In central nervous system, schwannomas, as ubiquitous tumors, mostly originate from sensory nerves like auditory and trigeminal nerves. However, intrasellar schwannomas are extremely rare. They are often misdiagnosed as pituitary adenomas.We report a rare case of schwannoma in the sellar region—a challenging diagnosis guided by clinical presentations, radiological signs, and postoperative pathological test.We represent a 65-year-old woman who had suffered from headaches, hypothyroidism, and visual disturbance. Her MRI revealed an abnormal sellar region mixed-signal mass lesion with suprasellar, left parasellar, and sellar floor invasiveness. We present detailed analysis of the patient's disease course and review relevant literatures. Written informed consent was obtained from the patient for publication of this article. A copy of the written consent is available for review by the editors of MEDICINE. Because this article does not involve any human or animal trials, there is no need to conduct special ethic review and the ethical approval is not necessary.When surgically treated, her specimen revealed a typical histopathology pattern of schwannoma. The patient's symptoms improved a lot after surgery and he continues to be under observation.Despite its rarity, intrasellar schwannoma should be considered in the differential diagnosis of sellar lesions that mimic pituitary adenomas. Here, we present an unusual case of schwannoma in the sellar region in a patient who presented with symptoms of headache, hypothyroidism, and visual disturbance. On MRI, the mass was signal-mixed with suprasellar, left parasellar, and sellar floor invasiveness, initially indistinguishable from an invasive pituitary macroadenoma.Schwannomas mostly originate from sensory nerves like auditory and trigeminal nerves in the nervous system, and account for 8% to 10% of all primary intracranial tumors. However, they are uncommon in the pituitary sellar region. The most common tumor type in the pituitary fossa and parasellar area is the pituitary adenoma, whereas schwannomas are among the rarest. In fact, only 24 cases of intrasellar schwannoma have been reported in the literature up to now.A 65-year-old woman came to PUMCH with headaches, fatigue, feebleness and depression, and visual disturbance of 4 months’ duration, which had worsen since the previous 2 weeks. She denied polydipsia, polyuria, sexual hypoactivity, or any symptoms of unconsciousness, epilepsy, convulsion, and cognitive disorders. Physical examinations revealed that her left visual acuity was 0.8 and the right was 0.6. Goldmann perimetry revealed a temporal hemianopia for the left eye and some severe temporal scotomas for the right eye. Other neurological examination results were normal. Her history was negative for head trauma. His social and family history and his system review were negative.Her MRI revealed an abnormal sellar region mixed-signal mass lesion with suprasellar, left parasellar, and sellar floor invasiveness Figure . The lesAs the patient's fatigue, weakness, and depression were most likely caused by anterior pituitary function damage, or more specifically a hypothyroidism, we performed a series of endocrine tests Table , which iShe then underwent a surgical exploration through a transsphenoidal approach. After drilling the sellar floor and opening the dura, a firm, tough, wheaten mass was found. As its consistency was too rubbery to be easily cut by a surgical blade, and it adhered so tightly to the left cavernous sinus and carotid, only subtotal resection was ultimately achieved. Some intraoperative photographs of the tumor during surgery are seen in Figure Against all expectations, a micrograph of the surgical specimen recommended its diagnosis as a schwannoma, revealing multiple portions of stromal-type tissue consisting of spindle-shaped cells with eosinophilic wavy cytoplasm and elongated nuclei. The Ki-67 proliferation index was approximately 2%. Immunohistochemical staining was positive for vimentin protein; immunohistochemistry was negative for human epithelial membrane antigen and S-100 protein. Together, these findings confirmed the schwannoma diagnosis Figure .The patient reported no side effects of surgery and was discharged from hospital 8 days afterwards in good clinical condition. The postoperative period was uneventful. An MRI of the sellar region 3 months after surgery showed subtotal resection of the tumor Figure . Her heaPituitary adenomas comprise the overwhelming majority of primary tumors in sellar region. The remaining neoplasms include other tumors of pituitary origin , tumors of nonpituitary origin , and other uncommon conditions .3 The origin of primary schwannomas in the sellar region is still unclear. As the sellar region has no obvious nerve, some authors hypothesized that tumors originate from lateral sellar nerve plexus, perivascular schwann cells adjacent to the medial wall of the pituitary fossa, or sensory nerves of the dura.2 Studies to elucidate the histopathogenesis of these neoplasms are warranted.Usually, differential diagnoses of sellar, parasellar, and suprasellar lesions include pituitary adenomas, craniopharyngiomas, meningiomas, and cholesteatomas alike. Schwannomas, however, are not commonly included, because of their exceeding rarity in this region, although they represent approximately 8% of all intracranial neoplasms.2 Few cases of real primary intrasellar schwannomas have been reported previously. Recently, Sharifi et al performed a literature review of intrasellar schwannomas reported before, all of which presented a suprasellar or parasellar extension similar to that of the present case.1Intracranial schwannomas can occur sporadically or present as manifestations of central neurofibromatosis (type 2), which are most often found in young males.4 Lateral expansion into cavernous sinus may compress oculomotor, trochlear, and/or abducent nerves, leading to diplopia and then ocular misalignment. Suprasellar expansion often presents with visual disturbances because of compression of the optic nerve or chiasm; similar to our patient, who developed headaches, and bitemporal hemianopia. When the schwannoma compresses the normal pituitary gland, pituitary hormone disorders may follow. Interestingly, most of these cases showed hypopituitarism.4Table 13 In our case, the pituitary dysfunction was hypothyroidism and hyperprolactinemia, probably occurring as a result of the compression against the hypothalamic-pituitary stalk. To our knowledge, this is the first intrasellar schwannoma case with such a hormone disorder.Generally, the anatomical location determines the clinical features of intrasellar schwannomas. Those located within the sella turcica typically present with symptoms similar to pituitary adenomas.1 the MRI findings of a intrasellar schwannoma showed the lesion was hard calcified. In our case, the MRI signals were quite mixed, indicating the internal components were complex and various.As with pituitary adenomas, intrasellar schwannomas often present as a mass lesion with an iso-to-hypointense signal on T1WI with homogeneous contrast enhancement; whereas on T2WI, they commonly show a more hyperintense signal than pituitary adenomas. In a study conducted by Sharifi et al,14 The radiological features dictate the surgical approach by revealing the location and extent of the tumor spread. A microscopic transsphenoidal approach is highly recommended, in that it renders good visualization, facilitates complete or near-complete removal of the tumor possible, and can be technically simpler if the mass has already widened and invaded sinus walls without high morbidity.4 Correct diagnosis is usually established by histopathological and immunohistochemical examination of surgical specimens15; most reported intrasellar schwannomas were initially misdiagnosed as pituitary adenomas or other tumor types before surgery.16 Although none of the previous reported cases underwent second surgeries for remnant or recurrent tumors, adjuvant treatment such as radiotherapy can be considered.The definitive treatment for intrasellar schwannoma is surgical excision, particularly for those with atypical radiological and clinical manifestations and hard-to-confirm diagnoses.Although intrasellar schwannomas are not common, they should be considered in diagnosing sellar lesions that mimic pituitary adenomas or meningiomas, especially when the final diagnosis of these rare sellar masses relies on pathology and immunohistochemistry results.


Colloid cysts are rare congenital benign tumors accounting for 0.2-2% of all intracranial neoplasms. They usually occur in the front part of the third ventricle. The clinical presentation is related to the increased intracranial pressure and is widely variable; sudden death associated with acute hydrocephalus can occur, therefore recognition of this rare condition is important in order to select an appropriate surgical treatment. We report a case of new onset headache secondary to a colloid cyst of the third ventricle.A 44-year-old woman with unremarkable medical history was admitted to our clinic for recurrent attacks of pressing headache, with abrupt onset and brief duration, accompanied by nausea, transient hearing loss and tinnitus. These symptoms were relieved by supine position. The clinical picture progressively worsened, with episodes of vomiting during headache. Neurological examination was negative. A magnetic resonance imaging (MRI) scan showed a spherical mass lesion with lipid signal at the intraventricular foramina of Monro causing compression of the third ventricle and expansion of the ventricular system, suggestive of hydrocephalus.After neurosurgical evaluation the patient underwent an endoscopic removal of the lesion. Histological findings were compatible with colloid cyst of the third ventricle. After surgical treatment the patient recovered completely from symptoms and the follow-up MRI demonstrated the complete excision of the lesion.Colloid cysts are congenital, slow growing, benign intraventricular lesions usually arising in the third ventricle. They may cause obstruction of the foramen of Monro with blockage of cerebrospinal fluid (CSF) flow, producing progressive or intermittent elevated intracranial pressure with chronic or acute hydrocephalus . The onsOur case highlights the importance of early detection and prompt treatment of this potentially life-threatening cause of headache.Written informed consent to publish was obtained from the patient(s).


