Awareness of this rare condition and possible differential diagnosis in this age group is also discussed. The rarity of neonatal appendicitis together with lack of specific signs and low index of suspicion make it very difficult for the attending clinicians to make an early diagnosis. Moreoverth day, plain radiographs of the abdomen showed free air in the peritoneal cavity, dilated ileal loops, some air–fluid levels in the lower abdomen but no evidence of pneumatosis intestinalis. After placement of a left-sided flank drain, the baby’s respiration improved. However within 24 h, small bowel prolapsed through the drain site and the patient had to be taken up for emergency laparotomy. The peritoneal cavity was contaminated with fibrinopurulent debris especially in the right lower quadrant. The appendix, measuring 3.0 cm in length was perforated near the tip. There were no obvious signs of NNEC, Hirschsprung’s disease or other obvious anomalies in the intestine. An appendicectomy was performed and full-thickness biopsies taken from multiple sites from the large bowel. Histologic examination of the appendix showed that the lumen was filled with concretions with dense fibrinosuppurative infiltrate and transmural granulation at the site of perforation. Normal ganglia were found in all specimens of the large bowel. The postoperative period was uneventful. The child was thriving at six-month follow-up and his milestones were normal.A 23-day-old full-term male weighing 2700 g, born by normal delivery to a second gravida mother with an uncomplicated perinatal history was brought to the pediatric emergency. The baby was apparently normal in the first 15 days of life, but when he refused to feed, he vomited and developed abdominal distension. After one week of treatment at a local hospital, the patient was referred to our center. On the eighth day of his illness, the patient had high fever, tachycardia, increasing abdominal distension, and increasing oxygen requirement. The physical examination revealed mild respiratory distress and a markedly distended, tender abdomen with no skin changes. The leukocyte count was 14,500/dL. The renal function tests were within normal limits. A diagnosis was made of peritonitis, possibly secondary to neonatal necrotizing enterocolitis (NNEC), and supportive treatment was started. On the 9While acute appendicitis is a common occurrence in childhood, it is hardly ever considered in the differential diagnosis of acute abdomen in neonates. The incidence of neonatal appendicitis has been variously reported as 0.04 to 0.2% and is more frequent in premature males.–3 This lIn neonates, it is extremely difficult to properly assess the presentation leading to a delay in diagnosis. This leads to increased rate of perforation, peritonitis, and mortality. However some features are indicative. The baby may have irritability, distressed breathing, and wriggling indicating peritoneal inflammation. There may be abdominal distention, bilious vomiting with induration, edema and erythematous rash over the abdominal wall. This may be associated with swelling of the scrotum and a right lower quadrant mass. Other less consistent findings are anorexia, fever and leucocytosis. Abdominal radiograph may show abnormal gas pattern, free peritoneal fluid and air, thickened abdominal wall, a right scoliosis and obliteration of psoas margin. The presence of calcified appendicoliths, a radiographic presence seen in good number of patients in the older age group has never been reported in newborns.–7On abdominal ultrasonography, presence of intra-abdominal abscess, absence of gas in appendiceal lumen or evidence of collection in the right iliac fossa strongly suggests acute appendicitis. Spiral computed tomography can also be a very useful diagnostic tool.–10Perforation is a significant factor in determining the prognosis. As the signs and symptoms are not characteristic, the incidence of perforation is high in neonatal appendicitis. The other reasons are a thin appendiceal wall and an indistensible cecum. A relatiHowever, contrary to the late childhood, perforation of appendix in this age group may be due to Hirschsprung’s disease, meconium plug syndrome, cystic fibrosis, necrotizing enterocolitis, and gastroenteritis. These co12In cystic fibrosis, although the most common system to be affected is the respiratory system, abdominal complications including appendicitis can also be observed. However,In the great majority of cases, acute appendicitis is rarely diagnosed preoperatively and is discovered only on postmortem examination. In this age group, symptoms such as irritability, drowsiness, nausea, vomiting, distension, and abdominal tenderness are not specific to appendicitis and can be found in myriad other abdominal conditions including generalized septicemia. In conclusion, judicious analysis of clinical symptoms and imaging findings and a high index of suspicion and the fact that the appendicitis should also be considered in the differential diagnosis with related clinical conditions can lead to early diagnosis and more timely surgical intervention to reduce the mortality of neonatal appendicitis.


