Perivascular epithelioid cells (PECs) are constantly present in a group of tumors called PEComas, including angiomyolipoma (AML), clear-cell "sugar" tumor (CCST) of the lung and extrapulmonary sites, lymphangioleiomyomatosis, clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres and rare clear-cell tumors of other anatomic sites. PECs have distinctive morphologic, immunohistochemical, ultrastructural and genetic characteristics, including an epithelioid appearance with a clear to granular cytoplasm, a round to oval, centrally located nucleus, and an inconspicuous nucleolus. PECs also express melanocytic and myogenic markers like HMB45 and smooth muscle actin. PEComa is rare in human, and multifocal PEComas (PEComatosis) is extremely rare. Up to now there have been only less than 5 cases described in the literature. Here we report a 46-year old Chinese woman who had PEComatosis arising from the genital tract and pelvis. To our knowledge, this is the first case of PEComatosis ever reported in China.http://www.diagnosticpathology.diagnomx.eu/vs/1293097548652023.The virtual slide(s) for this article can be found here:  Perivascular epithelioid cell tumor (PEComa) refers to a family of neoplasms showing at least partial morphological or immunohistochemical evidences of a putative perivascular epithelioid cell (PEC) differentiation. PEComa may involve the visceral organs, including the lung, kidney, liver, nasal cavity, small and large bowel, appendix, prostate and uterus, and also the retroperitoneal and abdominopelvic sites. The currently reported PEComas include renal and extrarenal angiomyolipoma (AML), lymphangioleiomyomatosis (LAM), clear cell "sugar" tumor of the lung (CCST), clear cell myomelanocytic tumors (CCMMT) of the falciform ligament/ligamentum teres and distinctive clear cell tumors at other anatomic sites. PECs have a clear or acidophilic cytoplasm and express the biomarkers of melanocytic and smooth muscle. The mean age of patients with PEComa is reportedly 45 years (ranging 40-75 years old), and there is a marked female predominance . Here weIn Nov, 2010, a 46-year-old woman was referred to Department of Ggyneco-obstetrics of Changhai Hospital due to a uterus mass and a left pelvic adnexal mass, which were palpated during a routine physical examination, but presenting no abnormal vaginal bleeding and stigmata of the tuberous sclerosus complex. Serum CA-125 and CA-19-9 levels as well as other laboratory findings were all within the normal limits. Transabdominal sonography revealed a uterus mass (diameter 3.2 cm), a left pelvic adnexal cystic mass (diameter 3.7 cm) and a left pelvic mass(diameter 4.9 cm). The initial clinical impression of the lesions was uterus leiomyoma and left ovary chocolate cyst. Then the patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Intraoperative findings included smooth surface masses arising from the posterior wall of the enlarged uterus (diameter 3 cm), right part of cervix (diameter 3 cm) and the left broad ligament (diameter 4 cm), with hypertrophic ovaries, normal fallopian tubes, without ascites. An intraoperative frozen section of the uterine mass was interpreted as leiomyoma.The surgical specimens were collected and fixed in 10% formalin for H-Estaining and immunohistochemical staining. The immunophenotypes of tumor cells was examined by EnVision Plus system (DAKO Corporation). The antibodies used included HMB-45 (1:50), Melan-A(1:50), smooth muscle actin (1:50), calponin (1:100), CD34 (1:50), CD10(1:100), S-100(1:200), p53(1:50), estrogen receptor (1:80), progesterone receptor (1:40) and MIB-1 (1:200). The surgical specimens included a 9 cm × 6 cm × 6 cm uterus with left adnexae and right cavitas pelvis masses. Grossly, two round masses were found in the uterine, with a 3 cm × 3 cm × 2 cm mass in the myometrium and a 3 cm × 3 cm × 3 cm mass in the subserosa of posterior wall; a mass measuring 5 cm × 5 cm × 4 cm was found in the left broad ligament, multiple masses were found in the right cavitas pelvis, with the largest diameter being 3 cm Figure  and MelaOther specimens revealed chronic cervicitis, endometrium of proliferative phase, follicular cyst of left ovary, normal right ovary, and fallopian tubes. The postoperative course was uneventful, and the patient was discharged 4 days after surgery. No further treatment was needed; and no recurrence was noticed 12 months later as shown by transabdominal sonography of the abdomen and pelvis. She is currently followed up regularly by ultrasonography.PECs, first described by Bonetti et al , do not PEComas may arise from multiple anatomical sites, including the kidney, lung, pancreas, liver, uterus, breast, ligament teres, falciform ligament, orbit, skin and soft tissue, with the uterus and retroperitoneum appearing to be the most frequent sites of origin. The first case of uterus PEC tumor was reported by Pea et al . PEComasUterine PEComa lacks characteristic clinical and imaging changes; the diagnosis mainly relies on pathological approach, and should be differentiated from the following tumors: (1) Epithelioid smooth muscle tumor(ESMT)- the ESMT cells are round, polygonal and spindle-shaped; the spindle-shaped cells had cigarette-like nuclei with rounded ends; there was typical smooth muscle transition; they are usually HMB45 negative, without characteristic capillarity network of bloodvessels. PEComa is supplied by rich blood vessels, and the tumor cells surrounded the blood vessels, forming into patches and nests; they are often HMB45 positive. It has also been reported that a small number of ESMT cells could be HMB45 positive, but Melan-A negative; therefore double positive for SMA and Melan-A indicate a higher probability of PEComa. (2) Endometrial stromal sarcoma(ESS)-the tumor cells are spindle-shaped with less cytoplasm and negative for HMB45; PEComa cells are large and round or polygonal in shape, with rich eosinophilc cytoplasm, and are HMB45 positive. Some PEComa patients also have lymphangioleiomyomatosis and tuberous sclerosis, which has not reported in ESS patients. (3) Uterine clear cell carcinoma (UCC)-the tumor is composed of cells with clear cytoplasm and a hobnail morphology, which forming into solid, ductal or papillary shapes, negative for HMB45 and positive for CK. (4) Metastatic renal clear cell carcinoma (MRCcc)-the cytoplasm is clear and polygonal shaped; the round tumor cells arranged into nest, alveolar, ductal, or papillary shapes, but with no perivessel structure; the tumor is HMB45 negative, and CK, EMA positive. (5) Paraganglioma-it should be differentiated with PEComa when the cytoplasm is clear. Paraganglioma cells are arranged in streaks, glands, or nests, with flat supporting cells lining around, and with distinct organoid and pseudorosette structure; besides, they are HMB45 negative, and NSE, Syn, CgA and NF positive; the supporting cells are positive for S-100 protein.PEComas have been reported as predominantly benign. Greene  believedDue to the multicentricity of the lesions, we favor the designation of PEComatosis proposed by Fadare et al . PEComatWhether the multiple foci of PEComas are truly multicentric or whether they originate from a single primary site remains to be further investigated. To the best of our knowledge, the present case is the first PEComatosis in China. Treatment of PEComas is challenging since chemotherapy and radiotherapy seem not effective, and surgery is currently the most important treatment of these tumors.The authors declare that they have no competing interests.WY first identified this case,retrieved clinical information and wrote the manuscript. GL and ZW-q proposed the studies and provided valuable insight during manuscript preparation. All authors read and approved the final manuscript.


Cystic hygroma is a rare congenital malformation of the lymphatic system. It is most frequently detected in the head and neck region. Ultrasonography is performed as the first step for radiological diagnosis of these lesions. Magnetic resonance imaging provides important information for diagnosis and to guide treatment. In this paper, a case is reported of cervicomediastinal large cystic hygroma in a male infant. Cystic hygroma is a rarely encountered congenital malformation of the lymphatic system, which is most often observed in the head and neck region. Ultrasonography (US) is used as the first step in radiological diagnosis of these lesions. However, magnetic resonance imaging (MRI) is being more frequently used to evaluate the lesion structure, dimensions and extent and to determine the treatment plan and appropriate treatment approach . The aimA 2-year-old male patient presented at the clinic with complaints of a swelling on the left side of the neck, which had been present since birth and seemed to have increased in size over the previous 2 - 3 months. Physical examination determined a mass lesion in the left cervical region reaching approximately 6 cm in size, slightly mobile and pressurized, with no changes in skin color or temperature, leading to posture impairment of the neck. In the laboratory findings, in the acute phase reactants there was a slight increase in lymphocytes and a mild level of iron deficiency anemia. Other routine laboratory results were normal. B-mode USG examination was directed to the cervical region. A complex cystic lesion was observed to be located in the left anterior cervical region, extending intrathoracically.MRI examination was made to evaluate the extent of the lesion and details of the internal structure. From the MRI examination, the lesion was determined to be located on the left half of the neck in the infra-suprahyoid region, extending into the retropharyngeal cavity, to the posterior cervical triangle and the intrathoracic left superior mediastinum. The internal jugular vein was completely surrounded by the lesion. The multilocular, septated lesion with lobular contours and thick walls was separated from the surrounding tissue with clear borders. In T1-weighted images, the lesion was seen on intermittent signals depending on hemorrhagic and/or proteinous content and on T2-weighted fat suppressed images there was evident hyperintensity. On contrast T1-weighted fat suppressed images there was only contrast of the thick septa and wall , 2a, 2b.Cystic hygroma (lymphangioma) is a rarely seen congenital malformation of the lymphatic system, which most often involves the head and neck region . Half ofIn the diagnosis of cystic hygroma, US, CT and MRI can be used. Various thicknesses of septa are seen as anechoic, multilocular cystic masses on US. However, US is not sufficient to extend to the retropharyngeal, axillary and mediastinal view . On CT iSmall lymphangioma which do not create functional impairment or aesthetic problems do not require interventional treatment. However, treatment is unavoidable for lesions located in the cervicomediastinal region, particularly when respiration and feeding are inhibited. Most lesion treatment is based on surgical removal , 9. AnotIt can be concluded that in cystic hygroma located in the cervical region, MRI examination is of great benefit for correct evaluation of the lesion extent, dimensions and internal structure and thus selection of the correct treatment.


