Giant cell tumor (GCT) is a benign neoplasm arising most commonly in the long bones. GCTs of the larynx (GCTL) are relatively rare, and only individual case reports are documented in the literature. Patients with such tumors may present with hoarseness and anterior neck swelling. We present a 59-year-old man with hoarseness and enlarging anterior neck mass for 3 months. A fiberscopy revealed a submucosal swelling of the left subglottic trachea. Computed tomography and magnetic resonance imaging of the larynx demonstrated a large, well-defined, inhomogeneous enhancing mass at the left thyroid cartilage, which was obstructed entirely. The anterior neck mass was biopsied for histopathological analysis, which showed multinodularity with intervening vascularized connective tissues. The mass was made up of mononuclear cells and distributed multinucleated giant cells. The mitotic activity of the mononuclear cells was as high as 6 per 10 high-power fields. Pathologic consultation resulted in a diagnosis of giant cell tumor. The patient underwent total laryngectomy and, postoperatively, he did well without recurrence or metastasis for two and a half years. Giant cell tumor (GCT) is classified as a benign bone tumor, and it represents 4 to 9.5% of all bone tumors and 20% of all benign bone tumors . It occuA 59-year-old man was referred to our department with a 3-month history of gradually worsening hoarseness and enlarging anterior neck mass. On physical examination, a firm and fixed mass measuring 3.0 cm at its maximum diameter was noted in the left region of the thyroid. Endoscopic examination revealed a submucosal swelling of his left vocal fold with intact movement. The laryngeal mucosa did not appear to have an ulcerative or hemorrhagic lesion . A compuGiant cell tumors (GCTs) are relatively frequent skeletal tumors that occur mainly at the distal end of the femur and the proximal end of the tibia. In the head and neck region, GCTs are found mainly in the maxillary region  and at tThe common head and neck sites for these tumors correspond to the cranial bones formed by endochondral ossification. The laryngeal skeleton is primarily composed of hyaline cartilage until approximately the second decade of life, at which time it may gradually begin to be replaced by bone. This occurs at different rates in different individuals but occurs earlier in males .The treatment of GCTs is controversial, and no consensus exists on their management. A review of the literature reveals that the majority of patients were managed surgically . SurgicaGCT of the bone is a locally osteolytic tumor with variable aggressiveness. In rare cases, pulmonary metastasis can be observed . AdditioGCT of the larynx is an uncommon entity, with very few cases reported in the literature. GCT of the larynx should be treated with complete surgical removal and has an excellent prognosis. We performed total laryngectomy on a patient with GCTL. The patient is free of recurrence after two and a half years, but long-term observation is still required.


Bronchogenic cysts (BC) are congenital abnormalities that occur most commonly within the mediastinum, and rarely occur within the diaphragm. We present the 21st case of an intradiaphragmatic bronchogenic cyst in the English literature, and review all previous published cases. Analysis includes presenting clinical symptoms, relevant radiologic studies, surgical approaches to resection, and management of the diaphragm, among other relevant data. These lesions should remain on the differential diagnosis in cases of unusual masses in the region of the diaphragm. Bronchogenic cysts (BC) are congenital abnormalities that arise from the ventral foregut and occur most commonly within the mediastinum. Bronchogenic cysts rarely occur within the diaphragm. Up to June 2015, there were twenty cases of intradiaphragmatic bronchogenic cysts reported in the English literature. We present a case of bronchogenic cyst located within the diaphragm in an adult male patient. This lesion was removed via a left thoracotomy approach and diagnosis was confirmed with surgical pathology.A 41 year old male presented with 6 months of back pain located around his lumbar spine. He had no history of trauma. An MRI of the spine revealed an ovoid soft tissue structure located medially at the left lung base, measuring approximately 4.5 cm by 5 cm Fig. . An old The patient was taken to the operating room for a left posterolateral serratus sparing thoracotomy with resection of the left diaphragmatic mass. The diaphragm was reconstructed with a synthetic patch. The post-operative course was uneventful and the patient was discharged home on post-operative day five. Final pathology was consistent with a benign intradiaphragmatic bronchogenic cyst with clear margins. The mass was composed of benign ciliated respiratory epithelium, cartilage and seromucinous glands surrounded by benign skeletal muscle.We performed a combined MEDLINE, PubMed, ScienceDirect, and SCOPUS Database search for “bronchogenic cyst” AND “diaphragm” without date limitations. Case reports of intradiaphragmatic bronchogenic cysts were selected for analysis and review. Non-English language articles were excluded. Citations of these case reports were inspected for references to other case reports, and these were investigated for appropriateness and included if relevant. Patient demographics, case specifics, including imaging, and details of operative approach were noted in each case report and pooled together for analysis. If a particular case report did not include pertinent data, such as operative approach or diaphragmatic management, they were excluded from that particular analysis.A total of twenty English language case reports for intradiaphragmatic bronchogenic cysts were published from 1952 to 2014. The age of presentation varied significantly and ranged from 19 months to 74 years old, with a mean age of 42.7 years old. 57 % (12/21) of the patients were female. 71.4 % (15/21) presented on the left side. Most frequently, presentation was either for back pain or cough, each of which were present in 19 % (4/21)  Table . LaparotOn gross examination, six (28.6 %) were noted to be multilocular, while three (14.3 %) were unilocular, with the remaining not describing loculations. Four cases (19.0 %) described calcification present in specimen, three noting the calcification to be focal. One of these cases did not show radiologic evidence of calcification. All pathologic analysis denoted absence of malignancy; however, two cases denoted the presence of squamous metaplasia within the resected bronchogenic cyst. Sample size would limit any analysis of predictors of metaplasia in this series.Bronchogenic cysts are congenital lesions that are thought to arise from an abnormally budding ventral foregut, which then develops into a blind ending fluid filled pouch . HoweverSymptoms that are seen are frequently nonspecific and may not be related to the bronchogenic cyst itself, but rather prompting workup, which then reveals the lesion. When symptoms arise, typically patients present with pain, but they can also have respiratory symptoms such as intermittent or persistent cough. Symptoms are likely due to compression or irritation of adjacent structures. Bronchogenic cysts, as a group, can also present with symptoms from cyst communication with aerodigestive structures, bleeding, or localized infections, but none of these were present with bronchogenic cysts in the intradiaphragmatic location . ImagingIt appears as though surgical approaches for resection of these lesions are dependent on the most likely diagnosis as well as the anticipated extent of diaphragmatic reconstruction. Frequently, preoperative localization of these lesions are difficult, and they may be mistakenly thought to be either within the abdominal or thoracic cavities, rather than within the diaphragm itself. However, our review notes that virtually all approaches are suitable for successful resection of intradiaphragmatic bronchogenic cysts, including minimally invasive approaches. We can speculate that minimally invasive approaches would yield improved short term outcomes, but with such a small sample size, and each unique case presenting its own challenges, we could only speculate as to that conclusion.The pathologic hallmark of bronchogenic cysts is the presence of ciliated pseudostratified columnar epithelium, cartilage, and smooth muscle within the cyst wall. Grossly, there is a variable presentation, which likely contributes to their variable radiologic appearance. Reason for resection of these lesions is their unclear diagnosis, potential for harboring malignancy, infectious complications, erosive complications, and symptomatology, if present . While pIntradiaphragmatic bronchogenic cysts are very rare, and this review characterizes the variable nature of clinical presentation, radiologic appearance, surgical techniques, and pathology. These lesions should remain on the differential diagnosis in cases of unusual masses in the region of the diaphragm.


Leiomyosarcoma of the uterus is an extremely rare but highly aggressive tumor that accounts for only 1–2 % of uterine malignancies, and is usually associated with a dismal outcome.The authors present an unusual case of pedunculated subserosal leiomyosarcoma of the uterus mimicking ovarian carcinoma. A 57-year-old postmenopausal woman presented with progressive low abdominal pain and urinary frequency. Pelvic magnetic resonance imaging revealed a large adnexal mass with carcinomatosis peritonei. Laboratory examination revealed an elevated serum CA-125 level (398.4 U/ml). The patient underwent exploratory laparotomy under suspicion of ovarian malignancy. Frozen section indicated malignancy originating from the uterus, and thus, total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic and para-aortic lymph node dissection, and mass excision were performed. Subsequent histopathologic examination resulted in a final diagnosis of leiomyosarcoma of the uterus. The patient’s postoperative course was uneventful, and gemcitabine and docetaxel adjuvant chemotherapy was administered.The authors report an unusual case of pedunculated subserosal leiomyosarcoma of the uterus mimicking ovarian carcinoma. Uterine leiomyosarcoma (LMS) is a rare uterine malignancy that accounts for approximately 1–2 % of uterine malignancies and 25–36 % of uterine sarcomas. It occurs mainly after menopause  depicted a large, complex, adnexal mass, suggestive of ovarian carcinoma. Axial T2-weighted imaging showed a solid, cystic mass with heterogeneous signal intensity in the pelvic cavity. Contrast-enhanced fat-suppressed T1-weighted imaging showed heterogeneously marked enhancement of the mass. On sagittal and coronal T2-weighted images, the mass abutted the uterus  of the uterus is an extremely rare but highly aggressive tumor and is associated with poor prognoses, even for early-stage disease. LMS is usually a large, yellow or tan solitary myometrial mass with some hemorrhagic and necrotic areas. Necrosis, moderate-to-severe cytologic atypia, and > 15 mitotic figures per 10 high power fields (HPF) are considered typical histopathologic criteria. It may be near impossible to differentiate benign and malignant uterine tumors; however, pathological features, such as, larger tumor cells, fewer stromal fibers, increased mitotic activity, and nuclear pleomorphism may aid differentiation. Nevertheless, the presence of mitoses is a hallmark of malignancy.The signs and symptoms of LMS are similar to those of benign leiomyoma. Presenting symptoms are nonspecific and include abnormal vaginal bleeding , abdominal pain, vaginal discharge, urinary frequency, constipation, and abdominal distention. However, in our case, the patient had no complaint of vaginal bleeding possibly because of an unusual subserosal location.per se [Serum CA125 levels are occasionally elevated in LMS. It has been reported preoperative serum CA125 levels are significantly higher in LMS than in uterine leiomyoma . AlthougMRI is very useful for determining anatomic features, such as, the locations, sizes, and numbers of pelvic masses. In addition, MRI may provide diagnostic information that supports a preoperative presumption of leiomyosarcoma; however, it is not entirely accurate. LMS usually manifests as a large infiltrating myometrial mass with high signal intensity on T2W images and low ADC values. Final diagnosis of uterine leiomyosarcoma is made by histopathological findings. In our case, an incorrect initial diagnosis of ovarian malignancy was made because ovarian cancer is more common in postmenopausal women and MRI showed a large, adnexal mass, ascites, omental smudging, and misty mesentery.Pedunculated masses may have obscure origins and be misinterpreted as ovarian masses. The detection of a vascular pedicle can provide information on the origin of a mass. When normal ovaries are visualized separately, a mass of ovarian origin can be excluded. In our case, we retrospectively analyzed MR images, and noted bridging vessels between the uterus and a juxta-uterine mass, suggesting a pedunculated subserosal uterine mass. However, normal ovaries were not visualized because ovaries were atrophied and obscured by the mass. Several case reports have been issued on uterine masses considered preoperatively to be ovarian tumors, due to similar locations and imaging findings. For example, a large pedunculated leiomyoma with cystic degeneration mimicking ovarian malignancy , and an Immunohistochemistry may be helpful for differentiating leiomyosarcoma from other tumors. In our case, immunohistochemistry revealed diffuse strong positivity for smooth muscle actin (SMA), desmin and h-caldesmon, and SMA and desmin are usually expressed in LMS, whereas h-caldesmon is highly specific for LMS .Total hysterectomy and bilateral salpingo-oophorectomy are recommended for LMS grossly confined to the uterus, but dissection of pelvic and para-aortal lymph nodes is not routinely recommended. Lymphatic spread is not common and lymph node involvement is only encountered in <3 % . HoweverLMS is characterized by early dissemination and metastases to multiple organs and primarily presents with hematogenous spread. Interestingly, in the described case, metastases occurred to omentum and mesentery around the rectosigmoid via direct peritoneal spread from the pedunculated subserosal leiomyosarcoma. Prognosis primarily depends on the extent of disease at diagnosis and mitotic index. In addition, several authors have suggested that tumor size may be an important prognostic factor , 13. GarIn summary, we present an unusual case of pedunculated subserosal leiomyosarcoma of the uterus that mimicked ovarian malignancy, based on anatomic location, imaging findings, and serum tumor marker level, in a postmenopausal woman. This case cautions pedunculated subserosal leiomyosarcoma of the uterus with a highly elevated serum CA-125 marker may mimic ovarian carcinoma and should be considered in the differential diagnosis of an adnexal mass in postmenopausal women.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.