Anterior abdominal wall fibroid are uncommon and could be a cause of pain and discomfort. Very few cases have been reported in the literature but none in our region.We present an uncommon case of a 31 year old para 2+2 trader from the Itsekiri tribe of the Niger Delta region of Nigeria who presented with a one year history of a periumbilcal mass, had surgical removal of the mass and histology revealed leiomyoma.Abdominal wall fibroid is a good differential diagnosis to be considered in any woman of reproductive age with an anterior abdominal mass and previous uterine surgery, including laparoscopic surgeries. Fibroids also called leiomyoma are said to be the commonest benign tumour of the reproductive tract and are clinically apparent in 20% of women of reproductive age. The may be present in as many as 70% of uteri removed at hysterectomy . The comWe present an unusual case of abdominal wall leiomyoma in a 31 year old multipara with 2 previous Caesarean sections and no evidence of uterine fibroid.We present a case of a 31 year old para 2+2  female trader from the Itsekiri tribe in the Niger Delta region of Nigeria. She was first seen at the gynaecological clinic with a one year history of progressive periumbilical swelling which later became associated with pain. The mass at initial presentation measured about 2.0/1.5 cm and a year later became 10.0/8.0 cm. It was located on the left periumbilical area, roundish, mildly tender, firm, slightly mobile and not attached to the skin or underlying tissue. She has 3 living children having had a set of twin and both deliveries were by Caesarean section. Indications for her 2 previous Caesarean sections were twin gestation and cephalopelvic disproportion respectively There were no findings of uterine fibroids from previous ultrasound scans and Caesarean sections done. Abdominopelvic scan done at presentation was essentially normal except for the anterior abdominal wall mass measuring 100 mm/96 mm of mixed echogenicity suggestive of anterior abdominal wall cyst or fibroid.Her general clinical state was normal. She was counseled for surgery. The abdomen was entered via a previous midline scar which was extended slightly above the umbilicus to reach the mass. The incision was developed into the subcutaneous tissue and rectus sheath and the mass was enucleated from its capsule figure , the pelThere is paucity of findings of isolated abdominal wall fibroids in the literature without previous surgeries for myomectomies or presence of uterine fibroids. She has never been managed for infertlity nor has any diagnosis of uterine fibroids been made. Indications for her 2 previous Caesarean sections were twin gestation and cephalopelvic disproportion respectively. There was no evidence of uterine fibroids in all surgeries. This supports the thinking that leiomyomas can be found anywhere there are smooth muscles .Abdominal wall fibroids are commoner after laparoscopic surgeries are done compared to open surgeries ,7,8. IncThis is the first reported case of abdominal wall leiomyoma in the medical literature from this region. Laparoscopic myomectomies are not widely done in our country because of lack of skills so many gynaecologist resorts to open myomectomies. This may be the reason why it is not common. The uniqueness of this case is the fact that the patient does not have uterine fibroid. There is the possibility that uterine seeding into the abdominal wall could have occurred in one of the Caesarean sections.Surgical removal was the mode of treatment and she did well postoperatively with disappearance of symptoms of pain.In conclusion, abdominal wall fibroid is a good differential diagnosis to be considered in any woman of reproductive age with an anterior abdominal mass and previous uterine surgery, including laparoscopic surgeries.Written informed consent was obtained from the patient for publication of this case presentation and accompanying image. 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.GO did the recent surgery and the second Caesarean section, provided data read and approved the manuscript. TO assisted recent surgery and the second Caesarean section, provided data read and approved the manuscript. PN provided data read and approved the manuscript. HO provided data read and approved the manuscript


A surgical compress retained in the abdominal cavity following surgery is a serious problem. Here, we describe a 33-year-old female who was admitted with abdominal pain, vomiting, no passage of gas or feces, and abdominal distension for 3 days. She had a splenectomy at another medical center 4 years previously. An upright plain abdominal film revealed small bowel obstruction with marked small bowel air-fluid levels. The physical examination revealed muscular guarding and rebound tenderness in the periumbilical region. Therefore, a laparotomy was performed. A surgical compress was removed at enterotomy and the final diagnosis was gossypiboma. Because a retained surgical compress may lead to medicolegal problems, it is important to count the material used before and after a surgical procedure to reduce the risk of this problem. Gossypiboma is the term used to describe a surgical swab retained after surgery. Synonyms are textiloma and cottonoid . FortunaA 33-year-old Turkish woman was admitted to our emergency unit with abdominal pain, severe nausea and vomiting, constipation, no passage of gas or feces, and abdominal distension for 3 days. She had a splenectomy at another center 4 years previously. The previous year, endoscopy had been performed because of dyspepsia and stomach ache. A peptic ulcer crater and a mass compatible with a phytobezoar in the duodenum were observed via endoscopy . LaboratPostoperative surgical complications are frequently unavoidable. However, some complications result from human error in both the intra- and postoperative periods. One such complication, which is frequently under-reported, is a retained compress or gossypiboma . Retaineet al. [et al. [Gossypiboma is difficult to diagnose, but plain X-rays, ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI) can help to make the diagnosis . In thiset al.  examined [et al.  investigIn conclusion, small sponges should not be used during laparotomy. Compresses should only be used intraperitoneally, one at a time, mounted on forceps. Before closing the peritoneum, the surgeon should explore the abdominal cavity completely.


Angiosarcoma of bone is an exceedingly rare primary bone malignancy that can present as an aggressive osteolytic lesion. Histological diagnosis can be extremely challenging, as the pathological features often resemble that of aneurysmal bone cysts. We report an interesting and peculiar case of an intraosseous angiosarcoma that presented as a diagnostic dilemma and discuss the relevant radiological and pathologic findings. Angiosarcomas are aggressive, malignant, vascular tumors derived from mesenchymal cells. They can occur in any organ of the body, however the development of intraosseous angiosarcoma is exceedingly uncommon . The cliMr. RJ was a 70 year old gentleman who presented with right knee pain. The pain in his leg had been present for many months and was progressively becoming more swollen to the point that he had difficulty ambulating. This was on the background of a past history of bilateral popliteal artery aneurysms that were ligated and bypassed surgically. Examination of his knee revealed a large knee joint effusion with considerable restriction in motion due to pain.Radiographs of the right knee demonstrated multiple permeative lytic lesions surrounding the distal femoral condyle and metaphyseal region, resembling a "soap-bubble" appearance. Magnetic resonance imaging (MRI) of the knee revealed numerous rounded areas that were hyperintense on T2-weighted imaging in the subcortical bone of the distal femur Figure . Some ofComputer tomography (CT) guided closed biopsy of the lesion