A 72-year-old Caucasian male who presented with haematuria in July of 2000 was found to have a large left-sided bladder tumour. He underwent a transurethral resection of the tumour and surveillance program. In October 2008 he underwent a transurethral resection of the prostate (TURP). Histology of theprostatic chippings showed poorly differentiated TCC with prostatic invasion. A CT of his chest abdomen and pelvis revealed no lymph node involvement or metastatic spread. He therefore underwent a cystoprostato-urethrectomy withileal conduit formation, in December 2008. In May 2010 the decision was made to perform a left inguinal orchidectomy as he presented with a craggy mass of his left testis, and there were clinical concerns that this was atumour. Histology revealed that the left testis had been wholly replaced by a tumour. Taking into account his previous urological history, the features of this tumour are consistent with metastatic TCC, which is very rare. A 72-year-old Caucasian male who presented with haematuria in July of 2000 was found to have a large left-sided bladder tumour, and urine cytology revealed malignant cells. A renal ultrasound scan (USS) at the time was normal. Transurethral resection of the tumour (TURBT) showed a G2 pT1 transitional cell carcinoma (TCC) of the bladder. A check cystoscopy in 2002 revealed a red patch over the previous resection site; and biopsies revealed transitional cell carcinoma in situ (Tis) which was managed by a six-week course of intravesical BCG. He continued cystoscopic surveillance, unfortunately in March 2005 further cystoscopic biopsies again revealed transitional cell carcinoma in situ (Tis), this was managed by further instillations of intravesical BCG. He again underwent a surveillance cystoscopy programme with biopsies when indicated, which were unremarkable until June 2008, where biopsies showed a reoccurrence of Tis again managed by six-week course of intravesical BCG.In October 2008 he underwent a transurethral resection of the prostate (TURP) as he had presented with acute retention of urine. Histology of the prostatic chippings showed poorly differentiated TCC with prostatic invasion. A CT of his chest abdomen and pelvis revealed no lymph node involvement or metastatic spread. He therefore underwent a cystoprostato-urethrectomy with ileal conduit formation, in December 2008. Surveillance CT scans (including urogram phases) in June and November 2009 were unremarkable.He presented to the urology department in January 2010 complaining of a 2-week history of left testicular pain. Examination was unremarkable, but a scrotal USS suggested epididymo-orchitis as a potential diagnosis; thus, a 2-week course of ciprofloxacin (500 mg twice daily) was prescribed and his symptoms improved. However his pain never really settled but he did not represent until May 2010. On this occasion examination revealed a craggy mass and generalised scrotal swelling. A scrotal USS revealed an enlarged testis with hypervascularity compatible with orchitis . The decision was made to perform a left inguinal orchidectomy to clinical concerns that this was a tumour. Histology revealed that the left testis had been wholly replaced by a tumour composed of islands of pleomorphic cells with surrounding desmoplastic stroma and the ghost outlines of atrophic seminiferous tubules. Immunohistochemical studies showed the tumour to be strongly positive for AE1/AE3 and CK7, whilst it was focally positive for CK20 and negative for PLAP, PSA, CEA and vimentin. Taking into account his previous urological history, the features of this tumour are consistent with metastatic TCC.Urgent CT and bone scans were arranged (June 2010). The bone scan was unremarkable; unfortunately CT revealed abdominal para-aortic lymphadenopathy which is new since the CT in November 2009 and almost certainly due to further metastases , lung (36%), bone (27%), and adrenal glands (21%). It is important to note that haematogenous spread may involve any organ .There are a number of important learning points from this case. This gentleman presented with Tis in 2002, 2005 and 2008, which seemed to respond to BCG each time it was reinstigated, and perhaps a radical cystectomy should have been performed at this point as recommended by the European Association of Urology (EAU) [A key learning point is the fact that metastatic disease to the testis was never considered as a diagnosis until the histology was obtained from orchidectomy; however, this is understandable due to its rarity. Also, as this man no longer had a bladder, therefore he is unlikely to develop epididymitis secondary to urosepsis.In conclusion a diagnosis of metastatic transitional cell carcinoma of the bladder presenting at any site must be considered in all men with bladder neoplasia.


Arthroplasty is a well-established routine elective surgical procedure in orthopaedics. To a great extent, diagnosis, treatment and post-operative rehabilitation in these patients is standardised. In a busy clinic, surgeons from time to time tend to focus their attention on common causes of joint pain, but it may lead them to overlook sinister but less common pathologies. Here we report a case of a patient with groin pain due to pre-operatively undetected pelvic metastases from a pyeloureteral carcinoma who underwent total hip arthroplasty. There are several case reports which deal with primary or secondary tumours which were either discovered at the time of replacement surgery or developed at the site of prosthesis years after total hip or knee replacement. To the best of our knowledge, this is the first case report in which a metastatic cancer was missed pre-operatively and intra-operatively both by the radiologist and by the orthopaedic surgeon and should be reported so that surgeons are reminded to be careful when dealing with seemingly routine cases.A 79-year-old Caucasian woman presented to the arthroplasty clinic with groin pain. Initial radiographs showed subtle bilateral abnormalities in the pelvis. Neither the radiologist nor the orthopaedic surgeon recognized it. A diagnosis of osteoarthritis of the hip was established, and she underwent total hip arthroplasty. Despite initial improvement, the patient came back with worsening hip pain three months later. Further radiological examination revealed multiple metastatic lesions throughout the pelvis due to a pyeloureteral carcinoma.This case report emphasizes the importance of meticulous, unbiased pre-operative assessment of patients and their radiographs, even in so-called routine clinical cases. Often subtle radiological changes are classed as normal, especially if they are bilateral. Further radiological imaging should be recommended in all cases where unexplained clinical features or radiological findings are present. Total hip arthroplasty (THA) is a well-established routine surgical procedure in orthopaedics -3. To a Here we report a case of a patient with groin pain due to pre-operatively undetected pelvic metastases from a pyeloureteral carcinoma who underwent THA. There are several case reports that deal with primary or secondary tumors which have either been discovered at the time of replacement surgery or developed at the site of prosthesis years after total hip or knee replacement -9. To thet al. [Upper urinary tract tumours are uncommon and constitute 5% to 7% of all urinary tract tumours . A ratioet al. ,13 showeet al. ,13.A 79-year-old, active Caucasian woman with left groin pain was referred to the orthopaedic clinic by her general practitioner to undergo THA. She complained about progressive left groin pain for 12 months that was worst in the morning and exacerbated by physical activity and prolonged sitting. Clinical examination revealed left-sided antalgic gait and tenderness in her left groin. Left hip internal rotation was limited to 10°. Anteroposterior pelvic and true lateral hip radiographs revealed typical signs of osteoarthritis Figure . FurtherMany case reports have been published with regard to tumours developing at the site of arthroplasty -9. It maInterestingly, the patient's surgery was postponed because of microscopic haematuria, which was interpreted as a lower UTI. However, haematuria is one of the most common findings in urothelial carcinoma, although it is not specific and is also present in cases of UTI . The quesine qua non for the establishment of the correct diagnosis.This case report is of particular importance to all radiologists and any surgeon involved in elective orthopaedic surgery. It highlights the importance of avoiding the pigeon-holing of patients with specific symptoms into specific diagnostic categories to correctly diagnose the outliers. Further, radiographs should be analyzed in a systematic, standardized and complete manner, taking every visible structure into account. In the presence of bilateral subtle abnormalities, further radiological imaging such as magnetic resonance imaging or single photon emission tomography should be recommended. In conclusion, the importance of an unbiased assessment of patients and their radiographs is a 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.PK reviewed the case and drafted the manuscript. FI participated in drafting the manuscript and case review. TS participated in drafting the manuscript and literature review. NFF participated in drafting the manuscript and case review. MTH reviewed the case and drafted the manuscript. All authors read and approved the final manuscript.


