Head and neck sarcomas are relatively rare tumors, with angiosarcomas representing a small subset. Angiosarcoma is a malignant endothelial neoplasm characterized by atypical, multilayered, or solid endothelial proliferation with vasoformative architecture. The global incidence of irradiation-associated sarcoma is estimated as between 0.03% and 0.08%. Here we reported the case of an elderly woman previously treated with radiation more than 20 years ago for an unknown primary of head and neck. This interesting case presented as a diagnostic challenge, and multiple biopsies were required to eventually establish the diagnosis of laryngeal angiosarcoma. We additionally have confirmation from our prior radiation records that the patient did, in fact, receive a substantial dose of radiation to the site previously. To our knowledge, this case represents the first report of a documented radiation-induced multifocal laryngeal angiosarcoma. Head and neck sarcomas are relatively rare tumors, with angiosarcomas representing a small subset. Angiosarcomas have a higher propensity to affect the elderly and prior studies exist correlating them to radiation exposure. Angiosarcomas tend to originate either from blood vessels or lymphatic vessels. Most head and neck angiosarcomas tend to be observed in the face or scalp. In nonvisible areas of the body, the tumor generally presents as a palpable mass or with symptoms specific to the site. After treatment, the tumor tends to recur locally; however, distant metastases may also occur. The best determinant of overall survival is the ability to completely resect the tumor with a negative and wide margin. While surgery is the main form of treatment, the overall survival for patients with angiosarcoma is low and opportunity of a wide resection margin is rare in the head and neck region. For these reasons, patients with a high-grade lesion and/or positive or close margins may benefit from adjuvant chemoradiation therapy.We report the case of an elderly woman previously treated with radiation more that 20 years ago for an unknown primary of head and neck who now presented with a fungating supraglottic mass. This interesting case presented as a diagnostic challenge, and multiple biopsies were required to eventually establish the diagnosis of laryngeal angiosarcoma. An 83-year-old female was directed to the emergency department by her primary care physician three weeks after falling on her left flank for a CT of her abdomen. This CT showed concern for bile duct dilatation, but as she was asymptomatic, she was discharged home with followup. Several days later an endoscopic retrograde cholangiopancreatography (ERCP) was performed, and a large hypopharyngeal mass was incidentally noted. A biopsy was taken at the time of her GI procedure. The patient was subsequently admitted, and ENT was consulted the following day. Her past medical history includes hypertension, congestive heart failure, stroke, and history of cervical metastasis from unknown primary that was treated with radiation therapy 21 years ago.This patient was originally diagnosed with a T0N1M0 cancer of unknown primary in 1987. A primary site was not identified on CT of the head, neck, chest, abdomen, and pelvis as well as quadruple endoscopy. Fine-needle aspiration of the neck mass was positive for a poorly differentiated carcinoma. The lesion was 1–1.5 cm in size in the right jugular digastric region just below the angle of the mandible. She received definitive radiation therapy to the head and neck region at Gershenson Radiation Oncology Center, Detroit, MI, from February 11th, 1987 to May 5th, 1987. She received treatment to the neck node and potential sites of primary via left and right lateral opposing ports to 3600 rads by means of Cobalt 60. After this dose, off-cord treatment was delivered to the left and right anterior neck to an additional 1440 rads. Finally, the site of cervical adenopathy was given an additional boost of 900 rads with 10 MV electrons. The lateral supraclavicular field and posterior neck were also treated. On review of the treatment portals and prescribed therapy, the patient received a cumulative dose of 5940 rads to the anterior neck 20 years prior. The anterior neck irradiated included epiglottis and larynx.On further questioning, patient admitted to one-month history of dysphagia to solids and liquids. She reported a globus-type sensation as well as discomfort when eating. She also had intermittent hot potato voice and coughing episodes. She has had a twenty-pound weight loss and was more easily fatigued. However, she denied any fevers, night sweats, shortness of breath, or otalgia. The patient is a former heavy tobacco user, about a pack a day to a third of a pack a day. She denied any alcohol or drug use. On physical examination, a large and fungating epiglottic mass extending into the base of the tongue was noted on fiberoptic nasopharyngoscopy. Neck examination revealed no obvious lymphadenopathy. CT scan of the neck and thorax with IV contrast performed the same day demonstrated a heterogeneous enhancing epiglottic mass which involves the preepiglottic space and extends inferiorly along the mucosal pharyngeal space more on the right involving the laryngeal surface of the aryepiglottic folds. This mass measures 2 × 1.8 × 1.6 cm. The mass extends to involve the false cords and possibly the superior aspect of the right true vocal cord. There is no cartilage invasion, retrocricoid invasion, or subglottic extension. No significant lymphadenopathy. The original biopsy at the time of ERCP returned necrotic debris with focal squamous features and cellular atypia. Numerous clusters of bacterial and fungal elements were consistent with candida. The patient was then taken back to the operating room for triple endoscopy, tracheostomy, and biopsies. Direct laryngoscopy revealed a friable, exophytic mass involving the base of tongue, vallecula, and the oral surface of the epiglottis as well as the laryngeal surface of the epiglottis down to the aryepiglottic fold but does not involve the false vocal cord, arytenoid, piriform sinus, or the postcricoid space. The mass was biopsied. Direct bronchoscopy was negative. Direct esophagoscopy revealed a duodenal mass which was biopsied. A PEG tube was placed. The pathology returned predominantly fibrinopurulent exudates and necrotic debris with bacterial and fungal organisms. Because of the negative biopsy, the patient was once again taken to the operating room for biopsy. This was biopsied several times and sent off for frozen section. Frozen section was negative. We then sent off for another set of biopsy specimens. Again this was negative. We sent a third set of biopsy specimens, and this was deferred for permanent. Pathology returned benign squamous mucosa with marked subepithelial inflammation, florid granulation tissue with prominent endothelial proliferation and extensive necrosis. Sections show extensive necrosis with prominent exuberant granulation tissue with spindle-cell proliferation and a chronic inflammatory infiltrate. The spindle cells do not show much cytologic atypia, and no mitotic figures were noted. Some of the spindle cells appear to form vascular channels. The differential diagnosis included florid granulation tissue formation, a poorly differentiated spindle-celled carcinoma or a spindle-cell sarcoma. Immunohistochemical stains were performed and included actin (smooth muscle marker), CD31 , P63, CAM5.2, and AE1/AE3 epithelial markers. Sections showed the spindle cells to stain strongly with the CD31, while the other stains were essentially negative in the spindle cell area. This is consistent with a florid endothelial proliferation or granulation tissue formation. No unequivocal malignancy is seen in this specimen.After three negative and inconclusive biopsies, patient's case was discussed in the Multidisciplinary Head and Neck Tumor Board, and a recommendation for a larger and more aggressive biopsy was recommended. Infectious disease has also been consulted regarding this patient, and they requested tissue for cultures. After extensive discussion with the patient and her family, patient was taken back to OR for extensive debulking of the epiglottic mass. The final pathologic diagnosis was angiosarcoma. The biopsy shows a highly cellular neoplasm with necrosis. Tumor cells are positive for CD31, CD34, and negative for Factor VIII, cytokeratin AE1/AE3 and CK903. The morphology and immunoprofile are diagnostic of epithelioid angiosarcoma. Outside consultation from the University of Michigan ultimately agreed with the above diagnosis. The patient then underwent a total laryngectomy and cricopharyngeal myotomy with tracheoesophageal puncture. During the surgery, 2 primary foci involving the epiglottis and base of the tongue were noted . The finPrimary laryngeal sarcomas have been determined to comprise only 0.32%, 0.55%, and 0.87% of all laryngeal malignancies based on previous studies .Angiosarcoma is a malignant endothelial neoplasm characterized by atypical, multilayered, or solid endothelial proliferation with vasoformative architecture . The gloOnly 13 case reports of primary laryngeal angiosarcoma have been reported in the English literature to date Tables Radiation therapy has been well documented as a causative factor in the formation of basal-cell carcinoma and squamous cell carcinoma, but only rarely angiosarcoma . It is vImmunohistochemical and histologic features, are required for identification. Histologically, in angiosarcomas the most common type is epitheliod angiosarcoma . FrequenThe overall prognosis of angiosarcoma is poor. They spread by both the lymph system and via blood vessels . RegardiThomas  reports In addition to the pathologic diagnostic dilemma associated with this condition, cases must also fulfill criteria for radiation-induced sarcoma. Although several authors report prior radiation in association with cases of angiosarcoma of the larynx , specifi


Intraspinal neurocysticercosis is an uncommon manifestation that may present as an isolated lesion. Furthermore, acute hydrocephalus caused by isolated intraspinal neurocysticercosis without concomitant cerebral involvement is extremely rare.A 64-year-old man presented with a history of severe headache, an unsteady gait, and occasional urinary incontinence. Magnetic resonance imaging of the thoraco-lumbar spine revealed multiple, cystic, contrast-enhancing intraspinal lesions. A computed tomographic scan of the brain showed marked ventricular dilatation but no intraparenchymal lesions or intraventricular cysticercal lesions. This case of acute hydrocephalus was found to be caused by isolated intraspinal neurocysticercosis and was treated by ventriculoperitoneal shunt placement and surgical removal of the intraspinal lesions , followed by administration of dexamethasone and albendazole.Isolated spinal neurocysticercosis should be considered in the differential diagnosis of acute hydrocephalus when no explanation is found in the brain, particularly in geographical regions endemic for cysticercosis. Taenia solium. Neurocysticercosis (NCC) of the central nervous system is classified by location as parenchymal, subarachnoid, intraventricular, or spinal. The spinal type is rare and accounts for only 1.0-5.8% of cases, and isolated spinal NCC without intracranial involvement accounts for ~25% of these cases [Cysticercosis is a common parasitic disease that invades the central nervous system; in humans, this condition is caused by encysted larvae of se cases . Furtherse cases .Here, we report a rare case of isolated intraspinal NCC exhibiting clinical features of acute hydrocephalus. In addition, we discuss the principles of the diagnosis and treatment of intraspinal NCC and provide a review of the literature.3 (eosinophils 3%) and a hemoglobin level of 12.8 mg/dL, although the hematological findings were unremarkable. Motor examination showed normal strength in both upper extremities but reduced strength in both lower extremities . Under a diagnosis of acute hydrocephalus, lumbar tapping was attempted repeatedly, but cerebrospinal fluid (CSF) was not obtained despite adequate lumbar puncture. Magnetic resonance imaging (MRI) of the thoraco-lumbar spine revealed multiple cystic contrast-enhancing intraspinal lesions extending from C7 to S1  scan of the brain showed marked ventricular dilatation but no intraparenchymal lesions or intraventricular cysticercal lesions Figure . In addiPostoperatively, an anticysticercal agent  and an oral steroid were administered for 6 weeks. During the early postoperative period, the patient remained awake, alert, and oriented, although his muscle strength and occasional urinary incontinence were not improved. After the 6-month follow-up period, these symptoms remained, and the patient was unable to ambulate without assistance despite improvements in ventricular dilation Figure .T. solium. NCC typically involves the brain parenchyma, intracranial subarachnoid space, or ventricular system and can cause ventricular dilatation. The disease is often self-limiting unless hydrocephalus requires surgical intervention [et al.[NCC is the most common parasitic disease capable of invading the human central nervous system and is caused by encysted larvae of rvention . Intrasprvention -7. Callan [et al. reportedn [et al..et al.[Sotelo et al. reportedet al.. The freet al.. Acute het al.. TreatmeIn this case, brain imaging analysis showed symmetric dilatation of all 4 ventricles without any obvious cause in the brain. Therefore, the pathophysiology of hydrocephalus may have been secondary to chronic arachnoiditis due to cysticercosis . HoweverIn conclusion, isolated spinal NCC should be considered in the differential diagnosis of acute hydrocephalus when no explanation is found in the brain, particularly in regions endemic for cysticercosis.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.NCC: Neurocysticercosis; CT: Computed tomographic scan; MRI: Magnetic resonance imaging; CSF: Cerebrospinal fluid.The authors declare that they have no competing interests.SK and HW performed the diagnosis and surgery. SK and CJ drafted the manuscript and revised it critically for important intellectual content. CJ and DK made substantial contributions to the study conception and design. SK and DK granted final approval of the version to be published. All authors read and approved the final manuscript.


