Supratentorial primitive neuroectodermal tumors predominantly occur in children, and are rare in the adult population. Less than 100 cases of supratentorial primitive neuroectodermal tumor have been reported in adults internationally. Our case study reports this rare incident.A 22-year-old Hispanic man presented with headaches, blurry vision, diplopia, intermittent vomiting, and grossly decreased vision. A magnetic resonance image showed a left posterior parietal heterogeneously enhancing mass measuring 4.2cm × 7.2cm × 7.0cm. After craniotomy for resection and decompression, the mass was histologically revealed to be a supratentorial primitive neuroectodermal tumor. Standardized immunohistochemical studies for this mass were carried out.We have concluded that immunohistochemical and genetic workup should be included in the standardized pathological workup for primitive neuroectodermal tumors in order to provide more prognostic information. Based on our current literature review, we propose an immunohistochemical panel. Supratentorial primitive neuroectodermal tumors (sPNETs) are known to be tumors of the pediatric population. According to the World Health Organization, sPNETs are described as a cerebral or suprasellar embryonal grade IV tumor made up of undifferentiated or poorly differentiated neuro-epithelial cells which have the capacity for, or display, divergent differentiation along neuronal, astrocytic, ependymal, muscular or melanocytic lines. Approximately 1% of pediatric brain tumors are sPNETs. However, they can sporadically occur in adults. To date, only less than 100 sPNET cases have been reported in adult patients [et al., it was determined that the mean age of diagnosis in adults was 35.2 years of age, with one peak between the ages of the second and third decades. Throughout all age groups, more males were prone to be diagnosed with pNETS than females [In an extensive case review series by Ohba  females .Of the PNETs in the central nervous system (CNS) 5.6% are supratentorial. Moreover, the locations of these tumors are almost equally distributed in the frontal, temporal, and parietal lobes . Less thHistologically, the highly cellular tumor consists of anaplastic cells with small round to oval hyperchromatic nuclei surrounded by scanty cytoplasm Figure . After aA 22-year-old Hispanic man was referred to a neurosurgery clinic by his ophthalmologist, who was concerned about increased intracranial pressure after noticing papilledema on fundoscopic examination. The patient had been complaining of headaches, blurry vision, diplopia, and intermittent vomiting for a month. He reported that his symptoms had been worsening over the past week. The patient has no other significant past medical history and has been in general good health besides the recent onset of headaches. He was a resident of California. Furthermore, on physical examination, left horizontal nystagmus and grossly decreased vision on the right temporal and right nasal hemifield were noticed.A brain MRI revealed a left posterior parietal, irregular, hemorrhagic mass measuring approximately 4.2cm × 7.2cm × 7.0cm, enhancing heterogeneously, and associated surrounding vasogenic edema and a 8.7mm midline shift from left to right. Compressions of the atrium of the left lateral ventricle, and the third ventricle, as well as some effacement of the quadrigeminal plate cistern, particularly on the left were noted  is phenomenal in that it is unique to central and not peripheral PNETs. Thus, the usage of immunohistochemical assay for CD99 and fluorescence in situ hybridization (FISH) assay for the  translocation have granted the capability to distinguish between intracranial peripheral and intracranial central PNET [et al., it has been suggested that mutations in IDH1, which encodes for cytoplasmic isocitrate dehydrogenase, are among the most frequent mutations in sPNET, and are much more prevalent in adult cases in comparison to pediatric cases [et al. have shown that a high incidence of TP53 mutations and the absence of amplification of the c-myc/N-myc genes in adult sPNET, in comparison to pediatric PNET, suggest that adult sPNET should be an independent subset of tumors among CNS-PNETs [Therapeutic approaches are close to non-existent because the pathological mechanisms underlying sPNETs are poorly understood. Some of the proposed genetic alterations include: isochromosome 17q; losses of chromosomes 6q, 9q, 10q, 11, and 16q; trisomy-1q; and mutations of ic cases . FurtherEven though no prospective treatment studies have been conducted to date, and no optimal treatment regimen for sPNET has been established, a complete surgical excision followed by radiation therapy has been proved to be crucial in many retrospective studies -12. RadiAn extensive review of the current literature suggests that immunohistochemical and genetic assays are becoming essential markers for prognosis and possible treatment of sPNETs. As we discover more about all the mentioned studies, it will become essential to integrate them into our standard pathological workup for brain masses in adults that are highly suspicious to be PNETs based on radiographic studies and preliminary intra-operative pathological diagnosis.IDH1  to differentiate adult sPNET from other possible pediatric PNETs. Furthermore, an evaluation of TP53 mutations (high incidents in adults) and c-myc/N-myc genes (absence of amplification in adults) should be evaluated in order to differentiate between a true sPNET and any pediatric PNETs. Subsequently, a CD99 and a FISH assay for the  translocation  should be conducted in order to distinguish between intracranial peripheral and intracranial central PNETs, because the prognosis of central and peripheral PNETs are drastically different, as mentioned previously.We propose immunohistochemical and genetic assays panels, which to the best of our knowledge has not been proposed in the literature before, and we have decided to use this panel in our institutions. We believe that initially a genetic assay should evaluate any potential mutations to Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.SL was the physician for the patient in the manuscript and was a contributor in writing the manuscript. OH was a major contributor in writing the molecular neurobiology segments of the manuscript, as well as the main editor. DM was the chief editor of this manuscript. JA and TM have also contributed to the various editorial comments and to preparing the manuscript, and histological examination. JS contributed to the surgical segment of the manuscript and was the main attending neurosurgeon for this case. RG contributed the neuro-radiology included with the manuscript. All authors read and approved the final manuscript.