The malignant transformation of abdominal wall endometriosis is a rare event and poorly understood. Less than 30 cases have been reported in the literature. Most of the reported cases have a solitary tumor in the abdominal scar. A few cases have metastasis. Here we report a case of clear cell carcinoma in abdominal wall endometriosis with bladder and lymph system metastasis. The patient had a history of abdominal wall endometriosis and recently developed symptoms of urgent urination and inguinal mass. Physical examination and a computed tomography (CT) scan detected lymph node metastasis. CT and cystoscopy confirmed bladder involvement. The patient underwent extensive surgery and chemotherapy. Pathological analysis made a diagnosis of clear cell carcinoma with bladder and lymph node metastasis. The patient was followed up and died of the disease. Symptoms of bladder invasion and lymph node spread could be a sign of malignant transformation. Local invasion and lymph node spread are two important forms of tumor metastasis. Extensive lymph nodes metastasis might be related with poor prognosis. Endometriosis is defined as the presence of endometrial glands and stroma outside the uterine cavity. It affects about 15% of women of childbearing age. The most common site of endometriosis is in the pelvic cavity but it occasionally occurs in the extrapelvic area. Abdominal wall endometriosis represents 1% to 2% of all endometriosis lesions [A 39-year-old woman with a history of abdominal wall endometriosis presented to our clinic complaining of urgent urination and an emerging inguinal mass. She had a CS due to a breech presentation in 1994. She noticed a mass in the CS scar with cyclic pain in 1999. The mass was excised and diagnosed as abdominal scar endometriosis histologically. The margin was free and no further treatment was given after surgery. In 2004 a similar mass with cyclic pain was detected in the scar again. The mass grew gradually and was diagnosed as recurrent abdominal wall endometriosis without further treatment. She also developed urgent urination and dysuria during her menstrual period. In the last 4 months she noticed masses emerging in the bilateral inguinal area with mild pain.A physical examination showed a solid 6 cm × 5 cm mass without a clear borderline near the symphysis pubic in the previous longitudinal scar. Two enlarged lymph nodes with diameters of 2 cm and 1 cm in the right groin, and one lymph node with a diameter of 1 cm in the left groin were detected. Serum CA125 was 22.1 U/ml. Computed tomography (CT) scans showed that the mass had invaded the bladder and that the inguinal lymph nodes were enlarged (Figure The patient recovered uneventfully but refused further treatment after three cycles of chemotherapy with carboplatin and paclitaxel. She sought traditional Chinese herbal medication. Ten months after chemotherapy, tumor recurrence was detected in the pelvic cavity and the patient died of the disease two months later.Malignant transformation of endometriosis is quite rare, affecting 1% of women suffering from endometriosis. The most common sites of malignant transformation of endometriosis are the ovaries. About 20% of cases occur in extragonadal sites including the rectovaginal septum, colon and vagina [Sampson  proposedMalignant transformation of endometriosis in the abdominal wall can invade all layers of the abdominal wall and grow as large as 10 cm in diameter . HoweverLocal invasion is one important biological characteristics for malignant transformation of endometriosis in the abdominal wall; however, it can also spread through the lymph system. Three other cases with lymph metastasis have been reported in the literature Table . For theThe diagnosis of malignant transformation of abdominal wall endometriosis is still a challenge for gynecologists. There are no characteristic symptoms and markers during malignant transformation. Imaging can detect endometrioma and its fast growth. Malignant transformation is only suspected when the tumor has a solid or mixed component. In the present case, the patient developed urgent urination and an inguinal mass, which could be a sign of tumor invasion. Therefore, malignant transformation and metastasis were highly suspected before surgery. Accordingly, we were able to make a detailed plan and prepare for extensive surgery.The best treatment for malignant transformation of endometriosis in the abdominal wall is unknown. Radical surgery with a wide resection is believed to be the primary treatment . ChemothMalignant transformation of endometriosis in the abdominal wall is a rare complication and poorly understood. Symptoms of bladder invasion and lymph node spread could be a sign of malignant transformation. Local and lymph system invasion are two important routes for tumor metastasis. Extensive lymph node metastasis is probably related with poor prognosis.Written informed consent was obtained from the patient for publication of this case report.CS: Cesarean section; CT: computed tomography.The authors declare that they have no competing interests.HL collected clinical information, searched the relevant literature and wrote the draft. JL provided the case, followed up the patient and approved the manuscript. JL and HS participated in the surgery and revised the manuscript. QC performed the pathological analysis and provided the images in the manuscript. All authors approved the final manuscript.


Mediastinal tuberculous lymphadenitis is a rare disease in adults. Dysphagia as the accompanying symptom is even a rarer manifestation. Cases with esophageal symptoms may present as esophageal ulceration, mucosal or submucosal mass, fistula or sinus formation, extrinsic compression or displacement of the esophagus. Extrinsic compression may radiologically or endoscopically present as a submucosal tumor. Our case is a 30-year-old woman with dysphagia for a month. Extrinsic compression was seen endoscopically on the mid-esophagus. On thoracic CT and MRI images, a multiloculated cystic/necrotic mass 5.5×4.8×3.1 cm in size consisting of multiple septa was located subcarinally in the middle mediastinum. In Wang needle aspiration, a mucopurulaent liquid was aspirated from the subcarinal localization by bronchoscopy. Diagnostic thoracotomy was carried out because histological and bacteriological examinations were not diagnostic. It was reported as tuberculous lymphadenitis pathologically. The control thoracic CT performed after antituberculous treatment showed complete regression of the mass. We herein report a rare form of tuberculous lymphadenitis. Dysphagia due to mechanical obstruction of the esophagus is often related to a malignant esophageal disease. Benign lesions causing such obstructions are quite rare and they tend to manifest themselves as intramural tumors or extrinsic compressions. Lymphadenitis due to mediastinal tuberculosis is rarely seen in adults; dysphagia caused by this disease is much more rare. DysphagiA 30-year-old female patient was admitted to an internal medicine clinic with the complaint of difficulty on swallowing for a month and endoscopy revealed external compression on the middle part of the esophagus. She was referred and admitted since the chest MRI revealed a subcarinal mass in the middle mediastinum. The past medical history revealed a surgery for hydatic liver cyst.The physical examination revealed a blood pressure of 110/70 mmHg, pulse of 88/ min and a respiratory rate of 18/min. All system findings were normal. Laboratory findings revealed minimal hypochromic microcytic anemia in complete blood count. Biochemical tests were normal. The erythrocyte sedimentation rate was 32/h. Arterial blood analysis revealed slight hypoxemia. Lung function tests revealed medium obstruction and restriction .The chest X-ray was normal. Lung CT and MRI examinations showed a subcarinally located 5.5×4.8×3.1 cm mass in the middle mediastinum, which had a multilocular cystic/necrotic appearance with multiple septa . No endoMediastinal tuberculous lymphadenitis cases with esophageal symptoms may admit in forms of esophageal ulceration, mucosal or submucosal mass with ulceration, sinus or fistula formation, external compression or dislocated esophagus. In case of extreme external compression, the disease may be presented as submucosal tumor radiologically or endoscopically. Our caseDifferential diagnosis of formations radiologically presenting as a mediastinal mass is quite important. Generating a differential diagnosis for a mediastinal mass starts with a classification scheme. The system used by Felson divides the mediastinum into anterior, middle and posterior compartments by drawing a line along the front of the trachea and the back of the heart and a second line 1 cm posterior to the anterior margin of the thoracic vertebra. This system does not classify the superior mediastinum as a separate compartment.For anterior mediastinal masses, the classic differential diagnosis is the “4 Ts”; namely, thymoma, thyroid, teratoma and terrible lymphoma. Further clues may be obtained from the radiographic appearance, the patient’s age and associated clinical manifestations. For example, mediastinal thyroid is rare if there is not demonstrable direct extension from the neck. Calcification, teeth and/or fat in a lesion are in favor of teratoma. Lymphoma is the likeliest diagnosis when there is a multilobular mass. Thymoma often, although not always, overlies the aortopulmonary window.6]Swollen lymph ganglions due to inflammation are called reactive adenopathy, which are seen as oval formations, up to less than 1.5 cm in diameter with a homogeneous content and contrast uptake on CT and MRI images. Central nodal necrosis secondary to intranodal abcess formation may develop in some infections such as staphylococcal infections and is called suppurative adenopathy. Differential diagnosis of suppurative adenopathy and malignant adenopathy is not possible with imaging tool examinations. Multiple lymph ganglions with contrast uptake, central necrosis and globular calcifications are seen radiologically in tuberculous adenitis. Nodal calcifications are mostly seen in tuberculosis, but may also be seen in metastatic papillary thyroid cancer, in lymph ganglions after radiotherapy or in necrotic ganglion abcesses in the healing period. Egg shell calcifications are typically seen in silicosis, sarcoidosis, tuberculosis and amiloidosis.[Lymphadenitis is the most common form of extrapulmonary tuberculosis. The infection often begins in the lungs; therefore, the most common infected sites are the regional lymph nodes, into which the lung parenchyma drains. To understand the course of spreading of the infection one should know the lymph node drains well.1-Anterior mediastinal group: They receive afferent vessels from the thymus, thyroid, heart and pericardium, diaphragmatic and mediastinal pleura and middle diaphragmatic nodes.2-Paratracheal and tracheobronchial groups: These groups receive drainage from most parts of the lungs and bronchi, thoracic trachea, heart and some efferents from the upper para-esophageal nodes of the posterior mediastinal group. The nodes comprising these groups include the upper  and lower  paratracheal, subaortic , retrotracheal (station 3), and subcarinal station 7) nodes. The subcarinal nodes are contiguous with the hilar nodes and drain into the paratracheal nodes, preferentially to the right.[ nodes. T3-Posterior mediastinal group: The posterior mediastinal nodes comprises the para-esophageal (station 8) and pulmonary ligament (station 9) nodes. The efferents from the posterior mediastinal nodes.communicate with the tracheobronchial group, particularly subcarinal nodes and drain chiefly into the thoracic duct, but also drain to the subdiaphragmatic para-aortic or coeliac nodes.Lymph nodes are located along the bronchi and may be divided into hilar  and intrapulmonary nodes. The latter consist of interlobar , lobar , segmental , subsegmental  and intraparenchymal intrapulmonary nodes. Most of During primary tuberculosis infection, bacilli reach from the primary focus in the lung to the nearest lymph nodes  through the lymphatic way and then to the further lymph nodes. They may also spread hematogeneously to reach other lymph nodes, in which they survive for years as dormant bacilli. Tuberculous lymphadenitis most commonly develop after primary infection, followed by endogenous reactivation of dormant bacilli after years and finally as a result of an infection in the neighborhood. AlthoughSuccessful control of tuberculosis decreases lung tuberculosis cases, but extrapulmonary tuberculosis (EPT) cases do not decrease in a similar ratio. ReportedTreatment with anti-tuberculosis medications is effective and surgery is only needed in the presence of complications. Our case


Dear SirImperforate hymen is the most frequent obstructive anomaly of the female genital tract. It can be diagnosed on prenatal ultrasound as bladder outlet obstruction, due to hydrocolpos or mucocolpos  and postCase 1: A 21-day-old female baby was presented with complaint of urinary retention. Since bladder was palpable on examination, catheterization was done by the nursing staff on advice of a resident. Urinalysis and ultrasound were advised which turned out to be insignificant. On 5th day of admission it was noticed that despite catheterization lower abdomen was still distended. Perineal examination done at that time revealed imperforate hymen (Fig. Case 2: Twelve years old female presented with lower abdominal mass. She was catheterized elsewhere for urinary retention but mass persisted. She was admitted and CT scan abdomen advised which picked up a cystic mass in pelvis (Fig. Critical thinking is about how we approach to the problems, questions and issues . LearninHyposkilliacs are “physicians who cannot take an adequate medical history, cannot perform a reliable physical examination, cannot critically assess the information they gather, cannot create a sound management plan, have little reasoning power, and communicate poorly. They learn to order all kinds of tests and procedures but don't always know when to order [them] or how to interpret them” The requisite of producing good clinicians is to create an environment of inquiry. The learner or clinician will analyze and evaluate the patient and disease with his/her cognitive skills only if his brain is primed to do so. Otherwise the culture of technological overuse will keep on flourishing; and we will end up in producing clinicians unable to pick up simple clinical diagnoses with poor basic knowledge and skills. This is high time to revisit the training methods for junior doctors, to be addressed conscientiously.Source of Support: NilConflict of Interest: None declared