Multiple paragangliomas of the head and neck are rare conditions. Carotid paragangliomas are most common multiple paragangliomas. Laryngeal paragangliomas are very rare neuroendocrine tumors and usually are seen as symptomatic solitary lesions. We present multimodality imaging findings of incidentally detected laryngeal paraganglioma in a woman with synchronous carotid body paraganglioma and positive family history. To the best of our knowledge, this is the first case of laryngeal and carotid body paragangliomas in a patient with positive family history. Radiologists should keep in mind that paragangliomas may occur in various locations as multiple tumors. Head and neck paragangliomas (HNP) are rare neuroendocrine neoplasms. They represent 0.03% of all neoplasms and 0.6% of head and neck neoplasms . The mosA 32-year-old woman presented with swelling in the left neck for the past 1.5 years. She was a nonsmoker and nondrinker. Her past medical history was negative but her mother was operated on for carotid body paraganglioma. On physical examination, mobile, soft, 1.5 cm mass was palpated anterior to the sternocleidomastoid muscle on left side. There were no cranial nerve palsies or neck lymphadenopathy. A thorough review of other systems was negative. Laryngoscopic examination was not remarkable. Blood and urine catecholamine levels were normal.On ultrasound (US) exam well defined, oval shaped, hypoechoic, solid, 25 × 32 mm hypervascular mass was seen at the left carotid bifurcation Figures . SplayinBecause of multicentricity and positive family history, Gallium-68 (Ga-68) DOTA-peptide positron emission tomography/computer tomography (PET/CT) was performed to verify the diagnosis and disclose whether or not another focus of paraganglioma exists anywhere in the body. 68Ga-DOTANOC PET/CT showed the laryngeal lesion with intense tracer uptake (SUV max: 35.8) and carotid body paraganglioma (SUV max: 37.5) Figures . 68Ga-DODigital subtraction angiography (DSA) demonstrated right superior thyroid artery supplied laryngeal mass and left ascending pharyngeal artery supplied mass at carotid bifurcation Figures . The masThe pathologic diagnosis was paraganglioma. Microscopically, the well encapsulated tumor made characteristic clear cell nests with Zellballen pattern. Immunohistochemical analysis showed tumor cells positive for chromogranin and S-100 (sustentacular cells). Staining for Ki67 showed <1% positive cells. Five months later, second operation was performed for laryngeal tumor following superior thyroideal artery embolization . The diaMultiple paragangliomas may occur in 40% of familial paragangliomas and 10% of sporadic tumors . Chase dLarynx is a very rare location for paragangliomas of the head and neck. The majority of laryngeal paragangliomas arise in the supraglottic larynx. They occur most commonly in the fourth to sixth decades and show a female preponderance (1 : 3), unlike other neuroendocrine tumors of the larynx . HoarsenLaryngeal paragangliomas must be differentiated from other neuroendocrine tumors of the larynx, including typical carcinoid, atypical carcinoid, small cell neuroendocrine carcinoma, and large cell neuroendocrine carcinoma . ParaganDoppler US is a first-line imaging technique in patients with suspected carotid body paraganglioma. Well defined solid mass at the carotid bifurcation, splaying of the internal and external carotid arteries, and high vascularity are typical findings of these tumors. US cannot demonstrate jugulotympanic and most of vagal paragangliomas due to their farther cephalad localization. Contrast enhanced computed tomography is a useful technique for defining bone changes in patients with jugular paraganglioma such as irregular widening of the jugular foramen with cortical erosion and permeative bone destruction. MRI provides better tissue characterization and allows detailed evaluation of tumor localization, size, vascularity, its relationship with major arteries and surrounding structures, and presence of intracranial extension. The typical MRI finding of paragangliomas is “salt and pepper” appearance that results from slow flow or hemorrhage and signal void areas due to high velocity flow, respectively. CT and MRI may also show asymptomatic synchronous HNP especially in patients with familial paraganglioma. Imaging findings of laryngeal paraganglioma are similar to other HNP. In our case, similar imaging features of both lesions confirmed diagnosis of multiple paragangliomas.DSA is most commonly used for preoperative vascular mapping and tumor embolization rather than diagnosis. Also, balloon occlusion test can be performed to assess collateral cerebral perfusion in patients with carotid body paragangliomas to determine the risk for ischemia and stroke following ICA ligation.68Ga-DOTA-peptide PET/CT has higher sensitivity and advanced spatial resolution for the detection of well differentiated neuroendocrine tumors than conventional somatostatin receptor scintigraphic agents. Apart from characterizing a lesion as neuroendocrine tumor, 68Ga-DOTA-peptide PET/CT can reveal extension of disease and exclude synchronous and metastatic lesions. 68Ga-DOTA-peptide PET/CT study may further assist in choosing the right treatment and selection of patients for peptide receptor radionuclide therapy with 177 Lu or 90 Y labeled peptides in such patients with extensive disease.In conclusion, radiologists should keep in mind that paragangliomas may arise in head and neck region as synchronous or metachronous multiple tumors. All areas within the images should be carefully evaluated to avoid missing asymptomatic lesions. Earlier detection and treatment of these tumors can reduce morbidity and mortality rates of surgery.


Malignant fibrous histiocytoma (MFH) is a tumor that occurs throughout the body as a relatively uncommon entity. The current study presents two cases of primary malignant fibrous histiocytoma of the liver. The first case was of a 67-year-old male who exhibited no symptoms or abnormal physical signs, and in whom the lesion was found by ultrasound examination during a routine physical examination. The second case was of a 35-year-old male who presented with persistent malaise, weight loss and intermittent right upper quadrant pain. The presence of liver cirrhosis due to hepatitis B virus, which was identified 10 years previously, and the clinical appearance caused MFH to appear as hepatocellular carcinoma at the time of the initial diagnosis. Abdominal magnetic resonance imaging scans were the main tools of diagnosis, but the MFH mimicked hepatocellular carcinoma due to the similar morphological characteristics, the rare occurrence of MFH and the underlying diseases of the liver. The first patient underwent a complete resection and recovered well, while the second patient underwent palliative treatment due to the large size of the tumor and the obstructive emboli in the portal vein. The diagnoses of the tumors were confirmed as MFH by histopathology and immunohistochemistry. Malignant fibrous histiocytoma (MFH), also known as undifferentiated pleomorphic sarcoma, is the most common type of sarcoma in adults aged ≥50 years . AlthougThe present study reports two cases of primary hepatic MFH, with analysis of the ultrasound and MRI findings. The first case was of a 67-year-old male who had no symptoms or abnormal physical signs on routine physical examination. Other possible significant laboratory abnormalities, which included changes in serum transaminase , serum bilirubin  and serum α-fetoprotein  concentrations, were not present. The patient was referred to the Department of Surgery  for further investigation of a hepatic tumor detected by a B-scan ultrasound.The second case was of a 35-year-old male who was serologically positive for hepatitis B surface antigen and was admitted to the Department of Surgery with a history of persistent epigastric abdominal pain and weight loss. The serum concentrations of alkaline phosphatase and fibrinogen were increased to 259 U/l  and 4.65g/l , respectively. Serum α-fetoprotein  concentrations were also normal. The patient underwent a liver ultrasonogram, which revealed a large hypoechoic lesion occupying the greater portion of the right lobe.2) and use of post-contrast liver acquisition volume acceleration sequences, including arterial, portal venous, delayed phase and coronal T1WI with fat suppression.The two patients underwent MRI prior to therapy. MRI was performed using a 1.5-T MRI system . Overall, the imaging examinations performed consisted of axial T1 and T2 weighted imaging (WI) with fat suppression, in and out phase imaging, diffusion weighted imaging . Following three cycles of chemotherapy the tumor increased in size. The patient then received cyroablation treatment, however the patient succumbed to a bleeding complication.The post-operative period was uneventful in case one. However, the tumor of the second patient was unresectable and the patient was then referred to the oncology service for palliative chemotherapy , considered to be a rarely occurring soft-tissue sarcoma of the liver in adults, has previously been defined as a pleomorphic malignant spindle cell neoplasm exhibiting fibroblastic and facultative histiocytic differentiation. However, the pre-operative diagnosis of hepatic MFH is made extremely challenging by the lack of clearly characteristic features. In order to diagnose primary hepatic MFH, two notable characteristics are necessary. The first involves the histopathology of the hepatic tumor. The second is that the clinicopathological criteria that define the tumor as hepatic in origin must be met. Li et al  proposedThe imaging features of the tumors in the two cases were within the extent of the soft-tissue MFH classification. It has been reported that MFH appears as a well-defined mass that shows hypoechoic, mixed or hyperechoic patterns with variable anechoic areas. The complex internal pattern of MFH depends on the solid portion of high cellularity and the necrotic regions on the ultrasonogram . The feaet al reported a case of the simultaneous occurrence of MFH and hepatocellular carcinoma in a patient with a cirrhotic liver (MFH has been reported as inhomogeneously hyperintense on T2WI and inhomogeneously enhanced on gadobenate dimeglumine-enhanced T1-weighted gradient-recalled echo imaging ,7, whichic liver . AlthougMFH of the liver is generally recognized as having a high local recurrence rate and a significant metastasis rate. The risk of local recurrence and distant metastasis correlates with the depth and size of the primary tumor. Recurrence of the tumor is not uncommon, even when the resection margin is tumor-free . DistantThe size and location of the tumor and the efficacy of the initial surgical removal all contribute markedly towards the prognosis of a patient with soft-tissue MFH . The tumIn conclusion, the present case study described two cases of rare MFH in the liver, which tended to appear as hepatocellular carcinoma. MRI of a primary liver MFH may reveal a well-encapsulated, inhomogeneously enhancing mass. Although MFH is a rare entity, the possibility of hepatic MFH should be acknowledged during differential diagnosis, even in cirrhotic patients, and hepatic spindle cell tumors require comprehensive tissue sampling and immunohistochemical analyses in order to be diagnosed.