The primary and metastatic gastrointestinal synovial sarcoma is rare with a wide differential diagnosis. It usually expresses cytokeratins EMA, BCL2 with an occasional CD99, and S100 positivity but not desmin. We present a case of metastatic synovial sarcoma with unusual immunophenotype causing diagnostic challenges. The tumor cells showed focal cytokeratin, EMA, and, unexpectedly, desmin positivity. Additional intranuclear TLE-1 positivity and negativity for CD34 and DOG-1 were also identified. A diagnosis of monophasic synovial sarcoma was confirmed by using FISH break-apart probe. RT-PCR revealed the SYT-SSX1 fusion gene. Intra-abdominal synovial sarcoma, either primary or metastatic, with unusual desmin positivity raises the diagnostic challenge, since a wide range of differential diagnoses could show a similar immunophenotype . Typical morphology and focal cytokeratin/EMA positivity should alert to this tumor, and FISH and RT-PCR remain the gold standard for the confirmation. Synovial sarcomas are rare, unique spindle cell tumors probably of mesenchymal cell origin . They ocThis is a consulting case of a 26-year-old Hungarian male patient presenting with an intra-abdominal mass. The computed tomography (CT) showed a relatively circumscribed retroperitoneal lesion measuring 6.3 × 5.7 cm in size. The lesion was closely attached to the pancreatic body, spleen, a segment of colon, and most of the stomach. En bloc resection including distal pancreatectomy, subtotal gastrectomy, splenectomy, and partial colectomy was carried out. Macroscopically a well-circumscribed mass measuring 6.5 × 5.5 × 5.0 cm in size was found in the retroperitoneal region with adjacent gastric and colonic wall invasion. It showed a grayish-white cut-surface with focal punctate hemorrhage. The spleen was nearby but free of tumor grossly.Histologically the tumor showed spindle to ovoid cells with relatively bland nuclear features forming dense cellular sheets and vague fascicles. Mitoses are numerous . Focal a The postoperative condition of the patient was stable and no further recurrent tumor was found up to the recent followup. Synovial sarcoma, a misnomer of possibly mesenchymal cell-derived soft tissue tumor, accounts for 5 to 10% of soft tissue sarcomas with 80% of the cases affecting the extremities around the large joints . Neverth Similar ancillary approaches can be applied to differentiate from myogenic tumor; nevertheless, desmin is usually negative in synovial sarcoma . It is k We reported a case of a metastatic intra-abdominal synovial sarcoma from a 26-year-old man. The histological study showed a monophasic pattern. The tumor cells demonstrated diffuse intranuclear TLE-1 with focal EMA and cytokeratin positivity and DOG-1 negativity. Unusual desmin expression was identified. Fluorescent in situ hybridization and polymerase chain reaction confirmed the diagnosis, with detection of the t translocation and SYT-SSX1 fusion gene, respectively. Intra-abdominal synovial sarcoma, either primary or metastatic, with unusual desmin positivity raised the diagnostic challenge, since a wide range of differential diagnosis could show similar immunophenotype. Typical morphology and focal cytokeratin/EMA positivity should alert to this tumor, and TLE-1, a relatively sensitive marker for synovial sarcoma, should be used to avoid the diagnostic dilemma. FISH and RT-PCR remain the gold standard for the diagnostic confirmation.