Arteriovesical fistulas are extremely rare. Only eleven cases were previously reported in the literature. They can occur iatrogenically, traumatically or spontaneously.We report an unusual case of a 62-year-old woman with arteriovesical fistula that developed fatal hematuria after transurethral electrocoagulation. Computed tomography (CT) and selective angiography revealed a pseudoaneurysm of the right superior vesical artery with arteriovesical fistula formation, which was managed by transarterial embolization.Contrast enhanced CT or CT angiography should be performed when a pulsatile hemorrhage is revealed during cystoscopy. Therapeutic vesical arterial embolization should be considered as a safe and effective procedure for arteriovesical fistulas. Transurethral electrocoagulation may cause severe hematuria for pulsatile bladder bleeding in patients with pelvic vascular malformation. Arteriovesical fistulas are extremely rare. To our best knowledge, only eleven cases were previously reported in the literature. They can occur iatrogenically, traumatically or spontaneously . We repoA 62-year-old woman presented to our outpatient clinic with an 8-day history of painless gross hematuria. She had seen local general physician a few days before this presentation and was prescribed antibiotic for a presumed urinary tract infection, which helpless. She has no significant past medical history and physical examination was unremarkable. Ultrasonography revealed normal upper urinary tracts and a long strip, 4.2 cm × 1.2 cm sized, hypoechogenic mass floating in the bladder, with its pedicle located at the bladder base  revealed complete occlusion of superior vesical artery, and a duplex cystoscopy showed nomoral bladder Figure . The patHematuria due to arteriovesical fistula is an extremely rare clinical entity and we identified only a few cases in the literature. Arteriovesical fistulas can occur iatrogenic injury -6, seconA pseudoaneurysm, also known as a false aneurysm, is a leakage of arterial blood from an artery into the surrounding tissue with a persistent communication between the originating artery and the resultant adjacent cavity. The development of pseudoaneurysm of a vesical artery is extremely rare, and Pontin et al reported one similar case due to radiation therapy . In thisEstablishing a precise diagnosis of superior vesical arterial pseudoaneurysm is often difficult since it used to be of rare occurrence. Still, it is important to be aware that vascular abnormality, such as aneurysm, fistula or malformation, could be a possible diagnosis for hematuria, especially when the bladder hemorrhage refractory to bladder irrigation, intra-vesical instillation or endo-urological intervention. Or characteristic pulsative bleeding was presented in cystoscopy inspection. Contrast enhanced CT or CTA would be a useful tool to identify the bleeding source.There is no agreement in the literature on a specific course of treatment for arteriovesical fistula. Management options for the patients include embolization, open repair and aneurysm resection . SelectiIn summary, we report a case of arteriovesical fistula formed spontaneously that developed fatal hematuria after transurethral electrocoagulation. Awareness of this as a possible cause of hematuria can assist in immediate diagnosis and appropriate treatment. Contrast enhanced CT or CT angiography should be performed when a pulsatile hemorrhage is revealed during cystoscopy. Therapeutic vesical arterial embolization should be considered as a safe and effective procedure for bleeding control. Transurethral electrocoagulation may cause severe hematuria for pulsatile bladder bleeding in patients with pelvic vascular malformation.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 of this journal.CT: Computed tomography; CTA: Computed tomography angiography.The authors declare that they have no competing interests.XYZ, YWL drafted the manuscript. BC, XYZ, XFZ and LPX also assisted with manuscript preparation. YHS revised the manuscript. All authors have read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2490/13/68/prepub


Vaginal primary malignant melanoma is a rare and very aggressive tumor. It most commonly occurs in postmenopausal women, with a mean age of 57 years. Our patient is an 80-year-old, postmenopausal Greek woman presented with a complaint of abnormal vaginal bleeding. On gynecologic examination there was a pigmented, raised, ulcerated, and irregular lesion 5 × 4.5 cm in the upper third of anterior vaginal wall. She underwent a wide local excision of the lesion. The histopathology revealed vaginal primary malignant melanoma with ulceration and no clear surgical margins. She denied any additional surgical interventions and underwent to postoperative adjuvant radiotherapy. Follow up 5 months after initial diagnosis revealed no evidence of local recurrence or distant metastasis. The prognosis of vaginal primary malignant melanoma is very poor despite treatment modality, because most of the cases are diagnosed at advanced stage. Particularly patients with no clear surgical margins and tumor size >3 cm needed postoperative adjuvant radiotherapy. Vaginal primary malignant melanoma (VPMM) is a rare and very aggressive tumor , 2. It aThe estimated incidence of VPMM is 0.026/100,000 women per year . It mostThe precise etiology of VPMM is relative unknown . It is tOur aim is to present a case of VPMM that underwent a wide local excision and postoperative adjuvant radiotherapy.The patient, an 80-year-old, gravida 3, para 2 postmenopausal Greek woman, presented to the Department of Obstetrics and Gynecology of the University of Patras Medical School with a complaint of abnormal vaginal bleeding. Her medical history included hypertension and diabetes mellitus. She had menopause at the age of 50. Her surgical history was unremarkable. Her family history revealed no evidence of cancer among the first-degree relatives.On gynecologic examination there was a pigmented, raised, ulcerated, and irregular lesion 5 × 4.5 cm in the upper third of anterior vaginal wall. There were no palpable inguinal lymph nodes, and the rest of pelvic examination was normal. Preoperative computer tomography (CT) of the abdomen and pelvis, abdominal ultrasound (U/S), chest X-ray, intravenous pyelography (IVP), colonoscopy, and urethrocystoscopy was normal.She underwent a wide local excision of the lesion. The histopathology showed a malignant neoplasm consisting of tumor cells mainly with epithelioid and less with spindle cell morphology . There w192Ir due to age, performance status, and comorbidities. The entire length of vagina was treated with HDRB in five weekly outpatient fractions using a vaginal cylinder 3 cm in diameter. Relying on the radiobiology of cutaneous malignant melanoma (CMM), a dose of 8 Gy given to a depth of 0.5 cm into the vaginal mucosa was administered with each treatment, for a total dose of 40 Gy [The patient denied any additional surgical interventions and underwent postoperative adjuvant radiotherapy. She received only high dose rate brachytherapy (HDRB) with of 40 Gy .Followup 5 months after initial diagnosis, with CT of the abdomen and pelvis, abdominal U/S, chest X-ray, IVP, colonoscopy, and urethrocystoscopy, revealed no evidence of local recurrence or distant metastasis.The precise histogenesis of VPMM is relatively unknown . ProbablAlthough VPMM might arise anywhere, it is primarily found in the lower one third 34%) and mostly on the anterior wall 38%) of the vagina , 4. VPMM% and mos% of the The most common symptoms and signs in patients with VPMM are vaginal bleeding 80%), vaginal discharge (25%), palpable vaginal mass 15%), and pain (10%) 0%, vagin, 15. The%, and paEpithelioid is the most common histologic cell type of VPMM (55%) , 8, 13. Although there are several treatment options for VPMM, an appropriate and effective treatment protocol has not been defined yet . In thosLymph node dissection is not recommended in patients with VPMM, because the rate of lymph node metastasis is low . Also thRadiotherapy can be applied as primary treatment for patients who are unable or unwilling to have surgery , 18, 19.Dacarbazine (DTIC) has been the standard of care for many years in patients with advanced stage CMM, with response rates of 7.5% to 12.1% . HoweverImmunotherapy with interferon (IFN) or interleukin-2 (IL-2) confers survival benefits in patients with VPMM at high risk for recurrence, but toxicity is important , 22–24. The combination of chemotherapy and immunotherapy (biochemotherapy) in patients with advanced stage CMM is associated with an increased response rate . HoweverVPMM is a very aggressive tumor, and most patients are diagnosed at advanced stage , 22, 28.Despite treatment modality, 5-year survival in patients with VPMM ranges from 8.4% to 17.5% , 9. TumoIt is obvious that the prognosis of VPMM is very poor despite treatment modality, because most cases were diagnosed at advanced stage , 15. Als