Due to episodic epigastric pain this 32-year-old woman had undergone computed tomography (CT) 20 months previously. Contrast enhanced CT showed a hypodense lesion between liver segments IV and VIII with a diameter of 4 cm and inhomogenous early enhancement suggesting hemangioma. Eight more lesions (diameter up to 1 cm) that were only visible in the early arterial phase were found in segments VI and III. Magnetic resonance imaging (MRI) 13 and 8 months before admission showed slight progression (from a diameter of 4.0 to 4.6 cm) of the lesion in segment IV. At that time, the lesion appeared to be lobulated with a central hyperintense scar and arterial enhancement, primarily compatible with “atypical” focal nodular hyperplasia (FNH). Except for occasional abdominal pain and a 10-year history of histamine intolerance, the patient was free of symptoms. She had taken thyroid replacement therapy (Euthyrox® 100 µg per day) for years; she had no previous surgery, had never received a blood transfusion, and was not vaccinated against hepatitis A or B; she neither smoked nor drank and her family history was unremarkable. The patient is a single parent of a healthy 10-year-old boy. Physical examination was unremarkable; she weighed 56 kg and her height was 174 cm. Except for lactate dehydrogenase  routine laboratory tests were negative. A panel of antibodies to detect autoimmune and collagen vascular diseases was negative; thyroid stimulating hormone (TSH) was 1.3 µU/ml .During a follow-up exam in the outpatient liver clinic, she showed a facial flush that lasted for 2 min, although the situation was not psychologically upsetting. She said facial flushes are part of her histamine intolerance. A diagnostic test was performed, and she was admitted to the hospital for further management.Abdominal multidetector CT revealed a 4.0 × 3.3 cm, well circumscribed, heterogenous, hypodense lesion in liver segments VIII/IV with significant contrast enhancement during the arterial and portal venous phases and with contrast washout during delayed phases Fig. . Eight mTaken together, the MRI studies showed hypervascular lesions in both lobes of the liver compatible with several diagnoses. The hyperintensity of the larger lesion and the early arterial contrast enhancement also seen in the smaller lesions are typical for hemangiomas. Moreover, upon MRI hemangiomas show a quite homogenous portal venous contrast enhancement due to accumulation of contrast medium during the examination. So in this case, MRI suggests a hemangioma for several reasons. On the other hand, the central hyperintense scar found in one of the lesions could be characteristic for FNH; however, the MRI findings do not clearly allow a definite diagnosis.The patient under discussion is a 32-year-old woman with nodules in the liver. Except for occasional abdominal pain and histamine intolerance, she is asymptomatic. Twenty months earlier, CT of the liver showed what was first thought to be a hemangioma and later interpreted as an “atypical” FNH. Further MRIs revealed progression of the lesion (from a diameter of 4.0 to 4.6 cm) and identified eight additional lesions (diameter up to 1 cm) in the liver. Facial flushes were thought by the patient to be due to histamine intolerance diagnosed 10 years before. Physical examination did not show any abnormality and, except for LDH, routine laboratory tests were within normal limits.echinococcus; however, the MRI lesions are not typical for echinococcal cysts [Bartonella henselae due to contact with cats and a resulting bacillary peliosis hepatis could be considered, but this disease predominantly occurs in immunosuppressed patients, is associated with fever, and runs a much shorter course. As to the episodic abdominal pain, a regular recurrence of symptoms could parallel the menstrual cycle, but endometriosis would not really explain the liver lesions. Since the panel of antibodies for autoimmune and collagen vascular diseases was negative, an autoimmune disease can alsobe ruled out. The patient said she was histamine intolerant, but unfortunately no further information was available as to how this diagnosis was established 10 years earlier (genetic variant? activity of diaminooxidase?). FNH could still be considered as a possible diagnosis, but it would not have required prompt admission to the hospital. The assumed diagnosis of a liver hemangioma based on the CT scan, which showed a hypodense lesion with inhomogenous early arterial contrast enhancement, appears unlikely since the lesion had progressed in size.As the patient is a native of Austria, hepatocellular carcinoma (HCC) in a noncirrhotic liver due to chronic hepatitis B is unlikely. Since hepatitis serology is routinely tested during pregnancy in this country, an infection would have been revealed much earlier when the patient had been pregnant. It is unclear whether the patient had consumed foods such as wild berries or raw vegetables and so might have been infected with al cysts . An infe99mtechnetium octreotid scintigraphy and 68gallium-DOTATATE (= DOTADOC) PET-CT are parts of the further workup.For my differential diagnosis, I have to address the facial flush presumed to be due to histamine intolerance. Flush symptoms can be caused by enhanced release of vasoactive substances such as serotonin and bradykinin, mostly due to a gastroenteropancreatic neuroendocrine tumor (GEP-NET). Frequently, flush symptoms in GEP-NETs occur in the setting of liver metastases. This is because most of the bioactive substances that are released by a GEP-NET may be metabolized by the liver on first pass from the splanchnic area and, more importantly, liver metastases provide for more tissue for production and release of bioactive substances into the circulation. Laboratory tests showed elevated serum LDH, with an increased ratio of LDH/AST, a possible marker for the presence of liver metastases. An important diagnostic test that could suggest a GEP-NET is the analysis of serum chromogranin A. Chromogranin A is a protein found in the secretory granules of neuroendocrine cells, and its concentration correlates with tumor mass . FurtherGEP-NET with liver metastases; clinically “carcinoid syndrome.”This patient does indeed have a gut neuroendocrine tumor (NET). Further history revealed that flush symptoms as noticed in the outpatient liver clinic occur 10 to 50 times per day. The patient complained of bloating but denied diarrhea. The following laboratory results were obtained: serum chromogranin A 1061 ng/ml , serum serotonin 2063 ng/ml , and urinary 5-HIAA 118 mg/24h . For further staging, MRI of the small intestine and the liver was performed.Just a short remark—the patient was admitted immediately so that somatostatin analog therapy could begin without delay.Some epidemiological facts: Although previously regarded as rare, GEP-NETs represent the second most common digestive malignancy after adenocarcinomas , 4. Base111In-DTPA-octreotide (OctreoScan™) and 99mTc-tektrotyde®, or a positron emitter  such as 68Ga-octreotide, 68Ga-DOTA-TOC, and 64Cu-DOTA-TATE is used.NET cells express somatostatin receptors (SSR) which are the target for radionuclear tracers during somatostatin-receptor scintigraphy (SSRS). For SSRS, a gamma emitter  such as 18Fluro-DOPA PET/CT is another radionuclear method to assess the metabolic activity of GEP-NET cells independent of the SSR status. In NET cells the activity of DOPA-decarboxylase that decarboxylates the aminprecursor DOPA to a biogenic amin is increased [18fluro-DOPA differently. In one third of GEP-NET patients this investigation provides pivotal and therapeutically important information that cannot be obtained by other morphologic and functional imaging methods [ncreased –11. Depe methods .18fluro-DOPA PET/CT  , 15.MRI has a higher sensitivity for identifying the primary tumor. Tumors usually have low signal intensity on T1-weighted sequences (75 %) and high signal intensity on T2-weighted sequences (94 %), being hypervascular on arterial post-gadolinium images: 15 % of liver metastases were only seen on the immediate post-gadolinium images. The tumors are most conspicuous on fat-suppressed T1-weighted images . Both CTAfter the GEP-NET had been diagnosed the patient was immediately treated with somatostatin analog (SSA) and 3 months later underwent tumor debulking, i.e. resection of the primary tumor and liver metastases. Dr. Kornprat was the surgeon and will explain and comment on the treatment.Surgical resection of liver metastases is known to have a positive effect on overall survival and quality of life due to alleviation of symptoms related to secretion of serotonin or other mediators in functioning tumors . SarmienWhen NETs are associated with endocrine syndromes, debulking surgery is attempted whenever feasible. Debulking procedures include not only resection of the primary tumor, liver metastases, and lymph nodes but also ablative therapies that remove > 90 % of the tumor , 18–20. A two-step surgical approach was chosen for this patient: First, a 75-cm-long segment of the lower small bowel with adjacent mesenteric nodes was resected. There were no intra- or postoperative complications.Depending on the number and localization of the metastases, up to 65–70 % of the whole liver volume  can be removed surgically , 24, andMacroscopically, the resected segment of ileum was 75 cm long and contained three tumors of 2, 1.5, and 3 cm in diameter. The tumors were 1.5 and 6 cm apart and were located 20 cm from both, the oral and aboral margin of resection. All three tumors extended beyond the mucosa into the small bowel wall. The serosa was intact. The mesentery showed another mass of 5.0 × 3.5 × 3.0 cm that extended to the resection margin and five lymph nodes with diameters of up to 2 cm.Microscopically, all three tumors found in the resected ileum and the tumor in the mesentery could be identified morphologically and immunohistochemically as NETs Fig. . They weSystems of nomenclature reflect differentiation and grading features of NETs Table . GeneralThe proliferative rate of a NET provides significant prognostic information and can be assessed as the number of mitoses per unit area of tumor  or as the percentage of neoplastic cells immunolabeling for the proliferation marker Ki67 , 27.In this patient, fewer than two mitoses/10 HPF and a Ki67 index ≤ 2 % were found, corresponding to NET grade 1 (NET G1). The tumor in the mesentery extended to less than 1 mm from the resection margin, so that there was some uncertainty as to whether the tumor had been completely resected. There were neural sheath and venous invasions at multiple sites in the area of the mesenteric root. Moreover, a metastasis was found in one of the five lymph nodes in the resected mesentery. The tumor cells were immunohistochemically positive, with antibodies against chromogranin and synaptophysin. Some of the tumor cells reacted with antibodies against serotonin but not with those against gastrin, adrenocorticotropin, somatostatin, and pancreatic polypeptide.The liver specimen measured 17.0 × 10.5 × 5.0 cm. A tumor of 5.5 × 4.0 × 4.0 cm extending to the resection margin was found on the cut surface, which on histology proved to represent a liver metastasis of the NET described above.According to the UICC 2009  the tumoThe prognosis for jejunoileal NETs depends on tumor size and the extent of tumor infiltration, the grade of differentiation, and the presence of metastases. With liver metastases, the 5- and 10-year survival rates amount to 35 and 15 %; without liver metastases the rates increase to 72 and 60 %, respectively .The term NET was based on the hypothesis that the cells of neuroendocrine neoplasms originate from the embryonic neural crest. Based on findings that most of these neoplastic cells resemble cells of endodermal origin, this concept was discarded years ago . HoweverG protein-coupled receptors (GPRs) in NETs have been studied extensively. Since stimulation or inhibition of such receptors can influence tumor growth, a number of GRPs have been found to be excellent targets for GEP-NET diagnosis and treatment . SomatosA Randomized Double-Blind Placebo-Controlled Study of Lanreotide Antiproliferative Response in Patients with Gastroenteropancreatic Neuroendocrine Tumors) study, lanreotide (Somatuline Autogel®) significantly prolongs the progression-free survival (PFS) of patients with GEP-NETs . The antiproliferative effect of lanreotide is statistically significant for patients with midgut NET and clinically relevant for those with pancreatic NET. As treatment with lanreotide also prolongs the PFS of GEP-NET patients with high-grade tumors  and higher hepatic tumor burden (> 25 %) [After resection of the primary tumor, subsequent extended hemihepatectomy and continued lanreotide treatment, chromogranin A levels further decreased to 71 ng/ml. Due to vertigo and hypotension as side effects, the dosage of lanreotide had to be reduced from 120 mg/month to 90 mg/month. One year after surgery, staging revealed micrometastases in the remaining liver on PET scan, but they were not detectable by MRI; the disease state so was classified as “stable.” The patient has no more flushes, and urinary 5-HIAA is not elevated. According to the CLARINET ((> 25 %) , treatmeWhich further or future therapeutic options could be considered for this patient? Could liver transplantation be an option?According to the ENETS Guidelines , SSA theLiver transplantation may be a therapeutic option for patients with functioning NETs refractory to medical therapy that can lead to life-threatening hormonal disturbances, or for patients with nonfunctioning tumors with diffuse unresectable liver metastases refractory to all other possible treatments . ConsideThis case clearly shows that initial imaging studies can sometimes be misleading and that facial flush is not necessarily due to histamine intolerance. In a recent case report, a patient had been followed for a serous cyst in the liver for many years before frequent flushing developed and a NET was diagnosed . On the Patients should always be examined very carefully even if the primary assessment suggests such harmless conditions as hemangioma of the liver or histamine intolerance. A history of flushes should lead the physician to rule out a dangerous cause of flush, such as malignant “carcinoid syndrome” (metastatic NET).Three neuroendocrine tumors of the ileum  with metastases to the mesenteric lymph nodes and the liver.