A possible association between Friedreich's ataxia (FA) and neoplastic development has been recognized. FA patients have low frataxin levels and insufficient response to oxidative stress. In these patients fibroblasts are characterized by a high rate of mutations. Herein, a case of a 39-year-old woman with FA tetraplegia, who was admitted in our department with intestinal obstruction due to a huge uterine tumor, is described. An abdominal CT revealed a huge intra-abdominal mass originating from the right cornu of the uterus. Tumor excision and adhesionlysis were performed. The histological examination of the tumor revealed a leiomyoma. FA patients seem to present with a variety of neoplasms uncommon for their young age. This is the first report of a leiomyoma originating from the genital system in a female patient with FA tetraplegia. Therefore it is important to identify neoplasms at an early stage in patients with FA and start immediate therapy. Friedreich's Ataxia (FA) is an autosomal recessive disease of the cerebellum, spinal cord, and peripheral nerves described by Friedreich in 1863 –4. AlthoA 39-year-old white female patient was admitted to the emergency department of our hospital with symptoms of acute intestinal obstruction, first noted 48 hours before . The patient was tetraplegic due to Friedreich's disease diagnosed 25 years ago and was hospitalized permanently in a center for chronic disorders.Upon physical examination, a prominent abdominal mass distorting her trunk shape was noted. In palpation, the abdomen had considerable distension with diffuse tenderness and moderate rebound tenderness. Despite the distension in the upper abdomen, a huge tumor could easily be palpated occupying mainly the lower abdomen.The neurological examination revealed good responsiveness of the patient, quadriplegia, loss of tendon reflexes, and positive Babinski sign bilaterally. She was then referred for cardiac evaluation, where mitral insufficiency and left ventricular hypertrophy were found. μL with 79.1% neutrophils, which was further elevated the second day; urea was found 51 mg/dL and γGT 54 mU/mL. The rest of the blood tests were normal.The white blood cell count was 18,100/The most possible diagnosis was intestinal obstruction probably due to the tumor. An abdominal computed tomography was performed, which revealed a huge intra-abdominal mass originating most possibly from the right cornu of the uterus Figures . An emergency exploratory laparotomy followed with a midline incision. After the peritoneal cavity was opened an enormous circumscribed mass measuring 29.5 × 22 × 12 cm was revealed displacing the bowel to the abdominal periphery. The mass originated from the right cornu of the uterus. After lysis of the adhesions between the tumor and the greater omentum, the tumor was excised  and the The tumor, as well as the peritoneal washings, was sent for histological and cytological assessment, which did not reveal any evidence of malignancy. Histological assessment of the specimen revealed a uterine leiomyoma with increased mitotic activity. The postoperative course was uneventful, and the patient had no further complications. At present, two years after surgery the patient fares well and lives in a center for chronic disorders. FA is an autosomal recessive spinocerebellar neurodegenerative disease with a wide range of clinical signs and symptoms, the onset of which in the majority of the cases occurs during adolescence. Although FA is a rare condition, it is the most common hereditary ataxia. The disease results in a relatively low life expectancy, and therefore the survival beyond the forth decade is unusual . The neurological findings of the disease include limb and truncal ataxia, dysarthria, loss of lower-extremity tendon reflexes, and progressive corticospinal tract disorder as well as loss of vibratory and proprioceptive sensation. The disease has also nonneurological manifestations such as kyphoscoliosis, pes cavus, diabetes mellitus with insulin resistance and heart disease, atrial fibrillation with resultant tachycardia, and cardiomyopathy , 5. FA is caused by a pronounced lack of fraxatin, a mitochondrial protein of not well-understood function . The und Frataxin is a mitochondrial protein found predominately in the brain, heart, and pancreas. Although the main function of frataxin is still a matter of discussion , some ev Fraxatin not only protects tumor cells against oxidative stress and apoptosis but also acts as a tumor suppressor. Fraxatin expression is upregulated in several tumor cell lines in response to hypoxic stress, a condition often associated with tumor progression. The ability of tumor cells to maintain a balance between adaptation to hypoxia and cell death is mediated by hypoxia-inducible factors (HIFs), which regulate the expression of hypoxia-responsive genes . Therefo FA patients have low frataxin levels and insufficient response to oxidative stress . In this Recently, two enzymes involved in the tricarboxylic acid (TCA) cycle, fumarate hydratase (FH), and succinate dehydrogenase (SDH), which also plays a role in oxidative phosphorylation, have been shown to be tumor suppressors. Indeed germline mutations of FH predispose individuals to the development of uterine or cutaneous leiomyomas, whereas mutations in SDH cause hereditary paragangliomas and pheochromocytomas . On the  Although tumor development is not a typical manifestation of the disease, these patients seem to present with a variety of neoplasms uncommon for their age. To the best of our knowledge, six cases of neoplasias with FA have been described in the literature, but none of them was associated with leiomyoma. In summary, in 1986, Barr et al.  presente Although the question of a possible association between FA and malignancy in general was raised by Kidd and his colleagues , it has  However, it is important to identify tumor development at an early stage in patients with FA and start immediate therapy; therefore the clinician should be sensitive enough to detect them.The authors declare that there is no conflict of interests.


Mantle cell lymphoma is one of the several subtypes of non-Hodgkin’s lymphoma. Mantle cell lymphoma is the rarest of the subtypes, accounting for about 6% of all non-Hodgkin’s lymphoma cases in the United States and Europe. Lymphoid neoplasms of the urinary tract and male genital organs are relatively rare, accounting for less than 5% of extranodal lymphomas. We present a rare case of mantle cell lymphoma infiltrating the ureter causing pelvi-ureteric junction obstruction on tissue diagnosis.A 78-year-old Caucasian woman was referred to our department with right flank pain, pyrexia and features of a urinary tract infection. A nephrostogram revealed a grossly distended right pelvicalyceal system in a pelvi-ureteric junction obstruction pattern. She underwent an elective pyeloplasty after her acute management and the results of histological examination revealed mantle cell lymphoma.de novo or a transformation from her previous splenic lymphoma with villous lymphocytes.We describe a rare presentation of mantle cell lymphoma as a pelvi-ureteric junction obstruction. To the best of our knowledge, there has not been any previously published report of the above finding. Our patient had a history of a previous lymphoma but the aim of this manuscript is to highlight a possible presentation rather than determining whether the mantle cell lymphoma was  Mantle cell lymphoma (MCL) is one of the several subtypes of non-Hodgkin’s lymphoma. MCL is the rarest of the subtypes, accounting for about 6% of all non-Hodgkin’s lymphoma cases in the United States and Europe. It is the result of a malignant transformation of a B lymphocyte in the outer edge of a lymph node follicle, called the mantle zone. Those cells can spread through the lymphatics and blood to other lymph nodes or tissues such as the bone marrow, liver and gastrointestinal tract. MCL has the worst prognosis among lymphomas, with a median survival of approximately three to four years ,2.Lymphoid neoplasms of the urinary tract and male genital organs are relatively rare, accounting for less than 5% of extranodal lymphomas. We conducted a literature search of PubMed and MEDLINE using the keywords ‘ureter’, ‘mantle cell lymphoma’ and ‘lymphoma’. We found no reports of MCL involving the ureter.We present a rare case of MCL infiltrating the ureter causing pelvi-ureteric junction obstruction (PUJO) on tissue diagnosis.A 78-year-old Caucasian woman was referred to our department with right flank pain, pyrexia and features of a urinary tract infection. Part of her investigations included an emergency ultrasound scan. This suggested right hydronephrosis with debris, suggestive of infective material, but with no obvious obstruction, mass or calculi seen. In terms of her past medical history, she had a splenectomy 15 years ago for splenic lymphoma with villous lymphocytes (SLVL), and had undergone laparoscopic cholecystectomy, a left total hip replacement and hysterectomy. She had been under regular follow-up by the hematologists and been managed conservatively. Part of her follow-up with the hematologist included ultrasound and computed tomography scans, which suggested a long-standing right PUJO and some prominent lymph nodes in her porta hepatis Figures .9 cells/L), C-reactive protein level of 202mg/L and a creatinine level of 113μmol/L (with a baseline of 60μmol/L)  20 and CD79a, staining with markers B-cell lymphoma 2 and cyclin D2, weaker diffuse staining with CD5 and a low proliferation fraction, less than 5%, with marker Ki67. These features are consistent with MCL  chromosomal translocation in the deoxyribonucleic acid (DNA). The cause is unknown and no inherited predisposition has been identified [Ki-67 is an indicator of how fast cells mature: the lower the percentage, the lower the speed of maturity, and the more indolent the disease. In our scenario, our patient expressed less than 5% staining [She had previously been diagnosed with SLVL, after which she had a splenectomy. Unfortunately, it was unclear how this diagnosis was made. She had been asymptomatic until her admission for pyonephrosis and follow-up scans after her splenectomy attributed her right hydronephrosis to a ‘congenital’ PUJO.With good renal function demonstrated on her dimercaptosuccinic acid scan and her history of SLVL, one should be suspicious of attributing the finding to a congenital condition.SLVL is a lymphoproliferative disorder characterized by the presence in the peripheral blood of atypical B-lymphocytes. Clinical features include massive splenomegaly and absence of peripheral lymphadenopathy. In our patient, an incidental finding of splenomegaly led to her having a splenectomy for SLVL .In retrospect, we could argue that with good renal function on her previous imaging and with her history of SLVL, her right-sided hydronephrosis was secondary to the lymphoma process rather than to a congenital finding.de novo or a transformation from her previous SLVL.We describe a rare presentation of MCL as a PUJO. To the best of our knowledge, there has not been any previously published report of the above finding. Our patient had a history of a previous lymphoma but the aim of this manuscript was to highlight a possible presentation rather than determining whether the MCL was 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.IN and RZ performed the main authorship and data collection. RZ reviewed the literature. TL revised the manuscript. All authors read and approved the final manuscript.