Osteosarcoma (OS) is a malignant mesenchymal tumor, which rarely occurs in the maxilla. Although variable histologic and radiographic features of OS have been reported previously, in the majority of the cases painful swelling of the jaw is mentioned as the first clinical presentation. Furthermore, early diagnosis and wide surgical resection of the tumor are the most important determinant factors of prognosis. Therefore, the unusual clinical presentations of OS should be considered meticulously to expedite the diagnosis process. We describe a case of OS of the maxilla with extremely unusual presenta-tion in a 42-year-old female, that was initially designated as “epulis fissuratum”. Here, we highlight the importance of com-bining the clinical, radiographic and histopathologic examination to obtain a definitive diagnosis and also the significance ofearly effective surgical intervention in evaluation of pathologic lesions. The maxilla is less commonly affected than the mandible and in the maxilla the majority of OSs arises in the alveolar ridge and the maxillary antrum. Symptoms usually include painful swelling in the area and loosening of teeth, although paresthesia, nasal obstruction and ophthalmic complications such as proptosis may be noted.3 Hence, early diagnosis, especially in OSs with unusual clinical presentations, has a great impact on treatment planning and prognosis of the patients.The management of all variants of osteosarcoma, including low-grade, intramedullary, and juxtacortical osteosarcomas, are identical with complete surgical excision of the tumor together with wide safe surgical margins. Although other treatment modalities such as chemotherapy and radiation therapy have been proposed, the majority of the tumors are clinically identified in advanced stages and require combination treatment.The authors would like to report a case of OS of the maxilla with extremely unusual presentation in a 42-year-old woman and also describe a comprehensive evaluation of the patient, which led to definitive diagnosis and early effective treatment.A 42-year-old female patient was referred to the Faculty of Dentistry of Babol University of Medical Sciences by a dentist due to dull pain on the left side of her face.On the clinical examination the patient, who had been wearing an upper denture for about 5 years, reported a dull pain since two months earlier on the left side of her face, which was attributed to the irritation of the prosthetic appliance fabricated by the general practitioner, who had examined the patient previously and made a diagnosis of “epulis fissuratum”.Extra-oral examination was unremarkable, no palpable lymph node at any level of the head and neck region was detected and no contributory past medical history was noted.Intra-oral examination revealed a firm and mild swelling overlaying the buccal and palatal aspect of the left maxillary alveolar ridge at the left premolar teeth area . It exteConventional panoramic radiograph revealed a poorly defined mixed-density lesion . FurtherTherefore, after combining the clinical examination and radiographic evaluation, under the title of an “aggressive bone-forming neoplasm” the following differential diagnoses were proposed: 1) intramedullary osteosarcoma; 2) aggressive osteoblastoma; and 3) ossifying fibroma. Then the patient was referred to the Oral and Maxillofacial Surgery Department for an incisional biopsy.Microscopic examination of the incisional biopsy revealed heterogeneous neoplastic tissue composed of interlacing fascicles of spindle-shaped cells with plump nuclei and prominent nucleoli . In addiConsidering the size of the lesion and its extension according to the clinical findings and radiographic evaluation on the CT scan , the surFinally, the gross surgical specimen was sent for detailed histopathologic examination. The patient was supposed to be rehabilitated occlusally and functionally afterwards, using an obturator.Histologic evaluation of the excised specimen confirmed the initial diagnosis. All the surgical margins were free of tumor.The patient has been under close supervision clinically and radiographically for six months after the surgery and no evidence of local recurrence or distant metastasis has been observed to date.6OSs of the jaws are very rare and have a mild male predilection. At the time of diagnosis, the patients are approximately 2 decades older than those with extragnathic OS, which have a peak incidence between the ages of 10 and 14 years. Head and neck OSs have lower tendency to distant metastasis than their long bone counterparts, and they have a better 5-year survival rate.7 and demonstrate a high mortality rate associated with local invasion that is usually difficult to control.8 Early diagnosis and wide surgical resection is the most important factor for prognosis, which results in a 5-year survival rate of 80%.7 Therefore, when the pathologic features on a biopsy specimen of the jaws are in favor of a bone-forming neoplasm, such as the current case, clinical, radiographic and histopathologic correlation is strongly recommended in establishing an accurate diagnosis.However, most Gnathic OSs are high-grade lesions10 or small cell11 subtypes and variable radiographic appearances3 have been reported previously, which confirm the importance of meticulous evaluation of the patients with intraosseous lesions resembling OS; but a few cases with uncommon clinical presentation have been described and based on our knowledge the present case is the first reported OS with clinical presentation similar to “epulis fissurarum”. In other reported rare clinical or radiographic presentations of OS, Soares et al2 reported a patient with OS erroneously diagnosed as a periapical lesion and this delay in accurate diagnosis led to death of the patient after about 2 years. Yamamoto et al12 described a case of maxillary OS with extremely unusual image findings in an 11-year-old boy and associated this strange feature with initial phase of tumor progression. Babazade et al13 reported an uncommon bilateral metachronous OS of the mandibular body in a 27-year-old man and recommended that physicians should be aware that OS can occur in different sites as a true multicentric lesion.Several histologically unusual features of OS such as epitheliod14 The presence of extremely heterogeneous features makes it very difficult to diagnose OS by only interpretation of a small incisional biopsy, especially when similar, osteoblastoma-like features are encountered.15 In our case, excluding the radiographic findings, the fascicular pattern of spindle-shaped cells, in the absence of distinct pleomorphism and significant numbers of mitotic figures, could be easily misinterpreted as a benign mesenchymal origin spindle cell tumor such as ossifying fibroma or desmoplastic fibroma. Therefore, it is strongly recommended that the incisional biopsies of the lesions suspected to be OS should be the best and the most abundant specimens possible and conventional radiographs be requested on peripheral reactive lesions such as peripheral ossifying fibroma, pyogenic granuloma and epulis fissuratum and the final diagnosis be rendered essentially after correlation of radiographic and pathologic findings.3Microscopically, the essential criterion of OS is direct production of osteoids or immature bone by tumoral cells. Three more common histologic variants including osteoblastic, chondroblastic and fibroblastic and several less common subtypes such as epithelioid and telangiectatic have been described. It seems these histopathologic subtypes do not have any great impact on the prognosis.8 Fernandes et al16 in a retrospective review of 16 gnathic OSs demonstrated no statistically significant differences in survival between patients who had received chemotherapy and those who had not been treated this way. In addition, Granowski-LeCornu et al17 determined improved survival in treatment of OS of the jaws in patients benefiting from improved imaging, earlier diagnosis and more aggressive treatment that included improved surgical clearance and additionally demonstrated no clear survival advantage for neo-adjuvant chemotherapy. However, it should be pointed out that some other studies have indicated that radiotherapy in addition to surgery improves local control of the tumor and survival rate of the patients significantly.8According to several investigations the main treatment modality is still wide surgical excision and it has been demonstrated that usefulness of adjuvant chemotherapy in patients with OS of head and neck region is a matter of debate.The authors made an attempt to verify the importance of combining the clinical, radiographic and histopathologic examinations to achieve a definitive diagnosis. It should also be emphasized that early diagnosis of OS facilitates the treatment procedure, reduces associated morbidity, significantly improves prognosis and ultimately leads to successful treatment outcome.


Intra-peritoneal rupture of hydatid cyst is a rare complication and there is no consensus about its treatment.The reported case concerns a 25 years old female patient who had been complaining for four months from a moderate pain in the right upper quadrant. No clinical or biological signs of sepsis or allergic reactions were witnessed. Ultrasound and CT examinations showed a multilocular hepatic cyst in addition to multiple unilocular cysts in the abdomen. The suspected diagnosis was hepatic and peritoneal HC and a surgical treatment was scheduled four weeks later. Surgical exploration showed a large ruptured HC on the left lobe of the liver, with daughter cysts in the peritoneal cavity. Left lobectomy of the liver with complete ablation of all daughter cysts and a wide peritoneal lavage were performed. For the three months following the surgery, Albendazole had been given to the patient. No recurrence occurred after four years of follow-up.Intra-peritoneal rupture of liver HC could be asymptomatic. This case showed that in some cases, occurrence of complications is not systematic. This suggests that urgent surgical treatment is not always mandatory in the absence of alarming signs. Well-conducted medical treatment would reduce the risk of occurrence of secondary peritoneal hydatidosis. Intra-peritoneal rupture is a serious turning point in the evolution of hepatic hydatid disease. It is a rare complication and there is still a debate in the literature about a reliable treatment. The reported case concerns a patient suffering from an asymptomatic intra-peritoneal rupture of a hydatid cyst in the left liver, which was discovered intra-operatively, highlighting the implications of this atypical mode of presentation.A twenty-five years old female patient was referred to our unit after showing symptoms of abdominal hydatid disease. Her medical history was uneventful and she had been complaining for four months from a moderate pain of the right upper quadrant. Clinical examinations revealed an absence of fever or allergic symptoms and a soft abdomen. However, there was a firm painless mass in the right upper quadrant. Abdominal ultrasonography (US) showed a cystic mass of the left hepatic lobe, corresponding to a type II hydatid cyst (HC). In addition to this, it also showed a multilocular cystic in the pelvis. CT scan revealed Figures , 2 and 3Intra-peritoneal rupture is a rare complication of hepatic hydatid disease even in endemic areas. Its incidence varies in the literature from 1.75 to 8.6% . The rupMost frequently, the rupture in the peritoneum is symptomatic, with constant abdominal symptoms ,5 (abdomThere is no consensus regarding the treatment in the case of an intra-peritoneal rupture. The majority of authors recommend an emergency surgical treatment  due to tThe recurrence rate following an intra-peritoneal rupture of the liver HC could reach 21% . The useThe intra-peritoneal rupture of liver hydatid cysts could be asymptomatic and the occurrence of complications is not systematic. This suggests that in case of early diagnosis of intra peritoneal rupture, urgent laparotomy should not be mandatory in the absence of alarming signs, and could be performed after correct preparation. Well-conducted medical treatment would reduce the risk of occurrence of secondary peritoneal hydatidosis.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 of this journal.The authors declare that they have no competing interests.MA and AM wrote the paper, HA provided data; AM, RM, EM, SF and HA revised the manuscript. All authors read and approved the final manuscript.


