We present a case of post-traumatic diaphragmatic herniation of the liver, which mimicked an intrathoracic tumor. After an automobile accident, the patient underwent thoracotomies for hemothorax and lung cancer in the right chest. Seven months later, computed tomography (CT) demonstrated a round tumor in the thorax adjacent to the right diaphragm with a higher density than the liver parenchyma. An intrathoracic tumor including a primary or metastatic lung cancer was suspected. However, positron emission tomography (PET) showed that the uptake of fluorine-18-fluorodeoxyglucose (FDG) was identical to that in the liver, and the tumor appeared to be contiguous with the liver. Thus, we suspected liver herniation. Core needle biopsy revealed liver cells without neoplastic tissue. Upon surgical exploration, herniation of the liver was found and repaired. PET was helpful in providing morphological and functional information leading to accurate diagnosis of liver herniation in this unusual case. Diaphragmatic herniation of the liver following blunt trauma may develop long after the initial trauma and remain clinically silent. Unless a large portion of liver and/or other abdominal organs are herniated, it is often difficult to distinguish diaphragmatic herniation of the liver from an intrathoracic tumor . PositroA 68-year-old woman was involved in an automobile accident and was transferred to the emergency department at the Hiroshima Prefectural Hospital. Computed tomography (CT) on admission demonstrated traumatic aortic injury, multiple rib fractures, and bilateral hemo-pneumothoraces as well as a spiculated mass, 2 cm diameter with pleural indentation in segment 6 of the right lung. She underwent emergent repair of the descending aorta and right pleural drainage. On the fourth post-operative day, bloody drainage from the right chest suddenly increased in volume. The patient was taken back to the operating room and at right thoracotomy, a bleeding point was found on the surface of the diaphragm. Hemostasis was established by using polypropylene suture.Four months later, the size of lung mass was unchanged, and PET showed little FDG uptake. Because malignancy was suspected and her general condition improved, she underwent surgical resection of the tumor. After meticulous dissection, the right lower lobe was partially resected, but systematic lobectomy and radical lymph node dissection was not feasible due to significant adhesion. Histological examination revealed a well-differentiated adenocarcinoma with clear tissue margins.The follow-up CT at 3 months revealed another tumor in the right lower lobe adjacent to the diaphragm, which had not been recognized before. Twelve months after lung resection, a discrete ovoid mass 3.7 × 2.7 cm in diameter with slightly higher density than that of liver parenchyma was apparent Figure . SubsequTraumatic rupture of the right diaphragm following blunt trauma is uncommon. The extent of herniation varies, from a small portion of liver, to the entire liver plus other abdominal organs. Small herniations are typically asymptomatic, and diagnosis can be delayed for many years -7. The dIn our case, several factors contributed to the difficulty in making an accurate diagnosis of diaphragmatic hernia. These include small herniation of the liver, concomitant lung cancer with suboptimal resection, and elevated CT density in the herniated portion of the liver. At first, as an intrathoracic tumor or metastasis from a lung cancer was suspected, a PET study was performed. Identical FDG uptake in the intrathoracic lesion to that in the liver was seen, leading to a diagnosis of liver herniation. However, since the patient's previous lung cancer showed little FDG uptake, and other neoplasms could not be differentiated solely by PET findings, additional supportive evidence was needed to make a definite diagnosis. US and MRI could not be performed, because of difficulties with the patient's control of breathing during the examination. As the tumor was adherent to the chest wall, we decided to perform a needle biopsy. This provided a conclusive finding of liver cells without neoplastic tissue thus confirming the diagnosis of liver herniation. The CT findings could be explained by strangulation of the herniated liver likely inducing congestion, which was confirmed at operation. This might have led to the higher density in the herniated portion on CT.Increased FDG uptake in PET is an important finding for differentiating benign lesions from malignant ones and is interpreted by calculation of the SUV . HoweverIn this case, histological examination of the specimen by needle biopsy revealed inflammatory cell infiltration around normal liver cells and fibrosis of Glisson's sheath. Yoshimura et al.  reportedSince this report involves only one patient, and there are no other reports in the literature, we cannot assume that herniated liver always exhibits FDG uptake at the same level as liver parenchyma. Hepatic hernias should be included in the differential diagnosis of a right basal mass in the thorax, in the patient with a history of thoraco-abdominal trauma. Recently, PET study has been used frequently in the differential diagnosis of intrathoracic neoplasms. The authors believe that knowledge of this case will be important for diagnosis and decision-making in other cases of ambiguous intrathoracic masses.We present a case of post-traumatic diaphragmatic herniation of the liver masquerading as an intrathoracic mass. Although the herniated liver had inflammatory cell infiltration, PET did not show increased FDG uptake above that of the normal liver level. In this case, PET information was helpful for diagnosing even a small liver herniation, due to its normal FDG uptake pattern, informing the subsequent management and repair of the diaphragmatic defect.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.KS, NM, SH, NC and YH participated in the care of the patient, including the operative part.TN participated in the pathology.KS wrote the first draft of the manuscript.KO and YH critically reviewed the manuscript.All authors read and approved the final manuscript.


Congenital cholesteatoma may be expected in abnormally developed ear, it may cause bony erosion of the middle ear cleft and extend to the infratemporal fossa. We present the first case of congenital cholesteatoma of the infratemporal fossa in a patient with congenital aural atresia that has been complicated with acute mastoiditis.A sixteen year old Egyptian male patient presented with congenital cholesteatoma of the infratemporal fossa with congenital aural atresia complicated with acute mastoiditis. Two weeks earlier, the patient suffered pain necessitating hospital admission, magnetic resonance imaging revealed a soft tissue mass in the right infratemporal fossa. On presentation to our institute, Computerized tomography was done as a routine, it proved the diagnosis of mastoiditis, pure tone audiometry showed an air-bone gap of 60 dB. Cortical mastoidectomy was done for treatment of mastoiditis, removal of congenital cholesteatoma was carried out with reconstruction of external auditory canal. Follow-up of the patient for 2 years and 3 months showed a patent, infection free external auditory canal with an air-bone gap has been reduced to 35db. One year after the operation; MRI was done and it showed no residual or recurrent cholesteatoma.Congenital cholesteatoma of the infratemporal fossa in cases of congenital aural atresia can be managed safely even if it was associated with mastoiditis. It is an original case report of interest to the speciality of otolaryngology. Congenital cholesteatoma (CC) is thought to occur secondary to failure of normal involution of epidermoid formation within the middle ear cleft. A collection of stratified squamous epidermoid cells appears during embryonic development and are instrumental in the development of middle ear mucosa. These remaining squamous cells grow slowly and become clinically apparent as a cholesteatoma usually during childhood . HoweverAlthough it is rare, the incidence of congenital cholesteatoma is higher in ears with congenital aural atresia (CAA) than in normally developed ears; in normal case, the ectoderm canalizes from medial to lateral to form the external auditory canal (EAC). If this process is arrested, stenosis or atresia of the EAC is a result. In such developmental disorders, when tympanic membrane is not formed, ectodermal epithelium is lost next to tympanic cavity. This lost ectodermal epithelium may initiate the growth of cholesteatoma .We report a very strange case of unilateral congenital aural atresia who presented with acute mastoiditis with congenital cholesteatoma of the infratemporal fossa.th post-operative day and re-packing of the external canal was done weekly for six weeks. Histopathological examination of the mass confirmed the diagnosis of cholesteatoma, also to confirm the existence of squamous epithelial cells; immunohistochemical staining with involucrin was performed on paraffin-embedded sections of the mass, using the avidin-biotin-peroxidase complex immunoperoxidase technique [A sixteen year-old male patient referred to our institute with post-auricular discharging sinus on his right side associated with swelling and redness around it, examination revealed that the patient had aural atresia dating since birth and microtia with acute mastoiditis that led to sinus formation on the right side, the left ear was completely normal. Two weeks before presentation, magnetic resonance imaging (MRI) was done in a private hospital due to the patient has complained of pain in right side of his head and face, it showed a soft tissue mass in the right infratemporal fossa Figures , surgicaechnique , involucCongenital cholesteatoma is a very rare disease; however it could be expected in the presence of congenital anomalies of the external ear, however it is more frequent in cases of EAC stenosis rather than in cases of complete atresia . LevensoRegarding the site of occurrence of CC, it is usually located intradurally; less frequently (20%), an extradural location is noted. There are five general sites of extradural occurrence: the middle ear, external auditory meatus, the mastoid, squamous portion of temporal bone and the petrous apex . In 2010Acute mastoiditis has been reported to be the first presentation of CC in a normally developed ear; Hidaka et al  has repoAlso, CAA may be presented with acute mastoiditis; Zalzal  has publPawełczyk and Czarski  publisheFinally, we conclude that CAA may be associated with CC that may extend outside the temporal bone reaching to the infratemporal fossa, also it may cause complications like mastoiditis. To avoid complications, radiologic evaluation of children with CAA is highly important for early detection of CC. Such cases should be managed surgically to drain infection and to remove the cholesteatoma mass, also atresia repair can be done successfully in the same setting.Written informed consent was obtained from the parents of the patient for publication of this case and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CC, Congenital cholesteatoma; CAA, Congenital aural atresia; EAC, External auditory canal; MRI, magnetic resonance image; CT, Computerized tomography.The author declares that he has no competing interests.This author is the sole author for this work.The study was carried out at the Department of Otolaryngology of Cairo Univesity.The study has been funded by the author with no financial conflict of interest.It is an original article, it has not been published before, and it is not considered for publication elsewhere.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1472-6815/12/6/prepub