was performed. The histological examination showed fibrotic changes and was deemed non-diagnostic. Because of the bizarre clinicopathological picture, an open biopsy was performed and yielded two grams of haemorrhagic soft tissue. Histological examination showed a reactive process resembling aneurysmal bone cyst formation similar to the CT percutaneous biopsy. There was no evidence in either biopsy sample to indicate malignancy or infection.Following this, the patient continued to experience ongoing symptoms over the knee. Repeat MRI revealed progressive multiple, destructive bone lesions involving the right femur, with some haemorrhagic components in the soft tissue around the knee. It was decided that a wide en bloc resection of the distal femur and proximal tibia would be performed. A rotating hinge megaprosthesis  was used for reconstruction of a mobile knee joint. Histological analysis of the resected specimen showed cystic spaces which were lined by fibrous septa that contained blood vessels Figure . PromineTwo months after the index operation, he presented with an acute extensive and painful haemarthrosis. Angiogram of the right leg was performed and revealed extravasating collateral branches from the profunda artery around the knee joint. Exploration and ligation of the right popliteal aneurysm and evacuation of the knee joint haematoma was performed. The patient was also anaemic, however, haematological assessment showed no underlying blood malignancies that could account for this, leaving chronic blood loss the most likely clinical cause.The patient continued to have unremitting limb pain, and an x-ray of the knee showed progressive osteolytic destruction over the bone-implant junction Figure . It was The patient's disease progressed and he subsequently developed pulmonary and intra-abdominal metastases. Lung biopsy confirmed tumor metastasis. He died of metastatic disease and sepsis one year after initial presentation.Angiosarcoma is a tumour derived from mesenchymal cells, which have undergone angioblastic differentiation, resulting in the formation of blood vessels . PrimaryReports of cardiac and pulmonary angiosarcoma highlight the difficulty in making the initial diagnosis due to the relatively non-specific clinical course and apparently normal radiological findings ,5 High-get al, reported that angiosarcoma arising in soft tissue demonstrate early and continuous enhancement on Gadolinium-enhanced T1-weighted SE MR images [MR imaging findings of angiosarcoma are relatively non-specific. Characteristically, they demonstrate low signal intensity on T1-weighted images and heterogeneously intermediate to high signal intensity on T2-weighted images . In our R images . MoreoveR images .As seen in this case, the histologic features of aneurysmal bone cyst can mimic that of angiosarcoma. On histopathology, aneurysmal bone cysts (ABCs) are blood-filled cystic cavities that are lined by a thick, fleshy membrane that has an endothelial-like inner layer. Nearly 50% occur as a secondary lesion in another tumor. These lesions include giant cell tumor, chondroblastoma, osteoblastoma and some malignant tumors . Often, et al reported that doxorubicin-ifosfamide chemotherapy produces a modest response rate in angiosarcoma [The 5-year survival rate for patients with angiosarcoma is reported to be 12% to 31% ,19 ReporAngiosarcoma of bone is a rare primary bone sarcoma. The diagnosis is often delayed because of the non-specific clinical presentation and radiological features. Clinicopathological correlation is of paramount importance to differentiate aneurysmal bone cyst from the aggressive type of vascular malignancies. Timely surgery is potentially curative although prognosis of most of the cases is often dismal.The authors declare that they have no competing interests.The authors equally contributed to the preparation and drafting of this manuscript.


Patient. We report a 22-year-old male presenting with extraskeletal osteosarcoma of the orbit.Discussion. Extra skeletal osteosarcomas are uncommon tumours, usually arising from the lower extremities or girdle. These are aggressive tumours with high metastatic potential and poor outcome. Optimal treatment is undefined, and the role of radical surgery, radiotherapy and aggressive chemotherapy is currently being evaluated. The orbit is a rare site forextraskeletal osteosarcoma, with the only previous case reported in an 11-year-old male, who was irradiated in infancy for a retinoblastoma.


Desmoid tumors (DTs) are neoplasms of fibroblastic origin characterized by lack of a capsule. They are nonmetastatic and locally aggressive. Intraabdominal DTs are often observed in familial adenomatous polyposis and Gardner syndrome or subsequent to localized traumatic injury. Sporadic forms are defined as nontrauma- or nongenetic-related DTs. Isolated, sporadic pancreatic DTs have been considered anecdotal, with only 9 cases described in the literature. We report the case of a 68-year-old man with a case of sporadic cystic DT localized to the pancreatic tail. The tumor was discovered incidentally during computerized tomography performed for an unrelated condition. The patient was asymptomatic; however, biopsy was performed on the clinical suspicion of cystic cancer of the pancreas. Pathology analysis showed fibroblastic proliferation, and the diagnosis of DT was confirmed by immunohistochemical staining for beta-catenin. The patient underwent resection with no further treatment and remain disease-free 60 months after surgery. Surgery was performed on the suspicion of cystadenocarcinoma. During the surgical exploration, the mass appeared to be localized completely to the tail of the pancreas, with no invasion of adjacent structures. There were no enlarged nodes surrounding the tumor. A left pancreatectomy was performed with spleen preservation and lymphadenectomy of the celiac axis and peripancreatic nodes. The transection margin was tumor-free. The gross appearance of the mass was of a dense grayish tumor containing a cystic cavity, with no signs of a capsule and no evidence of necrosis.Histologic sections showed proliferation of spindle-shaped or stellate cells, with a fasciculate and storiform growth pattern within a background of myxoid intercellular matrix . Glassy,The tumor infiltrated the pancreas and surrounding adipose tissue. The patient developed an abscess of the pancreatic stump, which was treated by CT-guided percutaneous drainage and resolved on postoperative day 28. Oral intake was initiated on postoperative day 5, and patient was discharged on postoperative day 16. The patient is doing well and remained disease-free according to 60-month follow-up CT .Desmoid tumors are rare, benign, soft-tissue tumors characterized by fibroblastic proliferation within a collagen matrix. Recent classification includes abdominal and nonabdominal DTs; among abdominal DTs, a further distinction is made between abdominal-wall and intraabdominal DTs . IntraabWnt gene and activation of the Wnt signaling pathway [Pancreatic DTs are extremely rare. A review of the literature showed 9 reported cases , 8–14, w pathway .Diagnosis of a rare case of extraintestinal gastrointestinal stromal tumor was excluded on the bases of the morphologic findings and negative immunostaining for CD34 and CD118/c-kit. Recurrences at the site are frequent in FAP and Gardner syndrome but appear to be absent in sporadic pancreatic forms, according to the follow-up times reported in the limited literature , 13–15. In conclusion, sporadic pancreatic DT is an extremely rare finding in common clinical practice. There are no symptoms, signs, or imaging features to aid in diagnosis. Fine-needle aspiration should be considered for incidentally discovered small lesions. However, according to current guidelines, surgery must be performed if there is any doubt as to diagnosis. Follow-up is also necessary, regardless of the low-rate of tumor recurrence.