Most primitive neuroectodermal tumor of the chest wall destroy the rib, chest wall muscles, diaphragm, and lung or extend into the spinal compartment, resulting in a large-sized tumor and symptoms. In contrast, we recently encountered a rare case of Askin’s tumor presenting with early-onset chest pain despite the small size. After resection of the tumor and adjuvant chemotherapy, the patient remains disease-free over 3 years of follow-up. Chest radiograph showed a small nodular opacity in the right lung field, suggesting focal pneumonia or mass, and CT scan revealed a small mass with a broad base on the pleura of the right anterior hemithorax and pleural effusion  ,3. In coWritten informed consent was obtained from the patient’s parent for the publication of this report and any accompanying images.


DFSP rarely leads to a metastasis (Less than 5% have metastasis), but DFSP can recur locally. We publish a rare case of a recurrent dermatofibrosarcoma protuberans and its management with radical excision and interval skin grafting There is only about one case per million per year. DFSP is a fibrosarcoma, more precisely a cutaneous soft tissue sarcoma. In many respects, the disease behaves as a benign tumor, but in 2–5% of cases it can metastasize, so it should be considered to have malignant potential. It occurs most often in adults in their thirties; it has been described congenitally, in children, and the elderly. It accounts for approximately 2–6% of soft tissue sarcoma cancers.2,3Dermatofibrosarcoma protuberans is diagnosed with a biopsy, when a portion of the tumor is removed for examination. In order to ensure that enough tissue is removed to make an accurate diagnosis, the initial biopsy of a suspected DFSP is usually done with a core needle or a surgical incision. Dermatofibrosarcoma protuberans begins as a minor firm area of skin most commonly about to 1 to 5 cm in diameter. It is a slow growing tumor and is usually found on the torso but can also be found on the arms, legs, head and neck. About 90% of DFSPs are low grade sarcomas.About 10% are mixed; they contain a high-grade sarcomatous component; therefore, they are considered to be intermediate-grade sarcomas. DFSP rarely leads to a metastasis (fewer than 5% do metastasis), but DFSP can recur locally. DFSPs most often arise in patients who are in their thirties, but sometimes have been described in children or the elderly. More than 90% of DFSP tumors have the chromosomal translocation t. The translocation fuses the collagen gene (COL1A1) with the platelet-derived growth factor (PDGF) gene. The fibroblast, the cell of origin of this tumor, expresses the fusion gene in the belief that it codes for collagen.2,3However, the resulting fusion protein is processed into mature platelet-derived growth factor which is a potent growth factor. Fibroblasts contain the receptor for this growth factor. Thus the cell “thinks” it is producing a structural protein, but it actually produces a self-stimulatory growth signal. The cell divides rapidly and a tumor forms. The tissue is often positive for CD34.2,3The standard of care for patients with DFSP is surgery. Usually, complete surgical resection with margins of 2 to 4 cm is recommended. The addition of adjuvant radiotherapy improves local control in patients with close or positive margins during the surgery. Patients who have a recurrent DFSP can have further surgery, but the probability of adverse effects of surgery and/or metastasis is increased in these patients. Imatinib is approved for treatment. Imatinib is a small molecular pathway inhibitor and inhibits tyrosine kinase. It may be able to induce tumor regression in patients with recurrent DFSP, unresectable DFSP or metastatic DFSP. There is clinical evidence that imatinib, which inhibits PDGF-receptors, may be effective for tumors positive for the t translocation.In the year 2014, a 60 year old male from a Sikh community presented to Plastic Surgery OPD of our institution with a huge recurrent swelling in left upper back since last 4 years. He had first noticed a 1-cm nodule on his left upper back which had grown gradually since then to attain its present size of 10 cm x 25 cm. The swelling was oval in shape with surgical scar and bossellated surface . He was Margin clearance had not been achieved and wound was closed primarily during all previous surgeries. Previous histopathology report was suggestive of DFSP with margin involvement. Metastatic work up was normaland there was no evidence of metastasis. Patient was operated under general anaesthesia, Radical (wide) local excision of tumour was donetaking a margin of 4cm from indurated margins. Resection included subcutaneous tissue, Latissimus dorsi (LD) muscle and part of serratus posterior muscle. The medial border of scapula was stabilised with sutures.Macroscopic examination of the excised tumor revealed potato like bulbous extensions on its surface and cut surfaces were pink-white in colour . The posSkin grafting was done after 3 weeks . Patient4 in 1925 when he reported three cases, however; Darrier and Ferrand established these interesting lesions as a distinct clinical entity in 1924.5 It is described by other terms like, progressive and recurrent dermatofibroma by Darrierand Ferrand,5 hypertrophic morpheaby Sherwell,6,7 sarcomatoustumor resembling keloid by Taylor,8 fibrosarcoma of the skin(Stout).9 Malignantfibrous xanthoma, was included by O’Brien and Stout10 in dermatofibrosarcoma protuberans. Dermatofibrosarcoma protuberans was first named byHoffmann1 is a rare neoplasm of the dermis layer of the skin, and is classified as a sarcoma. There is only about one case per million per year. In many respects, the disease behaves as a benign tumor, but in 2–5% of cases it can metastasize. The treatment of choice is complete resection with a wide margin of surrounding tissue, including the underlying fascia. The safety margin should reach 3 to 4 cm beyond the macroscopic lesion and the margins must be histologically negative, otherwise the recurrences occur in approximately 70% of patients.Dermatofibrosarcoma protuberans (DFSP)11 Mohs micrographic surgery has the advantage of high oncologic effectiveness and maximal tissue savingand is increasingly accepted as the treatment of choice.11Therefore, in management of locally malignant tumours one should not be lenient in the fair of development of large post surgical defect. Thus in the management of postsurgical defects, planning in reverse is very important. Another alternative treatment is Mohs micrographic surgery, but this is usually left for specific locations such as the face or eyelids, where large resections may lead to functional problems.12Further outpatient care is important as the tumor has a proclivity for recurrence. Poor prognosis is characterized by its late presentation, aggressive local invasion, regional nodal involvement and distant metastasis. Some histologic features serve as poor prognostic indicators; high number of mitotic figures, increased cellularity, DNA aneuploidy, TP53 gene over expression and fibrosarcomatous change..We conclude that in management of locally malignant tumorsone should not be lenient and planning in reverse is very importantand we shouldnever compromise to excise in fear of residual defect size and always consult your colleague for any help


No case of solitary fibrous tumor of the pleura with Doege-Potter syndrome hasbeen reported in China. This study was to report a rare repeatedly recurrent caseof solitary fibrous tumor of the pleura with Doege-Potter syndrome diagnosed inChina and a three-decade literature review of solitary fibrous tumor of the pleurawith Doege-Potter syndrome worldwide.A rare case of solitary fibrous tumor of the pleura with Doege-Potter syndromewas diagnosed in 2005 with follow-up to 2011. All medical records were collectedand literature of solitary fibrous tumor of the pleura with Doege-Potter syndromefrom 1979 to 2011 was obtained through Medline. This typical case, diagnosed andconfirmed by histopathologic results, was a 72-year-old Chinese woman who had acomplaint of night sweat for a month. A localized mass 12 cm × 11 cm × 8 cm insize was found associated with pleural effusion in her left low chest cavity, andblood tests showed severe hypoglycemia. Removal of the mass solved thehypoglycemia. The case was repeatedly recurrent in April, 2010 and March, 2011 andhad no signs of recurrence up to the end of 2011 after surgery. A review of 45cases of solitary fibrous tumor of the pleura with Doege-Potter syndrome comparedand summarized clinical characteristics, treatments, and outcomes by benign andmalignant tumor nature.Incidence of solitary fibrous tumor of the pleura with Doege-Potter syndromeis similar between genders. There are no significant differences in clinicalcharacteristics between benign and malignant cases. Surgery is the first effectivetreatment for solitary fibrous tumor of the pleura with Doege-Potter syndrome andthe completeness of the initial resection is the key to preventing recurrence.Routine follow-up examinations are recommended for early detection ofrecurrence. Solitary fibrous tumor of the pleura (SFTP) is a rare primary tumor, occupyingless than 5% of all pleural tumors .It arisA 72-year-old Chinese woman was hospitalized with a complaint of night sweat forone month in January, 2005. A localized mass 12 cm x 11 cm x 8 cm in size was foundassociated with pleural effusion in her left low chest cavity. Blood tests showedshe was severely hypoglycemic. Removal of the mass solved the hypoglycemia. No othertreatments were given. The histopathologic result showed a solitary fibrous tumor,potentially malignant.The patient was followed up until April, 2010 when symptoms of night sweatsreturned. A physical examination showed she had no breath sound in the left lowcavity, and the percussion note was dull on the left side of her thorax. Laboratoryevaluation revealed no significant abnormalities in hematological or biochemicalprofile except low blood glucose. The complete resection of the mass revealedmultiple well-capsulated masses in the left low chest cavity, the largest one 9 cm ×5 cm × 4 cm in size. Local tumor necrosis and mitosis up to 4/10 high power fieldswere microscopically identified. In March, 2011, the patient again had symptoms ofnight and morning sweats, and computed tomography and X-ray revealed a largerecurrent tumor mass. The extensive excision of mass plus extensive excision of thepleura was performed revealing multiple recurrent parietal pleural masses in theleft low chest cavity, the largest measured 14 cm × 10 cm × 10 cm. The episodes ofhypoglycemia abated postoperatively and the patient seemed in good health after thesurgery and had no signs of recurrence when we followed her up to the end of2011.et al. [We reviewed the literature in both English and Chinese languages from 1979 to2011 through Medline and summarized 45 cases of SFTP with DPS and classified theminto 22 benign and 23 malignant cases based on histopathologic results, referringto the criteria by England The age at diagnosis among 22 benign patients ranges from 38 to 79 yearsold, with a mean age of 60.8 ± 10.0 (standard deviation) and 23 malignantpatients 31 to 79 years with a mean