Langerhans cell histiocytosis (LCH) is a very rare disease of female genital tract, most commonly seen in vulva and unusual in postmenopausal period. Herein, we report the 8th case of pure vulvar LCH in a postmenopausal woman. We pay attention to the differential diagnosis in postmenopausal state, features of pathologic diagnosis, and treatment options. Langerhans cell histiocytosis (LCH) is characterized by clonal neoplastic proliferation of bone morrow derived Langerhans cells in various tissues . LCH is To the best of our knowledge, there are only 25 cases of pure vulvar LCH reported in the English literature, and approximately, only 7 cases are reported to be in the postmenopausal status , 4. TherA 60-year-old woman was referred to our gynecology unit with vulvar itching, burning, and puffy skin lesions for 2 months. On physical examination, both labia majora and posterior fourchette ulcerated, and papillomatous lesions were detected without existing inguinal lymphadenopathy . She wasMicroscopic findings revealed ulcerated, keratinized stratified squamous epithelium and, under this, neoplastic cell infiltration both in the superficial and deep dermis. These uniform cells are ovoid in shape, with lobulated nucleus, and have large eosinophilic cytoplasm and ambiguous nucleoli . ImmunohTo exclude systemic metastasis, high-resolution computed tomography of thorax and positron emission tomography scans were performed. These investigations revealed no metastatic disease except a vulvar hypermetabolic area 2 cm in the long axis that was diagnosed on PET scan. Additionally, a Pap smear test of the cervix and endometrial sampling was performed. Pathological reports of the smear test and endometrial sampling were appropriate with postmenopausal status. Tumor markers were also tested and found within normal limits.The patient was informed about the treatment options, and she was offered surgical treatment  combined with radiotherapy according to the Gynecologic Oncology Council opinion.In our case, we reported a very rare pure vulvar LCH in a postmenopausal woman. This is the 26th pure vulvar LCH case in the literature and the 8th pure vulvar LCH case in a postmenopausal woman , 4. The To distinguish between many vulvar diseases and to make the correct diagnosis, suggesting vulvar biopsy to the patient is the appropriate option. Pathological diagnosis is the gold standard for LCH. First, the key step for LCH diagnosis is the detection of neoplastic Langerhans cells with characteristic lobulated nuclei within eosinophilic leukocytes infiltrating the dermis on hematoxylin and eosin stain. Second, a positive immunohistochemical staining for CD1a, S-100, or CD68 is necessary and sufficient for a definitive diagnosis. Additionally, presence of Birbeck granules of neoplastic Langerhans cells on electron microscopy may help to improve the diagnosis , 6.It is important to make the diagnosis of pure vulvar LCH in a postmenopausal woman, because many of the vulvar lesions in the postmenopausal period are usually treated with topical corticosteroids. In some cases, topical steroids may treat LCH, but, in some other cases, LCH may stay in remission during the treatment period, and after the cessation of therapy life-threatening distant metastases may occur if not checked .There is still no universally accepted treatment protocol available both for vulvar and systemic LCH. In the reported cases of vulvar LCH, vulvectomy, local excision, radiotherapy, chemotherapy, and topical and oral steroids were used as treatment options. Among them, complete surgical excision is the recommended treatment. Radiotherapy is also beneficial in new cases. Spontaneous remission may also occur in some cases –4, 6, 7.Although very rare, in a postmenopausal woman with symptoms like vulvar itching, burning, and ulcerated and papillomatous lesions, vulvar LCH should be considered in differential diagnosis, and definitive diagnosis should include immunohistological examination of biopsy material. Once the diagnosis is confirmed, distant organ metastases should be checked by radiology, and appropriate treatment options should be chosen.


Lymphomas represent the second most frequent malignant tumor (incidence 2.5%) in the head and neck region. Non-Hodgkin lymphomas (NHLs) present with cervical lymph node involvement, but in 40% extranodal site could be primary involved: nasopharynx, the lacrimal sac, the temporal bone, or the others areas. NHLs of the ear are rarely reported. In this report, we described a patient with primary NHL of the external ear canal who was successfully treated with surgical excision and chemotherapy. Lymphomas represent the second most frequent malignant tumor (incidence 2.5%) in the head and neck region , 2. Non NHL of the ear are rarely reported. Ogawa et al. found only eighteen reported cases of primary temporal bone lymphoma in the English and Japanese literature, including the cases of primary lymphoma of the middle ear . These tEven more rarely, primary NHLs arise from the external auditory canal (EAC) and fill it , 8. OnlyIn this report, we described patient with primary NHL of the EAC who was successfully treated with surgical excision and chemotherapy. A review of the literature regarding EAC lymphoma also is provided to describe the characteristics and management options for this uncommon manifestation of the NHL. D.G.E., a 46-year-old man, came to our attention in March 2009 for a left hearing loss started 3 weeks before. The patient referred only to aural fullness and he did not report any other symptoms like earache or effusion. Otomicroscopy revealed a polypus occluding the left EAC. The tympanic membrane was not visible. Pure tone audiometry showed a left conductive hearing loss and a normal hearing threshold at right . This peTherefore, a petrous bone computed tomography (CT) was performed. The CT scan showed a pedunculated polypoid mass occluding only the lateral portion of the EAC, with a normally aerated middle ear and mastoid, and a normal bone of the EAC without signs of erosion .We carried out a excisional biopsy under local anesthesia. The histologic examination of the mass revealed a peripheral B-cell NHL: diffuse large cells 60% and follicular 40%, with an immune-proliferative activity (Ki-67 index) of 60%. Immunoperoxidase staining of the atypical cells was positive for CD20, bcl2, bcl6, CD10, CD21 and negative for CD3, CD5, CiclineD1, and CD30. Clinical staging was completed with a total-body Positron Emission Tomography (PET), a CT scan of the chest and abdomen, and a percutaneous bone marrow biopsy were performed which resulted negative for other lymphoma's localitation. The patient was admitted to the Hematology Clinic and treated with six cycles of immunopolychemotherapy according to R-CHOP scheme . At 24 months, follow-up evaluation confirms stable complete remission. No localized recurrence or any systemic involvement was detected. An ear examination did not reveal any anomaly. The tympanic membrane was normal; the hearing threshold as well as the EAC was normal. A CT scan, performed after 9 months of follow-up showed a normal EAC .Although lymphomas are frequently observed in the head and neck compartment, they rarely involve EAC . To our The clinical presentation of primary lymphomas of the EAC is highly aspecific. Therefore these malignant disorders are usually misdiagnosed and treated as external otitis. As the lesion grows there is a sensation of blocked ear, transmission hearing loss, and increasing earache, sometimes without otorrhea or otorrhagia. Diagnosis is done by histological studies .In a recent review, Delgado et al. reported a 53-year-old woman with a Non Hodgkin's T cell lymphoma of the EAC . The patSometimes a single biopsy could not be sufficient, as described by Delgado et al. . An inciIn our case we decided for surgical excision of the whole lesion, considering also CT scans which showed absence of bone lesions, that allowed right and early diagnosis of NHL of EAC.The aspecific clinical picture makes diagnosis not easy: differential diagnosis includes benign, malignant process of the EAC, and external and media otitis. The lack of earache and of bone erosion (cleared with CT scan) made us rule out the malignant otitis external.  International guidelines for therapy have not been established yet due to the scanty number of cases.The surgical treatment of the EAC tumors of epithelial origin is largely accepted. However, in cases of high grade lymphomas or disseminated disease radiotherapy and/or chemotherapy could be considered as the primary and definitive treatments. For an isolate lymphomas tumor of the EAC, the surgical procedure should consist only in the excision biopsy of the tissue mass. This case was treated with excisional biopsy and six cycles of immunopolychemotherapy, according to known literature. Delgado et al. reported similar procedures, excisional biopsy and chemotherapy for one patient , as wellThe reported case highlights that malignant lymphoma should be suspected in case of a tissue mass of the EAC especially if not associated to ear discharge and not responsive to conventional treatment.Lymphoma arising from the EAC appears to be a particular biological entity and is commonly associated with a good prognosis. Novel strategies in the improvement of prognosis should be addressed in future studies by assessing prognostic factors implicated. Our case suggests that an excisional biopsy may be the best way for an early and right diagnosis and confirms that surgical treatment followed by chemotherapy could be an effective treatment.


Malakoplakia is a disease especially of the urinary tract with typical plaques most frequently observed in the urinary bladder's mucosa. In the context of immunosuppression malakoplakia can also occur in other organs. Some of these extravesical malakoplakias are associated with an infection by Rhodococcus equi, a rare human pathogen well known from veterinary medicine. Here we present the first case of a pleural malakoplakia without lung involvement caused by a proved Rhodococcus equi infection. Malakoplakia as a disease of the urinary tract is characterized by typical plaques consisting of accumulated macrophages  exhibitiA 60-year-old man was admitted to the hospital because of generalized lymphadenopathy, hepatosplenomegaly and suspicion of pneumonia. Based on lymph node biopsy the diagnosis of T-cell-prolymphocytic-leukemia was confirmed. Allogenic stem cell transplantation was performed with a matched related donor with a HLA-A-mismatch followed by immunosuppression with tacrolimus.Three months later the patient was admitted to the hospital with retrosternal pain and a reduced general condition. The clinical examinations revealed pneumonic rales over the right lung, slightly elevated temperature, and elevated infection parameters. Primarily the diagnosis of pneumonia was made and the patient was treated with antibiotics and antimycotics. The following computertomography of the chest revealed a 12 cm in diameter and well circumscribed round mass affecting the right upper lobe of the lung (Figure Surgery operation confirmed a tumor, which was adherent to the parietal pleura as well as to the lung and which was removed by lobectomy of the right upper lobe. Microbiological examination of tumor tissue revealed a positivity for Rhodococus equi.On macroscopical examination the right upper lobe of the lung showed a 12 cm large tumor connected with the visceral pleura and the lung without infiltration of the lung. The tumor exhibited a gray-white cut surface with central necrotic, disintegrated areas (Figure Microscopical examination revealed a tumor consisting of a monomorphic cell population, which was predominantly composed of macrophages. Granulomas, multinucleated giant cells or significant cellular atypia could not be observed. The immunohistochemical studies confirmed Ki-M1P/CD68 positive macrophages as the major tumor component. An increased proliferation was not detectable using the Ki67 staining. The macrophages showed intracellular deposits of concentric layers with a bright rim. The latter structures displayed PAS positivity and could be confirmed as calcifications by Kossa staining thus representing Michaelis-Gutmann-Bodies pathognomonic for malakoplakia Figure .We present the first case of a pleural malakoplakia caused by a proved Rhodococcus equi infection. The patient suffered from a rare T-cell-prolymphocytic-leukemia and was treated by stem cell transplantation. To prevent rejection, the patient received an immunosuppressive therapy with tacrolimus. Retrospectively, the patient confirmed a contact with horses most probably causing the here described pleural malakoplakia.Malakoplakia is a rare disease occuring particularly in the area of the urinary tract. It is based on an E. coli infection, usually followed by a macrophage-rich inflammation . During The first human infection by Rhodococcis equi was described in 1967 in a drug immunosuppressed patient . Since 1Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.CLB constructed the manuscript and carried out pathological examination. SN was responsible for the clinical data. SS participated in pathological investigations. HJR was responsible for critical revision of the manuscript and has been involved in drafting it. All authors read and approved the final manuscript.