We describe a case ofextramedullary tracheal plasmacytoma that wasincidentally discovered in a 73-year-old man on a PETscan performed for assessing the extent of coloncancer. CT scan showed the tumor; multiplanarreformation coupled with virtual bronchoscopy allowedproper treatment planning. The tracheal tumor wasresected during rigid bronchoscopy. Relevantinvestigations excluded multiple myeloma. Follow-up CTshowed persistent thickening of the tracheal wall, butthere has been no recurrence after one-yearfollowup. Extramedullary plasmacytoma (EMP) is a rare plasma cell malignancy described in soft tissues outside of the bone marrow. It arises in different sites in the body especially in the upper airway . The mosThis 73-year-old nonsmoker man had a history of colon cancer, for which he underwent a surgical treatment during autumn 2009. On a follow-up abdominal CT, a possible liver metastasis was discovered for which a PET scan was required which showed a localized and unique FDG uptake within the middle third of the trachea . He compThe patient underwent a CT scan of the chest with an acquisition performed at the end of inspiration. A mass, 15-mm in diameter, arising from the left anterior wall of the middle third of the trachea was confirmed. This exophytic tumor was well limited and grew in contact with the tracheal cartilage, without evidence of cartilage invasion. Coronal reformation showed the longitudinal extent of the plasmacytoma, location of its upper part was 8.5 cm distal to vocal cords, and its distal part was 3.5 cm above the carina . VirtualFiber optic bronchoscopy revealed a fleshy tracheal mass occluding approximately 40% of the tracheal lumen and located 10 cm distal to the vocal cords. Pathological examination of the biopsies suggested an extramedullary plasmacytoma. On protein electrophoresis there was a diffuse elevation in the gamma region, but the immunoglobulin levels were normal on immunoelectrophoresis, excepting a discrete elevation of IgG and IgM levels, without monoclonal band. Urine contained no Bence-Jones protein. Bone marrow biopsy showed an excess of plasma cells (4%), without dysmorphic cells. No lytic bone lesion was observed on plain radiography which eliminates multiple myeloma. The patient was treated endoscopically under general anesthesia using a rigid ventilating bronchoscope . The endoscopic intraoperative view showed an obstructive tracheal mass with a small insertion pedicle . The masTracheal tumors are uncommon and represent around 1 to 2% of all respiratory tract tumors . SquamouOn reported EMP cases, 80% of EMP are located in the upper aerodigestive tract and appear as a soft tissue mass , 8. TheyThe diagnostic approach of those tracheal tumors was completely modified by the introduction of CT scan as CT allows evaluation of the extent within the lumen, airway wall, and mediastinal structures . Two-dimTreatment of tracheal plasmacytomas remains controversial as radiotherapy , 10 or sEven if the prognosis of EMP seems to be better than that of disseminated myeloma, patients require careful monitoring and long-term followup as a local recurrence or a progression to multiple myeloma has been described in up to 20% of cases . The 5-yThis is, to our knowledge, the first reported case of tracheal extramedullary plasmacytoma discovered incidentally on a PET scan. The case demonstrates the value of CT scan for preoperative planning of endoscopic surgery. Annual followup is mandatory, not to mistake an evolution to multiple myeloma or a local recurrence.


Congenital melanocytic nevi are among the several known risk factors for the development of melanoma. Neurocutaneous melanosis is a rare, congenital, non-hereditary disorder characterized by the presence of multiple and/or giant congenital melanocytic nevi. It is a rare condition, with fewer than 200 cases reported in the literature. Its association with tuberous sclerosis complex, a form of the neurocutaneous syndrome, is an unusual finding which, to the best of our knowledge, has not been documented in the English literature so far. Herein we present the first case documenting such an association in a 16-year-old post-pubertal Indian girl.In this report, we describe the case of a 16-year-old Indian girl who presented to our hospital with swelling on the scalp which had progressed from the hairline to just above the left brow, causing mechanical ptosis. She was born with a black-pigmented triangular patch covered with hair over the scalp which had increased in size over a period of eight years after birth. An X-ray of her skull and ultrasonography revealed soft tissue swelling in the left temporofrontoparietal region. Magnetic resonance imaging of her brain showed the presence of 8.99 cm × 2.26 cm abnormal signal intensity involving the scalp, a few small tubers with cortical dysplasia in the left frontoparietal region with asymmetric dilatation, and the presence of calcified subependymal nodules within the left lateral ventricle. These findings were suggestive of tuberous sclerosis. A histopathological examination of the swelling was suggestive of congenital melanocytic nevi. The patient underwent surgery. Excision of the tumor with primary skin grafting was done, with the graft being taken from the medial aspect of the right thigh.This case warrants further research to provide concrete evidence of an association of neurocutaneous melanoma with tuberous sclerosis complex. Research should be conducted to prove whether this is an unusual association or a new syndrome. Also, similar cases in other parts of the globe should be documented, as they would provide substantial support for such an association. Congenital melanocytic nevi (CMN) are found in approximately 1% of newborn infants, but 90% of these nevi are very small. Giant congenital nevi (GCN), giant hairy nevi and nevocellular nevi represent a special group of melanocytic lesions that generally cover large areas of the body and carry a potential risk for the development of malignant melanoma .Neurocutaneous melanosis (NCM) is a congenital, non-hereditary disorder defined by the presence of multiple and/or GCMN associated with abnormal melanin deposits in the brain and/or leptomeninges documented by magnetic resonance imaging (MRI) or autopsy -6. It isA 16-year-old Indian girl presented to our hospital with swelling on the scalp. She was born with a black-pigmented triangular patch over the scalp covered with hair as well as multiple black hairy patches on her extremities, back, and most of her anterior trunk. The patch on the scalp had increased in size over a period of eight years after birth. Within the past three months, swelling had progressed from the hairline to just above the left brow, leading to unilateral mechanical ptosis. Upon applying pressure, there was a scanty, yellowish, non-foul-smelling discharge that was occasionally blood-stained and was associated with intense itching without any pain. Her sleep, appetite, bowel, and bladder were unaltered. Her menarche had begun at the age of 15 years, and her menstrual cycle was normal and regular. Her birth and developmental history were normal. The family history was negative for congenital nevi or melanoma.The patient's clinical examination revealed her to be afebrile, with a pulse rate of 64 beats/minute, a respiratory rate of 16 breaths/minute, and blood pressure of 120/70 mmHg. The examination of her cardiovascular system was normal. Her cranial nerve functions were normal, and no neurological deficit was found in the limbs. The plantar response in both the limbs was flexor. There was no evidence of meningeal irritation.A local examination revealed a single swelling of 10 cm × 8 cm on the left side of the scalp. The swelling extended anteroposteriorly from 3 cm in front of the left parietal eminence to just above the lateral part of the left supraorbital ridge. The transverse extension was from the line passing through the left parietal eminence to the right of the midline . Her serum urea and electrolyte levels were normal. A lumbar puncture revealed clear cerebrospinal fluid with a normal cell count and biochemistry and no growth on culture. Her liver and kidney function tests were within normal limits. Her electrocardiogram did not show any abnormal features.Routine blood investigations showed that her hemoglobin level was 11.6% and her total leukocyte count was 4200/mmAn X-ray of her skull (posteroanterior view) revealed a soft tissue swelling in the left temporofrontoparietal region. Ultrasonography of the swelling showed a large, homogeneous, hypoechoic, solid 9.4 cm × 6.8 cm × 1 cm lesion in her scalp in the left frontal region and partially extending into the parietal region on the left side. The underlying outer table of the skull and diploic spaces were normal with few hyper-reflective areas scattered throughout the lesion and distal shadowing raising clinical suspicions of tiny calcified foci. The interface between the lesion and the scalp was obscured. No significant color flow was noted on a color Doppler ultrasonogram. These findings were highly suggestive of a possible angiofibroma. Power Doppler ultrasonography showed a highly vascularized lesion.MRI of her brain (both plain and contrast images) was done by using a multi-echo, multi-planar technique, which showed the presence of a 8.99 cm × 2.26 cm abnormal signal intensity involving the scalp (subcutaneous plane within fat) in the left frontoparietal region. The signal intensity appeared isointense to hyperintense on T1-weighted MRI scans with few small tubers with cortical dysplasia in the left frontoparietal region and asymmetric dilatation of the left ventricle that were hypointense on T2-weighted MRI and fluid attenuated inversion recovery (FLAIR) images. Few foci were seen within the lesion, which appeared hyperintense on T2-weighted MRI and FLAIR images. The MRI impression revealed multiple intra-cranial lesions, which included a benign, homogeneously enhancing, subcutaneous scalp lesion in left frontoparietal region suggestive of angiofibroma; a few calcified subependymal nodules within the body of the lateral ventricles on the right side; a few small tubers with cortical dysplasia in the left frontoparietal region; and left maxillary sinusitis  which had gradually progressed in size to a cutaneous scalp tumor indicative of melanoma. NCM is known to be associated with the other NCSs, such as Sturge-Weber syndrome and von Recklinghausen's disease. Associations with Dandy-Walker complex, spinal lipomas, and arachnoid cysts have also been reported ,26. AbouThis case warrants further research to provide concrete evidence of an association of TSC with NCM. Research should be conducted to prove whether NCM associated with TSC is an unusual or new syndrome. Also, similar cases occurring in other parts of the globe should be documented, as they would provide substantial support for such an association. Genetic and molecular investigations with specific tumor markers should be conducted. However, in the present case, because of the paucity of facilities in the institution and the financial constraints on the patient, a detailed investigation could not be performed.Written informed consent was obtained from the patient and the patient's next-of-kin 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.SR, PK, and MMAS participated in the clinical diagnosis, sequence alignment, and drafting of the manuscript and made useful contributions to the review of the literature. GK, RJ, PK, and MMAS were the team of operating surgeons. PK and MMAS participated in writing the Discussion section. PK and MMAS and PJT helped in the revision of the manuscript. All authors read and approved the final manuscript.