Orocutaneous fistula (OCF)  is an uncommon but well-described condition in the literature. These are often misdiagnosed by physicians and dentists. Careful selection of investigating modality is important in case of diagnostically challenging cases. A 19-year-old female came with a complaint of a lesion on the chin reported with h/o trauma with the impact on chin presented as diagnostic dilemma because of unusual case history and clinical examination. Commonly used radiographic investigations like IOPA and orthopantomograph did not resolve the dilemma whereas advanced imaging modality like CT scan, 3D volume imaging, and contrast enhanced CT played an important role in the diagnosis of OCF and selecting the treatment plan. Orocutaneous fistula (OCF)  is an uncommon but well-described condition in the literature. Diagnosis of OCF is still challenging and should be done with careful history taking, clinical examination, and radiographic examination. Such conditions are often misdiagnosed by physicians and dentists as skin lesions or nonodontogenic lesion. Therefore, accurate diagnosis and use of correct investigating modality are important for the diagnosis of OCF  for prompt treatment and reducing complication such as asepsis, osteomyelitis, and patient discomfort. This case describes the patient with lesion on the chin presented as diagnostic dilemma because of unusual history and clinical presentation which resulted in confusion whether the lesion is because of dental origin or a separate entity. This case report also describes the importance of CT scan, 3D volume imaging, and contrast enhanced CT in the diagnosis of OCF and in visualizing the communication between the periapical region and the extraoral lesion. This imaging technique might be very helpful to improve the diagnosis and management of orocutaneous fistula.A healthy 19-year-old female came to the department of oral and maxillofacial surgery with a chief complaint of pus oozing from small opening above the chin since past 1 month. Past medical history revealed h/o trauma by falling 4 yrs back. Since then the extraoral lesion was present. Patient was unable to bite from lower front teeth since 1 month. Pain was dull, continuous, and nonradiating, which aggravates on mastication and relieves on taking analgesics.The patient stated that the lesion had been a frequent discharge of purulent material , but theOn radiographic examination, IOPA  was takeOn CT scan , involveTerm orocutaneous fistula is misleading especially if it is of dental origin because there is no direct contact of oral cavity and skin. We suggest the use of terms like odontocutaneous, dentocutaneous, or apicocutaneous fistula for OCF of dental origin. An extra oral opening or cutaneous sinus tract may be confused with wide variety of diseases including local skin infections, in grow hair or occluding sweat gland duct, osteomyelitis, neoplasm, tuberculosis, actinomycosis, congenital midline sinus of upper lip –8, congeRadiological examination plays an important role in diagnosis of OCF. IOPA and OPG are the frequently ordered radiographs for the diagnosis of OCF. Odontogenic cutaneous sinus tract on the face often develops as a result of chronic apical periodontitis caused by pulpal degeneration or necrosis . PeriapiA case of OCF is reported which presented as a diagnostic dilemma which was resolved with the help of advanced radiographic investigations. All orocutaneous fistula must be considered of dental origin. The diagnosis of orocutaneous fistula must be as quick as possible to improve the clinical outcome. Therefore, early and accurate diagnosis demands that the lesion should be investigated with advanced imaging modalities like CT scan, 3-dimensional volume imaging, and CECT. Hence, in case of confusion, a clinician should ask for advanced imaging modalities like CT scan, 3-dimensional volume imaging, and CECT rather than depending upon imagination and conceptualization of the pathology.


A 57-year-old woman with a previous history of aesthetic surgery for breast reduction presented with a subcutaneous mass in the right axilla. A CT scan showed a solid mass on the chest wall, and she underwent surgical resection with a diagnosis of aggressive fibromatosis. After a 10-month period of follow-up, a local recurrence occurred, and in accordance with the up-to-date approach, the recurrence has been treated with a conservative approach  with good control of the symptoms and downsizing of the lesion. Aggressive fibromatosis (AF), also known as desmoid tumour or musculoaponeurotic fibromatosis, is a monoclonal fibroblastic proliferative disease . The tumAvailable treatments include surgery, radiotherapy, systemic medical treatment, hormone therapy, anti-inflammatory drugs, and tyrosine kinase inhibitors. Recently, it has been demonstrated that an initial aggressive approach (surgery and/or radiotherapy) might overtreat 50% of the patients .A 57-year-old woman with a previous history of aesthetic surgery for breast reduction presented with a subcutaneous mass in the right axilla. The patient underwent a computed tomography (CT) scan, showing a contrast-enhanced solid mass in the right axilla, with a total extra-pleural growth . A positThe patient underwent a wide soft-tissue surgical resection including major and minor pectoralis muscles, partial resection of the serratus anterior, and of the right breast upper external quadrant. The macroscopic pathological report described a white mass with a hard consistency and irregular margins, and the final histological diagnosis was extra-abdominal AF, with free resection margins, and an immune-phenotype positive for beta-catenin.A follow-up CT scan performed 10 months after surgery disclosed local recurrence infiltrating the intercostal muscles, the pleura, and the pectoralis muscles . SubsequBased on the new strategies of approach, a treatment based on the use of tamoxifen and anti-inflammatory drugs, along with a gastric protection, was indicated for the patient. The therapy prescribed included tamoxifen 20 mg/diem and celecoxib 200 mg/diem (anti-inflammatory COX-2 inhibitor), with good control of the symptoms and  downsizing of the lesion after 12 months of treatment, as demonstrated by CT scan .AF (or desmoid tumour) is an uncommon mesenchymal tumour with a fibrotic band-like consistency, characterised by excessive proliferation of fibroblast-like cells .The incidence of fibromatosis in the general population is 2–4 cases/million per year, with a slight female preponderance and peak incidences in the 3rd-4th decades of life  .AF can be intra-abdominal or extra-abdominal, being located in the chest wall 8–10% of all the extra-abdominal sites .Before 2000, radical surgical resection was considered to be the standard treatment because complete excision with negative margins was considered to be the primary goal, such as indicated for sarcomas.However, nowadays, amputation is no longer considered the most appropriate treatment in locally advanced AF, because the consequences of radical excision can be worse than the disease itself. Moreover, the fact that some tumours recur after surgery, but then remain stable without treatment, suggests that growth factors released following surgery may promote recurrence in tumours that would be otherwise indolent.A recently proposed treatment algorithm for AF  begins wValid current alternative treatments to surgery and radiotherapy are the anti-oestrogen and anthracycline-containing regimen  of tamoxIn our case, surgical resection was performed because of unclear histology at biopsy; at the time of local recurrence, re-do surgery was avoided, and according to the reported advances in knowledge of therapies for AF, the patient has been treated with tamoxifen and celecoxib. We saw initial promising results with control of pain and partial reduction of the lesion after 12 months of treatment.In conclusion, the current treatment for AF should include medical treatments alternative to surgery and radiotherapy, as they are able to ensure good control of symptoms and reduce the possibility of postoperative complications.The authors have no conflicts of interest to declare.