A spontaneous rupture of hepatocellular carcinoma (HCC) can lead to extensive hemorrhage and is a rare but life-threatening event. A 58-year-old male patient with no history of trauma presented at our institution with severe epigastric pain and abdominal distension for 6 h. His blood pressure was a 60/40 mmHg, and pulse rate was 132/min. Abdominal contrast enhanced computed tomography (CT) imaging revealed a ruptured mass under the left diaphragm and fluid collection in the upper abdomen, flanks and pelvic cavity. Exploratory laparotomy confirmed the presence of an active bleeding tumor in the triangular ligament invading into the diaphragm. The tumor was resected with an appropriate diaphragm margin. The resected tumor was 5 cm in diameter and pathologically identified as hepatocellular carcinoma with a negative surgical margin. This case report shows that ruptured hepatocellular carcinoma should be considered in the differential diagnosis of non-traumatic hemoperitoneum. And it is necessary to set a surgical plan for unpredictable HCC rupture with direct diaphragm invasion. However, such extensive surgery was thought to present too high a risk of damage during the postoperative course. This case study looks at a previously undiagnosed patient with a spontaneously ruptured HCC in triangular ligament with diaphragm invasion.Hepatocellular carcinoma (HCC) is the fifth most common type of cancer diagnosed worldwide and the third leading cause of cancer-related mortality,2. Spont3. Liver function tests were within the normal range . Prothrombin time and International Normalized Ratio (INR) were prolonged with prothrombin time of 16.4 s , and INR of 1.43 (PT ratio). Abdominal contrast enhanced CT imaging revealed a mass invading the diaphragm with contrast extravasation in the left, lateral segment of the liver (Figure A 58-year-old man visited the emergency department with hypovolemic shock. His chief complaint was the sudden onset of epigastric pain with abdominal distension lasting 6 h. Physical examination revealed an ill appearance with a blood pressure of 60/40 mmHg and a pulse rate of 132/min. Conjunctiva were pale but not icteric. Breath sounds were clear, and heart sounds were regular and without murmurs. The patient had negative history of hepatitis B, hepatitis C or trauma. Hemoglobin was 6.9 g/dl, white blood count was 15,800/mm3 and platelet count was 176,000/mmet al. reported that an accurate preoperative diagnosis of ruptured HCC was predicted in only 25% of cases, despite shock being present in 33 – 90% of the patients[en bloc resection or blunt dissection (Table et al. and Lin et al. reported no significant differences in the surgical morbidity and mortality between patients who underwent a traditional hepatectomy and those who had diaphragm resection[et al. reported no significant difference in short- or long- term surgical impacts between patients who received en bloc resection and those who had blunt dissection and suggested that surgeons should consider the possibility of tearing the HCC during blunt dissection between the tumor and the diaphragm[et al. have reported that a resection margin of 1 cm was the only significant prognostic factor for poor disease-free survival after en bloc resection[et al. pointed out that there was a possibility of increased intraoperative blood loss and a longer surgery when the diaphragm was resected[HCC is the most common primary malignant tumor of the liver,2. Lai apatients. Dopplerpatients. In thispatients. On the patients. To daten Table . Lau et esection,19. Yamaiaphragm. Leung eesection. Howeverresected. Thus, ien bloc resection of the diaphragm seems appropriate; however, such extensive surgery is thought to present too high a risk of damage during the postoperative course, especially in emergency operation. For hemoperitoneum patients with unpredictable HCC rupture and diaphragm invasion, physicians should establish a therapeutic plan with consideration of a surgical approach.The prognosis of spontaneous rupture of HCC is poor with a high hospital mortality rate. A peripherally located large HCC lesion is clinically prone to grossly involve the diaphragm, either by dense adhesion or as a rare result of histological invasion. In such cases, Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the editor-in-chief of this journal.The authors declare that they have no competing interests.MHY coordinated the team, helped in literature research and edited the final version of the manuscript. PKK collected the information and wrote the article SIY researched the literature and wrote the article. All authors read and approved the final manuscript.


Tumors originating from ceruminous glands are rare lesions of the external auditory canal. The lack of specific clinical and radiological signs makes their diagnosis challenging. We report the case of an exceptionally rare benign tumor, a syringocystadenoma papilliferum (SCAP), in an atypical location in the bony segment of the external auditory canal with uncommon clinical signs. The special traits of the case included the following: the most lateral component of the tumor was macroscopically cystic and a granular myringitis with an obstructing keratin mass plug was observed behind the mass. The clinical, audiological, radiological, and histological characteristics of the neoplasm are consequently presented. Intraoperative diagnosis of the epidermal cyst was proposed. The final diagnosis of SCAP was determined only by histological analysis after the surgical excision. The educational aspects of the case are critically discussed. Syringocystadenoma papilliferum (SCAP) was first described in the dermatological literature in the beginning of the 20th century as a “naevus syringadenomatosus papilliferus” . OccasioTo date, fewer than 150 case reports of ceruminous gland tumors originating in the EAC can be found in the literature . Among tThe present case illustrates an atypical location for an exceptionally rare disease.A 61-year-old woman presented to the tertiary referral Republican Vilnius University Hospital with a 4-month history of moderate ear pain, fullness, hearing loss, tinnitus, and periodic otorrhea on the right side. She had no previous history of ear infection or discharge. Otoscopically, the bony part of the EAC was fully obstructed by a single pink ulcerated polypoid mass with a hole in the center . GranulaCeruminous glands are normally located in the outer one-third to one-half of the external auditory canal skin overlying the cartilaginous region. It is generally accepted that the bony canal is devoid of such glands. In the present report, the SCAP originated from the anterior wall of the bony part of the EAC. Ceruminous glands tumors have previously been characterized as well-differentiated localized benign neoplasms that are occasionally cystic and demonstrate the papillary proliferation of glands that are histologically similar to normal ceruminous glands . Some auOccasionally, only histological examination may discover the proper diagnosis. However, when an insufficient amount of material is obtained, the intraglandular papillary structures of an SCAP, for example, may be easily missed. The type of the biopsy and its extent in cases of EAC lesions are still controversial issues. The analysis of similar cases in general does not suggest an exact algorithm for specific diagnoses, treatment opportunities, and clinical outcomes for lesions in the EAC. Preoperative incisional biopsy to establish the diagnosis can be performed to resolve differentiation problems; nevertheless, such biopsies involve a certain level of risk because all ceruminous gland tumors, including SCAPs, are thought to be potentially malignant , 15. In High-resolution computed tomography and magnetic resonance imaging are important modalities for diagnosing and differentiating masses in the EAC. Recently, Kamakura et al. presented the MR imaging characteristics of an SCAP of the EAC: intermediate signal intensities on T1- and T2-weighted images and slight enhancement on gadolinium-enhanced T1-weighted images . Judging


Neuroglial heterotopia, heterotopic brain tissue, or differentiated neural tissue outside the cranial vault is uncommon, and these anomalies most commonly occur in the nasal cavity.We report a case of rare pure cystic heterotopic brain tissue in a two-month-old Caucasian baby girl that presented as a large cystic neck mass and was confused with a cystic hygroma. Her mother reported a progressive increase in the size of this swelling and mild respiratory difficulty when the girl was sleeping. A computed tomography scan of the brain and neck showed a large heterogeneous mass extending from the base of the skull to the left submandibular region; a cystic component was also noted. Our patient under went total excision of the cystic mass and prevention of airway obstruction by a left submandibular approach. The final gross pathology diagnosis was heterotopic brain tissue.Pure cystic neck heterotopic brain tissue lesions are very uncommon, and a preoperative diagnosis of this lesion is difficult. Brain heterotopia is a rare, benign condition that should be considered in the differential diagnosis of the neonatal head and neck mass. Neuroglial heterotopia, heterotopic brain tissue, or differentiated neural tissue outside the cranial vault is uncommon, and these anomalies most commonly occur in the nasal cavity ,2. In raA two-month-old Caucasian baby was admitted to our pediatric surgical ward because of swelling in her left neck region present since birth. She was noted by her mother to have a growing left-sided neck mass and mild respiratory difficulty when sleeping. There was no associated odysphonia or dysphagia. No neck stiffness and no upper respiratory tract infection had been noted in the few days before presentation. Her prenatal history was unremarkable. Our patient experienced snorting, nasal flaring, and an inability to feed on initial evaluation. A physical examination revealed no gross craniofacial abnormalities; our patient was a healthy baby with a large (6 cm × 8 cm) palpable mass in the left neck extending from the upper neck to the supraclavicular fossa. The mass was soft, compressible, non-tender, and fixed. There were no associated changes in the overlying skin. The remainder of the physical examination was normal. A chest radiograph showed a mass lesion in the left neck without extension into the mediastinum. The mass caused mild deformity and deviation of the left mandible and masticator muscles anteriorly. The airway at the pharyngomucosal space was compressed and stenosed. A CT scan of our patient's brain and neck showed a large heterogeneous mass extending from the base of the skull to the left submandibular region; a cystic component was also noted. Findings from a CT scan of the brain were normal. The initial diagnosis was cystic hygroma. Our patient then received total excision of the cystic mass and prevention of airway obstruction by a left submandibular approach Figure . During Heterotopic neuroglial tissue (HNT) was first described by Reid in 1852 . ComposeThe treatment for heterotopic brain tissue is complete surgical excision. Surgical intervention is necessary in patients with heterotopic parapharyngeal neuroglial tissue that causes airway distress, dysphagia, or failure to thrive. The timing of surgery is controversial. Proponents of delayed resection believe that resection might be safer in the older child, in whom vital neurovascular structures are more easily salvageable and blood volume is greater -6. RecurIn summary, pure cystic neck brain tissue heterotopias are very uncommon lesions, and a pre-operative diagnosis of this lesion is difficult. Heterotopic brain is a rare, benign condition that should be considered in the differential diagnosis of the neonatal head and neck masses.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The author declares that they have no competing interests.