Yolk sac tumor  is a rare malignant germ cell tumor arising in the testis or ovary. Extragonadal yolk sac tumor is even rarer and has only been described in case reports. Due to the rarity of the tumors, the appropriately optimal treatment remains unclear. We report a case of yolk sac tumor in the seminal vesicle.A 38-year-old Asian male presented with gross hematuria and hemospermia. Transrectal ultrasound scan showed a solid mass in the left seminal vesicle and the scrotal sonography showed no abnormalities. Bilateral seminal vesicles were resected, and histopathological examination showed a typical pattern of yolk sac tumor (YST). The patient responded poorly to comprehensive treatment of radiotherapy, chemotherapy and surgeries, developed systemic multiple metastases, and died of cachexia one and half years after diagnosis. Yolk sac tumor  most frequently occurs in the gonads and has a poor prognosis, if not treated aggressively .Extragonadal yolk sac tumor (YST) is rare, and the most common sites of origin are, in order of frequency, the mediastinum, the retroperitoneum, the sacrococcygeal region and the pineal gland . Due to 2 for four consecutive days), and etoposide (100 mg/m2 for four consecutive days). After the chemotherapy, serum AFP was found to be decreased to 58.09 ug/l, and the symptom of hemospermia disappeared.A 38-year-old Asian male presented to his urologist with gross hematuria and hemospermia of a half-year duration. The remainder of his physical examination, medical history and surgical history were unremarkable. He had no history of cryptorchidism, testicular swelling or testicular atrophy, and no family history of testicular cancer. A transrectal ultrasound scan performed on the day of presentation showed a solid mass of the left seminal vesicle. A puncture biopsy of the left seminal vesicle confirmed diagnosis of germ cell tumor. No tumorous lesions were found in other sites. At presentation, levels of serum tumor markers were obtained: α-fetoprotein (AFP) was found to be elevated to 1,740.9 ug/l ; serum β-human chorionic gonadotropin (β-hCG)  and prostate-specific antigen (PSA)  were within normal range. The patient was given four cycles of chemotherapy consisting of bleomycin , cisplatin  reported an abnormal signal mass in the left seminal vesicle Figure . Positro2 for five consecutive days), and etoposide (100 mg/m2 for five consecutive days). On completion of the chemotherapy, serum AFP was found to have decreased to 53.24 ug/l and remained stable. However, at week 6 after chemotherapy, serum AFP again elevated to 184.9 ug/ml. Positron emission tomography (PET) of the pelvis revealed lymph node metastases in the region of the left iliac vessels. Thus, resection of the pelvic lymph nodes was performed. Enlarged lymph nodes were not found in the areas of the external iliac, internal iliac and obturator. The left side of the prostate was fixed to the pelvic wall. Histological evaluation of the specimens exhibited proliferation in the left obturator lymph tissues but showed no evidence of lymphatic metastasis. Fibrous connective tissue outside the bladder was infiltrated by lymphocytes.Histological evaluation of the resected specimen exhibited a malignant tumor composed of neoplastic cells proliferating in a microcystic or reticular pattern of growth. The neoplastic cells had highly atypical middle- or large-sized nuclei, with Schiller-Duval bodies partially mimicking papillary structures Figure . ImmunohOn the postoperative Day 3, adjunct radiotherapy was introduced, but the response was not good, with AFP remaining at a high level since then. The patient presented rapid multiple systemic metastases and died of cachexia one and half years after diagnosis.YST is a malignant germ cell tumor that usually arises in the gonads and is characteristically accompanied by an elevation of the serum α-fetoprotein level. YST is rarely found extragonadally. Case reports have described the occurrences of this entity in the pineal gland, head and neck, mediastinum, lung, stomach, liver, omentum, retroperitoneum, prostate, intrarenal, vagina, vulva, urachus and presacral regions -6.The typical morphologic structures of endodermal sinus pattern known as sinuses of Duval, Schiller-Duval bodies, or glomerulus-like structures, superficially resemble immature renal glomeruli . The thrThe histogenesis of extragonadal YST remains controversial. These tumors appear to arise from germ cells misplaced during embryogenesis, and malignant transformation leads to primary germ cell tumors at extragonadal sites .Due to the rarity of the extragonadal YST, there is no detailed description of its epidemiology and its clinical features in particular. Additionally, due to the particularly aggressive nature of YST, the information derived from germ cell tumor (GCT) studies may have limited applicability. From the case reports we have, the age distribution of extragonadal YST, similar to that of the testicular tumors, appears to be bimodal with an early peak in early infancy and a later peak in adolescence. YST may grow to a large size with no or relatively few symptoms, and the symptoms may vary due to origin sites. The majority of YSTs produce high levels of AFP, which helps in monitoring the response to the treatment. In the past, YSTs were nearly uniformly fatal, regardless of the primary location. However, the survival rate of YST patients has been significantly improved because of the application of Cisplatin-based multiagent chemotherapy -6,9.In our case, the patient had no history of swollen testes or testicular atrophy and had normal testes on palpation.Also, a scrotal sonography was performed and revealed no abnormalities in either side of the testes. With spatial resolution boosted by a high-frequency transducer, a testicular tumor may be nonpalpable but still visible sonographically ,11. Sonoet al.[A correct diagnosis was made as late as half a year after the symptoms appeared due to the unspecific symptoms (gross hematuria and hemospermia) and rarity of the site of extragonadal YST . This may be one of the contributors to the poor prognosis. From the clinical courses of this patient, we can see that the patient’s general response to the treatment is not very good as compared with recent reports. Jay P. Shah  reportedet al. reportedet al.-17. To oWe present a rare case of yolk sac tumor originating in the left seminal vesicle. Extragonadal YST is an aggressive germ cell tumor, and the treatment results are still unsatisfactory. More investigations are warranted to define the optimal treatment.Written informed consent was obtained from the patient’s elder brother 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.YST: yolk sac tumor; AFP: α-fetoprotein; CT: computed tomography; MRI: magnetic resonance imaging; PSA: prostate-specific antigen; PET/CT: Positron emission tomography/computed tomography; GCT: germ cell tumor; EGCT: extragonadal germ cell tumor; NSEGCT: nonseminomatous extragonadal germ cell tumor.The authors declare that they have no competing interests.XDY and HYP conceived the concept for the study, participated in drafting the manuscript, and conducted a critical review. Both authors contributed equally to this work. DWY participated in revising the manuscript critically for important intellectual content. CFW prepared the histological figures. All authors read and approved the final manuscript.