Elephantiasis as a result of chronic lymphedema is characterized by gross enlargement of the arms, legs or genitalia, and occurs due to a variety of obstructive diseases of the lymphatic system. Genital elephantiasis usually follows common filariasis and lymphogranuloma venereum. It may follow granuloma inguinale, carcinomas, lymph node dissection or irradiation and tuberculosis but this happens rarely. Vulval elephantiasis as a consequence of extensive lymph node destruction by tuberculosis is very rare. We present two very unusual cases of vulval elephantiasis due to tuberculous destruction of the inguinal lymph nodes.Two Indian women - one aged 40 years and the other aged 27 years, with progressively increasing vulval swellings over a period of five and four years respectively - presented to our hospital. In both cases, there was a significant history on presentation. Both women had previously taken a complete course of anti-tubercular treatment for generalized lymphadenopathy. The vulval swellings were extremely large: in the first case report, measuring 35 × 25 cm on the right side and 45 × 30 cm on the left side, weighing 20 lb and 16 lb respectively. Both cases were managed by surgical excision with reconstruction and the outcome was positive. Satisfactory results have been maintained during a follow-up period of six years in both cases.Elephantiasis of the female genitalia is unusual and it has rarely been reported following tuberculosis. We report two cases of vulval elephantiasis as a consequence of extensive lymph node destruction by tuberculosis, in order to highlight this very rare clinical scenario. Elephantiasis, the result of chronic lymph edema, is characterized by gross enlargement of the arms, legs or genitalia, and it occurs due to a variety of obstructive diseases of the lymphatic system. Genital elephantiasis is a common result of filariasis and lymphogranuloma venereum. However, it may also follow granuloma inguinale, carcinomas, lymph node dissection or irradiation and tuberculosis, although this happens rarely -6. FilarA 40-year-old Indian woman presented with progressively increasing vulval swellings over a period of five years. She also described a loss of appetite and weight. There was a history of fever with a rise in the evenings, night sweats and vaginal discharge, although her menstrual periods were normal. Eight years prior to presentation, she had generalized lymph node tuberculosis with discharging cervical and inguinal sinuses, for which she received a full course of anti-tubercular therapy. Her tuberculosis was completely cured by the anti-tubercular therapy and she did not show any evidence of a recurrence. Her genital swellings were extremely large and caused her to experience difficulty in walking. Sexual intercourse was not possible.Our examination revealed that she was poorly nourished, with a marked pallor. There was no lower limb edema. She had two giant vulval swellings measuring 35 × 25 cm on the right side and 45 × 30 cm on the left side Figure  and 2. IBoth her inguinal and cervical regions had puckered scars of healed sinuses without any palpable lymph node. The rest of our physical examination, including her vaginal wall, chest and abdomen was normal.She was found to be severely anemic (Hb 6 gm%) with a normal leucocyte count. Our other investigations, including blood urea nitrogen, serum electrolytes, creatinine, Mantoux test, night blood smear, chest X-ray, ultrasonography of her abdomen and pelvis, and pap smear were normal.She was taken up for surgery after the correction of her anemia. A wide local excision with a primary closure was performed. Part of the fibro-fatty tissue of her labia majora was preserved to give them a natural bulging appearance Figures , 3, 4, 5A 27-year-old Indian woman presented to our hospital with a very similar history and findings to that of case report 1, although her swellings were not as large. She presented with progressively increasing vulval swellings over a period of four years. She had a past history of fever, night sweats, weight and appetite loss, and vaginal discharge. Her menses were normal. Seven years prior to presentation, she also had generalized lymph node tuberculosis with discharging cervical and inguinal sinuses, for which she had received a full course of anti-tubercular therapy. Her tuberculosis was completely cured by the anti-tubercular therapy and she did not show any evidence of a recurrence.Her general physical examination was normal and there was no lower limb edema. She had a 15 × 7 cm labial swelling on the left side and a 9 × 5 cm labial swelling on the right side Figure . The skiAll of our routine investigations, including hemoglobin, total and differential cell counts, blood urea nitrogen, Mantoux test, night blood smear, chest X-ray, ultrasound of her abdomen and pelvis, and pap smear were normal.She was taken up for surgery and a wide local excision with primary closure was performed. Both of her labia majora were given a natural soft and bulging appearance. There was slight oozing in the post-operative period, but healing occurred with primary intention . Her post-operative period was uneventful. A follow-up period of six years revealed a satisfactory recovery and, unlike case report 1, she experienced minimal discharge from the wound site and her recovery was uneventful.A histopathological examination of the specimen in both cases showed changes of lymphedema. The features were suggestive of non-specific inflammation. There was, however, no clear evidence of tuberculosis in the specimens (in the form of granulomas and/or acid fast bacilli), malignancy, filariasis or donovanosisFigure .Lymphedema occurs due to an inability of the existing lymphatic system to accommodate the protein and fluid entering the interstitial compartment at tissue level. In the first stage of lymphedema, impaired lymphatic drainage results in protein-rich fluid accumulation in the interstitial compartment. Clinically, this manifests as soft-pitting edema. In the second stage, there is an accumulation of fibroblasts, adipocytes and macrophages in the affected tissues, culminating in a local inflammatory response. This results in a deposition of the connective tissue and adipose elements at the skin and subcutaneous level, leading to non-pitting edema. In the third and most advanced stage, the affected tissues sustain further injury as a result of both the local inflammatory response and recurrent infections. Such repeated episodes injure the remaining, incompetent lymphatic channels, progressively worsening the underlying insufficiency of the lymphatic system. This eventually results in excessive subcutaneous fibrosis and scarring with associated severe skin changes characteristic of lymphostatic elephantiasis ,10.Lymphedema is generally classified as primary when there is no known etiology, and as secondary when its cause is a known disease . PrimaryWuchereria bancrofti, is the most common cause of secondary lymphedema [The most common form of lymphedema is secondary lymphedema. In developed countries, the most common causes of secondary lymphedema involve resection or ablation of the regional lymph nodes by surgery, radiation, tumor invasion, direct trauma, or, less commonly, an infectious process. Globally, filariasis, caused by infestation of the lymph nodes by the parasite mphedema ,11.et al. reported two cases of vulval elephantiasis as a consequence of tubercular lymphadenitis, however, both the cases had smaller-sized vulval swellings [Vulval tuberculosis leading to pseudoelephantiasis - direct infiltration of the vulva by tuberculosis - is rare; however a few cases have been previously reported . Vulval wellings . In our Vulval elephantiasis is very rare, and vulval elephantiasis as a consequence of lymph node destruction by tuberculosis, as evidenced in our case reports, is rarer still. We present our cases to draw attention to this rare condition.The authors declare that they have no competing interests.C was the chief operating surgeon who analyzed and interpreted the patient data. JPS, MT, RK, TA, SJ, NN, RB, YK and SSr were the surgical residents who assisted in the surgery and work up of both of the patients. They also contributed to the preparation of the manuscript. SSa was the histopathologist who reported on the specimen. All authors read and approved the final manuscript.Written informed consent was obtained from the patients for publication of these case reports and any accompanying images. Copies of the written consents are available for review by the Editor-in-Chief of this journal.