Severe benign lymphoid hyperplasia (LH) is unusual in the head and neck region, but the diagnosis of LH is of clinical importance as it may be confused with malignant lymphoma, both on clinical examination and pathologically. While the etiology is poorly understood, a number of previous theories exist, which are included here in the context of a literature review. In this paper we present a case of severe pharyngeal lymphoid hyperplasia causing airway obstruction and requiring tracheotomy and subsequent surgical debulking. A 64-year-old African Canadian female with a history of urinary incontinence was admitted for an elective bladder suspension procedure by the gynaecology service in our institution. At the time of induction, our service was called emergently due to failed intubation related to a “pharyngeal mass.” Upon examination with direct laryngoscopy a large, multiloculated, exophytic mass was identified, emanating from the oropharynx and extending distally to the level of the supraglottis, occupying >90% of the upper aerodigestive tract. The obstructive lesion was biopsied, and specimens were sent fresh for lymphoma histopathology protocol. The airway was subsequently secured, and the procedure was undertaken. Review of the preoperative anaesthesia records revealed no features of airway obstruction nor B symptoms on clinical history. Postoperatively the patient was deemed unsafe for extubation and transferred to the intensive care unit while placed on high-dose intravenous dexamethasone. CT scan revealed the epicenter at the base of tongue and an appearance suspicious for malignancy . There wOpen tracheotomy was performed on POD 3 due to the absence of a leak, and biopsies were again performed, which ultimately revealed the equivalent benign pathologic findings. The patient was kept on a three-week course of tapering prednisone and proton-pump inhibitors. Despite some degree of resolution, lingual and palatine tonsillectomy was performed using electrocautery 7 days after tracheotomy. This procedure was carried out under general anesthetic in the form of a modified adenotonsillectomy, using a Boyle Davis gag for exposure and a combination of monopolar cautery for the palatine tonsils and suction cautery for subtotal ablation of the lingual tonsils. The patient was decannulated and discharged home 14 days after tracheotomy. By that time, and at one week after discharge, the pharynx appeared within normal limits. Follicular lymphoid hyperplasia (FLH) is an uncommon benign entity related to a rapid increase in the abundance of lymphocytes contained within or outside of lymph nodes. It has been historically referred to as reactive lymphoid hyperplasia or pseudolymphoma . This enarrangement (expansion) in the local tissue DNA [The diagnosis of FLH is of clinical importance as it may be confused with malignant lymphoma, both on clinical examination and histopathology. The etiology is poorly understood, although some authors have postulated a relationship with chronic irritation  or a reactive lymphoid proliferation to an unknown antigenic stimulation . While assue DNA . LH most commonly affects older patients, with a mean age of 61 and female-to-male ratio of nearly 3 : 1. It tends to present as a unilateral, painless, slow-growing, nonulcerated mass. The mean size is 2.5 cm in the literature (range 1–5 cm). Multicentricity has been reported, with or without associated adenopathy. Clinical and laboratory investigations are routinely negative [The differential diagnosis includes lymphoma, mesenchymal tumors, salivary gland neoplasms, and adenomatoid hyperplasia . MorpholSurgical debulking/excision is the treatment of choice. External beam radiation has been successful in a single case . A minorSevere benign LH is unusual in the head and neck region, but the diagnosis should be entertained on the part of the clinician both clinically and histologically when lymphoma is suspected—particularly in the oral cavity.


Sebaceoma is a benign tumor composed of incompletely differentiated sebaceous cells of varying degrees of maturity. Sebaceomas was never reported as a known premalignant lesion.This is a report of a sixteen year old boy who presented with a malignant transformation of a recurrent sebaceoma which was excised twice by Moh’s surgery. Excision was done with a free margin of 1 cm down to the parotid fascia. Reconstruction was performed on the same set by using cervicofascial flap extending down to the supra-clavicular area. The patient had an uneventful postoperative period apart from distal marginal necrosis of the flap, which healed nicely with conservative measures and daily dressing and was sent to our cancer centre to start his adjuvant radiotherapy.Previous literature stated that sebaceoma is a distinctive benign tumor. We have presented a case of an unusual malignant transformation of a preauricular recurrent sebaceoma. This indicates that sebaceoma does have a potential risk of malignant transformation. We believe that managing recurrent sebaceoma more aggressively with wide local excision and postoperative adjuvant radiotherapy would provide better prognosis. Sebaceoma is a benign tumor composed of incompletely differentiated sebaceous cells of varying degrees of maturity. Sebaceoma was never reported as a known premalignant lesion. This is a report of a sixteen year old male patient who presented with a malignant transformation of a recurrent sebaceoma of the right pre-auricular area.A previously healthy sixteen year old male patient presented with a right pre-auricular lesion. This was excised by general surgery department and was reported as a sebaceous cyst. One year later he presented with a friable nodule measuring 1 cm × 0.75 cm in the scaphoid fossa of the right ear and incisional biopsy showed histological features of sebaceoma , 2. Moh’One year later the patient presented with a 0.5 cm × 0.75 cm soft purplish lesion. Incisional biopsy was consistent with sebaceous carcinoma , 4. MRI This was managed by wide local excision with postoperative radiotherapy. Excision was done with a free margin of 1 cm down to the parotid fascia. Local cervicofascial rotational flap area  was usedThe patient had uneventful postoperative period apart from distal marginal necrosis of the flap, which healed nicely with conservetive measures and daily dressing and was sent to our cancer centre to start his adjuvant radiotherapy. The patient was followed for 13 months with no evidence of recurrence.Sebaceoma is a new term proposed to designate a distinctive rare benign neoplasm of adnexal epithelium with differentiation toward sebaceous cells.The histologic spectrum extends from that seen in sebaceous adenoma to lesions that may be difficult to distinguish from sebaceous carcinoma.676 and complete surgical excision is required.9Sebaceous carcinoma is a rare, aggressive, malignant tumor that is derived from adnexal epithelium of sebaceous glandsIn a clinicopathological study of five cases with sebaceous carcinoma arising in nevus sebaceous of Jadassohn, it has been shown that sebaceous carcinoma was always accompanied by other benign or malignant adnexal lesions such as sebaceoma, syrigocystadenoma papilliferum, syringocystadenocarcinoma papilliferum, trichoblastoma, trichlemmoma, desmoplastic tricholemmoma, or syrigoma.We have presented a case of an unusual malignant transformation of a preauricular recurrent sebaceoma. This indicates that sebaceoma could have a potential risk of malignant transformation. Managing recurrent sebaceoma more aggressively with wide local excision and postoperative adjuvant radiotherapy would possibly provide better prognosis.


Soft tissue sarcomas are uncommon tumors, and intraduodenal soft tissue sarcoma manifestation is even more rare. Only three cases of intraduodenal sarcomas have been reported in the literature thus far. Here, we report a case of an intraduodenal recurrence of a retroperitoneal sarcoma causing bowel obstruction. This unusual recurrence pattern likely relates to the patient’s previous resection and radiation treatment, and highlights the benefits, limitations and follow-up strategies after multimodality treatment. Soft tissue sarcomas (STS) are rare tumors, accounting for less than 1% of all newly diagnosed malignancies . These tA 60-year-old male patient had previously undergone a radical resection of a T2bN0M0G3 Stage III retroperitoneal sarcoma, including right nephrectomy and cholecystectomy, in 2002. Pathologic evaluation demonstrated a 25 cm high-grade liposarcoma with different histologic components, including myxoid, round cell, well-differentiated, sclerosing and pleomorphic patterns. Surgical margins were negative as the tumor did not invade the kidney, and since the renal vein and ureter were free of tumor as well.The patient did not undergo any additional therapy. Surveillance magnetic resonance imaging in 2007 revealed an isolated tumor recurrence in the retroperitoneum. An exploratory celiotomy revealed tumor involvement of the duodenum, head of the pancreas, superior mesenteric vein, vena cava and left renal vein with severe adhesive changes, leading to the intraoperative assessment of unresectable disease. Subsequently, the patient underwent stereotactic body radiation to the retroperitoneal tumor in five fractions for a total dose of 30 Gy, without complication. Postradiation imaging revealed near complete resolution of the retroperitoneal mass.One year later, the patient presented with weight loss, postprandial abdominal pain, nausea and vomiting. Cross-sectional imaging revealed a 7 cm mass with involvement of the third portion of the duodenum Figure . HoweverIntraduodenal presentation of STS is rare. To the best of our knowledge, there are only three cases reported in the literature. In the patient described, duodenal involvement by the extraduodenal sarcoma may have developed from either contiguous extension or hematogenous metastasis. However, at the time of the duodenectomy, there was no evidence of any extraduodenal disease or any distant metastasis. The solely intraduodenal growth pattern suggests that the previous radiation therapy to the retroperitoneal area, with duodenal shielding, had an impact in generating such an unusual disease pattern. This duodenal shielding from radiation together with a complete extraduodenal response to radiation therapy may thus have facilitated the isolated intraduodenal recurrence.Liposarcoma is the most common histologic subtype of retroperitoneal sarcomas, with a higher local recurrence rate than non-liposarcomas (58% versus 22%) . Even whThe patient was symptomatic from the duodenal recurrence with partial bowel obstruction and weight loss. Given his excellent performance status and the lack of vascular involvement by the tumor, the decision was made to resect the lesion. Not resecting the tumor would almost certainly have guaranteed tumor progression and, ultimately, oncologic failure with a high risk for death from the disease. Radiation was not considered as part of the therapy for the duodenal recurrence as there was clear evidence of bowel involvement. Performing radiation therapy on the bowel for a malignant obstructive process would likely lead to significant gastrointestinal toxicity, with questionable outcomes regarding the resolution of the obstructive component.Complex retroperitoneal STS often mandate a multidisciplinary approach to treatment sequencing and selection. An awareness of sarcoma subtype-specific responses to radiation may help select patients who are more likely to demonstrate extraordinary responses. For patients with initially unresectable tumors and good response to radiation therapy, resection options should be re-evaluated as they can lead to lasting disease or symptom control.Written informed consent was obtained from the patient for obtaining intraoperative images for educational or publication purposes. The authors submitted a waiver from their institutional review board (IRB) stating that this case report does not require IRB approval or oversight.The authors declare that they have no competing interests.JB drafted and revised the manuscript. JM contributed to the content of the manuscript and critically reviewed the manuscript. RT contributed to the content of the manuscript and revised the manuscript critically for important intellectual content. AK contributed to the content of the manuscript. GE contributed to the content of the manuscript. RS conceived of the case report, contributed to the content of the manuscript, revised the manuscript critically, and approved the final version for publication. All authors read and approved the final manuscript.