Inverted Meckel’s diverticulum has usually been misdiagnosed in the cases based on computed tomography images presented in the literature. The final diagnosis was made intra-operatively or by pathology reports after surgery. Despite this, preoperative diagnosis could be made successfully by using endoscopic ultrasound with double-balloon endoscopy prior to surgery.A 60-year-old Japanese woman with severe anemia complained of several episodes of black stool over the preceding 2 years. Abdominal computed tomography showed a 3.0-cm low-density tumor in the ileum, suggesting a diagnosis of intestinal lipoma. Examination of the tumor by endoscopic ultrasound with double-balloon endoscopy revealed a hypo-echoic layer corresponding to the muscularis propria, and a hyper-echoic layer corresponding to the fat tissue. These findings, which suggested that the tumor included areas outside the intestinal serosa, are not typical for a lipoma, despite the existence of a hyper-echoic layer corresponding to fatty tissue. We then considered a diagnosis of inverted Meckel’s diverticulum.Lipoma and inverted Meckel’s diverticulum are difficult to differentially diagnose by computed tomography. Polypectomy is the preferred therapeutic approach when a lipoma is present; however, polypectomy in a patient with Meckel’s diverticulum requires full-thickness resection. Situations where polypectomy is performed without preparing for full-thickness resection can be avoided by first making a precise diagnosis using double-balloon endoscopy and endoscopic ultrasound. Inverted Meckel’s diverticulum has usually been misdiagnosed in the cases based on computed tomography (CT) images presented in the literature . The finA 60-year-old Japanese woman with severe anemia complained of several episodes of black stool over the preceding 2 years. Upper and lower gastrointestinal tract endoscopies were performed at another hospital and the findings were unremarkable. She was referred to our hospital for further evaluation. Blood tests confirmed the known anemia  with iron deficiency .An abdominal CT showed a 3.0-cm low-density tumor in the ileum, indicative of an intestinal lipoma Figure . Intermi, despite the existence of a hyper-echoic layer corresponding to fatty tissue. Based on these findings inverted Meckel’s diverticulum was suspected as possible diagnosis and laparoscopic surgery was considered to be the appropriate treatment. A surgical specimen showed ectopic pancreatic tissue around the top of a tumor-like elevation, thus confirming our diagnosis.DBE images displayed a dumbbell-shaped tumor 30 × 35mm) on the anti-mesenteric side of the ileum, approximately 85cm from the ileocecal valve Figure . A simil0 × 35mm The postoperative course was uneventful and the patient was discharged with an improvement in anemia.Lipoma and inverted Meckel’s diverticulum are difficult to differentially diagnose by CT. Their main difference is the existence of serosa inside of the polyp. However, if a polypectomy were to be performed in a patient with inverted Meckel’s diverticulum, a full-thickness resection should be necessary. The accurate preoperative diagnosis of inverted Meckel’s diverticulum by DBE and EUS could avoid unnecessary polypectomies without preparation for full-thickness resection and the subsequent complications. The diagnostic characteristics of Meckel’s diverticulum in this case included a tumor located between 70 and 100cm from the ileocecal valve on the anti-mesenteric side, and EUS findings of a hyper-echoic layer encompassing areas outside the intestinal serosa. This case is a typical example of inverted Meckel’s diverticulum diagnosed by DBE and EUS, and so an unforced polypectomy was avoided. The limitation of DBE is that it cannot be used without the agreement of the patient ,3. We spDBE is an innovative method that facilitates disease treatment and enables pathological assessment by biopsy throughout the entire small intestine . DBE canThe combination of DBE and EUS can diagnose an inverted diverticulum that appears to be a polyp.Written informed consent was obtained from the patient prior to 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., or drug mentioned in this article. The authors declare that they have no competing interests.The authors have no financial or other interests in the manufacture or distribution of any equipment, device; AA was a major contributor to the writing, analysis, and interpretation of patient data for gastrointestinal bleeding. RO and MW were major contributors to the writing and proofreading of the manuscript. All authors read and approved the final manuscript.AA, KT, SO, EO, SS, JMA and TF performed the DBE