age of 64.1 ± 10.8. Twenty-four cases  are females and 21  males.About 86.4% of benign and 87.0% of malignant SFTP patients are diagnosed at theages of 50–70 years old  are alsoseen in both benign and malignant patients Table .From the 13 benign and 20 malignant reports that have a detailed descriptionof the tumor mass, 100.0% benign and 80.0% malignant have sharply circumscribedborders. Most tumors are located in the right low area of chest cavity. The sizeof most benign and malignant tumors is greater than 10 cm. The average dimensionof these SFTPs with DPS is 21.7 ± 5.0 cm for benign and 17.6 ± 4.1 cm formalignant tumors. There is no significant difference in morphologic featuresbetween benign and malignant tumors  are removed by simple excision of mass, and five cases by complex excisionthat included adhesion tissue. Of the 20 malignant cases with detailed surgicalinformation, 11 (55.0%) are removed by simple excision, and 9 (45.0%) cases bycomplex excision. No distant and lymph node metastasis are found in all cases. Thefollow-up time in 29 (64.4%) of 45 cases ranges from 1 to 288 months . All but four  ofthe patients are free of recurrent SFTP with DPS. One recurrent tumor is performedby complex excision (recurrent tumor and adherent tissue). One recurrent tumor isperformed by recurrent tumor excision. But they all are experienced repeatedrelapses. The other two patients refuse medical advice based on poor lung functionand other reasons. Radiotherapy and chemotherapy are used in four malignant cases- two with single radiotherapy and chemotherapy but none of them show any effector benefit. Two of three cases having detailed information of positron emissiontomography-computed tomography examination show little to moderate uptake of[.2% are ret al. [Some criteria, especially England , are useet al.  include et al. , 4, 6.Fet al. ,4.Based on the information of 45 cases of SFTP with DPS in our study, the clinicalperformances between benign and malignant tumors are almost the same. Localrecurrence can occur in both benign and malignant tumors of SFTP with DPS . Surgery is the first effective treatment for boththe original and recurrent benign and malignant masses –9.TheseIncidence of SFTP with DPS is similar between genders. There are no significantdifferences in clinical characteristics between benign and malignant cases. Surgeryis the first effective treatment for SFTP with DPS and the completeness of theinitial resection is the key to preventing recurrence. Routine follow-upexaminations are recommended for early detection of recurrent SFTP with DPS.Written informed consent was obtained from the patient for publication of thisCase Report and any accompanying images. A copy of the written consent is availablefor review by the Editor-in-Chief of this journal.


Angiomatoid fibrous histiocytoma (AFH) is a rare, low-grade malignant soft-tissue tumor most commonly occurring in the extremities of children and young adults and has a low potential of local recurrence and metastasis. Here, we present a case of recurrent subcutaneous perianal AFH. After an initial diagnosis as a sebaceous cyst, we were able to use immunohistochemical findings to correctly identify the mass as an AFH. The patient was effectively treated after 3 surgical resections. This case emphasizes the need to correctly diagnose soft-tissue tumors using a variety of diagnostic modalities to ensure that the patient receives proper treatment. Because of the potential for misdiagnosis, molecular diagnostics are increasingly being used to detect gene fusions characteristic of AFH.2 About two-thirds of cases occur at sites with abundant lymph nodes, such as the antecubital fossa, popliteal fossa, axilla, and inguinal and supraclavicular areas.3 AFH is often associated with systemic symptoms such as anemia, fever, weight loss, enhanced cytokine production, polyclonal gammopathy, and a Castleman disease-like lymphadenopathy.4 Local recurrence is more likely in deeply situated tumors and those with infiltrative margins.5 Surgical resection is the most common management and can effectively control local recurrences and metastases. Only a few cases have been reported demonstrating AFH arising in sites other than the deep dermis and subcutaneous tissue of the extremities, trunk, head, and neck.6 Xiang et al7 reported a case of AFH arising from the retroperitoneum.Angiomatoid fibrous histiocytoma (AFH) is a rarely metastasizing soft-tissue tumor of low-grade malignant potential that mostly affects children and young adults.8 we also review the latest literature on the etiology, diagnosis, and treatment of AFH.Herein, we present a case of a 35-year-old woman suffering from AFH presenting as a recurrent perianal subcutaneous mass, who underwent 4 surgeries. To the best of our knowledge, this is the first reported case of AFH presenting in this manner. Because the rarity of AFH often leads to the misdiagnosis of either a reactive lesion or a benign or malignant tumor,A 35-year-old woman was admitted to our hospital complaining of a perianal subcutaneous mass. Two years ago, we treated her for a similar mass that we surgically resected . Both masses were similar in tenderness, texture, and mobility. The only difference of the current mass was in its location, on the left side of her anus and exposed to air. During this visit, the mass was the size of a peanut. Approximately 1 month later, however, it grew to approximately 4 × 2.5 cm  angiography showed no abnormalities of the abdominal arteries and revealed an abnormal soft tissue mass surrounding the anus. Magnetic resonance imaging (MRI) of her pelvic cavity revealed small multifocal areas on the left, right, and back side of the anus Figure . These sGiven this diagnosis, we performed a retroperitoneal tumor resection and transverse colostomy and removed a firm hemorrhagic mass measuring 8.0 × 4.0 × 3.5 cm  Classification of Tumours of Soft Tissue and Bone, AFH is described as an intermediate soft-tissue tumor that is often locally aggressive.6 To the best of our knowledge, our case is the first report to describe a recurrent perianal subcutaneous AFH with no usual systemic symptoms such as anemia, fever, and so on.Although AFH is most commonly associated with the extremities, there have been a number of reported cases of AFH arising at additional sites such as the mediastinum, bone, intrapulmonal tissue, intracranial tissue, retroperitoneum, lung, vulva, and ovary.12 On CT images, these confusable heterogeneous soft-tissue mass in the extremities are usually isodense to muscle with high attenuating areas corresponding with hemorrhaging. MRI is superior to CT in depicting fluid–fluid levels within the cystic component of the mass, indicative of intralesional hemorrhage. AFH lesions are often homogeneously hypointense (isointense to muscle) on the T1WI and heterogeneously hyperintense on the T2WI.1 In our case, the mass on CT is slightly hypodense to muscle and its MRI fit with the reported findings.Despite previous studies, it is still hard to differentiate AFH from malignant fibrous histiocytoma, sarcoma, or other tumors with imaging alone.13AFHs have a broad range of histological features. Typical AFHs are characterized by 3 microscopic findings: solid arrays or nests of histiocyte-like cells, hemorrhagic cyst-like spaces, and aggregates of chronic inflammatory cells. Multifocal recent and old hemorrhages are a striking feature of this tumor. These spaces resemble vascular spaces, but they are not lined by endothelium. Inflammatory cells present include lymphocytes and plasma cells. A thick pseudocapsule and occasional germinal centers give this tumor a resemblance of a lymph node.14 Bohman et al4 reported a series of cases with perineurioma-like areas, with eosinophils in the associated inflammatory infiltrate, and with numerous eosinophils. To clarify the cellular differentiation features of AFH, Hasegawa et al15 once examined 4 cases of AFH by clinicopathologic, immunohistochemical, and ultrastructural analyses. Seventy-five percent examined by electron microscopy had a mixture of fibrohistiocytic, myofibroblastic, and undifferentiated cells containing cytoplasmic processes and dense core granules. The authors pointed that AFH has a variety of immunophenotypes, including histiocytic, myofibroblastic, epithelial, and neural and may occasionally have predominantly round cell morphology.15In addition to features that have been previously described such as nuclear pleomorphism, increased mitotic activity, atypical mitotic figures, reticular, myxoid patterns, lymphoplasmacytic infiltration, pseudovascular spaces filled with blood, round cell morphology, and spindle cell patterns.2 Cytogenetic analysis of our case demonstrated positivity for desmin, CD68, and CD99.The immunohistochemical features sometimes present a unique immunophenotype. For instance, approximately 50% to 60% of AFH lesions coexpress desmin, EMA, CD68, and CD99 but are generally negative for keratins, S-100 protein, CD34, and follicular dendritic cell markers (CD21 and CD35).16 followed by EWSR1-ATF1 from t,17 and the least common FUS-ATF1 from t.18 Thway et al expanded the clinicopathological spectrum of AFH by characterizing 13 AFH cases by fluorescence in situ hybridization (FISH) and reverse transcription polymerase chain reaction (RT-PCR). Besides the EWSR1 gene rearrangement detected in 4 cases by FISH, EWSR1-CREB1 fusion was confirmed in 9 cases, including a schwannoma-like AFH, and a EWSR1-ATF1 fusion was detected in a myoepithelioma-like AFH.16 These studies suggest that combinatorial FISH and RT-PCR will likely be diagnostically powerful tools to identify AFH by evaluating EWSR1-CREEB1, EWSR1-ATF1, or FUS-ATF1 fusions.2Because the role of immunohistochemistry in establishing the diagnosis of AFH is still limited, additional markers will be important to distinguish this tumor. Recently, the molecular pathogenesis of AFH has become clarified by the discovery of fusion genes resulting from various chromosomal translocations, including the most prevalent EWSR1-CREB1 derived from t,19 In the currently reported case, the initial diagnosis was as a sebaceous cyst because of the resemblance in appearance. The imaging studies we obtained were not diagnostic, but a pathological review of the immunohistochemistry confirmed our AFH diagnosis.It is important to correctly diagnosis AFH patients to avoid overtreatment when misinterpreted as a more aggressive tumor such as malignant fibrous histiocytoma, nodular Kaposi sarcoma, Ewing sarcoma, and rhabdomyosarcoma. Considering its potential for recurrence and metastasis, it is also important to avoid undertreatment by misdiagnosing it as a sebaceous cyst, perianal abscess, aneurysmal benign fibrous histiocytoma, or inflammatory pseudotumor.We report an uncommon case of AFH located around the anus without systematic symptoms. This patient suffered a frequent recurring AFH mass and underwent 3 surgical resections. AFH is a rare disease that is often initially misdiagnosed. It is a challenge to avoid overtreatment or undertreatment. Our case emphasizes the need to correctly diagnose soft-tissue tumors using a variety of diagnostic modalities to ensure that the patient receives proper treatment.