PCR chimeric transcriptional products PAX3-FKHR and PAX7-FKHR were not found. Six months following testicular resection, the patient died of multiple metastases in the mediastinum, lung and right thigh.Sclerosing rhabdomyosarcoma (SRMS) is an infrequent variant of rhabdomyosarcoma characterized by extensive intercellular hyaline fibrosis. We report the case of a 37 year-old male with a 9 × 6 cm SRMS on the right elbow. Histologically, the tumor showed an abundant extracellular hyaline matrix with extratumoral vascular emboli and microscopic foci of fusocellular embryonal rhabdomyosarcoma (FRMS) separated by a fibrotic band from the sclerosing areas. One year later the patient presented with a right intratesticular tumor of 1.2 × 0.8 cm, which was reported as pure FRMS. Immunohistochemically, SRMS was positive only for MyoD1 and Vimentin and negative for Myogenin and Desmin. Both the elbow emboli with the extratumoral foci of FRMS and the intratesticular tumor were positive for Myogenin, MyoD1, Vimentin and Desmin. Using fluorescent  Rhabdomyosarcoma (RMS) is the most common sarcoma developed during childhood. It is a devastating tumor displaying characteristics of muscle differentiation. Rhabdomyosarcoma currently is classified into three main groups: (i) embryonal (ERMS), (ii) alveolar (ARMS) and (iii) pleomorphic (PRMS), each of which can show several significant clinical, morphological, molecular and prognostic differences. ERMS tumor cells, including its variants, botryoid, anaplastic and spindle or fusocellular cells (FRMS), often occur in infants and adults . In non-A 37 year-old male without significant previous medical history, presented in June 2007 with a solitary soft tissue mass in the posterior side of the right elbow, with rapid growth that did not infiltrate the bone or the skin. The patient underwent a tumorectomy with wide margins. Following histological study, he received three cycles of chemotherapy (CTX+VCR+Doxorrubicin). One year after tumorectomy, a "de novo" intratesticular tumor appeared in the right testis, not previously detected. Orchiectomy was subsequently performed. A second line of chemotherapy (Ifosfamide) followed, but without positive response. Eight months following the orchiectomy, the patient died with multiple metastases in the left thigh, lung and mediastinum. No autopsy was performed Figure ® (Sakura), embedded in paraffin, cut in 4 μm thick sections and stained with hematoxylin and eosin for routine histological examination. Immunohistochemical analysis was done on the paraffin-embedded sections using standard protocol. Primary antibodies were: MyoD1 , Vimentin , Myogenin , SMA (specific muscle actin) , Desmin , EMA , CD 99 , p57 , CD 56 , S100 protein , CEAp , CD31 , CD34 , MIB 1 , and pan-cytokeratin . Fluorescence in situ hybridization (FISH) and RT-PCR were carried out on the paraffin-embedded sections from the elbow and testicle tumors. For FISH we used the Poseidon Repeat Free FKHR (13q14) protocol . The probe used was FKHR  Break apart (POSEIDON). There are two critical regions: (i) critical region 1 (red), in which the distal FKHR gene region probe was directly labeled with Platinum Bright 550; and (ii) critical region 2 (green), in which the proximal FKHR gene region probe was directly labeled with Platinum Bright 495. Analysis was performed with a fluorescent microscope, and the software used for image interpretation was CW4000. RT-PCR was performed as previously reported [The tumors were processed in molecular fixative solution for Tissue-Tek Xpress Rapid Tissue ProcessorGrossly the surgical specimen from the elbow showed a 9 × 6 cms nodular solid tumor, white in color with a glistening surface, infiltrating the surrounding skeletal muscle.Histologically, the tumor had abundant esosinophilic hyalinizing sclerotic matrix, containing predominant small round cells growing in small nests of fascicular, pseudovascular cord-like or single file. In some areas a predominant perivascular tumor growth was observed. No microalveolar pattern was seen. Wreath-like tumor cells and strap rhabdomyoblasts were not identified. Mitotic activity was up to 4 mitotic figures/10 HPF. Coagulative necrosis occupied 10% of the tumor mass. On hematoxylin and eosin findings an initial diagnosis of sclerosing sarcoma, most probably epitheloid sclerosing fibrosarcoma was reached Figure .Immunohistochemically the tumor cells strongly expressed Vimentin, MyoD1, SMA and CD99, being negative for the remaining antibodies tested, including Myogenin and Desmin. The proliferation index measured with MIB 1 was about more than 25%-30% of tumor cells.FISH hybridization did not show FOXO1A: 13q14 translocation and the chimeric transcriptional products PAX3-FKHR and PAX7-FKHR were also not found . Immunohistochemically the cells stained positively for Vimentin, MyoD1, CD99, Myogenin, Desmin, SMA and WT-1. The proliferation index measured with MIB was about 20%. Again, FISH failed to show the FOXO1A: 13q14 translocation and the chimeric transcriptional products PAX3-FKHR and PAX7-FKHR were not found.After the histological diagnosis of the surgical specimen from the testicular lesion, a complete sampling of the sclerosing rhabdomyosarcoma from the elbow treated one year before was performed. An extensive histological study of the new samples demonstrated new histological findings, such as the presence of two large endovascular (venous) emboli and 3 minute foci of tumor  located in the non-tumoral soft tissue, next to the sclerosing rhabdomyosarcoma and apparently isolated from them by a fibrotic acellular band. Histologically, tumor cells from the emboli and extratumoral foci showed predominant fusocellular morphology. A few isolated round primitive cells without sclerosis and strap rhabdomyoblastic cells were also present. Immunohistochemically these cells expressed Vimentin, MyoD1, Myogenin, CD 99, SMA and Desmin, being histologically and immunohistologically identical to the testicular tumor studied one year later , and most frequently appeared as tumors of the extremities as well as the head and neck region -7; their9 years, As initially described by Mentzel and Katenkamp in 2000 [Immunohistochemically sclerosing rhabdomyosarcoma in adults shows a distinctive immunophenotype in the expression of muscle markers, characterized by positivity for MyoD1, negativity for Myogenin and variable expression for Desmin and Actin muscle specific ,7, as ocThe histogenic relationship between sclerosing rhabdomyosarcoma and the other types of rhabdomyosarcomas remains unclear although cytogenetic studies have suggested a link with embryonal rhabdomyosarcoma ,13,14.Previous histological studies have pointed-out that sclerosing rhabdomyosarcoma may be closely related to embryonal rhabdomyosarcoma. Intratumoral small foci of rhabdomyoblastic strap cells have been observed in cases of sclerosing rhabdomyosarcoma ,7,14. ThIn the present case we also observed spindle and rhabdomyoblastic differentiation, but our findings have two main differences with other cases previously reported. First, spindle or rhabdomyoblastic differentiation appeared only in extratumoral areas infiltrating preexisting normal tissue or embolizing peritumoral vessels. No fusocellular or rhabdomyoblastic differentiation was identified in the sclerosing rhabdomyosarcoma. Second, and the more important difference, the peritumoral and intravascular infiltrating cells growing in a predominant spindle cell pattern showed a similar histological, immunohistochemical and molecular profile than that of the cells from the testicular fusocellular rhabdomyosarcoma diagnosed one year latter.Fusocellular rhabdomyosarcoma is a type of embryonal rhabdomyosarcoma that was described by Mentzel et al. in 2006 . HistoloPrimary testicular spindle cell rhabdomyosarcoma is extremely infrequent in adults; only isolated cases have being described in the paratesticular region ,18.On the other hand, testicular metastasis from rhabdomyosarcoma is a very rare phenomenon, representing approximately only 1% of testicular tumors ,19. OnlyIf our case follows this rule, it further supports that testicular spindle cell rhabdomyosarcoma represents a metastases from the cells with spindle or rhabdomyoblastic differentiation infiltrating vessels or soft tissue observed in the peritumoral vicinity of the sclerosing rhabdomyosarcoma of the elbow. In our opinion, the reported case highlights the close relationship between fusocellular rhabdomyosarcoma and sclerosing rhabdomyosarcoma, supporting the hypothesis (at least in adults) that most probably sclerosing rhabdomyosarcoma represents a subtype or type of fusocellular rhabdomyosarcoma sharing a common origin.The authors declare that they have no competing interests.MM and CO conceived of the study and participated in the design of the manuscript and performed the histopathological examination. JAG carried out the molecular genetic studies and drafted the manuscript. All authors have read and approved the final manuscript.