Tumoral calcinosis is an uncommon disorder characterized by the development of calcified masses within the peri-articular soft tissues of large joints, but rarely occurs within the hand.We present the case of a 31-year-old pregnant Indian woman with a three-month history of painful swelling within the tip of her right middle finger following a superficial laceration. She was otherwise well and had normal serum calcium and phosphate levels. Plain radiography demonstrated a dense, lobulated cluster of calcified nodules within the soft tissues of the volar pulp space, consistent with a diagnosis of tumoral calcinosis. This diagnosis was confirmed on the basis of the histopathological examination following surgical excision.To the best of our knowledge, we present the only reported case of acral tumoral calcinosis within the finger, and the first description of its occurrence during pregnancy. We review the etiology, pathogenesis and treatment of tumoral calcinosis. The term tumoral calcinosis was coined by Inclan et al. in 1943 [Tumoral calcinosis is an uncommon pathological entity characterized by multiple circumscribed, tumor-like, calcified masses in peri-articular connective tissue. These lesions mainly comprise calcium hydroxyapatite crystals and amorphous calcium phosphate . They weThe most frequent cause of tumoral calcinosis is chronic renal failure, with a reported prevalence of 0.5% to 1.2% in patients undergoing hemodialysis . FamiliaThe most common locations of tumoral calcinosis are around the hip joint, elbow, shoulder, foot and wrist joints, with a predisposition for extensor surfaces . This coA 31-year-old, right-hand-dominant woman of Indian origin presented with a three-month history of increasing swelling overlying the volar tip of her right middle finger. She recalled sustaining a superficial laceration to the area with a clean kitchen knife, which was treated successfully by application of a clean, dry dressing. She subsequently noticed the gradual appearance and enlargement of a locally painful swelling at her fingertip over a three-month period. Two weeks prior to presentation her general practitioner prescribed antibiotics for a presumed infection, but she experienced no improvement in symptoms. Although she was 30 weeks into her uncomplicated pregnancy at the time of injury, she had no history of systemic disease or any relevant family history.The clinical examination revealed a tender, solitary 1.5 cm × 3 cm elliptical lesion located within the pulp space of the patient's right middle fingertip and extending to the distal inter-phalangeal joint. The swelling had a firm, non-fluctuant nodular consistency, with an overlying, centrally located punctum from which no discharge was expressible (Figure The laboratory test results, including complete blood count, urea and electrolytes, erythrocyte sedimentation rate, C-reactive protein, serum urate, calcium, phosphate and alkaline phosphatase were all within the normal ranges. Plain radiographs revealed multiple circumscribed, calcified masses located in the soft tissues adjacent to the distal phalanx involving the distal inter-phalangeal joint with some extension into the distal aspect of the proximal phalangeal region Figure . A diagnSurgical excision was performed with the patient under general anesthesia and tourniquet control through a 3 cm volar mid-line longitudinal incision overlying the finger pulp. Macroscopically, three pieces of firm, dense, chalky white nodular tissue ranging from 30 mm × 10 mm × 5 mm to 10 mm × 5 mm × 2 mm were excised and sent for histopathological examination Figure , and theet al.'s original series [de novo in the distal phalangeal and/or acral region of a finger alone. Additionally, the case we describe involving the combination of pregnancy with tumoral calcinosis is unique. A distinctive feature of tumoral calcinosis affecting the hand is that it is painful because of the superficial location and pressure on adjacent cutaneous nerves [Since the publication of Inclan l series , more thl series -9, with s nerves .et al. [et al. [The literature is inconsistent regarding the diagnostic criteria for tumoral calcinosis, with some texts stating that only the familial form, as originally described by Inclan et al. , deserveet al. . Smack e [et al. , having The etiology of primary as well as secondary tumoral calcinosis remains unknown, although it is accepted that a familial disturbance in phosphate homeostasis is a contributing factor . The mecThe treatment of tumoral calcinosis depends largely on its underlying cause. Surgical excision is the mainstay of treatment, but recurrences are common following incomplete excision or in cases involving actively progressing lesions . PhosphaTumoral calcinosis is an uncommon pathological entity characterized by multiple circumscribed, tumor-like, calcified masses in peri-articular connective tissue comprising calcium hydroxyapatite crystals and amorphous calcium phosphate. It does not commonly affect the hand. To the best of our knowledge, we present the only reported case of acral tumoral calcinosis linked to pregnancy. Surgical excision offers the best treatment, but other therapies have been suggested in the literature.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.NH wrote the case report and treated the patient pre- and post-operatively. DPSB was the treating consultant who performed the operation and was responsible for the patient's care. He also reviewed the article and approved it. VG wrote the article and reviewed the literature. PP wrote the article and reviewed the literature. MCB was the treating consultant who performed the operation and was responsible for the patient's care. He also reviewed the article and approved it. WAB was the consultant radiologist who performed all the imaging and made the radiological diagnosis. All authors read and approved the final manuscript.


Decompression of the L5 nerve and resection of the tumor was performed. Osteoblastoma is a rare tumor with an incidence of 1% of all tumors and 30–40% of cases involving the spine. Osteoblastoma occurs most commonly in males . The most common area of involvement is the cervical spine followed by the lumbar spine. Posterior elements of the vertebrae are commonly involved.To report a rare occurrence of osteoblastoma involving the L4 vertebra in an 8-year-old female child with histological features suggestive of osteoblastoma with secondary aneurysmal changes. The mean age incidence of osteoblastoma is 20.4 years. In our case, a rare presentation of osteoblastoma was seen in the first decade. The child was admitted with a 1-year history of increasing back pain and radiculopathy. The child was evaluated with X-rays, computed tomography scan and magnetic resonance imaging, which indicated involvement of the posterior elements of the 4 Osteoblastoma is a benign vascular bone-forming tumor. It was independently described by Lichtenstein and JaffAn 8 year old female child came to us in nov 2008 with complaints of back pain and restriction of lower back movements since 1 year. Initially, the pain was aggravated on standing and while walking. Later, she had rest pain. She also had tingling and numbness along the dorsal aspect of her left leg since 6 months. This sensory disturbance was associated with slippage of footwear while walking.On examination, there was localized swelling in the L4 region on the left side. There was also paraspinal muscle spasm. Lumbar spine movements were restricted. Localized and diffuse tenderness was present. Muscle power in extensor hallucis longus and extensor digitorum longus was grade 4 out of 5 in the left lower limb. Superficial sensory functions in the left L5 dermatome were diminished. Muscle stretch reflex in the left ankle was sluggish.We thoroughly investigated the patient with plain roentgenograms, computed tomography (CT) scan and magnetic resonance imaging (MRI). Plain roentgenogram showed expansion of the left fourth lumbar pedicle, superior articular facet and transverse process. Flecks We found a firm, reddish to brown mass extending from the spinous process to the pedicle. Pseudocapsule with little vascularity was seen surrounding the mass. The tumor mass was friable. Complete excision of the tumor was possible with pseudocapsule in toto. There was no evidence of any necrosis or cystic spaces in the tumor.th lumbar nerve. No fresh deficits were noted postoperatively.The patient was relieved of back pain. The patient was eased of signs of neurological compression of the 5Multiple grey white to grey brown soft tissue bits with bony spicules.Multiple sections show irregular interconnecting trabeculae of woven bone within a fibrous stroma. The traOsteoblastoma is a rare bone tumor and accounts for approximately 0.5–1% of all primary bone tumors.–13 Spina113131515131541516Delay of diagnosis is very common, and seems to occur on average 6–12 months or later following the initial presentation.1621–23 T163


A 47-year old man with HIV presented with a stroke. Imaging revealed a large mobile left-ventricular thrombus. The mass was resected using a small ventriculotomy with good early postoperative prognosis. Thrombus etiology is likely related to HIV pathology. LV thrombi typically occur in the case of impaired LV dysfunction as a result of dilated cardiomyopathy, aneurysm or a myocardial infarction . HoweverA 47-year old man with HIV presented with a stroke. Upon workup, he was found to have a large mobile cardiac mass in his left ventricle.An echo revealed a 5.4 × 2.3 cm smooth, highly mobile surfaced mass to be adherent to the left ventricular apex Figure . The lefThe mass was removed via ventriculotomy on cardiopulmonary bypass Figure . PatholoA thrombus of this size is uncommon, particularly in a patient under 50 years of age with no known cardiac disorder. The patient’s positive HIV status is a possible risk factor for general thromboembolism and microembolism of the coronary circulation. HIV and AIDS are known to be associated with various abnormalities predisposing patients to a hypercoagulable state -6. TheseThe management of LV thrombi is often restricted to anticoagulant and thrombolytic therapy . HoweverWritten informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.N.H. complied the images and drafted the manuscript. L.S. provided figures and added to the case presentation. R.L. performed the operation and oversaw the manuscript production process. All authors read and approved the final manuscript.The authors declare that they have no competing interests.