Lobular Capillary Hemangioma (LCH) is a benign tumour that is known to be hormone responsive and have a relatively high incidence during pregnancy, the most common site being the gingival surfaces. A tracheal origin for this tumour is extremely rare, with no case reported so far in this patient population, and the only reported clinical presentation of tracheal LCH in the literature is with haemoptysis.We describe a case of a 23-year-old known asthmatic who presented at 32 weeks gestation with life-threatening respiratory failure resembling acute severe asthma, requiring invasive ventilation which was extremely difficult. This was subsequently found to be due to a large tracheal LCH producing a ball-valve phenomenon and predominantly expiratory airflow limitation similar to acute asthma. The endotracheal tube was advanced past the lesion under bronchoscopic guidance, and urgent Caesarean section performed due to foetal distress. The tumour was subsequently debulked and the trachea stented, facilitated by bi-femoral veno-venous extra-corporeal membrane oxygenation with relatively low dose of heparin.To our knowledge, this is the first report of a unique presentation and management of largest tracheal LCH so far occurring during pregnancy. Pulmonary and critical care physicians should be aware of this unique differential of refractory asthma, the aggressive nature of this benign tumour due to hormonal influences during pregnancy, and feasibility of using bi-femoral veno-venous extra-corporeal membrane oxygenation with low dose heparin as a rescue, given the high risk of bleeding. Primary tracheal tumours are rare with an estimated incidence of about 2.7 new cases per million per year and are usually malignant in adults . LobularA 32 weeks pregnant, 23-year-old known asthmatic woman presented to a peripheral hospital with acute respiratory failure during winter. Cold air was a known precipitant for her asthma. She was intubated and ventilated for presumed severe acute asthma with refractory bronchospasm. Following intubation she was found to be extremely difficult to ventilate and was retrieved to our intensive care unit for further management.2O, respiratory rate six breaths per minute, a tidal volume of 300 ml, and inspiratory flow rate of 30 L/min. However, high peak airway pressures truncated each breath. Intrinsic PEEP was measured as 30 cmH2O, with evidence of dynamic hyperinflation and accompanying hemodynamic instability despite inspiratory to expiratory ratio of close to 1:16. An arterial blood gas sample revealed severe respiratory acidosis . There was evidence of foetal distress on Cardiotocograph (CTG). Bronchoscopy demonstrated a pedunculated tumour arising from the posterior tracheal wall, extending from the tip of the Endotracheal Tube (ET) and measuring approximately 4 cm by 2 cm. The tumour acted as a ball valve to cause severe expiratory airflow obstruction  0 cmH2 < 80%). She was promptly commenced on ECMO with recovery of arterial oxygen saturation and minimal disruption to surgery. A small bolus of heparin (2000 units) was given intravenously just prior to connection of the ECMO circuit, but no further heparin was administered until after surgery. On return to the ICU, a low dose heparin infusion (10 units/kg/hour) was used to maintain ECMO circuit patency. The histopathology revealed recurrent LCH  was established via a bi-femoral approach. Surgical debulking was complicated by significant haemorrhage causing desaturation SpO < 80%. SObstructing airway lesions are recognised in the differential diagnosis of refractory asthma. Primary tracheal tumours are rare with an estimated incidence of about 2.7 new cases per million per year and are usually malignant in adults . AmongstMicroscopically, LCH has a distinctive lobular arrangement of capillaries of varying calibre embedded in a fibromyxoid matrix without atypical mitoses . Conventin situ.The pathogenesis of this lesion is currently uncertain. Most theories on pathogenesis revolve around LCH as a hyperplastic, neovascular response to an angiogenic stimulus with imbalance of promoters and inhibitors . AngiogeThe gingival surface is the most common site of LCH during pregnancy, with no cases of tracheal LCH reported during pregnancy to date. None of the case reports have clearly revealed the recurrent nature and rate of growth of tracheal LCH as they were already large, symptomatic, and requiring treatment at the time of diagnosis. The current knowledge about these features of this tumour mainly originates from literature on extra-tracheal sites of LCH, particularly the skin. LCH rarely exceeds 2.5 cm in size and it usually reaches its full size within weeks or months, remaining static thereafter . The LCHThe mainstay of treatment, if symptomatic, is surgical debulking or excision using various methods -4,12. ThHere we describe a rare differential of refractory asthma - the largest LCH tumour reported to date, occurring at an exceedingly rare site and with rapid growth during pregnancy, possibly due to hormonal influence. Pulmonary and critical care physicians should be aware of this unique differential of refractory asthma, the association with pregnancy, and the feasibility of using bi-femoral veno-venous ECMO with low-dose heparin for its management.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.CTG: Cardiotocograph; ET: Endotracheal tube; ECMO: Extra-corporeal membrane oxygenation; LCH: Lobular capillary hemangioma; PEEP: Positive end expiratory pressure.The authors declare that they have no competing interests.SP and SB analysed and interpreted the patient’s data and drafted the manuscript. UW revised the clinical data and supervised the case report. All authors read and approved the final manuscript.SP and SB have MD in pulmonary medicine and currently hold Australian fellowship in intensive care. Their area of interest includes critical care aspects of pulmonary medicine. UW is a senior consultant in intensive care. He has expertise in the area of ECMO and has been the director of ECMO services at Flinders Medical Centre Intensive care.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2466/14/41/prepubVideo 1. Bronchoscopic view at the edge of endotracheal tube. A highly vascular polypoidal lesion is seen, which causes a ball-valve phenomenon during respiration to generate severe compromise of expiratory airflow.Click here for file


Synovial sarcoma (SS) is a high-grade, rare variant of soft tissue sarcoma (STS). The biphasic subtype is less common than the monophasic subtype. SS is very common around joint cavities in the extremities, but can be present elsewhere in the body. Tumor staging and therapeutic management are usually clear for a localized disease, but the proper management at the metastatic stage can be unclear. According to the literature, the histologic presence of an SS tumor thrombus affects tumor staging, making it unclear whether the tumor stage corresponds to localized or metastatic disease. An intravascular SS tumor exhibiting high metastatic potential is a rare finding that warrants thorough investigation.A 49-year-old woman presented with a biphasic SS intravascular tumor of the left inguinal area with femoral vessels involvement. Ten cases of intravascular SS have been reported in the literature and contain little information regarding the proper management of a local metastatic disease. Ours is a rare case of SS with an intravascular tumor occupying the femoral-iliac vein (as seen in metastatic disease) that has been treated as a local disease with a multidisciplinary therapeutic approach. As a result, our patient has been disease-free for two years and, during that time, has achieved an acceptable quality of life.We discuss the pertinent clinical findings of this rare tumor and review the literature of tumor thrombus by SS. We also present the multidisciplinary therapeutic approach realized and the history of this disease. Soft tissue sarcomas (STS) are rare tumors that develop in supporting tissues. Synovial Sarcoma (SS) accounts for 8 to 10% of all STSs, occurring slightly more often in men than women at a ratio of 1.2:1.0 . ImmunohTreatment for localized SS typically consists of a combination of surgery, radiotherapy (RT) and, in some cases, chemotherapy. Adjuvant therapy in localized SS shows a response rate >65% for chemotherapy and >90% for RT. Nonetheless, the exact benefit of administering systemic therapy once SS is nonlocalized is controversial [There are insufficient data at present about epidemiology, diagnosis, and standard treatment in intravascular synovial sarcoma. However, a few intravascular SS have been previously reported.Herein, we discuss a very rare case of histopathologically confirmed biphasic synovial sarcoma with intravascular tumor thrombus treated with a multidisciplinary therapeutic approach.A 49-year-old woman presented to the emergency unit with a history of swelling and erythema in her left limb after a long car trip. In the absence of a medical history, she was diagnosed with deep vein thrombosis (DVT) via Doppler ultrasound. A month after the diagnosis, she was admitted to an internal medicine service with DVT progression  showed the extension of the thrombosis  and a nodular form attached to the vessel wall with unclear borders as shown in Figure A biopsy of the nodular form indicated synovial sarcoma. After histological and immunohistochemical confirmation, the case was presented to the sarcoma committee. No clear evidence of metastatic lesions was observed in the diagnosis images. The multidisciplinary sarcoma board recommended treating the patient according to localized sarcoma protocol, which includes wide surgery excision, RT, and chemotherapy. The insertion of a vena cava filter after surgery was indicated.Wide surgical excision revealed tumor attachment to the sartorius muscle and tumor infiltration of the femoral vessels (vein and artery). Safe borders were macroscopically accepted. The blood flow was reestablished with a contralateral saphenous bypass. A vena cava filter was placed.The pathology was consistent with biphasic synovial sarcoma World Health Organization (WHO) Grade 3. The surgical specimen presented a maximum 9 cm diameter. Tumor embolization, thrombosis, and vascular recanalization were observed. Surgical borders were affected in the proximal area. Figure Immunohistochemistry was positive for CD99, CK1/3, and EMA. SMA, desmin, CD34, pS100, and bcl-2 were negative. Translocation t(X:18)  was confirmed by FISH.Ten days after the primary surgery, the patient presented a new episode of thrombosis in the left iliac vein. The thrombus, completely extracted via anterograde and retrograde thrombectomy, was compatible with biphasic synovial sarcoma.Afterward, adjuvant chemotherapy was administrated with four complete cycles of doxorubicin and ifosfamide.The adjuvant RT treatment was performed via 3D-CRT, 6MV Photon beam (Clinac iX VARIAN) through six isocentric and coplanar beams. The treatment dose was prescribed to 95% the planning target volume isodose: 66 Gy  and 50 Gy . The total dose was administrated in 33 fractions (2 Gy per fraction) daily with five fractions per week. Image guide RT was realized through Kv/Kv and Cone Beam. Figure The patient was disease-free for two years with strict medical visits and a very good quality of life. A new episode of tumor thrombosis in the femoral vein developed. We performed a thrombectomy and noted a synovial sarcoma thrombus with the same immunohistochemical characteristics.Given the patient’s exceptional case, multiple recurrence risk factors, and the controversy around the use of adjuvant therapy for local metastatic disease, the sarcoma board recommended eight cycles of trabectedin for the patient.As of this writing, the patient is free of disease and undergoes frequent medical follow-ups. The treatment was well-tolerated. No acute and late toxicities were reported.After a comprehensive review of the literature, we have determined that intravascular manifestations of sarcomas are extremely rare. Schreiner et al., reported only ten documented cases of intravascular synovial sarcoma . SchreinIn our case, it was very difficult to establish if the origin of the primary tumor was within the vessel or outside of the vessel. However, the radiological studies and pathological results were consistent with those expected in a rare case of intravascular sarcoma. One of the first areas of uncertainty, in this case, was the staging. The intravascular sarcoma (tumor thrombus) is not contained in the TNM staging system, but we believe it correlates to a 'pre-metastatic' disease due to its high metastatic potential.The treatment options available for SS are wide surgical removal of the lesion and RT, often in combination with chemotherapy .In this case, immunohistochemical determination of biphasic synovial sarcoma was established in the primary lesion (the intravascular tumor and mass attached to the vessel) localized in a femoral vessel. A new thrombosis episode presented ten days following primary surgery, localized in the iliac vein. The thrombus was removed, and the pathological analysis revealed the same histology and epithelial markers. Indeed, this new thrombosis was concerning because it may indicate the persistent presence of the microscopic primary tumor. Given the disease’s characteristics, RT to the primary site  and iliac vein was included in the treatment planning, and adjuvant chemotherapy (doxorubicin-ifosfamide) was administrated. A local metastatic treatment  may alleviate symptoms, prolong progression-free survival, or cure selected patients, depending on the metastatic burden. These treatments are associated with chemotherapy delivered sequentially, perioperatively, or as exclusive therapy. Kavanagh et al., note two premises justifying the therapeutic strategy for metastatic burden sarcomas: clinical experience suggests that selected patients benefit from metastatic ablation, and consolidative local therapy produces good results in selected partial responders to systemic therapy -10.Trabectedin, approved in Europe in 2007 for the treatment of advanced metastatic soft-tissue sarcomas, has shown beneficial effects after failure with standard chemotherapy. The evidence suggests that translocation-related sarcomas may be sensitive to trabectedin .Considering the extreme rarity of intravascular synovial sarcomas, the feasibility of adjuvant treatment in a metastatic burden stage using local interventions  and the use of new drugs must be evaluated to determine the future clinical indications in similar cases. In our case, the patient was disease-free for two years after primary treatment. Following a relapse, salvage surgery and trabectedin were used. At present, the patient has no evidence of the disease.Intravascular synovial sarcomas are extremely rare lesions, and the most effective treatment is unknown. There are ten similar cases reported, but this case is the first to use local interventions and trabectedin. As the efficacy of adjuvant treatment for 'pre-metastatic' intravascular SS has not been widely studied because the disease is rare, our case indicates traditional treatments and new systemic drugs can improve the quality of life and delay the metastatic stage.