Massive ovarian oedema is a rare non-neoplastic clinicopathologic entity has a higher incidence in women during their second and third life decade. The oedema can be presented in one or both ovaries as a result of partial intermittent torsion of the ovarian pedicle that interferes to the venal and lymphatic drainage of the ovary.We present a clinical case of a 16 year old with massive ovarian oedema and we performed a review of the literature. The pathophysiology of this entity is very complex. We tried to perform a complete review of the literature and focus on the complexity of this entity as far as its pathophysiological backround is concerned and as far as its clinical presentation is concerned.In conclusion, massive ovarian oedema is a rare, multi disease mimicking clinical entity, with an acute or progressive clinical presentation. It has also to be a part of our differential diagnosis in cases of acute abdominal pain and we have to try to treat her conservatively, in order to preserve fertility. Massive ovarian oedema is a rare clinical entity, which is benign and has a higher incidence in women during their second and third life decade. This lesion was first described in 1967 . The facA 16-year-old patient was admitted in our emergency department with persistent abdominal pain in her right lower abdominal quadrant. The patient claimed that the pain was colic like, persistent and gradually advancing the last 2 months. Her medical history was free, her menarche was in the age of 13 years and her menstrual cycle had a periodicity of 28 days. The clinical examination revealed a palpable abdominal mass extending from the fossa iliaca to the liver. The patient was hemodynamic stable. After two doses of intravenous paracetamol the pain was alleviated. We performed a radiologic and laboratory control; our imaging control included an Ultrasound of the lower abdomen and a Ct Scan of the upper and lower abdomen. An intravaginal ultrasound control could not be performed because the patient was still virgin. The CT scan revealed a cystic lesion 23x15cm located in the right oblique abdominal area, from the liver to the minor pelvis, which is causing pressure to the right kidney and the bladder and a small fluid collection in the Douglas region. Fig. . In the Massive ovarian oedema is a tumor-mimicking condition occurring in young women . It is oThe lymphatic vessels have a special morphology without a structural basement membrane and an overlapping layer of vascular endothelial cells. The functionality of these cells is controlled from the Vascular Endothelial Growth Factor (VEGF) and its receptor VEGFR2) . There a . There Massive ovarian oedema is a rare, multi disease mimicking clinical entity, with an acute or progressive clinical presentation. It has also to be a part of our differential diagnosis in cases of acute abdominal pain and we have to try to treat it conservatively, in order to preserve fertility.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.


Calcifying fibrous tumors (CFT) are rare benign tumors. They usually affect children and young adults and the incidence is equal in males and females. The usual clinical presentation is that of a painless mass. A computed tomography scan typically reveals a well-demarcated calcified lesion. CFT usually presents as a solitary mass and the commonest sites of occurrence are in soft tissues, the pleura, or the peritoneum. Multifocal occurrences at the same site have also been reported. The first case of CFT was reported in 1988. We present a rare case of multiple calcifying fibrous tumors at multiple sites in the same patient. To the best of our knowledge, this is the first ever reported case of multifocal CFT atsix different anatomical sites in one patient. Calcifying fibrous tumors (CFT) are rare benign tumors. They usually affect children and young adults and the incidence is equal in males and females. The usual clinical presentation is that of a painless mass. A computed tomography scan typically reveals a well-demarcated calcified lesion. CFT usually presents as a solitary mass and the commonest sites of occurrence are in soft tissues, the pleura, or the peritoneum. Multifocal occurrences at the same site have also been reported. The first case of CFT was reported in 1988 [A 31-year-old caucasian man presented with a three-week history of abdominal pain on urination and altered bowel habits. Apart from tenderness and fullness in the right loin, his clinical examination was unremarkable. There were no clinical signs of autoimmune disease. He was physically fit, with no history of major illness. The results of his renal function, liver enzyme, bone profile, thyroid function, and full blood count tests, as well as an ultrasound of his kidneys, were normal. A computed tomography (CT) scan of his chest, abdomen, and pelvis reported multiple calcified masses in his right adrenal gland, right paraspinal region, left pelvis, spleen, liver, and lung nodules Figures  and 5.He was initially investigated for metastatic adrenal cortical carcinoma, pheochromocytoma, or metastatic sarcoma due to the right adrenal mass found in his CT scan. His random serum cortisol levels were raised and his 24-hour urinary noradrenalin, adrenalin, and dopamine levels were normal. Serum tumor markers such as carcinoembryonic antigen (CEA), alpha fetoprotein (AFP), and beta human chorionic gonadotropin (β-HCG) were normal. An initial CT-guided biopsy from the pelvic mass showed only unremarkable fibrofatty tissue and was hence concluded as non-diagnostic  was considered as a possible diagnosis but CFT was considered to be more likely. Both these resection specimens were sent for a second opinion by a soft tissue pathologist who confirmed the diagnosis of CFT as there was no expression of anaplastic lymphoma kinase 1 (ALK1). The patient’s symptoms of pelvic pain resolved after surgery. His case was discussed in a specialist multidisciplinary meeting and it was decided not to resect other masses as he was currently asymptomatic. A multidisciplinary team meeting decided against biopsying other lesions as they were not showing any signs of change and were radiologically similar to the one resected. He had surveillance CT scans every four months and there was no change in the remaining tumors. He remained very well and asymptomatic more than two years after his diagnosis of CFT. He is currently continuing with six-monthly clinical follow-ups. He remains well and asymptomatic.et al. reported 10 cases of CFT in 1993 and named this tumor’calcifying fibrous pseudotumor’ for the first time [CFT is a rare benign tumor presenting as a solitary calcified mass or multiple masses in one particular organ. The pathogenesis is unknown. The mass or masses are characterized as a collection of dense hyalinized collagenous tissue interspersed with benign-appearing spindle cells, psammomatous or dystrophic calcifications with lymphoplasmacytic inflammatory infiltrate . It was . Inflammatory myofibroblastic tumor is, however, distinguished from CFT by being generally more cellular; less hyalinized and typically lacking in calcification. Unlike IMT, CFT rarely expresses ALK by immunohistochemistry, suggesting that CFT is a different clinicopathologic entity than IMT. CFT can be asymptomatic for many years before presenting with symptoms. Symptoms vary with the site of occurrence and common presentations are those of pressure symptoms. The treatment for CFT is as it was in our case - surgical resection with long-term follow-up. Recurrence is extremely rare [The most common site of CFT reported in the literature is the pleura ,4. On imely rare ,6.On a review of the literature we found 103 cases of CFT. Of these, a total of 41 patients were male and 62 patients were female. Eighty patients had solitary tumors and 23 patients had multiple lesions. All of the patients who had multifocal CFT were limited to one or two anatomical sites -10. To tCFT is a rare benign tumor with a good prognosis after resection. It can be misdiagnosed on imaging as metastatic carcinoma. Histological confirmation by an experienced pathologist can confirm the diagnosis. Recurrence after complete resection has not been reported.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.FA, Manuscript writing and proof reading. MC, Manuscript writing and proof reading. SK, Providing pathology specimen/slides and proof reading. MP, Providing pathology specimen/slides and proof reading. AA Proof reading. MFL, Manuscript writing and proof reading. EM Proof reading. All authors have read and approved the final version of the manuscript.Histological appearance of pelvic CFT.Click here for file


Several types of congenital lesions can cause complete or incomplete obstruction of the intestine. Our purpose is to present 3 neonates with dual intestinal type I atresia, i.e., simultaneous obstructive lesions at 2 locations in which the atresia manifested as diaphragm-like tissue.All 3 cases were female infants ranging in age from 2 to 14 months. The common symptom in all cases was intermittent persistent vomiting. In some cases the vomitus was bilious, and other symptoms included abdominal distention and delayed meconium passage. Prior surgeries at another hospital were unsuccessful at relieving the symptoms in one case. One case had dual lesions in the colon, one dual lesions in the duodenum, and one atresia at both the distal portion of the ileum and the descending colon. Surgical exploration and removal of the lesions at our hospital was successful in all cases, and the infants were discharged in good condition.Type I atresia can manifest as a diaphragm-like tissue obstructing the continuity of gastrointestinal tract, and in rare cases multiple areas may be present. Base on the intermittent nature of the associated symptoms, diagnosis can be difficult and is often delayed. Physicians should be aware of this condition during the work-up of an infant with persistent intermittent vomiting. Several types of congenital lesions can cause complete or incomplete obstruction of the intestines and atresia, failure of the lumen to canalize, accounts for approximately 95% of obstructions . The speIntestinal atresia can occur anywhere along the GI tract, and the anatomic location of the obstruction and the degree of blockage determine the clinical manifestations . CharactSmall intestine atresia accounts for more than 90% of all cases of infants with congenital occlusions, while colon occlusion due to variable septa is rare, with only 6 reports since 1966. Though intestinal atresia usually occurs in a single location (>90% of cases), 6-20% of cases have multiple areas of atresia , and morAll three patients had signed informed consent, and the project was approved by the Medical Ethical Committee, The First Affiliated Hospital, Sun Yat-sen University. The details of each GI atresia conditions were described below.A 2-month-old female presented with abdominal distension and defecation difficulties soon after birth. She was one of premature twins born at 32 4/7 weeks’ gestation. She was admitted to a local hospital for a barium enema, and the results indicated areas of atresia located at both the hepatic and splenic flexures of the colon. Dilation of the colon was noted proximal to the atretic sites Figure . When shA 14-month-old female presented with repeated emesis since 3 months of age. The vomitus was mainly milk with occasional bilious fluid. The vomiting often occurred at night, 2 to 3 times a week. When she was 8 months old the vomiting increased to 2 to 3 times a day, with larger portion of the vomitus containing bile. No evidence of blood in the vomitus or abdominal distension was noted. GI radiography was performed at a local hospital, and obstructions were seen at the third and forth portion of the duodenum. Three surgeries were performed at the local hospital including a Ladd operation and duodeno-duodenostomy; however, her symptoms were not alleviated after these procedures. Hence, she underwent a further exploratory operation at the local hospital, and an obstruction which has the appearance of a diaphragm at the third portion of the duodenum was noted, along with severe adhesions. A proximal duodenostomy, resection of the septum, and enterolysis was performed, and a jejunal nutrition tube was placed. Unfortunately, her symptoms worsened after surgery. She was then admitted to our institution, and reexamination of the GI radiographic studies indicated that there was still an obstruction at the duodenum.Surgical exploration at our hospital revealed significant dilation of the second portion of the duodenum, while the distal intestines were saggy and flat. The third portion of the duodenum was adhered in a U-shape. After releasing the adhesions, a diverticulum was visible at the lateral margin of the mesentery. We removed the duodenum drainage catheter to inject air and extrude the air towards the distal end, and air was noted to pass through. A further attempt was made to place a rubber tube in the third portion of the duodenum, but the placement was inhibited at the junction of the second and third portions of the duodenum. The diverticulum was removed by wedge resection . The perforation within the diaphragm is usually singular and centrally located within the lumen of the intestine. An intestinal diaphragm may be accompanied with other malformations of the digestive tract such as an annular pancreas . A windsThe characteristic clinical feature of almost all intestinal diaphragms is repetitive vomiting. In cases of a perforate diaphragm, the diameter of the opening directly determines the degree of obstruction, and therefore is inversely related to the level of symptoms. Nevertheless, in cases of colonic diaphragmatic atresia, vomiting typically occurs after a full stomach and stools tend to be unformed or thin strip-shaped . In caseIt is difficult to distinguish type I atresia from other causes of vomiting by clinical manifestations alone. GI radiography and endoscopy are the best methods to diagnose the obstruction before surgery . HoweverTo avoid misdiagnosis, a few key points should be noted before surgery. Surgeons should observe the barium enema being performed which will help them to distinguish abnormalities from normal intestinal movement. In addition, surgeons should fully understand the patient’s prior surgeries and pathogenesis to avoid misdiagnosis of an intestinal diaphragmatic atresia.Written informed consents were obtained from the patients’ parents or guardians for publication of the case report and any accompanying images. Copies of the written consents are available for review by the journal editor.GI: Gastrointestinal.The authors declare that they have no competing interests.We declare that all the listed authors have participated actively in the study, and all meet the requirements of the authorship. J-CL reviewed the medical records and performed research. H-DC wrote the first draft of the manuscript and performed research. HJ and L-EH performed research. AK, Z-CZ, and Z-HZ managed the literature searches and analyses. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/14/108/prepub