Mycobacterium tuberculosis is rare and the diagnosis is frequently made by means of an esophageal biopsy during the evaluation of dysphagia. There are few cases reported in the literature.Esophageal involvement by We present two cases of esophageal tuberculosis in 85- and 65-year-old male Caucasian patients with initial complaints of dysphagia and epigastric pain. Upper gastrointestinal endoscopy resulted in the diagnosis of esophageal tuberculosis following the biopsy of lesions of irregular mucosa in one case and a sessile polyp in the other. Pulmonary tuberculosis was detected in one patient. In one patient esophageal stricture developed as a complication. Antituberculous therapy was curative in both patients.Although rare, esophageal tuberculosis has to be kept in mind in the differential diagnosis of dysphagia. Pulmonary involvement has important implications for contact screening. Tuberculosis of the esophagus is a rare condition, even in countries with a high incidence of tuberculosis (TB) ,2, and sMycobacterium tuberculosis infection in immunocompetent persons.We present two case reports of esophageal involvement by A Portuguese Caucasian man, 89 years old, with a history of hypertension and benign prostatic hypertrophy, started to experience dysphagia, epigastric pain and anorexia one month prior to presentation. He was treated with omeprazole and sucralfate without any improvement. He had a normal blood count, with an erythrocyte sedimentation rate of 16 mm . An upper gastrointestinal endoscopy was performed. This revealed congestion of the entire esophageal mucosa, mainly in the proximal portion (20-25 cm from incisors), with easy bleeding to the touch, some irregular mucosa (biopsy one performed) and, in the lower part of the esophagus, an irregular mucosa with nodular areas (biopsy two performed). Diagnostic hypotheses were esophageal cancer and esophagitis. The histological examination for biopsy one revealed heavy lymphocytic infiltrate and polymorphonuclear cells, with ulceration, without lesions of malignancy. The histological examinations of biopsy two showed esophageal mucosa with extensive ulceration, inflammatory lesions with epithelioid granulomas, and acid-fast alcohol resistant microorganisms on staining . An abdominal ultrasonography showed mild polypoid thickening in his gallbladder without calculus, with no pain to elective area compression and no other changes. An upper gastrointestinal endoscopy revealed a sessile 6 mm polyp with an irregular surface in the distal third of his esophagus, located 2 cm above the junction with his stomach  in a first phase lasting for two months, followed by a period of four to six months with two drugs (isoniazid and rifampicin). There are cases where treatment was successfully carried out with only three drugs for six months, excluding ethambutol ,13. SurgEsophageal strictures may result from external compression of the esophagus due to mediastinal or cervical lymph nodes as well as mediastinal fibrosis induced by tuberculosis. This condition results in long and narrow strictures that are difficult to dilate, and in which dilation may be associated with a higher rate of complications .Esophageal stenosis as a complication of esophageal tuberculosis is rare and there are few reports in the literature ,17. The Although rare, esophageal tuberculosis must be kept in mind in patients with dysphagia, especially in countries with high prevalence of tuberculosis, even in immunocompetent patients. Active pulmonary tuberculosis should be ruled out, since early recognition of this infection is very important for public health. Treatment of esophageal tuberculosis with antituberculous drugs is curative, although complications may sometimes occur as in our case. Finally, esophageal cancer must be included in the differential diagnosis of the endoscopic findings in this situation.Written informed consent was obtained from both patients for publication of this case series and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.JG was a major contributor in writing and revising the manuscript. RD was involved in drafting the manuscript and revising it critically for important intellectual content. AC and AA analyzed and interpreted the patient data regarding mycobacteriological examination. All authors were responsible for the diagnosis, treatment and follow-up of the patients whose case reports were described. All authors read and approved the final manuscript.


Synovial sarcomas (SS) account for 5–10% of soft-tissue sarcomas and typically arise in the para-articular regions of adolescents and young adults. Nonetheless, SS can occasionally occur in other regions of the body. Here, we present a first clinical literature report of a patient with an SS arising from the vaginal wall. A 40-year-old patient who presented a necrotic polypoid lesion, measuring 50 mm and extending from the external urethral meatus to the middle part of the anterior vaginal wall. The biopsy showed a poorly differentiated SS with abundant necrosis and a SYT-SSX1 mutation. A staging CT scan was negative for distant metastases. The patient, prior to the radical surgery, received neoadjuvant chemotherapy (ifosfamide and epirubicin) for three cycles. She underwent post-operative external radiotherapy and brachytherapy (50 Gy) due to close margins (<1 mm) in the pathologic specimen. She relapsed 11 and 16 months later with lung metastases, which, both times, were successfully removed by surgical resection. At 24 months from diagnosis, the patient is alive without further evidence of disease. In summary, in the presence of unfavourable prognostic factors, neoadjuvant chemotherapy could be the primary approach to reduce the tumour size and the risk of distant micro-metastases allowing a less aggressive radical surgery if the tumour is located in a non-extremity site. Hence, a multidisciplinary approach, if not influencing overall survival and disease-free survival, may improve the quality of life. In fact, in our patient we obtained a complete clinical control in the pelvis, avoiding pelvic exenteration with neoadjuvant chemotherapy. Synovial sarcomas account for 5–10% of soft-tissue sarcomas, which typically arise in the para-articular regions of adolescents and young adults. Nonetheless, SS rarely occur in the mediastinum, head and neck region, heart, lung and retroperitoneum. Here, we present a clinical literature report of a patient with a synovial sarcoma arising from the vaginal wall. The immunohistochemical, molecular and ultrastructural details of this case have been previously reported [A 40-year-old patient was referred to the European Institute of Oncology  with a diagnosis, from elsewhere, of a high-grade vaginal leiomyosarcoma. She was severely anaemic due to abnormal vaginal bleeding, and pelvic examination revealed a necrotic, friable and bluish polypoid lesion, measuring 50 mm longitudinally and extending from the external urethral meatus to the middle part of the anterior vaginal wall. A staging CT scan was negative for distant metastases.2) and epirubicin (90 mg/m2), q 21 days, for three cycles, obtaining a partial clinical response . She then underwent wide local excision of the anterior vaginal wall, with preservation of the external urethral meatus.Histopathological review of the original sections revealed a poorly differentiated synovial sarcoma, and the patient was treated with neoadjuvant chemotherapy, including high-dose ifosfamide  for close margins (<1 mm).Eleven months later the patient experienced three metastases to the right lung, which were treated with wedge resection. Five months later, three other metastases were found on the contralateral lung and were successfully removed by surgical resection.At 25 months from diagnosis, the patient is alive without further evidence of pulmonary or other metastases or local recurrence.Microscopic examination of this case revealed a high-grade, biphasic, poorly differentiated synovial sarcoma with abundant necrosis and mitoses (more than 10/10 high-power fields). The immunohistochemical findings were consistent with the diagnosis of a poorly differentiated synovial sarcoma, arising from the vaginal wall.The rearrangement of the SYT gene, characteristic of SS, was detected confirming the diagnosis of synovial sarcoma. A reverse transcriptase-polymerase chain reaction assay revealed a SYT-SSX1 fusion gene product of 158 bp.Synovial sarcomas constitute approximately 5–10% of all soft-tissue sarcomas . AlthougThe chance of a synovial sarcoma occurring in other body locations apart from the extremities is supported by the hypothesis that they do not arise from synovial tissue. In fact, it has been suggested that they originate from unknown stem cells that are capable of differentiating into mesenchymal and/or epithelial structures (an hypothesis that still needs to be confirmed) . BiphasiOther types of synovial sarcoma include leiomyosarcoma (which account for the most common histology in any single tumour series of the vagina), the spindle cell variant of squamous cell carcinoma and the rare mixed tumour of the vagina, either benign or malignant.Synovial sarcomas are characterized by the presence a specific genetic translocation, the SYT-SSX translocation t , which represents a specific diagnostic marker, being present in 95% of all SS cases. Its presence, as the only cytogenetic abnormality, suggests that it is the primary cause of synovial sarcoma.There are several subtypes, numbered SYT-SSX 1, 2 and 4 ,11, and An essential first step in the successful treatment of synovial sarcoma is adequate surgical resection with tumour-free surgical margins –14.However, in cases with unfavourable localization such as the head and neck, retroperitoneum, chest and female genital tract, chemotherapy and, perhaps, pre-operative radiation therapy should be initially given, with the aim of reducing the tumour volume, thus facilitating surgical resection. Our patient received primary chemotherapy with epirubicin and ifosfamide, with good partial remission. Other active regimens can include vincristine, actinomycin D and cyclophosphamide (VAC).It is interesting to point out that the combination of doxorubicin and ifosfamide induced a more significant responses compared to VAC regimens, as described in two other studies ,16.et al [After surgery, our patient received 50 Gy of radiotherapy, as the tumour margins were close (<1 mm) in the pathologic specimen. Given sarcoma's relative rarity, the precise doses of radiotherapy to achieve local control in microscopic and gross residual disease categories are not well known. However, Raney et al  usually The natural course of the disease is such that many patients will experience recurrence of the primary tumour and/or metastatic disease. The reported five-year overall survival rates range from 38% to 76% ,19 and fThe unfavourable prognostic factors, described in several studies, include a tumour size more than 5 cm, tumour localization in a non-extremity site, primary treatment outside a referral centre, a patient age of more than 25 years, the presence of tumour necrosis areas and bone invasion ,25 and tOur patient was more than 25 years old, had a tumour of more than 5 cm with abundant necrosis and poorly differentiated histology, a non-extremity site location and an SYT-SSX1 mutation. All these factors indicate an unfavourable prognostic outcome and can probably explain the aggressive behaviour of the tumour.In summary, in the presence of unfavourable prognostic factors, neoadjuvant chemotherapy could be the primary approach to reduce the tumour size and the risk of distant micro-metastases and allowing a less aggressive radical surgery if the tumour is located in a non-extremity site. Hence, a multidisciplinary approach, even if having no influence on overall survival and disease-free survival, may improve the quality of life. In fact, in our patient we obtained complete clinical control in the pelvis with neoadjuvant chemotherapy, so avoiding pelvic exenteration.