Introduction. Tumoral calcium pyrophosphate dihydrate (CPPD) crystal deposition disease (CPPDCD), also known as tophaceous calcium pyrophosphate deposition disease (CPDD), is a tumorlike lesion, and it should be distinguished from usual CPDD that causes severe joint inflammation and arthralgia. A case of tumoral CPPDCD of the wrist joint that required differentiation from synovial osteochondromatosis is described. Case Presentation. The patient was a 78-year-old woman with a 5-year history of nodular lesions at the right wrist that had gradually increased in size. An excisional biopsy and a histological examination of the excised nodular lesions by hematoxylin and eosin (H&E) staining were performed, demonstrating numerous polarizable, rhabdoid, and rectangular crystals, surrounded by fibroblasts, macrophages, and foreign body-type giant cells, consistent with tumoral CPPDCD. Conclusion. Tumoral CPPDCD, especially at the wrist joint, is rare, and, to the best of our knowledge, only 2 articles have been published. This case seems to need further follow-up for recurrence, because tumoral CPPDCD may recur after complete or incomplete surgical excision. Usual CPDD is characterized by episodes of arthralgia and the presence of CPPD crystals in the synovial fluid of the affected joint . TumoralThe patient was a 78-year-old woman with a 5-year history of nodular lesions on the right wrist. She consulted a clinic and was referred to our hospital in August 2014 with only two nodular lesions on the right wrist. On physical examination, the radial nodular lesion was 2 cm × 2 cm in size, the dorsal nodular lesion was 2 cm × 2.5 cm in size, and these nodular lesions were not movable, painful, or tender . She hadRadiographs of the right wrist joint before surgery showed multiple periarticular nodular lesions with calcifications on the volar , radial, and dorsal aspects of the right wrist joint . On compThere were no abnormal findings on peripheral blood examination. Laboratory studies showed normal serum uric acid, calcium, phosphorus, alkaline phosphatase, and C-reactive protein levels. These clinical and radiographic findings suggested an initial diagnosis of soft tissue tumor, such as synovial osteochondromatosis, which may occur secondarily or haphazardly in combination with CPDD .An excisional biopsy was performed. The volar and radial nodular lesions were exposed via a volar incision. First, the volar lesion was excised piece by piece, avoiding the flexor tendons and the median nerve . Next, tOn histological examination of the excised tumor tissue with H&E staining, numerous polarizable, rhabdoid, and rectangular crystals, surrounded by fibroblasts, macrophages, and foreign body-type giant cells, were seen Figures . The calAt 6-month follow-up, she had no swelling of her right wrist, and the radiographs showed no evidence of recurrence .CPPD crystals were first identified in 1961 in the synovial fluid of patients with gout-like symptoms without sodium urate crystals, which were described by McCarty as CPDD . The risTumoral CPPDCD should be differentiated from tumoral calcinosis and malignant or benign tumors such as synovial chondromatosis. The location of tumoral calcinosis is similar to that of tumoral CPPDCD but it occurs predominantly in adolescents and young adults and is more often multiple than solitary. On the other hand, tumoral CPPDCD is usually seen in elderly patients. Histopathologically, the calcified material in tumoral calcinosis lacks a crystalline structure and is composed mainly of hydroxyapatite , which dTumoral CPPDCD may recur after complete or incomplete surgical excision. Ishida et al. reported that one of five cases of tumoral CPPDCD had recurrences on two occasions during a 20-year span . TherefoIn summary, this report described a rare case of tumoral CPPDCD at the wrist joint. Preoperative clinical and radiographic findings suggested an initial diagnosis of synovial osteochondromatosis, and an excisional biopsy was performed. However, the histological examinations were consistent with tumoral CPPDCD. Because tumoral CPPDCD is a rare disease and it is difficult to diagnose preoperatively, an excisional biopsy is necessary for pathological diagnosis.


Nonbacterial osteitis is a rare autoinflammatory disease. Often it is mistaken for a tumor or osteomyelitis. We present a case of a twelve-year-old girl referred to our hospital because of a lesion of the right clavicle. The differential diagnoses were sarcoma, osteitis, and Langerhans cell histiocytosis. After biopsy the diagnosis nonbacterial osteitis (NBO) was established. Treatment of choice is a nonsteroidal anti-inflammatory drug. This case report gives a complete follow-up of the disease, showing the pitfalls of the diagnosis. Nonbacterial osteitis is a rare condition, most often of the femur or tibia, which occurs in children around the age of eleven –4. We prA twelve-year-old girl was presented in our bone tumor meeting because of pain and swelling of the right clavicle. Four months earlier in April she had fallen during exercise. She was presented at the emergency room of a hospital in the periphery with a painful right shoulder. An X-ray was made because of a suspected fractured clavicle : in retrFour months later in August, the patient had persistent pain and a swelling of the clavicle during physical examination. A new X-ray was made : this shAfter a few weeks though the swelling increased and pain persisted. Blood tests showed a c-reactive protein (CRP) of 22 and a sedimentation rate (ESR) of 81.In retrospect especially the moth eaten aspect and periosteal reaction of the lesion were suspect. The differential diagnosis is osteitis/osteomyelitis because of the heterogeneous moth eaten aspect and the localization. The lamellated periosteal reaction however could also be apparent in Ewing sarcoma; however a more lytic lesion and more expansion in the soft tissue usually characterize Ewing sarcoma. Osteosarcoma was also thought of but is very rare in the clavicle and is not distinguished by a lamellated periosteal reaction, but by a more spiculated or sunburst periosteal reaction. The image however could fit Langerhans cell histiocytosis; a rare multisystem disease involving clonal proliferation of Langerhans cells. A moth eaten aspect and lamellated periosteal reaction on X-ray also distinguishes the early phases of the condition.It was decided to make a MRI scan to differentiate between a malign or benign origin. On this MRI made in the end of August, we observe a large bony mass, with a heterogeneous aspect and translucent areas. A lamellated periosteal reaction, especially in the superior part, was seen. There seemed to be no soft tissue involvement. The size of the lesion at that moment was 10 × 5 cm. The lack of soft tissue involvement made Ewing sarcoma and osteosarcoma less likely. The absence of osteolysis and a spiculated periosteal reaction attributed to the possibility of a benign origin. An osteitis seemed to be a plausible cause; although Langerhans cell histiocytosis could not be ruled out .As osteitis is sometimes multifocal a bone-scintigraphy was made on which a hot spot in the right clavicle was seen. Therefor a multifocal disease was unlikely .The patient was referred to our musculoskeletal referral center, as it was not clear if the lesion was malign or benign.After referral, the radiological material was reassessed. In retrospect the lesion was rather suggestive for osteitis, as is described above in the differential diagnosis. It was decided to do an open biopsy, to exclude malignancy or Langerhans cell histiocytosis. Cultures were taken and tissue was sent for pathological evaluation. Pathological investigation showed existing bone with extensive reactive bone formation along the periosteal surface. In both the trabecular and cortical bone osseous transformation was seen, resulting in a more plexiform aspect of the cortex. The stromal spaces and marrow existed of well-vascularized stroma and especially in the trabecular bone chronic and active inflammation was observed . In the Because an unknown bacterial pathogen could not be ruled out completely, antibiotics were prescribed pragmatically, first flucloxacillin for six weeks and then because of relapse a switch to clindamycin for three months. There was not much radiological or clinical improvement. Because of the unsuccessful antibiotic treatment, the negative cultures, and the relatively low infection parameters the diagnosis of nonbacterial osteitis was now confirmed.According to the reports on the topic nonsteroidal anti-inflammatory drugs (NSAIDs) are the preferred treatment for the condition. Therefor NSAIDs were started , 2, 4. TNonbacterial osteitis (NBO) is a relatively rare disease , 2. It iThe median age of presentation is eleven years, with local pain, swelling, and malaise as the leading symptoms , 7, 8. TImaging shows a characteristic poorly defined, moth eaten, destructive lesion, with expansion, sclerosis, and a solid or multi lamellated periosteal reaction , 10. SomMagnetic resonance imaging (MRI) is regarded as a better diagnostic tool than computer tomography (CT), for it excludes marrow infiltration and soft tissue swelling . Blood tGikas et al. argue, based on their experience, that a biopsy is no longer necessary. Imaging, clinical, and biochemical findings are sufficient to diagnose NBO .However we still deem biopsy an important diagnostic tool, especially valuable if other symptoms, for example, those of SAPHO, are not present , 7, 10.It is important to determine between malignant, infectious, and autoinflammatory etiology and so biopsy attributes to the choice of a proper treatment , 11.Traditionally antibiotics were prescribed in NBO, based on the thought that there might be an infectious agent involved that cannot be cultured. Though, as it is considered an autoinflammatory disease without an infectious cause, not antibiotics but NSAIDS are considered the best therapeutic choice , 7, 10.TNF-alpha blockage has been described to be successful for the treatment of relapsing cases of NBO , 12, supBecause of a minor trauma, our patient was seen at the first aid department and the early signs of NBO were not detected. In retrospect on the X-ray a subtle osteitis is already exposed. We see how the disease has evolved four months later; now the subtle change has transformed in a clear swelling with bone formation and a moth eaten aspect. To differentiate between a malign or benign origin, a MRI scan is made. As there is now extensive osteolysis or spiculated periosteal reaction a malign origin seems unlikely. The imaging is suspect for osteitis. A bone scintigraphy rules out multifocal osteitis, as there is only one hot spot visible. The biopsy and cultures exclude a malignant or bacterial cause. The further course of treatment confirms the diagnosis of nonbacterial osteitis: antibiotics have no effect; however symptoms subside and the lesion calcifies under treatment with NSAIDs, which is the preferred medication.As far as we know this is the first case with imaging of nonbacterial osteitis in its earliest stages, showing the pitfalls of this diagnosis as the condition can look like a malignancy or an osteomyelitis. One should be aware though that NBO exists, even without the accompanying symptoms of SAPHO. To exclude a malignant or infectious origin, especially if further symptoms of NBO are not present, biopsy is an important diagnostic tool. NSAIDs are the treatment of choice if NBO seems to be the most likely diagnosis. This case provides a complete follow-up of a patient with nonbacterial osteitis, from a subtle start to active inflammation and to calcification once more.