A 10-month-old intact male cat with a clinical history of a large mass in the left perineal region was submitted to the surgery department. The mass had reportedly been present as a small swelling after birth. Cytological evaluation using a fine-needle aspirate showed eosinophilic keratinaceous debris, and was not convincing for the definitive diagnosis. Complete surgical excision was performed. Postoperative function and aesthetics were excellent. Based on gross and histological features the definitive diagnosis of the tumor was mature teratoma with ectodermal and endodermal components. After a follow-up period of 4 months, no signs of recurrence were evident. Surgical excision of the teratoma in our case was considered curative. A perineal location has not been previously reported in the cat and should be considered a rare condition in this species. Teratomas are uncommon tumors containing elements that originate from more than one germ cell layer, are foreign to the organ in which they arise, and show independent growth . TeratomA 10-month-old, domestic shorthair intact male cat was presented for examination with a clinical history of an approximately 3 cm in diameter soft and painless mass in the left perineal region . The subcutaneous tissues were opposed with simple interrupted sutures (3–0 polydioxanone) and the skin was closed with simple interrupted no absorbable sutures (3–0 nylon) after a piece of skin was removed to eliminate the dead spaces and no drain was fixed.The entire mass was submitted to the pathology department for gross and histological interpretation. The gross aspect was of a large cystic mass, red to gray, with round to oval shape, measuring 90×70×60 mm. On cut section, approximately 50 ml of yellow-red, fine granulated liquid mixed with several hair fragments leaked out and a multinodular, dense, red to gray mass, measuring 40×30×30 mm, was observed attached to inner surface of the cyst wall.An area with several long dark brown to black hairs was present on the surface of the inner mass. Another focal area with a few thin white hairs was also seen Figure D. For hiOn the follow-up examination 21 days later, the wound had healed normally. No signs of local swelling, tenesmus or constipation were observed. After 4 months postoperatively the cat was presented for a routine examination, having a normal quality of life without any signs of recurrence.Beside the fact that the incidence of the teratoma in cats is low (0.7% to 3.6%) , a perinThe teratoma is based on primordial germ cells from the top cell layer of the blastocyst (the epiblast). From this arise the ectodermal, mesodermal and endodermal germ cell layers . During The content of teratomas is complex, reflecting their heterogeneity of germ-cell origin. Neural tissue, woven bone, hyaline cartilage, hair follicles, sebaceous and apocrine glands, respiratory epithelium and adipose tissue have all been reported in animals ,9. HistoBased on the degree of malignancy, the tumor had a benign nature pathologically and clinically, and the cat recovered completely after surgery. Teratomas with immature (poorly differentiated) histological components are often referred to as teratocarcinoma .Norris (1969) describes a solid teratoma in a cat with dysgerminomas; the tumor grew invasively and extended through the abdominal wall . AlthougAccording to other clinical case reports ,11,15 cyAlthough, the biopsy is recommended before surgical tumor excision for definitive diagnosis , the ownA differential diagnoses should be considered from perineal hernias in cats. Although a rare condition in cats, mostly in neutered males and typically bilateral , some caIn humans, primary retrorectal teratomas are also very rare and in addition to the anatomical position of tumors, difficulty in diagnosis and surgical management often occur . Even thBecause of intermittent constipation with tenesmus it was suggested that surgical excision provided a chance for local tumor control and clinical signs improvement. Four months after surgery the good healing of the tumor site, the normal position of the anus, good clinical condition and abdominal ultrasonography indicated no metastases in our case Figure B. This mAlthough this is a rare tumour, it should be included in the differential diagnosis for a perineal swelling in a young cat. Complete surgical excision has the potential to be curative in case of a mature teratoma.The authors declare that they have no competing interests.CAO and CP performed the anesthesia and surgery; although CAO is the main author of the paper. MT and LF performed the histopathologic examination and interpretation. LB is the referring clinician for the present case. LO and CC reviewed the paper. All authors read and approved the final manuscript.