Desmoid tumors  are rare slow growing benign and musculoaponeurotic tumors. Although these tumors have a propensity to invade surrounding tissues, they are not malignant. These tumors are associated with women of fertile age, especially during and after pregnancy. We report a young female patient with a giant desmoid tumor of the anterior abdominal wall who underwent primary resection. The patient had no history of an earlier abdominal surgery. Preoperative evaluation included abdominal ultrasound, computed tomography, and magnetic resonance imaging. The histology revealed a desmoid tumor. Primary surgical resection with immediate reconstruction of abdominal defect is the best management of this rarity. To the best of our knowledge and PubMed search, this is the first case ever reported in the medical literature of such a giant desmoid tumor arising from anterior abdominal wall weighing 6.5 kg treated surgically with successful outcome. These tumors often appear as infiltrative, usually well differentiated and locally aggressive in nature and also known as deep fibromatoses. Desmoid tumors may be classified as extra-abdominal, abdominal wall, or intra-abdominal ). It is thought that the lesions may develop in relation to estrogen levels or trauma/operations. They constitute 3% of all soft tissue tumors and 0.03% of all neoplasms [Desmoid tumors  are rare slow growing benign fibrous tumors without any metastatic potential but a strong tendency to invade locally and to recur. The term desmoids was coined by Muller in 1838 and is derived from the Greek word A 25-year-old female patient presented to surgery department with a painless mass in the lower abdomen for 15 months which was gradually increasing in size. The patient had no history of previous abdominal surgery or any abdominal trauma. On examination, a single lower abdominal mass involving right lumbar, umbilical, hypogastric, and both iliac regions and extending vertically 6 cm below xiphisternum to pubic symphysis and transversely from right anterior axillary line to left anterior axillary line . Lump was fixed to the anterior abdominal wall, nontender, globular in shape of size 25 × 15 × 20 cm with smooth surface, and firm in consistency. Ultrasonography demonstrated a large solid heterogeneous hypoechoic mass showing internal vascularity, few anechoic areas of necrosis, seen in pelvic cavity extending to right lumbar region displacing the bowel loops superiorly.CT scan revealed a neoplastic anterior abdominal muscle mass arising from right anterolateral abdominal wall muscles in right lumbar, right iliac fossa, crossing midline extending to involve contralateral anterolateral abdominal wall muscles, anteriorly causing stretching of subcutaneous tissue. Intra-abdominal extension of mass reaching up to anterior surfaces of lower lumbar vertebrae mildly compresses aorta and IVC and displaces underlying bowel loops and bladder. Differentials include desmoid tumor and rhabdomyoma. No evidence of lymphadenopathy was detected. No evidence of free fluid was seen in pelvic and peritoneal cavity . MRI abdomen and pelvis Figures  revealedThe postoperative course was uneventful, and patient was discharged on the 9th postoperative day. Histopathological reports were consistent with desmoid tumor with negative surgical margins. At 8 months of followup, patient did not have any recurrence or incisional hernia. Desmoid tumors are cytologically benign fibrous neoplasm originating from the musculoaponeurotic structures throughout the body. Desmoid tumors are benign deep aggressive fibromatoses, originating from fascia and muscle aponeurosis with an infiltrating growth . ApproxiAbdominal desmoid tumor usually presents as a firm mass with ill-defined margins and no distinct capsule . On cut On ultrasonography, desmoid tumors appear as well-defined lesions with variable echogenicity. On CT, they may appear as homogeneous or heterogeneous and hypo-, iso-, or hyperintense compared with the attenuation of muscles , 6, 8, 9Wide local excision with reconstruction of the defect is the treatment of choice. Peritoneum, intraperitoneal organs, or adjacent bony structures involved by tumor must be resected as well. Incomplete tumor removal or positive surgical margins may lead to local recurrence –6 (20% tThe history of painless abdominal mass, the age and sex of the patient, the location of the mass within the anterior abdominal wall, and the imaging features make desmoid tumor a strong primary diagnostic consideration even if it is a rare entity. Aggressive, wide surgical resection with negative margin is the best surgical option. Complete surgical excision of desmoid tumors is the most effective method of cure, sometimes necessitating removal of most of an involved anterior abdominal wall in such a giant desmoid tumor with immediate repair of resultant huge defect and reconstruction using prosthetic mesh for better functional results.


Littoral cell angioma (LCA) is a rare splenic vascular neoplasm that arises from the cells lining the red pulp sinuses. It is deemed to be a benign and incidental lesion. The earliest literature report of littoral cell angioma has been described by Falk. The examination of samples after splenectomy reveals similar pathological change and its change rule is summarized. However, many recent reports have described it to be a malignant tumor with congenital and immunological associations. Generally speaking, the definitive diagnosis can only be made after histological and immunohistochemical profiles. In this case report, we presented the case of a 48-year-old woman with multiple splenic LCAs. Initially, the patient was characteristics of abdominal distension, weakness and fatigue. Multiple hemangiomas were observed in the spleen through abdominal ultrasonic diagnosis. Computed tomography (CT) scans revealed the splenomegaly with multiple round and hyperdense lesions. The patient subsequently underwent splenectomy. Postoperative histological and immunohistochemical studies confirmed the diagnosis of LCA. Based on the presentation of this case, clinical, radiographic and pathological results of LCA as well as recent advances in our understanding of this uncommon splenic lesion were reviewed. LCA is an uncommon splenic tumor diagnosed in patients with or without abdominal discomfort. Only a few case reports regarding this kind of tumor have been published as inconsistent results. In the present paper, we have reported a case of LCA and reviewed the literature. LCA is a benign neoplasm of the spleen that was first described by Falk et al. in 1991 , when thA 48-year-old Chinese woman was admitted due to non-specific complaints of abdominal distension, weakness and fatigue. This patient had been diagnosed with an oophoritic cyst 11 years ago and right oophorocystectomy. Meanwhile, this patient did not have medical history of weight loss, fever, or changes in bowel habits. Physical examination was notable for only splenomegaly. Abdominal ultrasonographic examination showed the increase in volume and multifocal processes throughout the spleen Figure . The nodThe differential diagnosis of splenic vascular tumors are broad and may represent benign , indeterminate , or malignant neoplasm (angiosarcoma) . Since tThe exact incidence of LCA is unknown although the incidence of splenic haemangioma varies from 0.03% to as high as 14% in a series of autopsy reports . ClinicaUsually, the diagnosis is made after splenectomy for some other reasons. However, approximately 45% patients will present with splenomegaly, fever and features of hypersplenism (anaemia and thrombocytopenia) .LCA may occur at any ages without sex-based predilection. Clinically, the disease may manifest as splenomegaly, anemia, and, less frequently, thrombocytopenia. LCA may also appear as a single or multiple lesions in the spleen. Diagnosis is usually made incidentally during surgery. Massive splenomegaly because of LCA can be mistaken as a pancreatic tumor.Recent reports describe LCA as being associated with neoplasms of the colon, kidney, pancreas, lung and ovary ,24. AssoAs a result of its unique histological and immunohistochemical characteristics, the definitive diagnosis can only be made on tissue samples obtained either cytologically or post-splenectomy because radiological findings are not specific for LCA. Diagnosis can be made by fine-needle biopsy, but surgery seems crucial because of suspected malignancy. Therefore, an evaluation for concomitant malignancy and surveillance is recommended.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.ZQH performed the literature review and manuscript writing. YJA reviewed and revised the manuscript and provided radiographic images. QMS reviewed and revised the manuscript and provided pathological images. WL provided original idea and assisted the manuscript revision. All authors read and approved the final manuscript.