Congenital midline swellings of nose are encountered rarely, and nasal gliomas constitute about 5% of such lesions. Various theories have been suggested to explain the pathogenesis. Imaging preferably by MRI is mandated to study the extent and to rule out intracranial extension. Treatment is complete excision, and the approach depends upon the extent of the lesion and availability of expertise. We present the management of one such case of congenital intranasal glioma without any intracranial extension that presented as a septal polyp. A three-month-old male baby was brought by parents with complaint of a fleshy mass coming out of right nostril ever since birth. There was history of occasional nasal obstruction, and the mass was growing slowly. There was no history of epistaxis or any other nasal discharge. The baby was first in birth order, product of nonconsanguineous marriage and born at fullterm by a normal vaginal delivery. The family history was unremarkable. On general physical examination, the baby was playful and hemodynamically stable. Examination of nose revealed a lesion protruding through the right external nares like a polyp . The polCongenital midline nasal masses are rare anomalies that occur in about one in 20000–40000 live births . Nasal gThe term “nasal glioma” is a confusing misnomer as it implies a neoplastic condition, which it is not. It needs to be differentiated from glioma, which is a malignant tumor of the brain , 2. 60% The possible theories of development of nasal gliomas include the following: (a) sequestration of glial tissue of the olfactory bulb entrapped during cribriform plate fusion; (b) ectopic neural tissue cells; (c) encephaloceles with lost intracranial connection and meningeal continuity; (d) inappropriate closure of the anterior neuropore  , 4. In 1Clinically, these masses are firm in consistency, noncompressible, nonpulsatile, grayish or purple lesions. These masses can protrude through the nostrils and can be confused with a nasal polyp. The patient may suffer from nasal obstruction, epistaxis, cerebrospinal fluid (CSF) rhinorrhoea, nasolacrimal duct obstruction, epiphora, hypertelorism and cosmetic deformity .Histologically, these lesions are made up of astrocytic neuroglial cells interlaced with fibrous and vascular connective tissue  that mayCT scan or MRI forms the mainstay of investigation as fine needle aspiration cytology or excision biopsy carries a significant risk of meningitis or CSF leaks . CT scanThe clinical differential diagnosis of nasal gliomas includes several disorders, which can present as nasal masses , 8. Some The treatment of choice of nasal gliomas is complete surgical excision . GliomasNasal gliomas are rare congenital anomalies. It is mandatory to rule out intracranial extension by cross-sectional imaging, preferably by MRI before performing any invasive procedure. Surgical excision in the mainstay of treatment and the route depend upon the extent of the lesion and the available expertise.The authors declare that there is no conflict of interests.


Schwannomas are indolent benign lesions arising from schwann cells in the nerve. They are especially rare in the paediatric population. We report an interesting case of a swelling in the upper neck, which highlights an atypical presentation of this tumour, as well as the complex details of its clinical, radiological, and surgical management. Schwannomas are indolent benign lesions arising from schwann cells in the nerve. Their earliest description was at the start of the twentieth century, and they were referred to as neurinomas . They arA fourteen-year-old girl presented with a one-year history of an asymptomatic slowly enlarging mass on the right side of her neck. There was no history of hoarseness, nasal regurgitation or associated pain, fever, or trauma. Her medical history was significant for recurrent tonsillitis in early childhood, but the mass was found to be unresponsive to over three courses of oral antibiotics before being referred to our specialist centre for evaluation. On examination, she had a large six-by-four centimetre immobile solitary right-sided neck mass deep to the right sternocleidomastoid muscle with a smooth regular border. There was no associated palpable cervical lymphadenopathy. The patient's oropharynx revealed no displacement of the peritonsillar structures, and a further physical examination was non-contributory. Routine blood tests including LDH were normal. Ultrasound showed a solid, well-circumscribed homogeneous mass arising from within the fascial planes with low level Doppler venous flow. The mass was displacing the great vessels to the right . She wenThis patient was discussed at our joint radiology/surgical MDT, and a consensus was made to proceed with surgical resection of the mass. During surgery, there were two lymph nodes above the right internal jugular vein identified which were excised for histopathology. These were later confirmed to be reactive hyperplasia. The mass was identified posterior to the right internal jugular vein and mobilised with blunt and bipolar dissection medially off the carotid artery , and latPostoperatively the patient developed anisocoria and ptosis of her right eye. A diagnosis of Horner's syndrome was made, the description of which forms a vital part of the informed consent process prior to surgical resection. The final histology report confirmed a palisaded spindle cell lesion consistent with a nerve sheath tumour .Schwannomas are solitary benign indolent tumors that commonly occur in individuals with neurofibromatosis. Schwann cells are glial cells that myelinate the axons of nerve cells. When schwann cells proliferate out of control in a capsule, it is called a schwannoma. Though benign, they can sporadically undergo malignant transformation. They can become debilitating when the growing tumor compresses the nerve causing chronic severe pain ; howeverThe head and neck region is the most common site of origin of schwannomas, but they can develop from any peripheral, cranial, or any autonomic nerve that has a schwann sheath. In the head and neck, they arise medially from glossopharyngeal, vagus, accessory, or hypoglossal nerves or, as is this case, from the sympathetic chain. Laterally they may arise from the brachial or cervical plexus . SchwannThe cervical sympathetic ganglia are part of the sympathetic chain in the neck region and run longitudinally over the longus capitis and longus colli muscles, as far as the pre-vertebral fascia. They are comprised of three ganglia—the superior, middle, and the inferior ganglia. The superior ganglion is largest and lies at the level C2-4, the middle ganglion lies at the level of C6, while the inferior ganglion is variable in position. It can fuse with the first thoracic ganglion to form a stellate ganglion. In most cases reported in the literature, the SCSC arises from the superior or middle part of the sympathetic chain .Horner's syndrome is a common postoperative complication due to the close association between the nerve and the tumour, which makes surgical separation almost impossible in the majority of cases. There has been some evidence to support intracapsular enucleation in order to minimise the risk of nerve of origin palsies following resection . While cIn their case series of eight tumours, Colreavey and colleagues noted six benign and two malignant schwannomas. One of their eight cases (twelve percent) presented with a horner's syndrome and that malignant schwannomas were rare and often associated with familial disorders such as neurofibromatosis . The difIn summary, this particular case is important as it is the first reported case of a schwannoma of the cervical sympathetic chain in Ireland in the paediatric population. Secondly, it reiterates that the management does not differ between adults and children who present with these tumours and that complete surgical excision is the mainstay of treatment for both diagnostic and therapeutic purposes. The prognosis of these lesions is excellent, and recurrence is rare.