Schwannoma or neurilemmoma is an infrequent benign tumor in the oral cavity that originates from the Schwann cells on the neural sheath of the peripheral nerves. Schwannomas are frequently located in the soft tissues of head and neck region, but only a 1 to 12% of them are located in the oral cavity. Some histological variants of schwannoma have been described including the cellular, plexiform, epithelioid, ancient, and melanocytic types. The “ancient schwannoma” is an uncommon variant of this tumor that shows specific histological characteristics, and is rare in the oral cavity with less than 15 cases described on the literature. Most of them were located in the tongue or in the floor of the mouth, being the hard palate an extremely rare localization. We present a new clinical case of an ancient schwannoma with a long time of evolution, arising from the nasopalatine nerve, and located in the hard palate of a 35 year old female. We also review the main clinical and histological characteristics of this pathology. Key words:Ancient schwannoma, neurilemmoma, palate, schwannoma. Schwannoma or Neurilemmoma is a benign tumor of the neural sheath derived from the Schwann cells -3. It waSchwannomas most often occur in the fourth and fifth decade of life and seem to have a 1.6:1 female predilec-tion ,6-8. ThiClinically, a circumscribed slow-growing, firm and solitary tumor is normally observed -3. Most The definitive diagnosis is based on the histological and immunohistochemical study -10. SeveThe treatment of this neoplasm is surgical, with a low recurrence rate ,2. MaligAfter an extended review of the literature, we consider that this could be probably the first clinical case reported of an ancient schwannoma located on the hard palate, or at least the first in which the histology of this histological variant is quite clear in this particular location. The clinical case and the main clinicopathological and therapeutic aspects are described and discussed.A 35 year old female is referred by her dentist to the Unit of Oral Medicine  with a slow growing tumor on the anterior portion of the hard palate. Family and medical history was noncontributory and she could not precise the exact time of evolution of the lesion, although, she had no-ticed a marked swelling for at least the past five years. She also pointed out that the lesion probably originated even some years before.On physical examination, a firm, sessile based tumor of the submucosa of 2.0 x 1.5 cm in size was observed in the midline of the hard palate. The surface mucosa was intact except for an erythematous central area  and irregular and hypocellular areas (Antoni B) Fig. . DegenerThe postoperative period was uneventful, and a week after the surgery the sutures were removed. After the resection of the tumor, the patient wore a thermoplastic vacuum formed device to protect the surgical area and to get more comfort during the secondary healing process. After one month a complete healing could be observed and after two year of continuous follow-ups, no recurrence has been observed.Schwannoma is a benign tumor of unknown cause originated from the Schwann cells of the neural sheath of the peripheral nerves -3. Only  Regarding to the ancient schwannoma in the oral cavity, the first report was made by Eversole and Howell in 1971 . After tSchwannomas most often occur in the fourth and fifth decade of life and seems to have a female predilection 1.6:1) , and myxoid and hypocellular areas (Antoni B). In the Antoni A areas, characteristic eosinophilic formations can be observed, the Verocay bodies -3,15. ThThe tumor cells of schwannomas are strongly immunoreactive to S-100 protein and are consistently negative for CD117, smooth muscle actin and desmin, and usually negative for CD34 .Computed tomography (CT) and magnetic resonance are useful tools in evaluating the location and extension of the lesion, as well as the possible relation with adjacent anatomical structures -3,15. DuComplete excision of the lesion, preserving the nerve of origin is the elected treatment -3,6-9. NAncient schwannoma is a slow growing, benign, solitary neoplasm rare in the oral cavity. Histopathological findings are essential for a correct diagnosis. Surgery is the treatment of choice and the rate of recurrence and malignant transformation is exceptional.


Peritonitis due to rupture of liver hydatid cyst secondary to blunt abdominal trauma can present with fatal consequences. Timely diagnosis and appropriate surgical management can be life saving. We report a case of ruptured liver hydatid cyst in the peritoneal cavity following trauma and its successful operative management in a preadolescent previously asymptomatic boy. Importance of detailed physical examination and early diagnosis by using appropriate radiological investigations is highlighted. Hepatic hydatid cysts can present in a myriad fashion. Acute presentation with rupture into the peritoneal cavity is a rare form of presentation with an incidence ranging from 1% to 8% . Intra-biliary rupture is another form of acute presentation and is more common than intraperitoneal rupture [3]. Following intraperitoneal rupture, presentation is usually acute with abdominal signs, such as guarding, and rebound tenderness with anaphylactic reactions occurring in 1% to 12.5% of cases, which at times could be life threatening . Herein, we report a preadolescent boy who presented with peritonitis following traumatic rupture of hydatid cyst of liver. The role of clinical examination and ultrasonography is highlighted for prompt diagnosis and successful management of this form of acute abdomen.An apparently healthy 11-year-old boy presented with acute pain in central abdomen of 5 hours duration. He gave history of a trivial blunt abdominal trauma while at play. Patient also complained of a bothersome itching all over his body especially over limbs and the trunk starting soon after the injury. At presentation, the patient