A clear cell hidradenoma is a rare dermal tumor, which is believed to originate from the apical portion of the sweat glands. The usual size reported is 5–30 mm. It is generally found in the head, face, and upper extremity regions. This lesion has not been reported to be large enough to impinge a joint range of motion. Hence, its description in the orthopedic literature is extremely rare. We present a giant benign nodular hidradenoma presenting as painful restriction of the right shoulder joint in a 35-year-old male. A nodular hidradenoma (NH) is a rare benign sweat gland tumor that is believed to originate from the eccrine gland. The lesiA 35-year-old male presented with complaints of painful restriction of the right shoulder motion for the last 3 months. The pain was insidious in onset, dull-aching to pricking in character, occurring only while attempting extreme motions of the joint, more so in activities that involved raising the arm in an overhead position, e.g. bowling during the sport of cricket, reaching for articles placed high on the shelf or while sleeping on the affected side where it used to wake the patient in the night. There was no history of trauma, massage, morning stiffness, involvement of any other joint or any other medical or surgical history of illness. There was no history of any other lump in the body. On examination, the right shoulder appeared normal on inspection. There was no tenderness over the anterior, posterior or superior aspects of the shoulder as well as in the subacromion region. A mass was palpated in the right axilla, which was tender on deep palpation, well circumscribed, of the size of a small orange, firm in consistency and adherent to the underlying structures but not to the overlying skin. The mass became prominent on full abduction and used to move with the movement of the scapula. The mass was nonpulsatile and no bruit was heard on auscultation. The range of movement (ROM) was 0–160 degrees of abduction, with pain starting on 120 degrees and partially relieved on abduction beyond. Flexion, extension and internal rotation were comparable to the other  side. There was 30 degrees of painful restriction of external rotation on the right side as compared with normal. The patient on further questioning revealed that this mass started as a small nodule observed while playing cricket 20 years back. The mass increased in size gradually but the patient ignored it till such time that he developed restriction of right shoulder movements, abnormal fullness and discomfort in the right axilla. Thorough clinical examination of the affected upper extremity was carried out to rule out any chronic infection, leading to lymphadenopathy. Examination of the other sites did not reveal any similar swelling or lymph node enlargement. There were no tingling sensation or numbness in the fingers or any wasting or hair loss noted in the right upper limb. Hematological tests were performed to rule out tumoral calcinosis (serum phosphate), complete hemogram, erythrocyte sedimentation rate and C-reactive protein for any possibility of occult systemic ailment manifesting as axillary mass.per se. The patient was suggested further investigation of the shoulder.A plain skiagram of the shoulder showed a round area of diffuse amorphous calcification in the axilla , whereasA multiplaner magnetic resonance imaging (MRI) scan on a 1.5 Tesla magnet was performed and T1, T2 and proton density images were obtained using spin echo (SE) and gradient echo sequences. Gadolinum-enhanced T1-weighted SE images were subsequently acquired in the sagittal, axial and coronal planes . There wFine needle aspiration from the lesion done twice was inconclusive and revealed red blood cells only.A small biopsy was required in this case for establishing a definitive diagnosis before carrying out an excision of the mass. However, as the patient had already undergone fine needle aspiration cytology (FNAC) twice and did not give consent for a third biopsy in view of a strong clinical diagnosis of a benign condition supported by the MRI scan findings, the biopsy was not performed. A decision of excision biopsy was taken after weighing the various pros and cons and explaining to the patient that in the event of the tumor being unresectable, a small biopsy only will be performed.The patient was operated from an anterior approach to the shoulder extending up to the posterior axillary fold. The arm was kept semiabducted to avoid stretching of the axillary structures over the mass. The axillary vessels and nerves were separated carefully and the mass was dissected in the depth of the incision. The deltopectoral interval was demarcated in the upper part of the incision to reach till the coracoid process and the conjoint tendon was identified. The pectoralis muscle was not divided. Blunt dissection was performed above and below the muscle. The ascending branch of the anterior circumflex vessels was identified and cauterized. The mass was found adherent to the fibers of the subscapularis and capsule at the anteroinferior aspect of the glenoid.en block. The wound was closed in layers over a suction drain for 48 h. The postoperative period was uneventful. Stitches were removed on the 12th day and ROM exercises were started according to his tolerance.The tumor was removed The mass was an irregular, bosselated, grayish white, firm tissue measuring 80 mm × 55 mm × 40 mm, with few attached muscle fibers on the outer surface. On cutting open, the surface was grayish white with few circular homogenous areas.The lesion was well circumscribed and showed a fibrous capsule on one side and fibrofatty tissue on the other side. It consisted of nodules of various sizes separated with fibrous bands. Each nodule was partly solid and partly showing glandular or cystic spaces. The latter contained papillary structures. Cuboidal cells lined the glandular spaces and papillary structures with uniform overlapping pale staining nuclei. The spaces contained eosinophilic granular material. In between the glandular spaces and in the solid areas, the cells were either polyhedral or rounded having a clear cytoplasm and rounded nuclei or oval with elongated nuclei. Many psammoma bodies or large chunks of calcification were seen at many places. Mitotic figures were rare and there was no necrosis. The histopathological diagnosis of benign NH was made in view of the above-mentioned findings typical for this tumor Figures  and 4. NAt a follow-up of 6 weeks, the patient had full ROM at the shoulder; except extreme external rotation and abduction, he was symptomatically better and there was no feeling of fullness in the axilla. At the 6-month follow-up Figures  and 6, tBenign NH has many synonyms, namely solid cystic hidradenoma, clear cell myoepithelioma, eccrine sweat gland adenoma, large cell hidradenoma and eccrineacrospiroma. It affects women more commonly than men and is usually found on the scalp, face, anterior trunk and extremities. This tumor occurs at any stage of life, but is most common in the fourth decade. Skin color changes, skin thickening, serous discharge or tenderness can accompany the lesion. The lesion is superficial and hence it is diagnosed early and excised often without much investigation. A deep NThere have been reported cases of NH from various parts of the body, like eyelids, vulva, palmer aspect of the hand, ulcerated lesion on the face and axillary tail of the breast.–6 MalignExcision of the mass was a difficult task from an anterior approach as its location was posterior, but because vital axillary structures could be best protected from an anterior axillary approach, this was preferred. This tumor cannot be ignored as it can rarely undergo malignant transformation. In such a large tumor, of such long duration, such possibility could exist. However, there was no evidence of malignant transformation in this case as per the clinical and histopathological features. This case report is interesting in the fact that it has added NH in the differential diagnosis of an axillary mass presenting as painful restriction of motion of the shoulder joint.


Stercoral perforation of the colon is a rare but serious complication of chronic constipation. We present a case of stercoral perforation requiring subtotal colectomy in a 41-year-old female who had been on methadone maintenance for a history of long-term intravenous heroin use. Our case highlights the importance of prompt and thorough surgical intervention in the successful treatment of this rare condition. Stercoral perforation is recognized as a rare cause of colonic perforation that is most commonly seen in elderly, debilitated, and/or institutionalized patients who are immobile and on multiple medications. Many drugs such as narcotics, anticholinergics, antacids, and NSAIDs have been implicated as causes of fecal impaction resulting in stercoral perforation. The mortality has been reported to be as high as 57%, but prompt surgical exploration may decrease that rate . We report a case of subtotal colectomy performed for stercoral perforation in an otherwise healthy individual who was on methadone maintenance for a long history of intravenous heroin use. A 41-year-old Caucasian female presented with a 2-day history of abdominal distention and generalized pain that was worsened with motion. These symptoms were accompanied by nausea, several episodes of nonbilious vomiting, and poor appetite. Her last bowel movement had been a week prior to admission. Her past medical history was significant for type 2 diabetes and vulvar lichen sclerosis, which was being treated with steroids since its diagnosis one year prior. She had a 15-pack-year history of tobacco use, as well as a distant history of intravenous heroin abuse with subsequent long-term dependence on methadone maintenance at 180 mg. Vital signs were noted as temperature 98.7°F, blood pressure 121/68 mmHg, pulse 101 bpm, and respiratory rate 24. Physical examination revealed diffuse abdominal tenderness with maximal pain on the left side, as well as rebound tenderness, guarding, and hypoactive bowel sounds. A rectal exam revealed no masses, empty ampulla, and hemoccult negative stool. Laboratory studies revealed a white blood cell count of 22,200/mL with bandemia, glucose at 267 mg/dL, and lactic acid elevated at 4.7 mmol/L. Other laboratory values were all normal. A CT scan of the abdomen and pelvis with PO contrast revealed multiple foci of extraluminal air in the peritoneum, a large amount of fecal material in the colon, and extravasation of oral contrast consistent with perforation of the colon  being resection of the diseased segment of colon, end colostomy, and Hartmann's closure of the rectum . Moreove3% being