Introduction: Pericardial synovial sarcoma is an extremely rare tumor with poor prognosis. Timely diagnosis and aggressive multimodal management improves patient outcome. We present our experience of diagnosis and management of a young patient with monophasic synovial sarcoma arising from pericardium.Case: A 27-year-old man presented with dyspnea and cough of three weeks duration. Examination revealed sinus tachycardia, distant heart sounds and elevated jugular venous pressure. Chest X-ray showed widened mediastinum. Transthoracic echocardiogram (TTE) noted large pericardial effusion with tamponade physiology. Therapeutic pericardiocentesis yielded hemorrhagic fluid. Computed tomography (CT) of the chest showed persistent pericardial effusion and a left anterior mediastinal mass. Left anterior thoracotomy, pericardial window and left anterior mediastinotomy were done, revealing a well-encapsulated gelatinous tumor originating from the pericardium. Histology and immunohistochemical profile showed the tumor to be a monophasic synovial sarcoma. Fluorescent in-situ hybridization (FISH) was positive for SS18 (SYT) gene rearrangement on chromosome 18q11, substantiating the diagnosis. Work-up for metastases was negative. Neo-adjuvant chemotherapy with high dose ifosfamide led to substantial reduction in the size of the tumor. The patient underwent surgical resection and external beam radiation therapy (EBRT) post surgery. He had symptom-free survival for 8 months prior to local recurrence. This was managed with left lung upper lobectomy and follow-up chemotherapy with docetaxel. The patient is currently stable with an acceptable functional status.Conclusion: In patients with pericardial effusions of unknown etiology, multiple modalities of cardiac imaging must be employed if there is suspicion of a pericardial mass. CT and magnetic resonance imaging (MRI) are useful to evaluate for pericardial thickening or masses in addition to TTE. Treatment of synovial sarcoma is not well established. Surgery is the cornerstone of treatment. In non-resectable tumors, aggressive neo-adjuvant chemotherapy with ifosfamide followed by surgical resection and EBRT may lead to improved outcome. To the authors’ knowledge, only 20 cases of synovial sarcomas originating from the pericardium have been described in the English literature to date2. This type of tumor is known to have a poor prognosis with no clarity about the best approach in managing this disease.Synovial sarcomas are malignant soft tissue tumors in extremities with a predilection for juxta-articular location and are predominantly diagnosed in young adults. Synovial sarcoma arising from the pericardium is an extremely rare tumor. Although termed as synovial sarcoma, these tumors have now been recognized to arise from mesenchymal tissue and show epithelial differentiation3. Primary synovial sarcoma leading to tamponade is very rare, and to the best of our knowledge, has been reported only twice prior to our report5. We delineate the systematic approach to diagnosis using imaging modalities, histopathology and cytogenetic analysis. Due to its site of origin and histology, it was essential to distinguish this tumor from other soft tissue tumors, and cytogenetic analysis was crucial with regard to this. Although surgery is the cornerstone of treatment of this tumor, we used multiple modalities of treatment such as neo-adjuvant chemotherapy using high-dose ifosfamide and external beam radiation therapy (EBRT). Our patient had a good initial response to therapy and had improved survival compared to previous patients5. We believe that this case report and the discussion about diagnosis, management and interval monitoring for recurrence will add to the current literature and help in managing such cases in the future.This case report describes a young man who presented to us with a cough and dyspnea and was diagnosed with cardiac tamponade due to this rare tumor. Pericardial effusions and cardiac tamponade secondary to malignancy are mostly secondary to metastasesA 27-year-old Hispanic man with no significant prior medical history presented to the emergency room with shortness of breath and a cough for three weeks. He also complained of mild precordial chest pain and generalized fatigue. His family history was contained type 2 diabetes mellitus in both parents but reported no history of cancer or genetic diseases. His social history revealed that he worked as a gardener, was married with two kids, was a non-smoker, occasionally consumed alcohol and had no history of drug abuse. He gave no history of any psychological illnesses. A physical exam revealed sinus tachycardia, distant heart sounds and elevated jugular venous pressure and a normal blood pressure. Chest X-ray showed an impressive mediastinal enlargement. Transthoracic echocardiogram (TTE) showed a large pericardial effusion  and collin-situ hybridization (FISH) was done to confirm the diagnosis and to exclude Ewing sarcoma. It showed the presence of SS18 (SYT) gene rearrangement on chromosome 18q11, substantiating the diagnosis. Also the FISH assay for rearrangement or dosage abnormalities of the EWSR1 oncogene at Ewing sarcoma locus 22q12 was negative.Review of pathology on routine hematoxylin-eosin stain showed that the tumor was monophasic and was composed of numerous spindle to oval cells in a fascicular growth pattern. The cells showed scant cytoplasm, oval to spindle nuclei with granular chromatin and with inconspicuous nucleoli . The tum2/day intravenous over 3 hours for 4 days) and mesna (1200 mg/m2) repeated every 21 days between July and December 2012. On repeat CT imaging prior to planned surgical excision, the tumor had substantially decreased in size (> 50%) and the patient was noted to have elevation of left hemi-diaphragm indicating likely involvement of the left phrenic nerve.The patent was treated neoadjuvantly with high dose ifosfamide  lymph node was also resected and sent for pathology.2) every 21 days along with steroid premedication with dexamethasone (4mg) for 5 days prior to treatment. This resulted in minor improvement on repeat CT imaging, but more importantly there was no further symptomatic worsening. The patient is clinically stable 18 months after diagnosis.The resected mediastinal mass (6.5 × 4 × 3 cm) was confirmed to be a monophasic synovial sarcoma on histology and immunohistochemistry. The adjacent lung was not involved by sarcoma. The three lymph nodes resected along with the mass were benign with non-necrotizing granulomas. The level 5 lymph node also showed non-necrotizing granulomas and was negative for malignancy. One of the resection margins was positive for malignancy. The patient recovered well after the repeat thoracotomy and EBRT was initiated 1-month post surgery. He had no recurrence of tumor on CT imaging at the first and second three-month intervals post surgery. Chest CT repeated at 9 months  showed local recurrence. The patient was noted to have an anterior mediastinal mass extending to the left upper lobe of the lung and a left pleural effusion. There was no evidence for any distant metastases. The patient underwent resection of the mass and left upper lobectomy, left video-assisted thoracoscopy, drainage of pleural effusion and total decortication of left lung. Pathology confirmed the recurrence but the resection margins were free of malignancy. The pleural fluid was also negative for malignant cells. Chemotherapy was reinitiated with docetaxel  appears to be more common than the monophasic type (only spindle-cells) and the poorly differentiated type 12. Our patient had a monophasic variant with predominant spindle to oval cells in a fascicular pattern. Some areas showed myxoid background as well as large areas of necrosis and the tumor was mitotically active.Synovial sarcomas are malignant soft tissue tumors with an aggressive growth pattern. Although they are predominantly diagnosed in extremities with a predilection for juxta-articular location, in young adults, they have been reported to occur in almost any organ13.Various other mediastinal and pericardial tumors  can have a similar appearance to synovial sarcoma on histology. Further analysis using immunohistochemistry is commonly done to differentiate between them. The specimens of our patient were uniformly positive for Vimentin, BCL-2 and CD99  and showed focal positivity for EMA. We also noted absence of immunostain positivity for muscle markers , neural and melanoma marker (S-100), pankeratin (AE1/AE3), cytokeratin (CK5/6), CD57 and p63. This helped in arriving at a diagnosis of synovial sarcoma and to exclude other possible soft tissue tumors. In our patient, the tumor cells also stained positive for NSE and FLI1. FLI1 is a fusion gene that is usually positive in Ewing sarcoma, but some cases of synovial sarcoma also show FLI1 positivity17. Reverse transcription-polymerase chain reaction (RT-PCR) or FISH can both be used to detect this gene. FISH has a higher sensitivity for detection and a better success rate than RT-PCR17. In our patient, FISH assay was performed, and it showed the presence of SS18 (SYT) gene rearrangement on chromosome 18q11. Also, in our patient in view of the immunostain positivity for markers of Ewing sarcoma, the FISH assay was conducted for rearrangement or dosage abnormalities of EWSR1 oncogene at Ewing sarcoma locus 22q12, and the result was negative. This helped us to further confirm the diagnosis of synovial sarcoma and exclude Ewing sarcoma13.Despite arriving at a diagnosis based on histology and immunohistochemistry, in order to further confirm diagnosis, cytogenetic analysis was done in our patient. Cytogenetic analysis is increasingly being used, especially in diagnosing those tumors arising in unusual sites. Confirming the histological diagnosis is crucial in determining therapy. More than 90% of synovial sarcomas demonstrate the presence of a reciprocal translocation t  , and detection of this SSX-SYT fusion gene confirms the diagnosis2. One exception is the case report of a patient whose survival exceeded 14 years and involved recurrences and multiple surgical resections4. Surgical resection can lead to curative therapy. Most often, due to the site of origin, tumors are adherent to adjacent vital organs and are non-resectable at the time of diagnosis. Therefore, EBRT and less commonly neo-adjuvant chemotherapy are used to make the tumor resectable. In our patient, we could not attempt complete surgical resection. Therefore, after the initial biopsy and pericardial window, neo-adjuvant chemotherapy was done with six cycles of high dose ifosfamide (2000mg/m2) and mesna. Due to the rarity of these tumors, it is very difficult to establish an optimal regimen for chemotherapy. In general synovial sarcomas are chemo-sensitive to ifosfamide and show a good response. A case series of 13 patients with synovial sarcoma treated with high-dose ifosfamide showed a response in all patients, with 4 clinical remissions achieved18. Combining doxorubicin with ifosfamide may achieve a higher response rate (58%)19 but patient tolerability and side effects need to be considered. The initial favorable response to neoadjuvant high dose ifosfamide in our patient made the tumor resectable and may be a viable alternative to EBRT alone in non-resectable tumors. Guet al. have reported using the collagen gel droplet embedded culture-drug sensitivity test (CD-DST) prior to determining the therapeutic value of chemotherapy in their patient with synovial sarcoma20. It may be useful to have CD-DST as a screening method for the selection of effective anticancer drug regimen for each individual patient2.Management of synovial sarcoma of pericardial origin is challenging and needs a multimodal approach. It is an aggressive tumor with a poor prognosis. Most patients have a short survival time21. To the authors’ knowledge only EBRT has been employed in previous cases. Intraoperative radiation therapy (IORT) with a radiation dose of 15–45 Gray appeared to be useful for local control and to be more effective in patients without distant metastases at the first visit in a study that included 24 patients with soft tissue sarcomas 22. But, lack of institutional experience with IORT dissuaded us from using it in our patient. Close monitoring for recurrence is very crucial in management of this tumor. CT or magnetic resonance imaging should be performed every 6 months to detect subclinical recurrences4. We performed CT imaging every three months along with regular clinical follow-up. Repeat imaging is essential to be able to diagnose subclinical recurrence and initiate repeat broad surgical resection and follow-up chemotherapy if needed. We used the anti-mitotic agent docetaxel as well as left lung upper lobe resection for treating the recurrence. Adjuvant therapy with single agent docetaxel is not considered standard therapy for synovial sarcoma, and adjuvant therapy after resection of metastases is also controversial. Our patient was treated with docetaxel for his local recurrence based on evidence showing that docetaxel is an efficacious and tolerable treatment as rescue medication in ifosfamide-resistant locally advanced or metastatic soft tissue sarcoma23.EBRT is a very useful modality of treatment post surgery when the margins of resection are positive. In our patient, EBRT was initiated one month after surgery. Previous reports have shown similar favorable resultsThis case report describes a case of monophasic synovial sarcoma of pericardial origin presenting as pericardial effusion with cardiac tamponade physiology in a young man. It is an extremely rare tumor, and systematic approach to diagnosis and management are crucial. We emphasize the use of cytogenetic analysis in confirming the diagnosis and excluding tumors that might mimic synovial sarcoma on immunohistochemistry. This case also highlights the use of neo-adjuvant chemotherapy using high dose ifosfamide as part of multimodal approach to treatment.Written informed consent for publication of clinical details and clinical images was obtained from the patient. The changes are appreciated and the format is appropriate.