was lying still in bed with pulse rate of 110/min and BP 110/60 mm Hg. There was no pallor. Lesions resembling utricaria were seen over thigh and trunk. Abdominal examination revealed generalized rebound tenderness.Baseline investigations were normal except for leukocytosis (13200/ mm3). Roentgenograms of the chest and the abdomen were essentially normal. Ultrasonography revealed that liver was enlarged and there were 2 cystic lesions [6.4X4.6 cm and 8.1X 6.9 cm] in right lobe of liver with hypoechoic contents and floating echogenic membranes and peripheral calcifications. Lot of free fluid was also present.Intravenous fluids were started with a bolus of Ringer lactate (20ml/Kg) followed by Dextrose 5% in normal saline (0.9%). Intravenous Hydrocortisone and Pheniramine maleate were adminstered along with antibiotic prophylaxis. The clinical picture with sudden generalized pain in abdomen with rashes, frank peritonitis and sonographic findings were suggestive of ruptured hepatic hydatid cyst. Patient was taken for exploratory laparotomy. The peritoneal cavity was filled with approximately 500 ml of bilious fluid which was drained out. Inspecting the liver surface showed extruded bile stained flaccid hydatid cyst .Another intact hydatid cyst was palpable in segment VI which was removed by partial pericystectomy. The entire peritoneal cavity was lavaged with hypertonic (3%) and normal saline. The bed of the first cyst was inspected for any bile leak. As there was no evidence of large cysto-biliary communication, the cavity was packed with omentum. A tube drain was placed in right sub-hepatic space. Postoperative course was uneventful. The drain was removed on the on 7th post operative day. albendazole (15 mg/kg/day) was started and plan was to continue it for 6 months (3 weekly courses and drug free period of one week with a watch on the liver enzymes and counts).Four weeks later, the patient presented with upper abdominal fullness not associated with any other complaint. He was afebrile and hemodynamically stable. There was no icterus. On examination, there was distension of abdomen limited to the upper half of abdomen. There was no demonstrable free fluid and bowel sounds were normal. Laboratory values were: Hb-9.2 gms/dl, TLC-6900/mm3, serum bilirubin-0.8 mg%, ALT-17 U/ L, ALP 365 U/ L, AST-28 U/ L. Sonography revealed a large multiloculated cystic mass (15cmx13cmx18 cm) antero-superior to the liver. The intra-hepatic biliary radicals and the common bile duct were not dilated. There was no free fluid. CT scan was done to know further details. It showed 13cmx13cmx16 cm cystic lesion in the right lobe of liver with well defined septa of liver parenchyma within it . Another cystic lesion was found in the left sub-hepatic space and lesser sac. Based on the findings it was diagnosed to be a “walled off” bile collection. A pig tail catheter was inserted percutaneously into the bilioma under sonographic guidance which was both diagnostic and therapeutic. It drained about 500 ml of greenish brown fluid overnight and culminated with disappearance of abdominal distension. The catheter was removed after 4 days when the effluent was negligible. Patient was discharged and on follow up 3 weeks later, found to be doing well. Currently patient is on albendazole therapy.Ecchinococcus granulosus causes hydatid disease, most commonly in the liver. It is endemic in sheep farming areas such as South and Central America, Western Europe, the Middle East and some sub-Saharan countries. Humans are accidental intermediate hosts and in its life cycle, can be considered as a dead end. Presentation may be myriad ranging from asymptomatic to life threatening anaphylaxis. Rupture of a hydatid cyst after trauma is an uncommon presentation especially in a non endemic area. Few reports of intraperitoneal rupture have been reported from the Indian subcontinent. Ray et al [6] reported spontaneous intraperitoneal and intra-biliary rupture of hepatic hydatid cyst in a 34 year old man. Ahuja et al [7] reported a 10–year-old child with hepatic hydatid cyst rupturing into sub-diaphragmatic space and pericardial cavity. The presentation may be quite dramatic because of the gross spillage of the contents that not only cause chemical peritonitis, but sometimes can also cause anaphylaxis. If there is bile leak, it further exacerbates the inflammatory process in the peritoneum.Preoperative diagnosis can be missed especially in a patient like ours who did not have any suggestive history except for the utricaria like lesions. Both ultrasonography and computerized tomography are highly sensitive in demonstrating the cyst rupture [4]. However the combination of clinical examination and ultrasonography findings made the diagnosis quite obvious which was further confirmed on laparotomy. Serology is used to detect specific serum antibodies or circulating antigens by a variety of immunodiagnostic methods. The most commonly used technique is enzyme linked immunosorbent assay (ELISA) for detection of echinococcal antibodies (IgG) in the serum. A false positive value can occur in a normal person especially in endemic area and similar results can also occur in patients with other parasitic infestations [8].Treatment has to be done expeditiously to prevent spreading of chemical peritonitis and to prevent disseminated peritoneal echinococcosis. Surgical therapy is no doubt the mainstay but the exact approach, radical or conservative is still not defined. In an emergency setting, the conservative methods have shown to yield satisfactory results [9]. Various obliterative techniques  have been described in the literature [3]. Some authors have favoured pericystectomy and liver resections as preferred treatment for hydatid disease [10]. Pericystectomy are usually performed when the location of the cyst is away from major biliary or vascular structures. Some form of liver resection is possible in an emergency setting only if the cyst is peripheral