Desmoid-type fibromatosis (DTF) is an uncommon nonmetastatic fibrous neoplasm. Sporadic intraperitoneal DTF is rarely described in current literature. We herein report a case of DTF of unknown cause involving the pancreatic head. A 41-year-old man presented with recurrent epigastric pain and weight loss. An abdominal computed tomography scan showed a well-delineated solid cystic mass inside the pancreatic head. Pylorus-preserving pancreaticoduodenectomy was performed due to the patient’s debilitating symptoms and suspected malignancy. The pathological examination revealed massive fibroblastic proliferation arising from the musculoaponeurotic tissues, consistent with a diagnosis of DTF. Immunohistochemical phenotyping determined positive immunoreactivity to vimentin and β-catenin, but negative immunoreactivity to smooth muscle actin, CD117, CD34, or S-100, confirming the diagnosis of DTF. No local recurrence or distant metastasis was found during a 24-month follow-up. Radical resection is recommended as first-line treatment for pancreatic DTF. Long-term follow-up studies are required to establish the prognosis of pancreatic DTF. Desmoid-type fibromatosis (DTF), also known as desmoid tumor or aggressive fibromatosis, is a rare soft tissue neoplasm. It is locally invasive but without metastatic potential, and is slow-growing . DTFs, wDTF is more frequently reported among the pediatric population (infantile DTF), but can occur at any age. In children, DTFs have primarily been extra-abdominal, involving the skeleton, skeletal muscle, adjacent fascia, aponeurosis, or periosteum. Intra-abdominal DTF (8%) is seen more often in adults, mainly in the gastrointestinal and genitourinary tracts . AlthougIntra-abdominal DTFs derive primarily from mesenteric connective tissue or the retroperitoneum . These tHerein we report a patient with an isolated, sporadic, and non-trauma-related DTF, located at the pancreatic head and manifesting as a cystic lesion. The patient underwent a successful curative resection and exhibited no signs of recurrence or metastasis within a 24-month follow-up period.A 41-year-old Han Chinese male non-smoker was referred to our surgical unit due to a six-month history of progressive epigastric pain and loss of weight. He denied any radiating pain, nausea, or vomiting. His past medical history showed a healed pulmonary tuberculosis and excision of a neck lipoma, 15 and 10 years previously, respectively. No history of prior abdominal trauma or surgery was noted. The patient had concomitant essential hypertension, but received no antihypertensive treatment. No family history was reported that was suggestive of any genetic disease.His physical examination was clinically insignificant. The liver function test was normal except for elevations in conjugated and unconjugated bilirubin: total 60.2 μmol/L , conjugated 19.3 μmol/L (0 to 8.6 μmol/L), and unconjugated 40.9 μmol/L (3.4 to 11.9 μmol/L). The levels of serum tumor markers were within normal limits: carcinoembryonic antigen, 2.5 ng/mL (0 to 5 ng/mL); carbohydrate antigen 19 to 9, 30.3 IU/mL (0 to 37 IU/mL); and α-fetoprotein antigen, 4.2 IU/mL (0 to 9 IU/mL).1- and T2-weighted images  indicated that the intrahepatic duct, the common bile duct, and the main pancreatic duct were all moderately dilated  scan revealed a 1.9-cm solid cystic mass in the pancreatic head, with no signs of vascular or visceral invasion  7 but developed an infected intra-abdominal hematoma on POD 13. This condition resolved with antimicrobial therapy as well as peritoneal irrigation and drainage. Oral intake was initiated on POD 18, and the patient was discharged from the hospital on POD 21.The histological examination confirmed a clean resection margin. Histological sections showed a large number of spindle-shaped cells, with a regular nuclear pattern within a background of massive collagen fibers. The peritumoral parenchymal tissue was edematous and infiltrated by tumor cells and a small number of inflammatory cells Figure a. No marFurther immunohistochemical analysis was ordered to differentiate uncommon gastrointestinal tumors of mesenchymal or neural origin; that is, DTF, fibrosarcoma, gastrointestinal stromal tumor, and neurosarcoma. The analysis revealed that the tumor cells were immunopositive for vimentin Figure b and β-cThe patient received no adjuvant therapy after surgery. He was followed-up regularly at the outpatient clinic using routine hematological and biochemical tests, as well as abdominal CT scan. He exhibited good health and complained of no discomfort. The total bilirubin level returned to normal after the resection. No signs of local recurrence or distant metastases were identifiable on abdominal CT scan by the last scheduled follow-up visit at 24 postoperative months.Pancreatic tumors are usually (approximately 95%) primary cancers of exocrine origin, namely, adenocarcinomas; the remainder arise from neuroendocrine cells. Pancreatic DTFs are extremely rare and encountered incidentally in surgical practice. Only nine previous cases of pancreatic DTFs have been reported within the last three decades in the English medical literature -13 , estrogen might have a pathogenetic role . HoweverFAP is frequently associated with intra-abdominal DTF, but only one pancreatic DTF patient was reported to have concomitant FAP in the current literature . Two sibAPC) or β-catenin (CTNNB1) is the most significant risk factor for intra-abdominal DTF in FAP patients [COX2), platelet-derived growth factor (PDGF), matrix metallopeptidase (MMP), hyaluronan-mediated motility receptor (RHAMM), and retinoblastoma 1 (RB1) [A somatic mutation in the genes 3′-adenomatous polyposis coli  . These gPrevious abdominal surgery is said to initiate the proliferation of fibrous tissue and is therefore implicated in the occurrence and recurrence of intra-abdominal DTF. However, of the reported pancreatic DTF patients, only two had undergone previous abdominal surgery, one of whom developed a sporadic DTF at the pancreatic suture line , and theDTF is characterized by a highly variable and unpredictable clinical course of tumor growth, stabilization, and occasionally even spontaneous regression in the absence of medical intervention . DTF-assA confirmed diagnosis of sporadic intra-abdominal DTF prior to surgery is very unlikely, while a tentative diagnosis is mainly based on clinical suspicion combined with medical imaging evaluation. A history of FAP may raise the possibility of intra-abdominal DTF, although pancreatic DTF is usually sporadic. Medical imaging evaluation is important for characterizing the location of pancreatic DTF in relation to the surrounding tissues and organs  and for determining its resectability. On imaging studies, pancreatic DTF usually manifests as a well-defined solid or solid cystic lesion. On ultrasonography, it is homogeneous and hypoechoic; on CT scan, it appears as hypodense soft tissue; and on MRI, signal intensity is low. The tumor is expected to be mildly enhanced in the presence of contrast media, as it consists essentially of over-proliferation of fibrous tissue. EUS is very useful for visually defining the pancreatic tumor located in the pancreatic head, and to determine whether the major vessels and the duodenum are infiltrated by the tumor. Additionally, EUS allows fine-needle aspiration biopsy for cytological examination of superficially located tumors ,21. In tThe typical histology of DTF shows regular fibroblasts and fibrocytes in a well-oriented pattern, but also with an infiltrative growth pattern within a background of massive collagen bundles. However, it is histologically challenging to differentiate pancreatic DTF from other uncommon pancreatic tumors such as low-grade fibrosarcoma, neuroendocrine tumor, and gastrointestinal stromal tumor. DTF is sometimes thought to be a potential sarcoma due to its aggressive growth and local invasion, but pathologic mitosis or metastasis is very unlikely .Immunohistochemistry against specific cell markers of various origins is a very effective diagnostic tool for the differentiation of pancreatic tumors. Pancreatic DTF expresses the markers for mesenchymal cells (vimentin) but not markers for stromal cells (CD117 and CD34) or neural cells (S-100). β-catenin immunohistochemistry is reported to be useful for distinguishing deep DTF from other benign or malignant fibroblastic and myofibroblastic lesions . Thus, tMultimodal therapy has been reported for the treatment of intra-abdominal DTF, including pancreatic DTF. Watching and waiting is an acceptable option for patients with asymptomatic or minimally symptomatic DTF, as it is a non-metastatic and slow-growing tumor. However, surgical intervention seemed mandatory in our case as the presence of pancreatic DTF was seriously compromising the quality of life of this patient, and its obscure nature made it potentially life-threatening.The mainstay of pancreatic DTF treatment is radical resection. In most previous reports, patients underwent distal pancreatectomy or pancreaticoduodenectomy, except for three patients who received only a tumor biopsy. The primary concern of the physician regarding surgical resection is the high likelihood of local recurrence: the reported recurrence rate of DTF after resection is 19 to 77% , and is Alternative or adjuvant therapies have also been proposed for the management of intra-abdominal DTF. Non-steroidal anti-inflammatory drugs (NSAIDs) were reported to successfully downsize a pelvic DTF in a man with complicating lower limb vascular compression . HormonaThe prognosis of pancreatic DTF is not known. Recurrence after curative resection has not been observed during mid- and long-term follow-up, except for a single patient with a congenital generalized fibromatosis . A favorIn conclusion, sporadic pancreatic DTF is a rare non-metastatic soft tissue tumor that can be locally quite destructive, and in our case, debilitating. Initial diagnosis is mainly based on clinical suspicion and medical imaging. The diagnosis is confirmed via immunohistochemistry to differentiate DTF from other uncommon soft tissue tumors originating from the pancreas. Radical resection with a clean margin is recommended as the first-line treatment for DTF, although FAP patients are more prone to recurrence following surgical resection. Adjuvant therapies may be effective for unresectable or recurrent DTF. Long-term prognosis is currently unknown, and a regular follow-up is essential.DTF: desmoid-type fibromatosis; FAP: familial adenomatous polyposis; CT: computed tomography; MRI: Magnetic resonance imaging; EUS: endoscopic ultrasonography; MRCP: magnetic resonance cholangiopancreatography; POD: postoperative day; NSAIDs: non-steroidal anti-inflammatory drugs.The authors declare that they have no competing interests related to the publication of this report.CJ and XW prepared the manuscript and conducted the literature search; CD reviewed and edited the manuscript; XB corrected and revised the manuscript; CD, CJ, and FX treated and observed the patient; XW provided the radiographic and ultrasound images; BT performed the histopathological and immunohistochemical examinations. All authors read and approved the final manuscript.