Mature cystic teratoma is a common benign neoplasm of the ovary. Complications occur in approximately 20% of cases. Clinical manifestations, laboratory findings, and imaging studies can assist in making a diagnosis of ovarian torsion of mature cystic teratoma. Furthermore, serum tumor markers may be helpful for diagnosing mature cystic teratoma and its torsion and, thus, can lead to early surgical intervention. A 56-year-old woman presented with a huge pelvic mass and pelvic pain. Serum CA19-9, CA125, and carcinoembryonic antigen levels were abnormally elevated at >700 U/ml, 282.5 U/ml, and 3.94 U/ml, respectively. The tumor was surrounded by extensive adhesions and showed inflammatory changes. The serum levels of these markers returned to normal levels after surgery. Mature cystic teratoma (MCT) is the most common germ cell tumor of the ovary in women of reproductive age. Clinical characteristics of MCT have been well documented, and about 20% of women with MCT experience complications, such as torsion, rupture, infection, and malignant transformation. Ovarian torsion is a common complication and constitutes a surgical emergency, and the rate of torsion has been reported to range from 12.9% to 15% in MCT patients . Delay iHere we report a case of ovarian torsion of MCT with rapid, significant changes in the serum levels of CA19-9 and CA125 in a postmenopausal woman.A 56-year-old postmenopausal woman presented with a huge pelvic mass. The patient had experienced moderate pelvic pain for 5 days before visiting a local clinic. Pelvic ultrasonography revealed a huge mass 11 cm in diameter in the right adnexa. Contrast-enhanced abdominopelvic computed tomography (CT) revealed an 11.0 × 7.5 cm mass containing a fat component arising from the right adnexa. She was referred to our hospital department due to abnormally high tumor markers and suspicion of a coexistent malignancy due to elevated serum CA19-9, CA125, and carcinoembryonic antigen (CEA) levels of >700 U/ml, 282.5 U/ml, and 3.94 U/ml, respectively. These serum markers were measured again preoperatively in our hospital and were found to be higher (Figure MCT is the most common benign neoplasm of the ovary. Adnexal torsion is a gynecologic surgical emergency, as delay in surgical treatment may lead to serious inflammatory consequences . SometimUltrasonography is usually used for the diagnosis of ovarian MCT. However, accurate diagnosis is difficult because tumor contents vary. In addition, 80% of cystic teratomas have sonographic appearances similar to those of ovarian malignancies [The rates of elevation of CA19-9 and CA125 have been reported to be 39.6 to 59% and 13.5 to 25%, respectively . Emin anCA125 is an extensively studied marker in the context of ovarian tumors. Serum CA125 levels provide information preoperatively that aid discrimination of benign and malignant adnexal masses. However, CA125 is not a tumor-specific antigen, and is also elevated in patients with endometriosis, adenomyosis, leiomyoma, and pelvic inflammatory disease. Accordingly, knowledge of CA125 alone is unhelpful for the diagnosis of MCT, and elevated CA125 levels in MCT may be related to peritoneal inflammation, such as that associated with pelvic inflammatory disease. In our case, the CA125 elevation was probably caused by torsion and inflammatory processes.CA19-9, in combination with CA125, might be a useful marker for the discrimination of MCT from ovarian cancer . Our casThe rate of elevation of CEA in ovarian MCT has been reported to range from 9.1 to 16.3% . HoweverNevertheless, these three serum markers can provide valuable information for the diagnosis of MCT and for predicting its complication, although CA125 or CA19-9 testing is not sufficient for the diagnosis of MCT . Our casIn our case, prolonged torsion and delayed treatment were caused by a number of factors. First, symptoms were nonspecific, which delayed hospital visit. Second, the first gynecologist approached at the primary clinic under-interpreted CT findings and over-interpreted serum tumor marker findings. In particular, CT findings suggesting adnexal torsion and high tumor markers were interpreted as indicators of ovarian malignancy. Third, delayed referral to a tertiary center was attributed to adherence to a routine outpatient schedule.In conclusion, a highly elevated serum CA19-9 level may be an adjunct serum marker for the diagnosis of ovarian teratoma, and could also provide useful information on the presence of torsion. Furthermore, simultaneous elevations of CA19-9 and CA125 are not always associated with malignancy and may improve the detection of torsion and the prediction of the extent of necrosis. Perioperative assessment of tumor markers may provide valuable information on the progression or resolution of inflammatory processes following torsion of the ovary. The interpretation of tumor marker elevations should be made in light of the clinical condition. The dramatic changes in serum markers observed perioperatively in our patient gave us a better understanding of the inflammatory process following prolonged ovarian torsion.Written informed consent was obtained from the patient for the publication of this report and any accompanying images.


Extranodal presentation of lymphoma is a rare occurrence. It has been postulated that chronic antigen stimulation may predispose a patient to the development of lymphoma.We present a case report of a large extranodal B-cell lymphoma mimicking a postoperative abscess following surgery for an infected proximal femur nonunion in an 80-year-old Caucasian male of Italian descent.This case highlights the need to consider malignancy in revision surgery, careful examination of operative specimens and the need for further understanding of the role of metal implants in chronic antigen stimulation. Lymphomas can develop in any location where lymphomatous tissue exists, but extranodal presentation in the lower limb and pelvis are uncommon. We report an unusual case of large extranodal B-cell lymphoma initially presumed to be a postoperative pelvic and thigh abscess following multiple operations for an infected proximal femur nonunion.An 80-year-old Caucasian male of Italian descent presented to the emergency room with a two-week history of progressive left thigh pain and swelling. The patient denied any history of trauma and stated that the pain was aggravated by weight-bearing. He denied any fevers, chills, night sweats, constitutional or neurological symptoms. He had no drug allergies and his only medication was a statin. Past medical history included hypercholesterolemia, open cholecystectomy, and prostate cancer requiring resection remotely in the past.Staphylococcus aureus cultured from previous operations. His most recent surgery occurred one month prior to his presentation to the emergency room and involved removal of a left trochanteric fixation nail and excision of a pseudoarthrosis, followed by an osteotomy of the left proximal femur nonunion and fixation with a proximal femoral locking plate (Figure The patient’s surgical history was significant for six previous operations for a left intertrochanteric femoral fracture complicated by an infected non-union. An infectious diagnosis had been proven with e Figure . No compe Figure A, B.Aftee Figure . Tissue Diffuse large B-cell lymphoma is the most commonly occurring lymphoma, estimated to account for 35% of all lymphoma cases globally . It typiLymphomas can also involve any part of the musculoskeletal system and occasionally can manifest as a primary soft tissue mass that can be mistaken for infection or a soft tissue sarcoma -8. Soft Although poorly understood, it has been postulated that primary soft tissue lymphomas may be the result of chronic antigen stimulation in an individual with a deficient immune system. In addition to inflammatory connective tissue disorders and viruses, other potential causes of a prolonged local inflammatory environment in patients undergoing orthopedic surgery include shedding of local metal ions and debris from metallic implants, radiation from radiographs and CT imaging, surgical trauma and infection.Pre-clinical evidence examining the potential carcinogenic properties of metal implants found a trend towards sarcoma development in rats after 30 months when implants with a high content of cobalt, chromium or nickel were used. There was also an increase in bone-associated lymphoma development in rats with these metal implants . It is bNumerous studies have explored the topic of increased cancer risk in individuals with metal implants, but have shown discordant conclusions. Gillespie et al.  followedChronic inflammation as a result of infection is a possible contributing factor in the development of lymphoma, as implied by studies of non-infectious consequences of osteomyelitis -31. CheuTypically, soft tissue lymphomas present in patients older than 60 years as enlarging soft tissue masses associated with swelling or pain. In typical cases, there are usually no B symptoms such as weight loss and fever ,6. Our cDespite the presence of some risk factors, the malignancy in this case could also have been coincidental. Cases have been reported of patients who underwent total joint replacement and were found to have lymphoma on routine histopathological exams ,33. One This case underlines the importance of considering the possibility of malignancy with revision surgery, particularly in cases that present as infectious in nature. It also re-affirms the importance of routine pathologic examination of operative specimens. As the number of joint replacements and fixation of fractures increase with life expectancy, potential long-term adverse effects of metal implants need to be further investigated.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.BP was the surgeon involved in the operations and follow-up of this patient and conceived of the report; AE contributed to data acquisition; MM contributed the data on lymphoma and was involved in editing the drafts; RM and JM contributed to data acquisition, drafting and editing the manuscript. All authors read and approved the final manuscript.