Staphylococcus aureus. Any skeletal muscle can be involved, but the thigh and trunk muscles are commonly affected. Only three cases of extraocular muscle (EOM) pyomyositis have been reported. We herein present four cases of isolated EOM pyomyositis. Three of our cases presented with acute onset of proptosis, pain, swelling and redness. One patient presented with mass in the inferior orbit for 4 months. One patient had central retinal artery occlusion on presentation. None of them had marked systemic symptoms. Computed tomography scan of all patients showed a typical hypodense rim enhancing lesion of the muscle involved. Three patients were started on intravenous antibiotics immediately on diagnosis and the pus was drained externally. Two patients underwent exploratory orbitotomy. In conclusion, it should be considered in any patient presenting with acute onset of orbital inflammation. Management consists of incision and drainage coupled with antibiotic therapy.Pyomyositis is a primary acute bacterial infection usually caused by  Pyomyositis is an acute bacterial infection occurring in the skeletal muscle. It was first described in 1885 by Scriba as an endemic disease in the tropics.3 We presStaphylococcus aureus. Ampicillin plus cloxacillin 1 g 12 hourly and amikacin were started according to the sensitivity report. He was discharged with gatifloxacin twice daily orally. After 1 month, he had residual restriction in motility in medial and superior gazes and 1 mm of proptosis. After 1 year, there was no proptosis and motility restriction.A 15-year-old boy was referred with complaints of sudden dimness of vision, proptosis, pain and swelling in the left eye (LE) for 5 days in March 2008. On examination, visual acuity in the right eye (RE) was 20/20 and in the LE was 20/200. There was proptosis of 3 mm, edema, ptosis and congestion in the superotemporal quadrant . The pupA 22-year-old man presented with complaints of swelling below the RE for 3 months in December 2007. He had history of pain and redness of the eye 4 months back, which subsided spontaneously. On examination, visual acuity both eyes (BE) was 20/20. There was a palpable, ill-defined mass with a nodular surface. It became prominent on up gaze . The infA 10-year-old boy presented to us with complaints of sudden onset of swelling, severe pain and redness in the RE of 5 days duration in July 2006. On examination, visual acuity in both eyes was 20/20. There was edema, ptosis and chemosis. Ocular motility was restricted in down and lateral gazes . The posA 55-year-old diabetic female presented to us with complaints of sudden dimness of vision, swelling, pain and redness in the LE of 5 days duration in January 2006. On examination, the pin hole visual acuity in the RE was 20/40 and there was no perception of light in the LE. There was proptosis, edema, ptosis and chemosis. The pupil was dilated and was nonreactive to light. Ocular motility was restricted in all gazes. The posterior segment showed central retinal artery occlusion. CT scan showed an irregular, ill-defined rim-enhancing hypodense lesion in relation to the inferior rectus and inferior oblique muscle with soft tissue infiltration of the lid and cheek . ProvisiStaphylococcus aureus and the third was idiopathic. Staphylococcus aureus is responsible for 95% of the cases in tropical areas and 70% of the cases of pyomyositis in nontropical areas.[Of the three reported cases, two were due to al areas. The etioal areas. Given thet al.[et al. reported two cases of young boys with EOM abscess on MRI.[CT scan or magnetic resonance imaging (MRI) is required for precise localization and diagnosis. Haufschild et al. reporteds on MRI. In both In conclusion, pyomyositis of EOM should be considered in any patient presenting with acute onset of orbital inflammation and characteristic CT or MRI features. Once the diagnosis is suspected, incision and drainage coupled with antibiotics eradicate the infection in most patients.


Brunner's gland hamartomas (BGH) are uncommon benign tumors of the duodenum forming mature Brunner's glands. We report here an unusual case of a giant BGH that was not amenable to endoscopic or surgical local resection thus requiring a pancreaticoduodenectomy for extirpation. The relevant literature is discussed. Brunner's gland hamartomas (BGH) are uncommon benign tumors of the duodenum forming mature Brunner's glands. BGH have an estimated incidence of < 0.01% based upon review of one large autopsy series [A 62-year-old Asian male presented to an outside institution with chief complaints of epigastric abdominal pain and reflux symptoms. Review of systems, past medical history, physical exam, and laboratory values were unremarkable. Family history was notable for pancreatic cancer in his father at the age of 92 years. An upper gastrointestinal contrast study was obtained and revealed a 6 cm mass within the duodenum that resulted in significant compromise of the lumen. A computed tomography (CT) scan demonstrated a cystic and solid lesion located within the duodenum and impinging on the head of the pancreas. Esophagoduodensocopy (EGD) and endoscopic ultrasound (EUS) demonstrated a submucosal, cystic lesion in the wall of the duodenum distal to the ampulla of Vater. The patient underwent an endoscopic ultrasound with multiple biopsies and fluid aspirations. Microscopic evaluation revealed benign glandular cells with reactive changes. No malignant cells were identified. Endoscopic un-roofing of the cystic lesion was performed. Clear viscous fluid was noted to emanate from the lesion and again pathology demonstrated only benign glandular cells with reactive changes. Despite this procedure the mass was noted to recur and grow in size over the next three 3 years. Over this time the patient did not experience vomiting or weight loss but did have significant worsening of his reflux symptoms.The patient was referred to our institution and evaluated by a multidisciplinary gastrointestinal oncology team. CT imaging at that time demonstrated a massive intraluminal mass extending from the antrum through the duodenum Figure . Based oPathologic examination demonstrated a Brunner's gland hamartoma measuring 10.5 cm Figure . No dyspBrunner's glands are alkaline-secreting glands located in the submucosal layer of the duodenum. The majority of Brunner's glands are located in the first portion of the duodenum, with decreasing prevalence in the second and third portions of the duodenum. BGH follow this distribution, as a series of 27 patients with BGH found 70% in the duodenal bulb, 26% in the second portion of the duodenum, and 4% in the third portion . ChronicHelicobacter pylori infection [Differential diagnosis includes duplication cyst, leiomyoma, leiomyosarcoma, adenoma or adenocarcinoma, lymphoma, carcinoid tumors, heterotopic pancreatic or gastric tissue, or gastrointestinal stromal tumors. Pathologic review demonstrates the admixture of normal tissues including Brunner's glands, ducts, adipose tissue, and lymphoid tissue . The celnfection . HoweverThe description of BGH on computed tomography in the literature, particularly in the era of multi-slice helical CT scanners has been limited but varies from homogenous enhancement with intravenous contrast administration to heterogenous lesions with solid and cystic components . EUS exaet al recently described a patient who presented with gastrointestinal bleeding secondary to a 2 cm BGH [There have been rare reports of malignant transformation of BGH in the literature. Brookes 2 cm BGH . The les2 cm BGH . These rBiopsies are typically indeterminate given the submucosal location of the lesions. Treatment options can include endoscopic removal for those lesions on a pedunculated stalk ,8 to surThe authors declare that they have no competing interests.ZAS contributed to the study design, manuscript preparation and editing. RHH contributed to the evaluation of the histopathology, manuscript preparation and editing. EKF contributed to the interpretation of the cross-sectional images, manuscript preparation and editing. CLW contributed to the study design, manuscript preparation and final editing. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