The sellar and juxtasellar regions in the paediatric population are complex both anatomically and pathologically, with magnetic resonance imaging (MRI) being the “gold standard” imaging modality due to the high contrast of detail. Assessment requires a detailed understanding of the anatomy, embryology, pathophysiology and normal signal characteristics of the pituitary gland and surrounding structures in order to appropriately characterise abnormalities. This article aims to provide an overview of the imaging characteristics of developmental/congenital and acquired disease processes which affect the sellar and juxtasellar region in the paediatric population.Main MessagesThe sellar region is anatomically complex and covers a wide pathology spectrum.• MRI is the key imaging modality to assess sellar and juxtasellar pathology.• Numerous developmental anomalies may not be discovered until adulthood.• Knowledge of pathology alerts and guides the clinician towards appropriate management.•  The pituitary gland is comprised of two dominant lobes, the neurohypophysis and the adenohypophysis. It is situated within the sella turcica, a depression in the sphenoid bone which is covered by the diaphragm sella with a defect for the passage of the infundibulum. The pituitary stalk extends inferiorly as a continuation of the hypothalamic infundibulum Fig. .Fig. 1NoThe neurohypophysis receives its hormonal supply through direct contiguity with the hypothalamus, with axons extending from the hypothalamic supraoptic and paraventricular nuclei into the neurohypophysis. The hormonal supply includes oxytocin and vasopressin-containing vesicles. The adenohypophysis does not have axonal contiguity between the hypothalamus and the anterior gland and relies on the hypothalamic-hypophyseal portal system. Parvocellular messengers are transported from the hypothalamic median eminence into the capillaries surrounding the anterior gland. Hormones from the adenohypophysis include: follicle stimulating hormone, luteinising hormone, corticotropin (ACTH), thyroid-stimulating hormone, prolactin and growth hormone.The pituitary gland develops through a complex mechanism involving the developing diencephalon and the primitive oral stomodeum . The adeThe pituitary gland exhibits a wide range of appearances during the first 2 years of life, with considerable variability in size, shape, and signal. At birth, both the anterior and posterior pituitary gland have high signal intensity on T1-weighted images Fig. . The antAt birth, the gland is physiologically enlarged with a corresponding concave superior margin, flattening with age. The gland is traditionally symmetric in size; however there can be asymmetry in normally functioning glands. In preterm infants, the gland is taller than in normal-term infants, even when correcting for gestational age. This is thought to be the result of reduced insulin-like growth factor 1 and higher levels of growth hormone in premature infants . The pitThis is a rare congenital anomaly with a neonatal presentation. The neonates can present with metabolic acidosis, thyroid and adrenal insufficiency, hypoglycaemia, and/or seizures. Associated anomalies of the forebrain and midline craniofacial bones can be present. Newborns are normal in size at birth, which has been explained by the presence of maternal growth hormone. Standard treatment includes replacement of growth hormone, thyroid hormone and glucocorticoids. Magnetic resonance imaging (MRI) findings include the absence of the anterior pituitary gland with an associated shallow sella, and variable length of the infundibulum and position of the posterior pituitary, ranging from the hypothalamus to the sella . The infThis rare anomaly consists of hypoplasia of the anterior pituitary gland, and also potentially the infundibulum and posterior pituitary gland. It can be associated with varying degrees of endocrine dysfunction, including panhypopituitarism and other congenital anomalies. Some research suggests that traumatic transection of the pituitary stalk during birth or hypoxic injury to the hypothalamus could result in hypoplasia, whereas others suggest an error during embryogenesis –12. MRI This congenital anomaly results in an aberrant position of the neurohypophysis, often located at the undersurface of the hypothalamus. It is associated with pituitary dwarfism, delayed skeletal maturation, Kallman syndrome, septo-optic dysplasia, dysgenesis of the corpus callosum, lobar holoprosencephaly, Chiari 1 malformation or a persistent/enlarged craniopharyngeal canal. It affects males more than females. MRI may show a small anterior pituitary gland with the posterior “bright spot” on T1-weighted imaging (T1WI) located within the upper portion of the infundibulum or the undersurface of the hypothalamus instead of the dorsal portion of the sella –15.This congenital anomaly is related to abnormal separation of the anterior portion of the notochord and prechordal plate during embryogenesis, likely due to teratogenic factors occurring during early embryogenesis , 17. CliCongenital developmental non-neoplastic grey matter heterotopia involving the tuber cinereum, inferior hypothalamus and/or mammillary bodies which are composed of small neuronal cells within a neutrophil-like stroma and scattered fibrillary astrocytes. These lesions typically occur in children with isosexual precocious puberty (0-8 years) or seizures, both gelastic or partial complex, in the second decade , 21. TheLack of involution of the craniopharyngeal canal, which can contain pituitary tissue. The adenohypophysis develops from evagination of ectodermal cells at the upper posterior portion of the primitive oropharynx, Rathke’s pouch, at 4-5 weeks of gestation. Rathke’s pouch passes between the developing presphenoid and basisphenoid chondrification centres at the upper clivus and sella, where it associates with the descending neuroectoderm of the infundibulum and will go on to form the neurohypophysis. The pathway of Rathke’s pouch is the craniopharyngeal canal, which progressively involutes by 6-7 weeks of gestation. The persistent craniopharyngeal canal on MRI can be associated with trans-sphenoidal meningoencephaloceles, ectopic pituitary tissue, sphenoid teratoma and infrasellar craniopharyngiomas , 24. On Benign cystic lesion containing fluid and variable amounts of protein, mucopolysaccharides and/or cholesterol , 26. TheCongenital malformation involving the lack of separation of neuroectoderm from surface ectoderm with resultant localised failure of bone formation. There is a wide range of abnormalities which affect the skull base within the sella turcica, ranging from a persistent craniopharyngeal canal to trans-sphenoidal and sphenoethmoidal cephaloceles, many presentations of which are extremely rare. For example, the reported incidence of trans-sphenoidal meningoencephaloceles is 1 per 700,000 live births , 32. CliNon-neoplastic congenital or acquired extra-axial off-midline lesion filled with desquamated cells and keratinaceous debris, usually with mild mass effect on the adjacent brain. Epidermoids grow by accumulation of desquamated epithelial cells into the centre of the cyst –35. MRI Non-neoplastic congenital or acquired ectodermal inclusion cystic lesion, which like an epidermoid, is lined by squamous epithelium, and are filled with lipid material, cholesterol, desquamated cells and keratinaceous debris. They are distinguished from epidermoids by containing deeper dermal elements and commonly arise from the midline. They typically present in the 20- to 30-year age range, but can also be seen in adolescence, with males slightly more affected than females . MRI wilCongenital arachnoid cysts are found in 0.16 % of all newborns with approximately 9-15 % occurring in the sellar region, with suprasellar cysts more commonly seen than intrasellar cysts. Most cases are asymptomatic; however, they may cause headache, optic nerve compression, psychomotor retardation, endocrine dysfunction or hydrocephalus , 40. SecBenign fatty lesions resulting from a congenital malformation, often located at or near the midline. In the sellar region, they occur along the surface of the infundibulum, floor of the third ventricle, or adjacent to cranial nerves, and are usually discovered incidentally, unless they cause a mass effect , 43. TheCommon incidental finding involving the pituitary gland and sella, which can result from a weakened or fenestrated diaphragma sella allowing CSF pulsations to flatten the pituitary gland. They are associated with elevated intracranial pressure, a posteriorly placed optic chiasm, and are considered a normal variant or termed “primary empty sella turcica” in the absence of surgery, radiation therapy or medically treated intrasellar tumour . In chilIn comparison to adults, pituitary adenomas are rare in the paediatric population, accounting for approximately 2.7 % of all supratentorial tumours. Most paediatric adenomas are functioning, with only 3-6 % non-functioning, compared to one-third of non-functioning adenomas in adults –48. MicrMRI findings of a microadenoma (<10 mm) commonly have intermediate signal on T1WI and T2WI, and may show characteristics of cysts, haemorrhage or necrosis. They typically show less contrast enhancement than normal pituitary tissue, often best visualised with dynamic early-phase imaging. In macroadenomas (>10 mm), they commonly have intermediate signal on T1WI and T2WI similar to grey matter, and may also have cystic, haemorrhagic or necrotic components. They can extend into the suprasellar cistern with a waist at the diaphragm sella or into the cavernous sinus, with occasional invasion into the skull base.Usually histologically benign locally aggressive lesions which arise from squamous epithelial rests along Rathke’s cleft, occurring in a bimodal age distribution, in children (<10 years) and adults (>40 years). Craniopharyngioma’s account for 3 % of all intracranial tumours. They are categorised into adamantinomatous and squamous-papillary types. The adamantinomatous type is more common and has a bimodal age distribution occurring in children and adults, whereas the squamous-papillary type usually occurs in adults. Although histologically benign, their insinuating pattern of growth makes complete surgical excision very difficult and not often possible.The adamantinomatous type usually demonstrates circumscribed lobulated margins on MRI, with the suprasellar location seen at a greater frequency than intrasellar. They show variable low, intermediate and/or high signal on T1WI and T2WI, with or without a nodular or rim of contrast enhancement. They may contain cysts, lipid components and calcifications. In children, the cystic parts tend to predominate, and frequently contain highly proteinaceous fluid, which may be bright on T1WI .Lesions which may occur anywhere along the optic pathway and account for 4-6 % of brain tumours in children, with a median age of diagnosis from 5-9 years. Approximately half of patients with optic pathway gliomas have neurofibromatosis type 1 (NF1), with