Solitary extramedullary plasmacytomas (EMPs) are nonepithelial neoplasms of plasma cell origin categorized among non-Hodgkin lymphomas, without the bone marrow involvement and systemic spread seen in multiple myeloma. They are uncommon tumors comprising 3% of all plasma cell neoplasias. Although they usually occur in the upper respiratory tract, only 1 case of EMP localized to the frontal sinus has been reported in the English literature. We present in this report a rare case of EMP originated from the left frontal sinus leading to left eyeball proptosis and movement restriction. A survey of sinonasal EMPs in the Turkish literature is reported, as well. Paranasal computerized tomography and magnetic resonance imaging of a 69-year-old female who presented with left eyeball proptosis and left-sided headache revealed a solid mass in the left frontal sinus. Histopathological analysis of the completely excised mass supported the diagnosis of plasmacytoma. The definitive diagnosis of solitary EMP was confirmed with further investigations at hematology and oncology clinics. The patient was treated with surgery followed by local radiotherapy to the head and neck region, and she was disease-free at her 1-year follow-up. Treatment of sinonasal EMP is surgery alone or surgery combined with radiotherapy. Long-term follow-up is a requisite for systemic control because of the disease’s high potential to transform into multiple myeloma. Ekstrameduller soliter plazmasitomlar plazma hücrelerinden kaynaklanan, non Hodgkin lemfomalar arasında sınıflandırılmış, kemik iliğini tutmayan ve multipl miyelom gibi jeneralize olmamış formda non epitelyal tümörlerdir. Nadir görülen tümörler olup plasma hücreli neoplazilerin %3’ünü oluştururlar. Çoğunlukla üst solunum yolunda sinonazal yerleşimli olmasına rağmen frontal sinüs yerleşimli olanına literatürde 1 olguda rastlanılmıştır. Bu yazıda sol frontal sinüs yerleşimli sol gözde proptoz ve hareket kısıtlılığına sebep olan nadir bir extrameduller soliter plazmasitom olgusu sunulmakta ve Türk literatüründeki sinonazal yerleşimli EMP’ler gözden geçirilmektedir. Altmış dokuz yaşında baş ağrısı ve sol gözde dışa itilme yakınması ile gelen bayan hastada çekilen paranazal BT ve MR sol frontal sinüs kitlesini ortaya koymuştur. Açık cerrahi ile tamamı çıkarılan kitlenin histopatolojik analizi plazmasitom tanısını koydurmuştur. Soliter EMP tanısı hematoloji ve onkoloji klinikleri tarafından yapılan tetkiklerle kesinleşmiştir. Hastaya postoperatif radyoterapi uygulanarak olumlu yanıt elde edilmiştir. Hasta halen sağlıklı olup 18. ay kontrolunda nüks izlenmemiştir. Sinonazal yerleşimli ekstramedüller plazmasitomların tedavileri cerrahi, cerrahi ve/veya radyoterapidir. Uzun dönemde hastalığın multipl miyeloma dönüşme gibi bir potansiyeli olabileceği gözardı edilmeden takiplerinde tam bir sistemik kontrol yapılması zorunludur.Plasmacytoma is a neoplasm consisting of proliferated monoclonal plasma cells in either the bone or soft tissue. It presents either as a single lesion (solitary plasmacytoma) or as multiple lesions localized in bone marrow [multiple myeloma (MM)]. Solitary plasmacytomas most frequently occur in the bone (solitary plasmacytoma of the bone), but they can also be found in the soft tissues as solitary extramedullary plasmacytomas (EMPs). EMPs are categorized as very rarely seen forms of the non-Hodgkin lymphomas. They represent about 3% of all plasma cell tumors . The tonThe median age at diagnosis is 55 to 60 years, and approximately two-thirds of the patients are males .A combination of surgery (for resectable tumors) and radiotherapy is the treatment of choice . ChemothWe present herein a 69-year-old woman who was referred to the ear, nose, and throat (ENT) clinic with a left-sided frontal mass and protrusion of the left eyeball due to an EMP originating from the left frontal sinus. A review of the Turkish literature on sinonasal plasmacytomas is also provided.A 69-year-old woman was referred to the ENT clinic with left frontal swelling for 7 days. Her past medical history was unremarkable except for a chronic headache that had become refractory to analgesics in the last months and hypertension that was kept under control with a beta-blocker. No additional complaints such as epistaxis or nasal obstruction were presented.On physical examination, a swelling in the left supraorbital region and forward protrusion of the left eyeball were noticed. The mass was smooth, nontender, and 4x3 cm in size. The anterior wall of the left frontal sinus was dehiscent. Visual acuity and eye movements were normal. Nasal endoscopy was unremarkable. No lymph node swelling was noticed on palpation.Contrast-enhanced computerized tomography (CT) scanning demonstrated opacification of the left frontal sinus due to a soft tissue mass extending into the orbit . The antMagnetic resonance imaging (MRI) of the orbits revealed a large mass in the left frontal sinus extending into the orbit, isointense on T1A- and T2A-weighted sequences without involvement of the periorbital fat and extraocular muscles .The patient underwent left osteoplastic flap surgery. The lesion was nonencapsulated and multilobular . Frozen Postoperatively, the patient was referred to a hematology clinic for further diagnostic work-up, including bone marrow biopsy and aspiration, skeletal X-ray survey, serum electrophoresis, immunoglobulin quantification, serum and urine immunoelectrophoresis, β2-microglobulin assay, chest X-ray, and abdominal ultrasonography. The bone marrow biopsy showed a plasma cell infiltration of less than 5% of all nucleated cells. The absence of hypercalcemia, kidney failure, osteolytic bone lesions, and other organ involvement in a patient with localized disease suggested the diagnosis of solitary EMP. The blood work and urine electrophoresis results are given in Table 1.The patient was given adjuvant radiotherapy at a dose of 40 Gy in the radiation oncology clinic. She was disease-free at her 18-month follow-up with no progression to MM.EMP of the head and neck most commonly affects the sinonasal cavity’s mucosa  .The clinical presentation of paranasal sinus EMP depends on the mass volume and on the site of involvement. Nasal obstruction (80%), soft-tissue swelling, epistaxis, nasal discharge, pain, and proptosis are the main clinical findings observed in decreasing order of frequency. The tumor has a slow-growing nature and becomes symptomatic when the mass fills the cavity several months later. This condition results in treatment delay and transformation into systemic disease (MM). The early referral of the patient in this report was due to the obvious symptoms of the mass, which eroded the frontal sinus wall and pushed the eyeball anteroinferiorly. Had the sinonasal cavity been involved, the tumor would not have been diagnosed at an early stage and would have progressed into multiple myeloma, which usually has a fatal progression.CT and MRI are complementary techniques in evaluating the size and location of a tumor and the involvement of the adjacent structures; however, the role of MRI in staging EMPs is not clear . AlthougThe diagnosis of EMP depends on the morphologic and immunophenotypic analysis of the localized tumoral mass . As is tImmunophenotyping with CD15, CD20, CD45RB, CD45RA, epithelial membrane, IgA, IgD, IgG, IgM, IgE, kappa light chain, and lambda light chain antibodies is helpful to differentiate EMP from epithelial tumors . Once thControversy exists regarding the optimal treatment of EMP. Based on the well-known high radiosensitivity of plasma cells, a dose of 40-50 Gy radiotherapy is advised in the treatment . AccordiRecently genetic abnormalities have become more apparent in pathogenesis and progression to MM. Additional biological factors related to the proliferative activity of the malignant plasma cells are high levels of angiogenesis and higher histologic grades. Translocations involving the immunoglobulin heavy-chain locus on chromosome 14q32, resulting in transcriptional activation of MAF, MMSET, CCND1, CCND3, or MAFB, and numerous structural and copy number alterations are important genetic changes for the pathogenesis of MM. Complex karyotypic abnormalities in MYC, the activation of NRAS and KRAS, mutations in TP53, and inactivation of CDKN2A and CDKN2C are late-onset translocations and gene mutations that promote a higher rate of progression to MM ,13.When compared to solitary bone plasmacytoma, EMP demonstrates a relatively low risk of progression to MM. The 10-year overall survival rate was reported to be 70% in EMP cases . In anotIn the case of persistent M protein after completion of radiotherapy or suppression of normal immunoglobulin classes and suppression of BMP6 expression by intensive methylation of the genes at CpG sites of the BMP6 promoter region by plasma cells, with extremely high levels of serum lactate dehydrogenase, early intensive systemic therapy should be considered to prevent poor prognosis .Because of the bony destruction in the present case, our treatment of choice was surgery combined with a radiotherapy dose of 40 Gy. The patient’s lesion was localized in only one sinus and was totally removed from the cavity. In our opinion, complete surgery with postoperative radiotherapy is more promising in the treatment of solitary EMP. The patient is now in follow-up at hematology and ENT clinics and is still free of disease at 18 months. Factors that influenced the good prognosis in our case were, as per the case-based experience in the literature, the relatively small size of the tumor (<5 cm), early presentation, a single site of involvement, localization , female sex, and combined modality treatment.Reports on nasal and paranasal sinus EMPs are rare in the English literature. Few reports have been published in the Turkish literature, as well. A comprehensive search of a Turkish database for patients with nasal and paranasal EMPs identified only 10 reports. Among these 10 reports, only 2 were included in PubMed . The case reports on nasal and paranasal EMPs in the Turkish literature are listed in Table 2. However, follow-up information was missing for most of the patients and therefore we were unable to perform a documented analysis on disease recurrence or progression. This was the shortcoming of this report.In summary, solitary EMP usually develops in the head and neck soft tissues but may also involve sites such as the thyroid and brain and may be observed in a large age range (17-80 years). One should always include the possibility of plasma cell tumor in older patients presenting with symptoms of epistaxis, nasal blockage, or facial mass. Deep submucosal biopsies are usually required so as to not miss the lesion in frozen sectioning. This neoplasm is usually treated with radiotherapy or with a combination of surgery and radiotherapy. Progression to MM may occur 3 to 8 years after treatment. Long-term follow-up in terms of disease recurrence and progression is mandatory.The authors of this paper have no conflicts of interest, including specific financial interests, relationships, and/ or affiliations relevant to the subject matter or materials included.