A 77-year-old Japanese woman presented a dome-shaped pinkish nodule on the scalp. Dermoscopy demonstrated yellowish homogeneous ovoid areas with translucent whitish veil and arborizing vessels. No association with Muir-Torre syndrome was found. Histopathology revealed a smooth-bordered neoplasm in the dermis with partial connection to the epidermis. The tumor was composed mainly of germinative cells. The tumor focally showed a typical “rippled pattern”. There were only a few vacuolated cells suggesting sebaceous differentiation. These cells were highlighted with adipophilin antibody. No nuclear atypia or mitotic figures were observed. We regarded the neoplasm as sebaceoma. Dermoscopy demonstrated clearly visualized yellowish homogeneous ovoid areas. This feature usually corresponds to dermal conglomerations of the cells with sebaceous differentiation. However, this case histopathologically showed only limited area with sebaceous differentiation. We presented a case of rippled-pattern sebaceoma and described its dermoscopic features. This was the first report referring to the dermoscopic features of sebaceoma.  A 77-year-old Japanese woman presented with a tumor on the parietal region of the scalp, which had gradually enlarged over the previous several years. Physical examination revealed a dome-shaped faintly pinkish nodule, 10 × 8 mm in size. The surface of the tumor was covered with yellow papules . DermoscSebaceoma, originally described by Troy and Ackerman , is a diDermoscopy is now widely used as a tool to diagnose many pigmented and nonpigmented cutaneous lesions. In the present case, dermoscopic examination demonstrated two discriminating features. One was clearly visualized yellowish homogeneous ovoid areas. This feature usually corresponds to dermal conglomerations of the cells with sebaceous differentiation. However, interestingly, this case histopathologically showed only limited area with sebaceous differentiation. The other feature was arborizing vessels at the peripheral area of the nodule. There were no arborizing vessels in the center of the tumor on dermoscopy or on clinical image as described in basal cell carcinoma . MenziesIn this report, we presented a case of rippled-pattern sebaceoma and described its intriguing dermoscopic features.


Penetration of the colon to the posterior uterine wall secondary to diverticulitis is unusual, with diagnostic methods not yet established. Non-invasive imaging, such as computed tomography and magnetic resonance imaging may help to establish a proper diagnosis, but confirmation may be reached only after surgical exploration.We report the case of a 78-year-old Japanese woman who presented with a low grade fever and mild diarrhea which occurred two or three times a week. Computed tomography and magnetic resonance imaging demonstrated a capsular lesion including an air structure with a diameter of 5 cm, between the posterior aspect of the uterine body and the sigmoid colon. A gastrograffin enema and colonoscopy demonstrated a giant diverticulum of the sigmoid colon with no evidence of malignancy. These data confirmed the diagnosis of diverticulitis complicated by a giant diverticulum. Because of a relapsing fever after therapy with antibiotics, the patient had en bloc surgical treatment of the uterus, fallopian tubes, ovaries and sigmoid colon, the organs involved in the diverticulitis, followed by an uneventful recovery.This is a rare case report of penetration of the sigmoid colon to the posterior uterine wall secondary to diverticulitis. Diverticulosis is the most common colonic disease. Up to 30% of individuals are affected by the time they reach 60 and nearly 65% by the age of 80 . In thisA 78-year-old Japanese woman, with a previous medical history of arterial hypertension and who had not undergone any previous operations, was admitted to our hospital with a low grade fever and mild diarrhea, which had occurred two or three times a week during the six months before admission. Physical examination revealed no spontaneous pain, no tenderness, and no guarding in the abdomen, and no abdominal or pelvic mass was present on palpation. The patient was afebrile, but there were mild inflammatory signs in the laboratory data . The other biological values  were normal.First, we performed computed tomography (CT) and magnetic resonance imaging (MRI) as non-invasive imaging modalities. The CT scan revealed a capsular lesion including air density with a diameter of 5 cm between the posterior wall of the uterine body and the sigmoid colon Figure . The MRIAmong diverticulitis complications, fistula formation may complicate up to 20% of the observed cases. The urinary bladder is the most commonly involved organ. The uterus represents is rarely involved . InflammOur patient demonstrated a relapsing fever but with the symptoms of diverticulitis, such as abdominal pain and less obvious tenderness. In the findings from the gastrograffin enema and colonoscopy, the ring-shaped lesion was revealed as a giant diverticulum of the sigmoid colon without evidence of malignancy, but penetration was not detected. CT and MRI played an important role in the pre-operative surgical planning by demonstrating the extent and degree of pericolonic inflammation. Though identifying any penetration is important for the planning of appropriate surgical management, in this study, neither the imaging nor the colon examinations could detect the penetration.We report the case of a patient with penetration of the colon to the wall of the uterus secondary to diverticulitis, together with a relapsing fever. In our patient, pre-operative diagnosis of the penetration was impossible on any imaging and colon examinations. Therefore, in cases of diverticulitis with relapsing fever, and even where no typical symptoms of diverticulitis are present, surgical management should be recommended bearing in mind the possible complication of penetration to other organs.CT: computed tomography; MRI: magnetic resonance imaging.The authors declare that they have no competing interests.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.TA, YA, NK, KK and TS analyzed the upper endoscopies, collected the clinical data and wrote the manuscript, with contributions from MI. HI, HE, KF, MY, HK and HT collected the clinical data. SY and YR performed the pathological assessment. TA, MI and AN analyzed the endoscopies and participated in the design and coordination of the case report. All authors read and approved the final manuscript.