symptomatic lesions only occurring in 1-5 % of patients . Most tuRare neoplasms (WHO grade II) which contain prominent mucoid matrix and angiocentric pattern of bipolar neoplastic astrocytes. Typically, they occur in children in the first and second decades, with a mean age of 7 years . They teExtra-gonadal germ cell tumours include: germinoma (most common), mature teratoma, malignant teratoma, yolk sac tumour, embryonal carcinoma and choriocarcinoma. They account for 0.6 % of primary intracranial tumours, with an incidence of 0.09 per 100,000. Peak incidence occurs between 10 and 14 years, with 90 % occurring in patients less than 25 years. They occur more frequently in males. Prognosis depends on the histological subtype, with a 10 year survival rate for germinomas of over 85 %. Other germ cell tumours have a lower survival rate, particularly those containing non-germinomatous malignant cells. Tumours often have intermediate signal on T1WI, slightly high signal on T2WI, and show contrast enhancement. They are typically centred on the pituitary stalk with the posterior pituitary bright spot often absent. They may look similar to Langerhans’ cell histiocytosis (LCH) or lymphocytic hypophysitis . ContrasThe second most common type of germ cell tumour behind a germinoma. They occur in children, with a greater frequency in boys, accounting for 0.5-1.5 % of all childhood brain tumours, and >50 % of intracranial tumours in infants less than 2 months , 55. HisMycobacterium tuberculosis infection remains a major international concern, with increasing burden of disease in developed nations and young children being the most susceptible [ceptible , 57. MRIceptible . MRI finceptible .Rare malignant bone tumour comprised of proliferating neoplastic spindle-cells which produce osteoid and/or immature tumoural bone. They usually involve the endochondral bone-forming portions of the skull-base and are locally invasive with high metastatic potential. Over 75 % of osteosarcomas are diagnosed in patients between 8 and 25 years, and account for less than 1 % of all head and neck malignant tumours in the paediatric population . They ocBone tumour usually occurring between ages 5 and 30 years, with a greater prevalence in males and is the second most common primary bone malignancy affecting adolescents and young adults. It most commonly arises in the trunk and diaphysis of long bones, with only 4 % of cases within the head and neck region . TumoursThese malignant neoplasms of neuroectodermal origin, arise from olfactory epithelium in the upper nasal cavity and cribiform region. Tumours consist of immature neuroblasts with variable nuclear pleomorphism, mitoses and necrosis. Tumour cells occur in aneurofibrillary intercellular matrix. There is a bimodal age of occurrence in adolescents (11-20 years) and adults (50-60 years) , with a Benign medullary fibro-osseous lesion, which can involve a single site (mono-ostotic) or multiple locations (poly-ostotic), results from developmental failure in the normal process of remodelling primitive bone to mature lamellar bone with zones of immature trabeculae within dysplastic fibrous tissue. Craniofacial bones are involved in 10-20 % of patients with the monostotic disease and in 50 % of patients with the polyostotic form . PatientRefers to an uncommon disorder in which polyostotic fibrous dysplasia is associated with pigmented cutaneous macules (café-au-lait spots) with irregular indented borders, precocious puberty, and/or other endocrine disorders such as acromegaly, hyperthyroidism, hyperparathyroidism, and/or Cushing’s syndrome. McCune-Albright syndrome is uncommon and occurs in 2-3 % of patients with polyostotic fibrous dysplasia .Disorder of the reticuloendothelial system in which bone-marrow-derived dendritic Langerhans’ cells infiltrate various organs as focal lesions or in diffuse patterns. Langerhans cells have eccentrically located ovoid or convoluted nuclei within pale to eosinophilic cytoplasm. Lesions often consist of Langerhans cells, macrophages, plasma cells and eosinophils. Prevalence of 2 per 100,000 children less than 15 years , with onRare autoimmune inflammatory process involving the pituitary gland confirmed by biopsy showing varying degrees of lymphocytic infiltration and plasma cells with fibrotic changes without multinucleated giant cells. They are more prevalent in women (80 %) who are in late pregnancy or postpartum but can also occur in children. Clinical findings include headaches and pituitary hormonal dysfunction, with deficiency of ACTH in adults and growth hormone in children . LesionsA rare benign histiocytosis, also referred to as sinus histiocytosis with massive lymphadenopathy. It usually occurs in children and young adults, with a male-female ratio of 3:2 . PatientPrimary CNS lymphoma is extremely rare in children, and usually occurs as intra-axial lesions and infrequently as leptomeningeal tumour. Most cases are non-Hodgkin, high-grade B-cell lymphomas. There is a strong link in patients with AIDS or primary immunodeficiency . LeptomeNumerous vascular malformations can be seen in the sellar/juxtasellar regions, including arterial aneurysms , cavernous carotid fistulas, arterial-venous malformations, haemangiomas or cavernous malformations. Cavernous malformations are the most common CNS vascular lesions in children, although the incidence is lower than in adults . CompareImaging of the sellar and juxtasellar region in the paediatric population is ideally performed with MRI, and relies on a strong fundamental knowledge of the anatomy and signal characteristics of a normal gland and surrounding structures. The knowledge of key differentiating MRI characteristics in common and uncommon disease entities of the paediatric sellar and juxtasellar regions, in conjunction with the clinical findings, undoubtedly aids in alerting and guiding the clinician down the appropriate path of management. It is best to approach a suspected sellar or juxtasellar abnormality with an open mind and a broad differential, with MRI being the “gold standard” of imaging to accurately characterise these lesions.


Aggressive angiomyxoma (AA) is an uncommon mesenchymal tumor that is mostly derived from the female pelvic and perineal regions. AA is a locally infiltrative slow growing tumor with a marked tendency to local recurrence. Painless swelling located around the genitofemoral region is the common symptom; thus, it is often misdiagnosed as a gynecological malignancy or a groin hernia. A 35-year-old female patient who previously underwent surgery for left femoral hernia operation resulting in surgical failure was reoperated for a giant AA located in the pelvis. The tumor was completely excised with free margins. Histopathologic examination revealed an AA. The tumor size was measured as 24 × 12 × 6 cm with a weight of 4.2 kg. Immunohistochemically, the cells show positive staining with vimentin, desmin, estrogen, and progesterone receptor. S100, MUC4, CD34, and SMA were negative in the tumor cells. AA should be considered in the differential diagnosis of any painless swelling located in the genitofemoral region, particularly in women of reproductive age. The principle treatment should be complete surgical excision with tumor-free margins. Long-term follow-up and careful monitoring are essential due to its high tendency of local recurrence in spite of wide excision of the tumor. Adjuvant antihormonal therapy yields promising results for preventing recurrence. Aggressive angiomyxoma (AA) is an uncommon mesenchymal tumor which is predominantly encountered among adult females in reproductive age . The tumSince AA was first described in 1983 by Steeper and Rosai , approxiA 35-year-old nulliparous female patient with a previous history of left femoral hernia operation was admitted to our hospital. In her medical history, the patient declared that she had a large painless swelling extending from her left groin to her left leg which grew gradually during one year. No gastrointestinal symptoms were determined. She was initially diagnosed as a femoral hernia and underwent surgery one month ago at another state hospital. Accordingly, she was referred to our clinic because of surgical failure. On physical examination, she had a left groin incision scar. An immobile, painless mass which filled lower quadrants of the abdomen was palpated. The mass was also extended to the one-third upper level of left thigh. Digital examination of the rectum was normal. Vulva was examined macroscopically because the patient was virgin. Subsequently, abdominal ultrasonography (USG), contrast-enhanced abdominal tomography (CT), and magnetic resonance imaging (MRI) were performed for estimating the tumor size, invasion degree of the mass, distant metastasis, and also ruling out other intra-abdominal lesions. A giant intra-abdominal solid mass that is fully filling the pelvis and extending to the left groin was revealed by USG, CT, and MRI . All adjAA is an uncommon mesenchymal tumor which is mostly derived from the pelvic and perineal regions including vulva, vagina, bladder, and rectum , 6, 8. HThe pathogenesis of AA is not well understood. There is only one study evaluating the pathogenesis of AA in the current literature in which Nucci and Fletcher  suggesteThe diagnosis of AA is very difficult because it is often asymptomatic until the tumor reaches large sizes. Urinary, gynecologic, and gastrointestinal symptoms like dysuria, dysmenorrhoea, constipation, and chronic abdominal/pelvic pain occur when the tumor begins to depress the adjacent organs including bladder, rectum, ureter, and uterus. AA commonly manifests a painless swelling located around the genitofemoral region. For this reason, it is often misdiagnosed as a vulvar abscess, Bartholin's gland cyst, vaginal prolapse, gynecologic malignancy, and groin/femoral hernia that may lead to unnecessary surgical interventions, as in our case , 17. In The current treatment of AA is complete surgical excision with tumor-free margins , 6, 8. HAll adjuvant treatment modalities remain controversial . ChemothAlthough the majority of the authors have reported no advantage in using radiotherapy, it can be a good alternative treatment in patients who are resistant to antihormonal therapy, those with recurrence or in whom tumor resection would cause high morbidity. Rhomberg et al.  and SuleDespite its rarity, AA should be considered in the differential diagnosis of any painless swelling located in the genitofemoral region, particularly in women of reproductive age. The diagnosis should be confirmed by CT and/or MRI. The principle treatment should be complete surgical excision with tumor-free margins. The patient should be informed about the high morbidity of the surgical intervention. Long-term follow-up and careful monitoring are essential due to its high tendency of local recurrence in spite of wide excision of the tumor. Adjuvant antihormonal therapy yields promising results for preventing recurrence. However, long-term use of these drugs is still controversial because of their adverse effects.