Congenital nasal pyriform aperture stenosis is a rare and potentially lethal form of airway obstruction in newborns. Immediate recognition and appropriate therapy are mandatory for this potentially life-threating condition. This anomaly may present as an isolated malformation or may be associated with other cranial-facial anomalies. Clinically, CNPS shows unspecific symptoms of nasal airway obstruction such as apnoic crisis, episodic cyanosis and inability to nurse. The purpose of this report is to present author's experience in the surgical management of this rare pathology in 3 patients.Three cases of congenital nasal pyriform aperture stenosis were reviewed for presentation of the disorder, management and effectiveness of the surgical treatment.All the patients underwent a surgical correction of the pyriform aperture stenosis using a sublabial approach followed by nasal stenting. During the follow-up no cases of restenosis, respiratory failure or cyanosis were detected.Congenital pyriform aperture stenosis should be suspected in newborns with clinical signs of severe nasal obstruction associated with a difficulty to pass a small catheter though the anterior nasal valve. Timely recognition is mandatory to prevent a potential deadly outcome. Surgical correction of the stenosis though a sublabial approach followed by a nasal stenting revealed to be most effective treatment for these patients. Congenital nasal pyriform stenosis (CNPS) is an unusual, potentially lethal form of neonatal nasal obstruction . The pyrClinically, CNPS manifests as nonspecific symptoms of nasal airway obstruction, such as apneic crisis, episodic cyanosis, and inability to nurse. Because infants are obligatory nasal breathers until 6–8 weeks of age, any form of nasal airway obstruction could cause severe distress symptoms; thus, early diagnosis and treatment are mandatory ,4.The purpose of this report is to present the author’s experience in the surgical management of this rare pathology in three patients.A full-term male infant had Apgar scores of 6 at 1 min and 9 at 5 min after birth. He was 2.690 kg in weight and 51 cm in height, and had a cranial circumference of 34 cm and a thoracic circumference of 32.5 cm. The infant showed mild respiratory distress, ribcage asymmetry, a reduced vesicular murmur, stridor, and diaphragmatic retraction. Anterior rhinoscopy was not possible because of bilateral stenosis of the nasal fossae and interference of the turbinates, but transoral posterior rhinoscopy showed a normal right nasal fossa and left choanal stenosis. Computed tomography (CT) revealed stenosis of the pyriform aperture  and choanal atresia  were sutured to the columella to maintain airway patency and stabilize the surgical results. The correct positioning of the stents was checked endoscopically , and right deviation of the nasal septum.The infant was maintained on decongestant drops for several days with a reasonable respiratory result, but experienced difficulties in feeding and failure to thrive. Thus, we performed endo-oral pyriform stenosis enlargement at 26 days of age. In this case, debridement of the posterior nasal fossa and turbinate was not required because the mild obstruction was mainly related to the posterior part of the turbinate and was easily displaced, achieving satisfactory airway improvement. Standard nasal conformers usually employed in cleft surgery (16 × 23 × 7 mm) were applied and maintained for 3 weeks after surgery. Nasal decongestants and saline drops were continued for 6 days, then discontinued. The patient was discharged with no complication on day 12.A female infant born at 37 weeks had Apgar scores of 9 at 1 min and 10 at 5 min after birth. Her weight was 2.800 kg, length was 47 cm, and cranial circumference was 31 cm. After an initial discharge from the neonatal care unit, the infant was readmitted at 34 days of age because of multiple episodes of cyanosis, dyspnea, inspiratory stridor, and difficulties in eating. CT showed bilateral stenosis of the pyriform aperture  with normal choanal morphology.Given the persistence of symptoms despite conservative treatment with local decongestants, the infant underwent surgery at 47 days of age. The pyriform opening was widened by bone drilling through an endo-oral sublabial approach, as described for cases 1 and 2 Figure . The infBecause neonates are obligatory nasal breathers, any condition that prevents normal nasal airflow must be diagnosed and treated correctly. CNPS is a rare clinical condition that must be considered in the differential diagnosis with other breathing difficulties of an inflammatory, traumatic, or congenital nature. The cause of this pathology is unknown, but it arises in the fourth month of fetal development because of an overgrowth of the nasal process of the maxilla and may present as an isolated condition or in association with other congenital disorders. Several studies demonstrated a relationship between CNPS and craniofacial abnormalities such as holoprosencephaly, cleft palate, and the early presence of maxillary central incisors ,6. This CNPS clinically presents with unspecific symptoms, such as episodes of cyanosis, inspiratory stridor, sternal retraction, thoracic asymmetry, hypoxemia, and acidosis ,5. TheseThe diagnosis of CNPS is based on clinical evaluation, including nasal endoscopy and especially CT. The inability to pass a 5F catheter and a radiographically measured pyriform opening < 8–10 mm in a full-term infant are considered diagnostic. If holoprosencephaly is suspected by the presence of a central maxillary incisor, encephalic CT or magnetic resonance imaging should also be performed -8.Once tInitial management of this condition involves the establishment of a secure airway by McGovern nipple placement or endotracheal intubation, with appropriate monitoring in the intensive care unit until the exact cause and severity of nasal obstruction have been established. In cases of mild CNPS, a nonsurgical approach involving the positioning of silastic stents in the nasal cavity and the use of local decongestants is preferable,9. HowevIn cases of moderate or severe stenosis, the approach is surgical and involves pyriform aperture enlargement through an endo-oral sublabial approach to reshape the stenotic area with burs. This method is safe and enables good field exposure, prevents damage to the nasolabial soft tissues, and does not cause visible scarring. Morbidity is irrelevant and results are achieved immediately after surgery .A transnasal approach has also been described, but in our opinion, it is not advisable in neonates because of the reduced dimensions of anatomic structures, which increase the risk of soft-tissue trauma .The surgical procedure begins with bilateral exposure of the pyriform aperture to free its bony margin, leaving the mucoperiosteum intact along the nasal floor and pyriform aperture. Drilling of the nasal floor must be avoided to prevent damage to the tooth buds . The apeIn cases of associated choanal atresia, excess membrane and bony tissue should also be removed. The use of endoscopy is currently recommended to more safely control the posterior nasal fossa and the positioning of nasal stents.To reduce recurrence and scar-related stenosis, the use of nasal stents is recommended. The choice of device should be based on the presence of choanal atresia. In isolated CNPS, the use of short nasal conformers, such as those routinely applied in cleft patients, is preferable because of their easier management: they are more comfortable for the patient, cleaning and replacement are easier and safer, and damage related to aspiration and displacement are avoided. On the other hand, the use of longer soft silastic nasal stents  is mandatory in the presence of choanal atresia to prevent obstructive scarring in the posterior nasal area and to ensure the stability of the surgical enlargement. We typically retain the stents for about 3 weeks if choanal atresia is present and for only 6–7 days in cases of isolated CNPS .CNPS is a rare and potentially letal cause of airway obstruction in infants. It may occur isolated or in association with other anomalies. CNPS should be considered in the differential diagnosis whenever, in newborns, there are signs of severe nasal obstruction associated with a difficulty in passing a small catheter through the anterior nasal valve.In cases of severe obstructions, refractory to a conservative therapy, a surgical correction of the stenosis followed by an appropriate nasal stenting is in mandatory.The English in this document has been checked by at least two professional editors, both native speakers of English. For a certificate, please see:http://www.textcheck.com/certificate/sTncLX.Written informed consent was obtained from the parents of the patient for publication of this article and accompanying images.CNPS: Congenital nasal pyriform stenosis.We disclose any financial and personal relationship with other people or organizations that could inappropriately influence our work.ES, BB, GO and AF performed surgery, ML drafted the manuscript. All authors read and approved the final manuscript.No Funding to disclose.


Ameloblastomas frequently occur in relatively young people, but are rarely seen in people aged 80 years or older. We report a case of mandibular ameloblastoma in an elderly patient with a review of the literature. The patient was a 82-year-old man who noticed swelling of the gingiva approximately 2 weeks prior to his initial visit. Computed tomography showed a radiolucent area with little radiopacity. Internal uniformity was observed at the site, with thinning of cortical bone which lacked continuity in some areas. The excision and curettage were performed under general anaesthesia. No recurrence has been observed 14 months after surgery. Among odontogenic tumours, ameloblastomas have the highest rate of occurrence after odontomas . They arAn 82-year-old Japanese man presented with swelling of the gingival in the molar region of the left mandible. Approximately 2 weeks prior to the first visit, the patient noticed swelling of the gingiva, and panoramic X-rays were taken at a dental clinic. The images revealed radiolucent findings at the site, and the patient was referred to our facility for examination. The patient had a moderate physique and was well nourished, but he was taking medication for hypertension. Intraoral findings showed that the upper and lower jaws were edentulous, with a relatively irregular border from the centre of the mandible to the gingiva of the molar region on the left side. Diffuse swelling and surface ulceration were observed. There was neither tenderness nor numbness of the lips .Panoramic radiographs revealed a barely perceptible, polycystic radiolucent area with slightly irregular margins in the left molar region of the mandible .Computed tomography showed a radiolucent area with little radiopacity. Internal uniformity was observed at the site, with thinning of cortical bone which lacked continuity in some areas . After 1The extracted tumour measured 3 × 2.5 cm and was milky-white in colour, with a slightly rough surface. The transverse section was mostly cystoid, but solid portions were also observed . HistopaAmeloblastomas have a relatively high rate of occurrence and are seen across a wide spectrum of ages . The peaIn reports by Philipsen et al.  and WettAmong reported cases of intraosseous that were diagnosed preoperatively, there have been three cases, including the one described here, in which a benign tumour was suspected , 10: oneA number of therapies are being investigated for the treatment of ameloblastomas, but in elderly patients, considering their overall physical condition and age, it is necessary to select a minimally invasive surgical approach. Even when the tumour was resected, resection was sometimes performed under local anaesthesia, due to the patient's overall physical condition and the need to preserve the function of the affected area  and case