Segmental omental infarction (SOI) is a rare cause of acute abdominal pain. Depending on the site of infarction, it mimics conditions like appendicitis, cholecystitis, and diverticulitis. Before the widespread use of Computed Tomography (CT), the diagnosis was usually made intraoperatively. SOI produces characteristic radiological appearances on CT scan; hence, correct diagnosis using this form of imaging may prevent unnecessary surgery. We present the case of a young woman who was treated conservatively after accurate radiological diagnosis. Segmental infarction of the greater omentum was described over 100 years ago , 3. Most3/ml and a C-reactive protein (CRP) of 88 mg/dl. Other blood tests and erect chest radiograph were unremarkable. Microscopic haematuria was also noted on urinalysis. An unenhanced CT scan was subsequently performed and demonstrated a focal region of heterogenous increased fat density involving the right omentum between the hepatic flexure and anterior abdominal wall  pain and no other gastrointestinal symptoms. Examination revealed focal tenderness in the RUQ with mild peritonism. Murphy's sign was negative. She was apyrexial; pregnancy test was negative; routine blood investigations revealed a raised white cell count of 13.6 × 10nal wall . No otheThe incidence of SOI is estimated to be around 0.1% of all laparotomies performed for acute abdomen . VariousClinical findings for SOI tend to be nonspecific. Patients are constitutionally well and present with acute or subacute abdominal pain; gastrointestinal symptoms such as nausea, vomiting, anorexia, and diarrhoea are uncommon , 12. TemCorrect radiological diagnosis is important to establish the most appropriate treatment plan for the patient. Ultrasound scan (USS) or computed tomography can be used to make a reliable diagnosis. Typical CT findings of omental infarction include a well-circumscribed ovoid area of heterogenous fat stranding with hyperattenuating streaks located within the omentum between the rectus abdominis and colon , 11. TypOmental infarction can either be managed conservatively or surgically, and there are controversies about the correct treatment modality. Some authors recommend surgical intervention because it leads to a shorter hospitalisation period and a more rapid improvement of the patients' pain –19. AlsoOthers argue that unnecessary operations should be avoided because this disease usually runs a self-limited course , 21–23. Fifty-four patients underwent successful conservative management with no ensuing complications. However, 10 patients 15.6%) had subsequent laparoscopic resection of the infarcted omentum having failed conservative management. The reasons for failed initial conservative treatment were severe intractable pain and worsening of symptoms. The postoperative recovery of these patients was uneventful. The results from the above table demonstrate that non-operative treatment of omental infarction has been achieved in several series with successful outcomes provided that an accurate radiological diagnosis is obtained and the patient's condition remains stable. Moreover, with conservative management, most patients become symptom-free within two weeks , 5, 31. .6% had sSurgical exploration of the abdomen is mandatory in patients with unclear radiological findings or if the patient's clinical condition deteriorates . If surgSegmental omental infarction is a benign rare cause of acute abdomen, and hence it is seldom considered in the differential diagnosis of acute abdominal pain. It mimicks symptoms that often leads to misdiagnosis of appendicitis, cholecystitis, or diverticulitis . The use


A 30-year-old woman with a history of smoking presented with abdominal pain and haematuria. On physical examination, she had a palpable pelvic mass. Imaging revealed a large pelvic mass located on the dome of the bladder, extending from the urachus, with pulmonary metastases. After open biopsy, urachal adenocarcinoma was histologically confirmed. The patient received six cycles of palliative chemotherapy combination 5 fluorouracil and irinotecan with complete response on the pelvic mass and partial response estimated to more than 80% on pulmonary metastasis. Although single-institution series have suggested that urachal adenocarcinomas account for 25-30% of primary adenocarcinomas of the bladder, a recent population-based analysis indicated that urachal adenocarcinomas represent approximately 10% of this group [lidated. .A 30-year-old Moroccan woman presented to her primary care physician having experienced abdominal pain, dysuria, and episodic gross hematuria for 1 month. Her medical history was unexceptional except for a 10-pack-year smoking history. Physical examination revealed a palpable suprapubic pelvic mass. The rest of her examination including pelvic and complete lymph-node examinations was unremarkable. Initial laboratory tests were all normal. An MRI of the abdomen and pelvis obtained 3 months after surgery revealed a lobulated left pelvic mass measuring 7.8 × 6.6 cm in the area of the prior pelvic-sidewall mass Figure . No obvien bloc removal of the umbilicus, urachal tumor mass, the entire urachal ligament, and bladder dome [en bloc resection of the umbilicus, peritoneal involvement, and positive nodes and/or margins [et al. Reported the results of a retrospective review of 42 patients treated at the MD Anderson Cancer Center. Among the 26 patients who developed metastases, only 4 had significant responses to chemotherapy, and of the 9 patients who received chemotherapy with 5-FU or cisplatin-containing regimens three responded [Primary treatment of potentially localized disease includes wide local excision of the urachus, umbilicus, and surrounding soft tissue combined with partial or radical cystectomy and bilateral pelvic lymphadenectomy ,5. Althoder dome ,4,6. Desder dome . Risk fa margins . Owing t margins ,6. Chemo margins -12. Despesponded . Irinoteesponded . Currentesponded . Irinoteesponded ,16. UracCurrently, there is no standard chemotherapy regimen for the treatment of metastatic urachal adenocarcinoma. The demonstration that more recently developed agents have efficacy in adenocarcinomas from other tumor types should inspire treatment options for this difficult disease. In addition, agents that demonstrate efficacy in metastatic disease should be pursued in trials designed to clarify the role of neoadjuvant or adjuvant chemotherapy in urachal adenocarcinoma.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.ET analyzed and interpreted the patient data regarding the retroperitoneal disease. IL have made contributions to conception and design, and acquisition of data. YA and MT have been involved in drafting the manuscript and revising it critically for important intellectual content. HM and HE have given final approval of the version to be published. All authors read and approved the final manuscript.