Paragangliomas are rare tumours with a prevalence of 1/10000 to 1/30000. Tumors arising from the paraganglia are characteristically of low malignant potential. Vertebral metastases are exceedingly rare, and only isolated case reports have described them. The authors present the clinical course of a 47 years-old female patient with a familial paraganglioma [PGL] with vertebral metastastization, who underwent an intralesional tumor excision and corpectomy. Genetic screening demonstrated a new germinal frameshift mutation of the SDHB exon 6 [c.587-591DelC]. After surgery there was normalization of the analytical parameters and imagiologic screening. One year later she presented a new image in the the pedicle of T11 on the contralateral side of the surgical incision. She performed 2 treatments with MIBG and 1 cicle of radiotherapy that made the new lesion regress. Currently the patient does not present any clinical or analytical evidence of new metastasis.This case outlines the clinical course of a patient with a PGL syndrome for whom a rare vertebral metastasis was diagnosed. It highlights the importance of identifying patients with germline SDHB mutations, as these patients are at a high risk of developing malignant disease. The paragangliomas/pheochromocytomas are rare tumours with a prevalence of 1/10000 to 1/30000 .Hereditary cases represent 35% of all pheochromocytoma and paraganglioma and are associated with gene mutations. All cases display an autosomal dominant inheritance pattern with incomplete penetrance and variable expression .Tumors arising from the paraganglia are characteristically of low malignant potential, with only 10% developing distant spread. The great majority of metastasizing tumors has evidence of distant spread at diagnosis. Fifty percent are located at the cervical lymph nodes, and the others are evenly distributed among bone, lung and liver. Vertebral metastases are exceedingly rare, and only isolated case reports have described them .The authors present the clinical course of a 47 years-old female patient with a familial paraganglioma, for whom an unusual vertebral metastasis was identified and treated.37 years-old caucasian female, without known diseases until 1997, when she complains of headaches, restless feeling, anxiety and palpitations, symptoms that she attributes to stress. Routine screening showed hypertension that was resistant to medical treatment. Laboratory analyses demonstrated an elevation in the 24 h dosage of vanilmandelic acid [VMA]: 52 mg/24 h [N: 1.4 – 6.5]; epinephrine: 21 μg/24 h [N 0–20]; norepinephrine: 3161 μg/24 h [N: 23–105]; dopamine: 711 μg/24 h [N: 65–400]; metanephrine: 91 μg/24 h [N: 52–341]; normetanephrine: 3546 μg/24 h [N 88–444]. Abdominal CT showed a 5.2 cm mass in the region of the left adrenal gland, with a corresponding hyperfixation in the MIBG scintigraphy. She underwent surgery for exploration and removal of the lesion. Intraoperatively it was found that the lesion was separated from the adrenal gland. This finding together with the pathologic report of a neoplasia invading the capsule, not going beyond it, and images of venous invasion made the diagnosis of malignant paraganglioma. Genetic screening of the patient demonstrated a new germinal frameshift mutation of the SDHB exon 6 [c.587-591DelC]. There was a family history of a cousin diagnosed at 13 years of age with a dopamine producing malignant paraganglioma, that died at 30 years of age with wide spread metastasis [cranial and lumbar].In the post operative period there was a normalization of VMA, catecholamines and metanephrines concentrations. On March 2006 routine analyses showed an elevation of catecholamines. Abdominal MRI showed a recidiva that was confirmed by MIBG cintylogram. The patient underwent a new surgical removal of the tumor. Pathologic report confirmed this to be a local metastasis of the previous paraganglioma. Once again there was a normalization of the analytical parameters after surgery but 12 months after this episode she started complaining of back pain. Analytical evaluation showed a new elevation of VMA and catecholamines concentrations. This time scintigraphy revealed a solitary hyperfixation on T11 Figure . The patThe prevalence of catecholamines or metanephrines secreting tumors in cases of hereditary pheochromocytoma and paraganglioma disorders is 10–20% in patients with von Hippel–Lindau disease, approximately 40% in patients with multiple endocrine neoplasia type 2A and 2B, and 0.1–5.0% in patients with neurofibromatosis type 1,7.The incidence of pheochromocytoma is 0.001 in the general population. There aren’t reports stating the real incidence of paragangliomas, but it is consensual that it is far bellow the incidence of pheocromocytoma. The malignant diagnosis is noted according to whether metastasis or invasion exists.Paragangliomas are commonly bilateral and may be multicentric. Multicentricity occurs in approximately 10% of tumors. Paragangliomas characteristically have a slow and unpredictable growth pattern, making it difficult to determine the natural history of the disease .There are five types of familial paraganglioma [PGL] syndromes that have been identified with mutations in the SDH genes that encode subunits of the heterotetrameric SDH complex. SDH is involved in oxidation of succinate to fumarate in the Krebs cycle and provides electrons to the mitochondrial electron transport chain .Investigation has revealed that SDHD mutations [PGL type 1] are associated with multifocal head and neck paragangliomas, whereas SDHB mutations [PGL type 4] are associated with extra-adrenal disease and malignancy ,9.Several studies have suggested that patients who are carriers of the SDHB mutation [PGL type 4], like the patient we describe, are more likely to develop extra-adrenal lesions [abdominal or thoracic] and malignant disease .The WHO defines malignant pheochromocytomas and paragangliomas as metastatic disease at sites where chromaffin tissues are not usually present. Metastatic sites include the lungs, bones, liver, and lymph nodes. Malignant tumors are typically larger, have a higher mitotic count, have extensive local or vascular invasion and express fewer peptides on immunohistochemical studies compared with benign tumors .There is little correlation between histologic appearance and malignant potencial. Tumors with significant atypia and pleomorphism have often behaved in benign fashion, possibly because they were removed before metastasis. Benign-appearing tumors can behave in aggressive fashion. Several authors have found that the presence of mitosis in the pathologic specimen correlated significantly with malignancy . In thesParagangliomas often exhibit a prolonged time interval to the development of recurrence or metastasis. Recurrence and/or distant metastases have been reported to occur from 0 to 20 years after diagnosis ,12.In the current case the patient presented one local recidiva and a distant metastasis 9 and 10 years after the initial diagnosis, respectively. In both situations it was decided that the patient was eligible for surgical treatment with curative intention because these were always isolated lesions at the time of diagnosis .The patient underwent a biannual monitoring program with physical examination and measurement of blood pressure and levels of urinary catecholamines and metanephrines . The facThe reason to choose a surgical intervention instead of chemotherapy in the T11 lesion was also based in the risk of vertebral colapse with the consequent danger of neurological injury . The exeNevertheless, this proved to be at least partially a correct decision because the patient became assymptomatic and was able to resume working [she is still working and with no clinical complaints].One could argue that she should have had chemotherapy with MIBG imediately after the removal of the vertebral metastasis because it was the second recidiva, but it was decided against that in group reunion. It was taken in consideration that chemotherapy does not seem to increase the survival in women with metastatic malignant paraganglioma .When the local vertebral recidiva occurred it was decided that the patient would undergo MIBG treatment. This option led to a regression in tumour size. Even in this perpective the surgical procedure was benefic to the patient because the dose of MIBG used is size dependent.The patient's direct family was offered genetic screening, having been diagnosed 5 positive patients, and one already died of metastatic pheocromocytoma .This case outlines the clinical course of a patient with a PGL type 4 syndrome for whom a rare vertebral metastasis was diagnosed. The case highlights the importance of offering targeted genetic testing for the SDHB gene mutations to a patient with multiple, extra-adrenal paragangliomas and a family history of malignant paraganglioma. It also illustrates the importance of identifying patients with germline SDHB mutations, as these patients are at a high risk of developing malignant disease. On the other hand it also highlights the importance of a multidisciplinary approach to these patients, as well as an aggressive attitude towards metastasis, with radical surgical exertion of the tumor being, whenever it is possible, the authors’ choice.Written informed consent was obtained from the patient for publication of this Case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors did not receive any outside funding or grants in support of their research for or preparation of this work. Neither they nor a member of their immediate families received payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity. The author(s) declare that they have no competing interests'.MRS: acquisition of data, data analysis and manuscript drafting, MSC: acquisition of data, data analysis and manuscript drafting, PCR: acquisition of data, data analysis, NN: analysis and interpretation of data, revising, AMG: analysis and interpretation of data, revising, RP: analysis and interpretation of data, revising, DC acquisition of data, analysis and interpretation of data, revising. All authors read and approved the final manuscript.


Sunitinib, an oral multitargeted tyrosine kinase inhibitor, is widely used in the treatment of renal cell carcinoma and gastrointestinal stromal tumor and has had a variety of adverse events. However, sunitinib-related acute cholecystitis has been reported in only two patients with gastrointestinal stromal tumor and renal cell carcinoma (clear cell subtype).A 75-year-old Japanese woman with a right sided abdominal swelling was referred to our hospital. Computed tomography (CT) showed a hypervascular bulky tumor in her right kidney, suggesting right renal cell carcinoma in clinical T4N0M0. Although sunitinib therapy was started as neoadjuvant chemotherapy, during the fourth week of the first cycle, she developed acute acalculous cholecystitis and disseminated intravascular coagulation associated with sunitinib. Sunitinib therapy was discontinued immediately and she recovered after subsequent treatment with antibiotics and gabexate mesilate followed by percutaneous cholecystostomy. Cholecystectomy and right radical nephrectomy were performed and pathological examination showed that her renal tumor was a chromophobe renal cell carcinoma (pT2) with necrosis. Inflammation and ischemia were observed in the gallbladder wall, which was compatible with acute acalculous cholecystitis. There has been no evidence of disease recurrence for more than six months.We described the third case of sunitinib-related acute cholecystitis in a patient with chromophobe renal cell carcinoma. Attention is required to sunitinib-related acute cholecystitis which, while uncommon, could be life-threatening. Sunitinib, an oral multitargeted tyrosine kinase inhibitor, is widely used in the treatment of metastatic renal cell carcinoma (RCC) and gastrointestinal stromal tumor (GIST) and has been administered in the perioperative period . Althoug®) followed by percutaneous cholecystostomy.A 75-year-old Japanese woman with a right sided abdominal swelling was referred to our hospital. She had no history of medication or smoking and was a social drinker. Computed tomography (CT) showed a hypervascular bulky tumor in her right kidney with suspected liver invasion without distant metastasis Figure , suggestAfter three months, a follow-up contrast-enhanced computed tomography (CT) revealed a marked shrinkage of the gallbladder and a 21% reduction in the size of the renal tumor with decreased enhancement of its center. Cholecystectomy and right radical open nephrectomy were performed. Adhesions thought to be due to cholecystitis made the operation difficult although common bile duct stenosis or retraction by the tumor that could lead to cholecystitis was not observed. Pathological examination showed that her renal tumor was chromophobe RCC (pT2) with necrosis occupying more than half of the tumor Figure . InflammWe present the third case of sunitinib-related acute cholecystitis which developed in a patient with a chromophobe RCC. In the literature, sunitinib-related acute cholecystitis has been reported in only two other patients, one with GIST  and the A common clinical feature of sunitinib-related acute cholecystitis in three cases including our patient and the previous two cases ,3 was acWe described the third case of sunitinib-related acute cholecystitis in a patient with chromophobe RCC. Attention needs to be given to sunitinib-related acute cholecystitis which could be life-threatening albeit uncommon.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.KN prepared the manuscript. KS and TM interpreted the patient data and revised the manuscript. All authors reviewed and approved the final version of the manuscript.


Xanthogranulomatous inflammation is a well-described inflammatory process, which may involve any organ but is most frequently encountered in the gall bladder and the kidney. There are rare reports of xanthogranulomatous appendicitis (XA) in the adult population, but only one brief mention of such a diagnosis in a child. In this report, we describe the case of an 11-year-old boy who presented with clinical signs and symptoms of acute appendicitis necessitating appendectomy. Upon microscopic examination, the appendix showed the typical features of XA. To the best of our knowledge, this is the first well-described case XA in a noninterval appendix in a child. We also reviewed the limited medical literature on the subject. The patient is an 11-year-old boy who presented with a 1-day history of abdominal pain and emesis but no fever. His past medical history was unremarkable. In particular, there was no history of hemorrhagic problems or systemic disease. The family history was also unremarkable. On physical examination, he had persistent and well-localized right lower quadrant and right flank tenderness at the expected location of his appendix (McBurney's point). The laboratory findings were unremarkable. WBC was 4.9 K/microliter with the following differentials: 49% segmented neutrophils, 41% lymphocytes, 9% monocytes, and 1% eosinophils. There was no evidence of anemia or thrombocytopenia . A computed tomography scan (CT-Scan) of the abdomen showed an enlarged appendix without inflammation; however, ultrasound images showed a fluid-filled appendix with a diameter within the upper rages of normal. A subsequent physical examination revealed an increase in abdominal pain and tenderness, and, consequently, the patient underwent a laparoscopic appendectomy. Gross evaluation showed a pink-tan appendix, measuring 8.3 cm in length and 1 cm in diameter. The serosal surface was unremarkable and cut surface demonstrated no fecalith. Microscopically, hematoxylin-eosin-stained sections of the tip of the appendix revealed numerous lipid-laden xanthoma cells in the mucosa  which weThe postoperative clinical course was unremarkable. The patient was discharged home the following day and had an unremarkable physical examination on a follow-up visit three weeks later.Xanthogranulomatous inflammation is a rare form of chronic inflammation, manifested by the presence of lipid-laden macrophages admixed with lymphocytes, plasma cells, neutrophils, and often multinucleated giant cells with or without cholesterol clefts . It was Cozzutto and Carbone noted that hemorrhage plays a major role in the development of foamy macrophages, postulating that the ingested erythrocytes and platelets at the bleeding site overwhelm the lysosomal system of the macrophages causing deposition of phospholipids which results in a foamy appearance of the macrophages .Involvement of the appendix by xanthogranulomatous inflammation, or xanthogranulomatous appendicitis (XA), is a rare phenomenon with only 10 cases reported in the literature. Nine of these case reports were described in adults . One study described the case of a 12-year-old boy who had XA in an interval appendectomy, and specimen was removed 6 weeks after an episode of acute appendicitis [Guo and Greenson reviewed the histopathology result of all interval appendectomy specimens within a 4-year period and compared them with a control group of 44 patients who had acute appendicitis and appendectomy within 72 hours of symptoms onset [In 1993, Birch et al. published the first reported cases of XA . McVey eMunichor et al. reported XA in a 37-year-old female who presented with typical signs and symptoms of acute appendicitis . In thatIn 2005, Chuang et al. reported a case of a 39-year-old man who presented with fever, right lower abdominal pain, and a mass . A hemicOmar et al. reported a rare case of XA, and cecal angiolipoma in a patient who presented with acute appendicitis and appendicular mass .In 2011, Martínez-Garza et al. reported a case of XA and they also performed a literature review . In theiThe oldest patient diagnosed with XA was a 78-year-old Japanese man who presented with 2-month history of right lower quadrant abdominal pain .Finally, Singh et al. reported a case of XA in a 21-year-old woman, who presented with abdominal pain and underwent surgery for suspected appendicitis . They hiThe classic microscopic pathologic appearance of XA demonstrates numerous lipid-laden macrophages (foam cells), abundant hemosiderin, and multinucleated giant cells, admixed with cholesterol clefts and mixed inflammatory cell infiltrate .Several factors precipitating XA have been proposed as follows: organ obstruction, suppurative infection, hemorrhage, defective lipid transport, and local hypoxia which can trigger tissue damage within the involved organs, usually eliciting a microscopic response of the disease process , 5. TherOur case was remarkable for the finding of XA in a child with an acute  presentation and also a CBC that was not suggestive of an acute suppurative inflammation.Xanthogranulomatous appendicitis (XA) is a rare clinical entity, particularly in the pediatric population. It may be encountered in specimens resected in the acute phase, but is much more common in interval appendectomy specimens. As observed in the present case and in previous reports, most patients with XA present with a picture of acute or subacute abdominal pain, and occasionally with a mass lesion.