Bone and soft tissue malignancies account for a small portion of brain metastases. In this review, we characterize their incidence, treatments, and prognosis. Most of the data in the literature is based on case reports and small case series. Less than 5% of brain metastases are from bone and soft tissue sarcomas, occurring most commonly in Ewing's sarcoma, malignant fibrous tumors, and osteosarcoma. Mean interval from initial cancer diagnosis to brain metastasis is in the range of 20–30 months, with most being detected before 24 months , some at 24–36 months , and a few after 36 months (chondrosarcoma and liposarcoma). Overall mean survival ranges between 7 and 16 months, with the majority surviving < 12 months . Management is heterogeneous involving surgery, radiosurgery, radiotherapy, and chemotherapy. While a survival advantage may exist for those given aggressive treatment involving surgical resection, such patients tended to have a favorable preoperative performance status and minimal systemic disease. It is estimated that up to 30% of patients with cancer will develop brain involvement , 2. BreaAccounting for 0.8% of all cancers, musculoskeletal bone and soft tissue sarcomas make up a small portion of patients with brain metastases . It is eThe purpose of this review is to present the current literature on brain metastasis (BM) from bone and soft tissue cancers, with an emphasis on musculoskeletal sarcomas and those most commonly metastasizing to the brain. We describe the incidence, diagnostic strategies, treatment paradigms, and prognostic outcomes as well as relevant background information.A search of the published literature was conducted for patients with brain metastasis from musculoskeletal sarcomas. The national library of medicine search engine, PubMed, was utilized for the literature search. For each of the sarcomas, the search terms “brain” and “intracranial” were combined with the tumor's name: “osteosarcoma,” “Ewing's sarcoma,” “chondrosarcoma,” “chordoma,” “malignant fibrous tumor,” “malignant fibrous histiocytoma,” “fibrosarcoma,” “liposarcoma,” “alveolar soft part sarcoma,” “rhabdomyosarcoma,” “malignant peripheral nerve sheath tumor,” “MPNST,” or “angiosarcoma.” Relevant articles describing case reports or clinical studies were selected, and the reference lists from these articles were also inspected for other relevant articles. Each of the resultant articles were examined closely and reported in this review. Cases in which there was contiguous extension of a primary tumor into intracranial structures  as opposed to frank metastasis from a distant site were excluded. Only publications in English, peer-reviewed journals were included.The primary malignant bone tumors discussed are the most commonly reported to metastasize to the brain: osteosarcoma (osteogenic sarcoma), Ewing's sarcoma, chondrosarcoma, chordoma, and fibroblastic/fibrohistiocytic tumors.After plasmacytoma 33%), osteosarcoma (20%) is the most frequently occurring primary malignant bone tumor , charact3%, osteoYonemoto et al.  recommenAs a commonly diagnosed musculoskeletal cancer in children and young adults, osteosarcoma, when metastatic, mainly spreads to the lungs and other bones and rarely to the brain. As such, it is difficult to form consensus guidelines on treatment once BM occurs. As many of these lesions are solitary, surgical excision has been the standard of care, with chemotherapy and RT for palliative measures.Ewing's sarcoma is commonly seen in young adults and has a slight male predominance –31. Up tApproximately 40 cases of ESBM have been described in the literature  2, 4, 9, 34–38. Overall, the appearance of BM signifies a poor prognostic outcome in patients with ES. The current increased frequency of CNS metastases has been linked to prolonged survival and alteration of host response following chemotherapy . MoreoveChondrosarcoma is a malignancy of mesenchyme that is a common primary bone tumor subsequent to osteosarcoma in frequency . It has Treatment with chemo- and radiotherapy has resulted in poor results, postulated due to the resistant extracellular matrix, low mitotic index, and sparse vascularization of cells , 42. As Though chondrosarcoma accounts for 40% of primary bone cancers in adults , it rareChordomas are rare, malignant tumors, which arise from the embryonic notochord –51. TheiInherently, chordomas destroy and replace bone in which they develop . Until rMetastasis from chordomas depends on many factors such as histology, mitotic activity, and treatment regimens . Due to Previously designated under “malignant fibrous tumors,” these are rare malignancies characterized by pleomorphic, high-grade tumor cells with histiocytic, fibroblastic, and myofibroblastic features –59. “MalAlthough these tumors can originate from either bone or soft tissue, defining the source is difficult . SuccessAs with other sarcomas, pulmonary metastasis is most common, with its detection increasing the risk of future BM , 68. ThuPeriodic imaging of the brain, the primary site, and the metastatic sites has been suggested to prolong life expectancy, though it remains controversial . In addiThe primary malignant soft tissue tumors (STS) discussed are also the most likely to metastasize to the brain: liposarcoma, rhabdomyosarcoma, malignant peripheral nerve sheath tumor, angiosarcoma, and alveolar soft part sarcoma.Liposarcomas comprise about 10–20% of all STS –76. WhilEspat et al.  analyzedAs the lifespan of patients diagnosed with liposarcoma extends, the natural history of the disease leads to CNS metastases, which occurs in a large part after 10 years following original diagnosis , 76, 79.Rhabdomyosarcoma (RMS) is a malignancy of striated muscle and one of the common STS in those under 20 years . It usuaVezeridis et al.  reviewedLike other sarcomas, multimodality treatment is instigated for those with BM from RMS . GaspariRMS is a common STS affecting young patients, and it has been known to metastasize to different organs and tissues. Patients with a history of metastasis have a significant risk of future BM . SurgicaA number of entities are synonymous with malignant peripheral nerve sheath tumors (MPNST) including neurofibrosarcoma, neurogenic sarcoma, and malignant schwannoma . MPNST uAccording to Aggressive SR followed by WBRT is the basis of treatment for BM. However, because of the limited pool of patients, there are cases surviving for many months without treatment and others dying over a short period after aggressive intervention , 94, 99.MPNST is a very aggressive cancer associated with high recurrence and metastatic potential. Prognosis is generally poor and worse with metastasis , 116. ItAngiosarcoma is an exceedingly rare malignancy, which arises from endothelial cells of the vasculature. The least common among vascular tumors, angiosarcoma has a prevalence of <1% of all sarcomas , 118. CeCurrent understanding of the metastatic behavior of angiosarcomas is limited. Of reported cases with BM, the often involved primary site is the heart . Though,Overall, the prognosis of angiosarcoma is grim. Typical 5-year survival is 12%, with metastases and reoccurrence typically occurring within the first 2 years . PrognosAlveolar soft part sarcomas (ASPS) account for a small percentage of STS at roughly 1% , 89. In Portera et al.  studied The literature is abundant with reports of BM from ASPS. Neurosurgical resection can be favorable and may increase long-term survival , 135. A  fibroblastic and fibrohistiocytic tumors section. Also, Malignant histiocytomas (undifferentiated pleomorphic sarcomas) and fibrosarcomas originating from the soft tissues are covered under thePrevious landmark studies on the treatment of brain metastases have shown efficacy in surgical resection and postoperative WBRT , 142, asGenerally, BM from these cancers is very infrequent, most commonly occurring in Ewing's sarcoma, fibroblastic/fibrohistiocytic tumors, and osteosarcoma. Pediatric patients are more likely to have RMS. Though ASPS commonly metastasizes to the brain, it is a much rare cancer and thus accounts for a lesser proportion. Many of the larger studies suggest a slight preponderance in males 52–60%) [–60% 2, , 38, 144Comprehensive neurological evaluation and imaging should be performed on development of suspicious neurological signs or symptoms. Clinical presentation of BM is characterized by rapid onset of neurological symptoms, such as paresthesias, visual field defects, and headache. Generally, routine neuroimaging studies without these indications are not warranted because of the rarity of BM and because they almost always result in signs and symptoms if they are present though it has been suggested to screen patients with osteosarcoma and MFT with extensive disease, especially if there is a short disease free interval , 19, 64.The extent of systemic disease and Karnofsky Performance Scale (KPS), as well as sarcoma histotype are important factors in determining patient outcomes. The presurgical KPS gives an overall clinical picture, and a score above 60–70 has been cited as a good prognostic indicator associated with higher median survival , 38, 144While SR has been suggested to lead to improved outcomes , 38, 144Brain metastasis in bone and soft tissue cancers usually occurs late in the malignancy. As most of the data in the literature is from case reports and case series utilizing heterogeneous treatments, it is difficult to discern the best therapeutic strategy. Prolonged survival and an adequate quality of life are achievable in a small, select population of patients. While such a survival advantage may exist for those given an aggressive treatment course involving surgical resection, it should be reserved for those with a favorable preoperative performance status and minimal systemic disease. Future large prospective studies can help give more insight on this uncommon, yet growing group of brain metastases. Overall, the occurrence of BM in patients with bone and soft tissue cancers is a poor prognostic sign that suggests late stage disease.


Isolated or solitary neurofibromas of the paranasal sinuses are rare. Mostly, they involve the maxillary sinus and so far, a solitary neurofibroma of the frontal sinus has never been reported in the literature. We present a case of frontal sinus neurofibroma treated successfully with surgical excision. A 28-year-old male presented with forehead swelling and computed tomography images revealed a tumor involving the left frontal sinus. Histopathological examination showed the tumor to be a neurofibroma. We discuss the clinical and pathological characteristics of neurofibroma arising in the nasal cavity and paranasal sinuses. Neurofibromas are benign nerve sheath tumors that originate from the peripheral nervous system. More specifically, they arise from the nonmyelinating Schwann cells but are commonly referred to as schwannomas in the literature . GeneralNeurofibromas are usually found in individuals with neurofibromatosis, which is an autosomal dominant disease. There are two types of neurofibromatosis: type 1 (von Recklinghausen disease), which is more common, and type 2, which typically has a more severe course due to central nervous system tumors [On occasion, an isolated neurofibroma can transpire without being associated with neurofibromatosis. Mostly, these solitary tumors tend to occur in the gastrointestinal system , 4, and In this report, we describe a case of a solitary neurofibroma arising from the frontal sinus. To the authors' knowledge, no such case has been published to date.A 28-year-old man presented to the otolaryngology clinic with a central forehead swelling, which had been gradually enlarging over several months . There w Past medical history was unremarkable. There was no history of trauma or sinonasal disease. Social history was also unremarkable; the patient was a nonsmoker and an office worker with no prior exposure to toxic fumes or inhalants. On examination, there was a central forehead swelling just superior to the level of the medial eyebrows. The mass was estimated to be approximately 5 cm in size, oval shaped, and no overlying skin changes were noted . On palp A computed tomography (CT) scan of the head and paranasal sinuses was obtained, which revealed an isolated soft tissue mass involving the left frontal sinus . The mas On surgical exploration, the superficial component of the tumor, was found to be arising from a left frontal sinus bony dehiscence, which was approximately 1.5 cm in diameter located along the anteroinferior frontal sinus wall . The sub A diagnosis of neurofibroma was made on histopathological analysis . Of note Postoperatively, the incision healed well with minimal forehead scarring and deformity . No recuNeurofibromatosis can occur with multiple peripheral nerve-sheath tumors, which can at times involve the head and neck area . Of thesNeurofibromas are slow growing benign tumors, but can become very large causing compression of local structures, including expansion and erosion of adjacent bone . In our Clinically, neurofibromas present like any other benign lesions and the presenting signs and symptoms depend on the site of the tumor and subsequent involvement of surrounding structures . In our Imaging for these lesions include CT scanning and magnetic resonance imaging (MRI) to demonstrate the extent of the tumor, which is vital for planning of surgical excision. In most cases, MRI is thought to be superior to CT images since the former can allow better differentiation of the tumor from adjacent soft tissues, as well as improved evaluation of possible intracranial and intraorbital extension , 11. UnfThe differential diagnosis of benign nasal cavity and paranasal sinus tumors mainly include fibroma, neurofibroma, papilloma, leiomyoma, and schwannoma , 12. HisThe overall management of neurofibromas depends on the presenting signs and symptoms. Some lesions that are small and not causing any issues may not require any intervention, other than monitoring. As mentioned above, with growth however, there may be compressive symptoms, such as pain, cosmetic deformity, and neurovascular compromise. As well, there may also be a suspicion of the tumor being malignant, which necessitates surgical exploration, biopsy, and excision , 6.Hemorrhage may occur during the operative intervention for neurofibromas due to the rich vascular stroma  but thisRecurrences have been reported to be infrequent although it is mentioned in the literature that tumors with locally invasive tendencies may recur after removal , 12. WitAn isolated neurofibroma of the frontal sinus in a young male patient is presented. The tumor was treated successfully with surgical excision. To the authors' knowledge, no similar case has been reported.