Mantle cell lymphoma (MCL) is a subtype of non-Hodgkin's lymphoma (NHL) comprising around 7% of adult NHL. It is characterized by a chromosomal translocation t(11:14) and overexpression of Cyclin D1. The incidence of secondary gastrointestinal tract involvement in MCL ranges from 10 to 28% in various series. However primary gastrointestinal MCL is very rare, accounting for only 1 to 4% of primary gastrointestinal lymphomas. The most common endoscopic feature of primary intestinal MCL is multiple lymphomatous polyposis. In rare cases it presents as protruded lesions or superficial lesions. Single colonic mass presentation is an extremely infrequent presentation. MCL has an aggressive course with quick progression, and most cases are discovered in the advanced stages. Colonic biopsies with histologic examination and specific immunohistochemical staining are the gold standard for a proper diagnosis. We report a case of a single mass forming mantle cell lymphoma of the ascending colon in a 57-year-old female patient with unusual colonoscopic and radiologic features and describe the therapy the patient received, thereby adding to the spectrum of clinical presentations of this aggressive lymphoproliferative disorder.  Mantle cell lymphoma (MCL) is a small B-cell non-Hodgkin's lymphoma and is recognized as an aggressive B-cell lymphoma derived from a subset of naive pregerminal center cells with a propensity to involve extranodal sites. The gastrointestinal tract is the most common extranodal site involved by lymphoma, accounting for 5–20% of all cases of extranodal involvement . DespiteA 57-year-old woman with a history of hypertension and type 2 diabetes mellitus presented to our clinic with abdominal pain of 2 months duration, unintentional weight loss of 5 kg, night sweats, and fatigue. Abdominal physical examination revealed a palpable mass on the right side involving the right upper and lower quadrants at the level of the right colonic area. No organomegaly or palpable lymph nodes were noted. Her complete blood count was remarkable only for microcytic anemia, while chemistry studies including liver function tests and tumor markers  were within normal limits. An abdominal CT scan showed a large soft tissue lesion arising from the wall of the ascending colon, with surrounding soft tissue deposits and enlarged pericolonic lymph nodes . GastrosBiopsies were taken from the mass and showed lamina propria expansion by a diffuse population of medium-sized lymphocytes with mild to moderate nuclear irregularity and mildly increased mitotic activity . On immuSubsequently, staging radiologic studies were performed. Abdominal CT scan with intravenous contrast administration disclosed an enhancing solid mass replacing the entire right colonic area, with corresponding mesenteric blood vessels encasement. Positron Emission Tomography PET-CT scan showed hypermetabolic large right colonic mass with adjacent retroperitoneal, mesenteric lymph nodes, peritoneal deposits, and heterogeneous bone marrow uptake. Since no uptake was noted in the small bowel, balloon enteroscopy was not preformed. Additionally, a bone marrow core biopsy showed marrow involvement by MCL.In view of the above findings, the patient's stage was determined as IV E as per the Ann Arbor classification. She was treated by induction chemotherapy alternating R-CHOP/R-DHAP for a total of 3 cycles of each protocol. A very good response was achieved, and the patient subsequently underwent conditioning with high dose chemotherapy (BEAM) followed by autologous stem cell transplantation (ASCT). Her overall treatment was well tolerated except for fatigue and grade 2 mucositis as well as febrile neutropenia. Evaluations after ASCT with a PET-CT scan on day 100 and day 180 showed a continuous complete remission. However, the patient refused colonoscopy after completion of chemotherapy.Mantle cell lymphoma is a subtype of non-Hodgkin's lymphoma (NHL) comprising around 7% of adult NHL . It is cGastrointestinal tract involvement is recognized occasionally as being the presenting sign of lymphoproliferative disorders, and early recognition is important for staging, prognosis, and selection of appropriate treatment. Of note, the incidence of secondary gastrointestinal tract involvement in MCL ranges from 10 to 28% in various series . At endoIn summary, we described the case of primary colonic mantle cell lymphoma with an unusual presentation, thereby adding to the spectrum of clinical manifestations of this aggressive lymphoproliferative disorder. Although a rare entity, primary colonic lymphomas should be included in the differential diagnosis of single colonic lesions. Awareness of such occurrences is necessary and might help refine diagnostic methods for gastrointestinal lymphoproliferative disorders.


We report a huge pilomatrixoma of the left shoulder in anelderly female, raising fear of malignancy. MagneticResonance Imaging delineated the swelling and wide surgicalexcision was performed. Post operative recovery wasuneventful. Long term follow up is necessary in view of thepossibility of recurrence and malignant transformation.Pilomatrixoma, Pilomatricoma, Calcifying Epithelioma ofMalherbe, Shoulder, Upper Limb, Elderly, Female Pilomatrixoma  is abenign tumour of hair follicular matrix cells based onhistochemical and electron microscopic studiesA 69-year-old lady presented with left shoulder swelling forpast 20 years. The swelling was gradually enlarging andassociated with pain in the last two years. One month prior toconsultation, the swelling ulcerated with seropurulentdischarge. She had no fever nor constitutional sign andsymptom of malignancy. Clinical examination revealed an8cm x 5cm x 3cm firm exophytic swelling with centralulceration over the left deltoid region . It appeWide surgical excision of the left shoulder tumour wasperformed and wound was closed primarily .HistopaThe presentation of this lady with a rapidly enlargingswelling and ulceration with seropurulent dischargeassociated with pain, foci calcification on plain radiograph,as well as heterogenous signal on MRI alerted on the highpossibility of a malignant tumour. The clinical appearancemimicked the malignant picture of squamous cell carcinoma.Hence, without pre- excision cytological examination orintralesional biopsy, wide surgical excision of the tumourwas performed. This more extensive surgical approach wasperformed as if it is a malignant tumour. Adjuvantchemotherapy or radiotherapy might be indicated ifmalignancy was confirmed.The histopathological and microscopic examinationhowever, showed a benign partly ulcerated circumscribedlesion formed by solid, irregular islands of epithelial cellswhich were embedded within a fibrocollagenous stroma. The epithelial cells were of two basic types, with the predominantcell type comprising of eosinophilic shadow cells (Ghostcell) with distinct cytoplasmic borders and a centralunstained area due to lost nucleus . The secThe clinical picture of the ulcerating lesion suggested apossible suspicion of a malignant tumour and a wide surgicalexcision was therefore carried out promptly. A fine needleaspiration for cytological examination or intralesional biopsy would be most helpful to reach a diagnosis andassist in landscaping the excision margin, apart from theradiological findings. A malignant lesion could have beenruled out and a marginal surgical excision would have beenadequateThe surgical excision of the lesion for this case has broughtabout the cessation of a ulcerating lesion and its subsequentcomplications such as superimposed infection. The complaint of pain was being solved with the lesion beingexcised and the cosmesis of the shoulder preserved. Thelesion may be left untreated if it is at early stage withoutproducing significant impairment functionally orcosmetically . Although the reported local recurrence rate after complete surgicalexcision is rare


Desmoid tumors (DTs) are rare, benign soft-tissue tumors that have the potential for local invasion, but not for metastasis. The tumors are commonly characterized by a palpable mass, but present a variable and unpredictable clinical course. The current study presents the case of a giant mediastinal DT exhibiting lung involvement. A 50-year-old female was referred to the West China Hospital  due to a recurrent DT that was identified one year following radical surgery. The patient subsequently received radiation therapy. The DT arose from the mediastinum, unlike the usual presentation, and recurrence presented as extensive invasion into the lung tissue, almost being misdiagnosed as lung cancer with brain metastasis. Tumor recurrence was diagnosed through contrast-enhanced computed tomography and histological examination of the tumor. A routine follow-up revealed no further tumor progression at 9 months post-admission. Taking into account the unpredictable treatment complications, recurrent DTs can be managed simply and efficiently. A ‘wait-and-see’ policy could be a viable therapeutic option for this disease. Desmoid tumors (DTs) are histologically benign monoclonal myofibroblastic neoplasms. However, these tumors tend to be locally invasive and infiltrate into the surrounding soft tissue, but are lacking in metastatic potential .Numerous studies have demonstrated that DTs account for just 0.03% of all neoplasms and ~3% of all soft-tissue tumors –5. The mA 50-year-old female presented with a dull pain in the left scapular region and a decreased range of motion of the left upper limb that had been present for five years. The patient was treated at a local hospital in June 2009. A computed tomography (CT) scan revealed a homogenous soft-tissue density mass lesion in the superior lobe of the left lung. Surgery was performed within the left side of the upper mediastinum and the tumor was completely removed. The longest diameter of the mass was ~6.1 cm. Surgical biopsies of the mass revealed a fibroma. However, in the year following surgery, the pain in the left scapular region occurred again and slowly progressed. Tumor recurrence was diagnosed through a CT scan and histological examination of the tumor. The left chest wall of the patient was treated with radiation therapy (64 Gy for 32 fractions). However, no notable improvement was found. The patient was referred to the West China Hospital  for a recurrent tumor of the mediastinum in November 2012. A CT scan revealed that the left thoracic wall had collapsed and there were two soft-tissue masses, located in the left pulmonary apex and the costal pleura below the left plumonary apex, respectively. The maximum cross-section was ~3.3×2.6 cm. The first tumor was wrapped around the left subclavian artery and caused erosion of the adjoining ribs . The numIn the present case, the DT originated from the mediastinum, which is a rare location, and the recurrence occurred one year after the initial surgery. However, the range of the recurrent mass was wide and infiltrated up into the neck and down into the lung. When purely analyzed by imaging, the tumor could be misdiagnosed as a malignant tumor arising from the lung. The most puzzling finding is that MRI revealed a nodule that could be considered as brain metastasis in the right side of the pons.Thus, the definitive diagnosis, classification and differential diagnosis of DTs require a histopathological examination ,11. HoweComplete surgical excision with wide tumor-free margins is the current treatment for primary and recurrent DTs . HoweverAge, tumor location and margin status are all factors associated with recurrence . If the In conclusion, the early detection of DTs and the use of a complete surgical resection play an important role in the prognosis. Long-term follow-up is an indispensable guide to future treatment.