Hypothalamic hamartoma is a rare neoplastic heteropia caused by organic developmental failure. The most common clinical findings in hypothalamic hamartoma are pubertas precox with or without gelastic seizure, and behavioural disturbance. The aim of this case report to inform a rare case of hypothalamic hamartoma with pubertas precox and gelastic seizure in a boy.1P2G2-3, and neurological status was normal. Laboratory finding: FSH: 0,5 mlU/ml, LH: 0,85 mlU/ml, testosterone: 239,20 ng/dL, 17α-hydroxyprogesterone: 107 ng/dl. Cranial CT scan and MRI showed a space occupying lession in suprasellar region which was suspected as a tubercinerium hamartroma. Bone age result was appropriate for an 8 year and 6 month old boy. He was treated with leuprorelin 1x3,75mg intramuscular every 4 week and valproic acid 2x200mg. After 3 years evaluation, patient’s Tanner pubertal status was A1P2G2-3 and the bone age was appropriate for an 13 years old boy. No gelastic seizure.A 5 year and 7 month old boy, admitted to the hospital with a chief complain of premature pubic hair growth and frequent sudden laughing without apparent reason which seemed to be forced, uncontrolled, irregular without deteroratoin of consciousness. Physical examination found accelarate height growth with appropriate proportion. Tanner pubertal status was ALeuproleline effectivity as a therapy for pubertas precox with hypothalamic hamartoma is still a controversion, so management through surgical approach may be considered, and valproic acid has a good effectivity for gelastic seizure in this case.


During pregnancy, masses that are larger than 5 cm and appearing in the Doppler ultrasonography as having increased blood flow, echoes of heterogeneous density, and containing solid components are suspicious for malignancy; however, differential diagnosis of decidualized endometriomas should also be considered. The patient was an 8 weeks pregnant primigravida. The ultrasonographic evaluation showed a cystic mass of size 65 × 57 mm in the left ovary that was well circumscribed, heterogeneous, with highly dense internal echo, and containing a solid component of size 8 × 14 mm. In the 12th week, the ultrasonographic examination revealed an increase in the size of the mass and increased arterial blood flow in the mass. The patient underwent surgery. It was observed that both ovaries were adherent in the Douglas pouch and that the left ovary contained an endometrioma of size 8cm. While the capsule was being peeled, lesions of soft density, with irregular surfaces, and with adhesion in the Douglas pouch were observed. The results of the frozen section revealed decidualized endometrioma and decidual structures. Even in pregnant women when adnexal masses are encountered and the ultrasonography, Doppler, MRI, and CA 125 level analysis still do not favor endometriosis, decidualized endometrioma should be considered in the differential diagnosis. Diagnostic criteria of endometriosis are the presence of 2 of the following 3 features outside of the uterus: endometrial glands, endometrial stroma, and hemosiderin-laden macrophages. It is a common gynecological problem and it is estimated to affect approximately 6–10% of the women of reproductive age . HormonaAn 8-week pregnant 32-year-old primigravida patient without prior clinical symptoms of endometriosis applied to the polyclinic with complaints of hyperemesis. The subsequent pelvic ultrasonography showed a cystic mass of size 65 × 57 mm that was well circumscribed, heterogeneous, with internal echo, and containing a solid component of size 8 × 14 mm in the left ovary. CA 125 value was measured as 220. The patient was informed of the suspicion of malignancy and surgery was recommended; however, the patient chose to postpone the operation until the 2nd trimester because of the pregnancy. In the 12th week, the patient was admitted with complaints of left lower-quadrant pain, nausea, and vomiting; the ultrasonographic examination revealed an increase in the size of the mass, 77 × 65 mm, and increased arterial blood flow in the mass. The patient was operated based on her informed consent.In the examination of the abdominal cavity, multiple endometriotic foci ‘‘gunpowder burns” on the pelvic side walls, in the pouch of Douglas, and on the intestinal surfaces as well as wide-spread intra-abdominal adhesions were seen. It was observed that both the ovaries were adherent in the Douglas pouch and that the left ovary contained an endometrioma of size 8 cm, whose content was drained during manipulation. While the capsule was being peeled, lesions of soft density, with irregular surfaces, and with adhesion in the Douglas pouch were observed at the base of the capsule that were spreading out of the ovarian capsule, also covering the surfaces of the right ovary and the uterosacral ligaments Figures  and 2. TDuring pregnancy, decidualization may appear in the endometrial tissues outside the uterus, especially in the endometrial stromal cells of the ovarian endometriomas , 7. MoreIn our case, the findings suggestive of malignancy were the size, which was reaching 8 cm, the heterogeneous density and the papillary projections of the echo, and the Doppler ultrasound of the cyst showing the tumor tissue-like increased perfusion. CA 125 levels may also increase by average levels in normal pregnancies. Macroscopically, the presence of papillary projections and its spread out of the cyst capsule towards the pouch of Douglas, the opposite ovarian serosa, and the pelvic side walls pointed to ovarian neoplasia. On the other hand, the presence of adhesions throughout the intra-abdominal, powder-burn lesions on the surfaces of the pelvic side walls and intestinal surfaces, and the image of the cyst contents were findings in favor of the endometrioma. However, ovarian endometriomas may undergo malignant transformation , 22. TheDuring pregnancy, although, for the differentiation of the masses in terms of endometrial malignancy, ultrasonography and Doppler findings, CA 125 levels, and the use of MRI are beneficial, when compared to the histological examinations they are insufficient. This case presentation suggests that the lack of ability to clearly carry out the distinction preoperatively necessitates the decidualized endometrial cysts to be included in the differential diagnosis of the pelvic masses during pregnancy.


Plasma cell tumors are a diverse group of neoplasms characterized by monoclonal proliferation of plasma cells. Extramedullary plasmacytoma (EMP) is a rare form of localized plasma cell tumor that arises most often in the head and neck region. We present an unusual case of EMP of the palatine tonsil from a tertiary care university hospital. We discuss the histopathologic and radiologic evaluation as well as treatment of EMP. Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm that occurs outside of the bone marrow . EMP usuA 52-year-old man presented with a left tonsil mass. The patient was an amateur vocal performer and had noted tonsillar asymmetry during singing exercises. Except for mild odynophagia, the patient was asymptomatic. His medical history was unremarkable other than longstanding obstructive sleep apnea and obesity. He had a remote history of smoking and occasional alcohol use. Examination revealed an exophytic lesion of the left palatine tonsil. There was no cervical lymphadenopathy.Magnetic resonance imaging (MRI) demonstrated a 2.1 cm mass of the palatine tonsil with associated enhancement . PositroHistologic evaluation showed focal mucosal ulceration, a plasma cell infiltrate, and deposition of amorphous eosinophilic material. A congo red stain was performed, and the amorphous material showed apple-green birefringence characteristic of amyloid. Immunoperoxidase staining showed the plasma cells to be lambda light chain restricted, consistent with a monoclonal process. A diagnosis of extramedullary plasmacytoma with amyloid deposition was made.The patient underwent complete surgical resection of the left tonsil. Final pathology was consistent with EMP . HematolEMP is a plasma cell neoplasm that occurs predominantly in the upper aerodigestive tract . Most leWhile inhalant exposure has been proposed as a risk factor for EMP of the head and neck, evidence to support this has been inconclusive . PatientDiagnosis of EMP can be made by tissue biopsy or a fine needle aspiration (FNA) biopsy. Reports have highlighted the challenges in diagnosis by FNA due to similarities with inflammatory conditions ; howeverOnce a diagnosis of EMP is made, further workup includes imaging and hematologic evaluation. MRI is useful in determining the extent of local disease, response to treatment, and in the detection of recurrence , 3. PET Histologic examination of EMP usually shows a monotonous infiltrate composed of discohesive plasma cells characterized by eccentrically placed round nuclei with coarse clumpy chromatin. Immunochemical staining reveals reactivity for either the lambda or kappa immunoglobulin light chain and establishes monoclonality , 4, 5. Treatment approaches include surgery and/or radiation therapy . EMPs re