Meningioma, craniopharyngeoma and glioma are mainly intracranial lesions. Nevertheless, in rare cases these entities may occur solely as extracranial lesions that may present as intranasal/sinusoidal masses, with headaches and nasal obstruction. We present three cases of common intracranial tumors, with purely extracranial extension. The three described cases demonstrate, that preoperative MRI and CT imaging is important for differential diagnosis to exclude intracranial connections of the tumors. A definitive diagnosis requires specialized immunohistochemical examinations. In all cases of intranasal or pharyngeal neoplasm the diagnosis of meningioma, craniopharyngeoma and glioma should be considered as differential diagnosis to optimize the surgical procedure. Meningiomas, craniopharyngiomas and gliomas are mainly intracranial lesions. Nevertheless, in rare cases these entities may occur solely as extracranial lesions that may present as intranasal or sinusoidal masses, with vision disturbances, headaches and nasal obstruction. We present three cases of common intracranial tumors, with purely extracranial extension. In all patients, the tumor became manifest in symptoms resembling a primary extradural entity, like chronic sinusitis, chordoma or nasal polyposis. The true diagnosis is based mainly on histopathological examination.We report on a 60-year-old woman with swelling in the left periorbital region and frontal headache at the same side. Her medical history was significant for sinus surgery 9 months before elsewhere. General ENT examination showed no recurrent nasal polyposis. Contrast enhanced magnetic resonance imaging (MRI) revealed involvement of left ethmoid and frontal sinuses. Computed tomography (CT) scans confirmed the findings with the suspicion diagnosis of a frontal sinus mucocele. The patient underwent transfacial sinus surgery according to RITTER-JANSEN ,2 perforA 16-year-old girl presented to our department with nasal obstruction and headache in the occipital region. Her medical history was significant for two turbinoplasties within two months in the last year elsewhere with persistency of the disturbances. General ENT examination showed a soft tumor in the nasopharynx. The overlying mucosa was normal in appearance.Radiological examinations included contrast enhanced CT- and MRI scans. CT scans demonstrated a well-defined expansive growing mass with a diameter of 5 cm comprising areas of calcifications and thinning and remodeling of the walls of predominantly the right pterygoid processes and clivus, but with preserved posterior and upper margin. Contrast-enhanced T1-weighted MRI showed a multilobulated, partly solid and cystic mass with central calcifications of the sphenoid cavity and clivus reaching both internal carotid arteries, and invading the ethmoid sinuses and the nasal cavity. The caudal border was the plane of soft and hard palate and the cranial border was the sella turcica. Time resolved contrast-enhanced MR angiography did not reveal any tumor blush or arteriovenoud fistulae Fig .The patient underwent transnasal endoscopic biopsy that showed adamantinomatous craniopharyngioma with focal keratinization, corresponding to WHO grade I . NavigatHistopathological examination including immunohistochemistry was performed of the tumor specimens. The tumor cells consisted were arranged in cords and bridges of multistratified epithelium with palisading of basal nuclei Fig. . NodulesA 48-day-old boy was presented with polypous mass in the left nasal cavity with resultant breathing and feeding difficulties. The preoperatively performed MRI- and CT scans showed an endonasal soft-tissue mass without intracranial connection and according to the patient's age a not-yet ossified skull base Fig. . The masThe three reports demonstrated that in all cases of intranasal or pharyngeal neoplasm the diagnosis of meningioma, craniopharyngioma and glioma should be considered as differential diagnosis. These three described tumors rarely appear in an extracranial localization.Intracranial meningiomas are the most common adult benign intracranial neoplasms, whereas extracranial meningiomas are rare tumors comprising 1–2% of all meningiomas ,5. The pExtracranial sinonasal tract meningiomas often demonstrate an erosion of the sinus wall, with extension to the surrounding soft tissue, to the orbit and occasionally to the skull base. Complete surgical extirpation of sinonasal meningioma is the treatment of choice without the need for adjuvant treatment. Relapses are possible in case of incomplete removal of the primary formation. The prognosis of extracranial meningioma is always good if the excision is complete .Craniopharyngiomas are benign but aggressive tumors deriving from cell rests from the Rathke's pouch and account for about 3% of all intracranial tumors ,9. UsualNasal glial heterotopia are rare tumors, might derive from either separated neuroectodermal tissue during the closure of the covering brain, or from a nasal encephalocele which is covered by dura, pia, and arachnoid and later disconnected from the intracranial cavity during subsequent development. They manifest usually at birth or during early childhood and can cause a visible deformation, a nasal obstruction or chronic otitis media. Nasal gliomas may occur in extrananasal (60%) or intranasal localization (30%) or combined (10%) . The treAll three present cases demonstrate that preoperative MRI and CT imaging is important for differential diagnosis to exclude intracranial connections of the tumor. A definitive diagnosis requires histopathological and immunohistochemical examination. In all cases of intranasal or pharyngeal neoplasm the diagnosis of meningioma, craniopharyngeoma and glioma should be considered as a differential diagnosis, and intracranial connections should be excluded to optimize the surgical procedure.The author(s) declare that they have no competing interests.SA and AA drafted the manuscript, TW evaluated the immunohistochemical stainings and confirmed the diagnoses, SA, WM and AA performed the surgeries and compiled the clinical data. IM evaluated the radiological diagnostics, SA and WM mainly contributed to the discussion. All authors read and approved the final manuscript.