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. The changes made were satisfactory.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. This is an interesting paper and is well written. It would definitely help future clinicians who might encounter this rare diagnosis. However I think that for the benefit of these clinicians the authors should include more details on the chemotherapy regimen, including the number of days ifosfamide was administered for and the frequency of the cycle. More details about the surgery and radiation would also be helpful.I have read this submission. I believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. In this case report, the authors offer a clinicopathologic description of a case of pericardial synovial sarcoma.  The patient was treated neoadjuvantly with "high dose" ifosfamide, but the authors describe using 2000 mg/m2 per cycle, which is actually considered quite a low dose for treatment of sarcomas. This is either an error  or a very low dose was used intentionally. Regardless, the patient apparently benefitted with some tumor shrinkage, which allowed for surgical resection.  A description of the surgery is lacking. We do not know how much pericardium was removed or if lymph nodes were sampled, as sarcomas are known to metastasize lymphatically as well as hematogenously.  Although postoperative EBRT was administered, there is no discussion about whether intraoperative XRT or proton therapy was considered.  Upon disease recurrence with a mediastinal mass and pleural effusion, a lobectomy and decortication was performed. We are not told if malignant cells were seen in the pleural fluid, in which case the entire pleura would be considered contaminated.  Post-op, the patient was treated "adjuvantly" with docetaxel. Here, the authors need to be careful to point out that single agent docetaxel is not considered standard therapy for synovial sarcoma, and adjuvant therapy after a metastasectomy is also quite a controversial strategy. It would be wrong if readers interpreted this report as an endorsement of post-op single agent docetaxel after a metastasectomy. In summary, it is not clear what we learn from this case report except that the patient apparently sustained a partial tumor response to ifosfamide chemotherapy, tolerated an R1 or R2 surgery, and despite post-op radiation, sustained a regional/metastatic recurrence. A more thorough discussion about the complexities and controversies of clinical decision making in patients such as this would make such case reports more informative to the audience.There are several major points that need to be addressed:I have read this submission. I believe that I have an appropriate level of expertise to state that I do not consider it to be of an acceptable scientific standard, for reasons outlined above. et al., 2007Maki RG). It is unclear why patient was only given docetaxel. This is a very well written article, and I think it makes a positive contribution to our current knowledge of cardiac sarcomas. The authors appropriately mention that these tumors are rare and no standard of care exists. However, this patient in the relapse setting was treated with single agent docetaxel. In phase II trials of soft tissue sarcomas, docetaxel has been used in combination with gemcitabine due to their synergistic effect .et al.Maki (2007) showed that gemcitabine-docetaxel yielded a better response compared to gemcitabine alone when used for initial treatment rather than as a rescue treatment option. We are of the opinion that although our patient had a favourable response, it may be useful to have CD-DST as a method for the selection of an effective anticancer drug regimen for each individual patient for both primary treatment and to select the rescue chemotherapy.We thank Dr. Mukhi very much for his review and approval of our case report. Our patient was treated with docetaxel for his local recurrence based on evidence showing that docetaxel is an efficacious and tolerable treatment as rescue medication in ifosfamide-resistant locally advanced or metastatic soft tissue sarcoma (


Lymphoma is the most common malignancy of the spleen. The imaging features of splenic lymphoma are nonspecific and mostly lymphomas present as a diffusely enlarged spleen. Focal lesions are described but remain of low density or intensity on CT or MRI, respectively. We describe a histologically proven case of splenic lymphoma that showed an atypical hyperdense/hyperenhancing appearance on imaging suspicious for a vascular pathology. To the best of our knowledge and based on review of English literature, such an appearance of splenic lymphoma is extremely unusual and rare. Lymphoma is the most common tumor of the spleen and it may be primary or secondary to a diffuse lymphomatous process . Splenic3/mm3, and platelet count of 70 × 103/μL. Peripheral blood smear did not show any abnormal lineage cells.A 60-year-old Caucasian male presented to the ER with one-month history of weakness, fatigue, and left upper quadrant pain. Past medical history was significant for diabetes Mellitus, hypertension, and hyperlipidemia. Patient worked in a fertilizing chemical business with history of exposure to chemicals and pesticides over the last 2 decades. Physical examination was remarkable for pallor and splenomegaly extending 7-8 cm below costal margin. Laboratory analysis showed hemoglobin of 9 g/dL, hematocrit 28%, white count 4.0 × 10Noncontrast CT abdomen showed massive splenomegaly with multiple hyperdense foci within the spleen  revealed Bone marrow biopsy, however, revealed a B-cell lymphoma and an initial staging PET scan showed massively enlarged spleen with increased FDG uptake  with an CT is the most widely used and currently the imaging modality of choice for assessment of lymphoma. The overall accuracy of CT in depicting splenic lymphoma is approximately 22–65% , differeConventional T1- and T2-weighted MR sequences have poor sensitivity for splenic involvement in lymphoma, which is due to the similar relaxation times of lymphoma and normal splenic tissue using these sequences . IdentifThe differential diagnoses based on CT and MRI imaging characteristics involved pathologies with a high vascular content, namely, splenic peliosis, cavernous atypical hemangioma, or an angiosarcoma. The bone marrow biopsy obtained given the history of chemical exposure and the finding of pancytopenia, however, showed B cell lymphoma. This was confirmed by PET scan SUV 6.23) and splenic pathology when patient presented with a splenic rupture. In a study of 68 patients with known malignant disease, an SUV threshold of 2.3 correctly differentiated benign from malignant lesions with the sensitivity, specificity, PPV, and NPV of 100%, 100%, 100%, and 100%, respectively [.23 and sIn conclusion, splenic lymphoma could present with imaging features simulating a vascular malformation or tumor and should be considered in differential of a multifocal hypervascular lesion, in an appropriate clinical setting.


Autoimmune hypophysitis very rarely spreads to nearby organs outside the pituitary tissue, for unknown reasons, with only 5 reported cases of hypophysitis spreading over the cavernous sinus.Three patients presented with cases of non-infectious hypophysitis spreading outside the pituitary tissue over the cavernous sinus. All three cases were diagnosed with histological confirmation by transsphenoidal surgery, and the patients showed remarkable improvement with postoperative pulse dose steroid therapy, including disappearance of abnormal signal intensities in the bilateral hypothalami on magnetic resonance imaging, resolution of severe stenosis of the internal carotid artery, and normalization of swollen pituitary tissues. Two of 3 cases fulfilled the histological criteria of immunoglobulin G4-related disease, although none of the patients had high serum immunoglobulin G4 level.The true implications of such unusual spreading of hypophysitis to nearby organs are not fully understood, but the mechanism of occurrence might vary according to the timing of inflammation in this unusual mode of spreading. Pulse dose steroid therapy achieved remarkably good outcomes even in the patient with progressive severe stenosis of the internal carotid artery and rapid visual deterioration. Autoimmune hypophysitis is classified into subtypes based on the histological findings into granulomatous hypophysitis, lymphocytic hypophysitis, and xanthomatous hypophysitis, or based on the site of involvement or endocrinological behavior into adenohypophysitis, infundibulo-neurohypophysitis, and panhypophysitis. DefinitIn this report we describe three cases of autoimmune hypophysitis spreading to nearby organs from outside the pituitary tissue over the cavernous sinus, and discuss the limitation and problems underlying the classification of hypophysitis from the histological and endocrinological aspects.A 60-year-old woman was referred to our hospital because of drastically decreased visual acuity of the left eye. She had been treated for previous hypothyroidism with 100 μg levothyroxine daily. On admission, neurological examination detected three-fourths visual field defect of the left eye with severely decreased visual acuity. Endocrinological examinations showed low levels of serum cortisol and thyroid-stimulating hormone (TSH), and hyperprolactinemia . She received systemic chemotherapy with bleomycin, etoposide, and cis-diamminedichloroplatinum, and irradiation to the tumor bed (20 Gy). After completion of the initial treatment, MR imaging revealed complete resolution of the tumor. She had remained in good condition with hormonal replacement therapy, including dexamethasone, levothyroxine, and desmopressin acetate daily. No abnormal findings were seen at follow-up MR imaging examinations, which were performed every 6 months thereafter. Six years after the first therapy, she suffered headache and easy fatigability. Endocrinological examinations showed panhypopituitarism and hypothyroidism Table .Second surgery was again performed through the transsphenoidal approach. Intraoperative rapid diagnosis suggested inflammatory disorder with massive infiltration of lymphocytes in the pituitary tissue, dura mater, and extradural sphenoidal mucosa, so surgery was limited to biopsy. Pulse dose hydrocortisone treatment was started from the day of the surgery using intravenous 200 mg hydrocortisone initially and tapered gradually. Histological examination of the biopsy specimen showed massive infiltration of inflammatory cells including lymphocytes in both the internal and external tissue of the dura mater. Staining for IgG4 was positive in the cytoplasm of approximately 40% of invaded plasma cells Figure . The hisLymphocytic hypophysitis spreading to nearby organs is rarely reported. However, most cases are located in the cavernous sinus, and are only presumed diagnoses based on the clinical symptoms such as diplopia-17. SpreVery few reports have described hypophysitis spreading to the surrounding structures over the cavernous sinus. Consequently, the current classification cannot accommodate any of our 3 patients into the accepted categories of adenohypophysitis, infundibulo-neurohypophysitis, or panhypophysitis. In contrast, non-bacterial inflammatory spread to multiple organs sometimes occurs from extracranial lesions, and IgG4-related pancreatitis frequently spreads to extrapancreatic organs such as the bile ducts, collecting renal duct, or retroperitoneal space. The undTranssphenoidal surgery has been performed as the treatment for hypophysitis in most cases, and total removal of the inflammatory process continues to be recommended for patients with visual deterioration. Steroid therapy was found to be less effective in some cases, but a sThe present three cases of autoimmune hypophysitis spread outside the pituitary tissue with severe fibrosis. Pulse dose steroid therapy achieved remarkably good outcomes even in the patient with progressive severe stenosis of the internal carotid artery and rapid visual deterioration.The therapeutic protocol was approved by the internal ethics committee of Kohnan Hospital 2012.Written informed consent was obtained from the three patients for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.IgG4: Immunoglobulin G4; MR: Magnetic resonance; TSH: Thyroid-stimulating hormone.The authors declare that they have no competing interests.AK analyzed the patient data regarding the endocrinological outcome, and was a major contributor in writing the manuscript. YO performed transsphenoidal biopsy of all the patients. MW performed pathological examinations. TK performed tumor removal in the first surgery of Case 3. TT gave an essential suggestion and supervised this manuscript. All authors read and approved the final manuscript.