and pedunculated. We performed partial pericystectomy with drainage. Akcan et al in a series of 372 patients with hydatid cysts did partial pericystectomy with drainage in 70% of patients as their preferred surgical technique [3]. The authors found this technique superior to others because it was simple, applicable to almost all cysts irrespective of its location and a shorter operation time.After removal of the entire cyst and its contents, the peritoneal cavity should be liberally lavaged with scolicidal agents and normal saline. Various agents and hypertonic saline have been described in the literature for the purpose [11]. Gargouri et al used 3%-5% saline with equal efficacy but stressed on the importance of contact time for effectiveness [12]. The cystobilious communication should be dealt with depending on the size of the fistulae. For large communications T-tube drainage [13], choledochoduodenostomy or sphincterotomy has been described. Endoscopic biliary stenting has also been reported to be of value in treatment for biliary fistulas complicating liver hydatid cysts [14]. In our case, the delayed appearance of bilary collection was most likely because of minor cystobiliary communications which somehow remained patent even after 4 weeks of initial surgery. An intra-biliary rupture was ruled out because patient did not have jaundice or features of cholangitis. The sonography and CT scan were also not suggestive of intra-biliary rupture.To prevent recurrences, albendazole therapy is mandatory. It should be started immediately and given for a prolonged period of time [15]. Follow up with 6 monthly imaging is prudent to detect recurrences. If these basic principles are followed the recurrences in these cases are not as high as once thought. The purpose of this report is to sensitize the surgeons dealing with emergency and trauma patients to consider rupture of Hydatid cyst in the differential diagnosis of acute post traumatic peritonitis and to reemphasize that a carefully obtained history and physical examination aided by radiological investigation can help diagnose them without undue delay. Source of Support: NilConflict of Interest: None declared


Objective: Primary bony tumors of the chest wall are usually benign and most commonly located in the ribs or sternum. Chondrosarcoma is regarded as one of the most frequent primary malignancies of the chest wall and its incidence after a sternotomy for a cardiac procedure is extremely rare. We present a case of sternal chondrosarcoma. Methods: The patient presented with a sternal mass 4 years after undergoing coronary artery bypass grafting for ischemic coronary artery disease. The mass originally emanated from the upper portion of the patients’ sternum and then rapidly enlarged to include the anterior aspects of his neck. Radiologic imaging studies were undertaken: computed tomographic scan and magnetic resonance imaging, with surgical intervention for excision. Results: Computed tomographic scan and magnetic resonance imaging established an 8.4 × 6.2 × 8.6 cm3 complex solid tissue mass within the lower neck arising from the sternal manubrium, with extensive bone destruction. Computed tomography-guided biopsy showed cells of uncertain significance. Surgical excision was performed and the mass was diagnosed as a grade II chondrosarcoma. Discussion: Primary sarcomas of the sternum though uncommon are potentially curable with wide surgical excision. Success depends on tumor histologic type and grade, which dictate recurrence. The patient is a Vietnam War veteran and was in the war zone where Agent Orange was utilized. He presented to the cardiothoracic surgery office for outpatient evaluation after noticing an expanding upper sternal mass, which he first observed 3 months prior . The pat3 complex solid tissue mass within the lower neck arising from the sternal manubrium, with extensive bone destruction. Because of the clinical and radiological appearance, a provisional diagnosis of a sternal granuloma was made.A thorough work-up ensued yielding further details of the mass, as noted on the CT scan  and magn3 mass arising from the anterior portion of the sternal manubrium, extending to the neck and locally invading the skeletal muscle was partially resected . According to the Evans classification in a scale that can range from Grade I (well differentiated) to Grade III (poorly differentiated), grade is the most critical single predictor of recurrence and metastasis.Wide resection of malignant chest wall tumors includes the affected ribs with at least a 4 ± 5 cm free margin proximally and distally to the tumor, portions of the ribs immediately above and below the tumor, the adjacent muscles, and the underlying pleura. Any other tissue adherent to the tumor is also excised. For sternal chest wall tumors, the size and location of the tumor determines the extent of the resection. Lesions at the upper third of the sternum necessitate resection of the manubrium and most of the sternal body as well as the medial ends of the clavicles, the adjacent sternocostal cartilages, and subtotal sternectomy. Lesions at the middle third of the sternum are treated with resection of the sternal body, with preservation of the manubrium and the xiphoid process.1,11,1,11,12Of note, potential complications include multiple myeloma, infection, need for tracheostomy, additional chest wall reconstruction, and even death.1p-dioxin) cannot be discounted. Agent Orange is the code name for one of the herbicides and defoliants used by the United States military as part of its extensive herbicidal warfare program during the Vietnam War. A number of different types of cancers found in Vietnam War veterans have been loosely attributed to the use of Agent Orange.13,Our case demonstrates a Grade II chondrosarcoma, which occurred 4 years after a sternotomy for a coronary artery bypass grafting. The exact genesis of this tumor is not known. Being that this patient was a Vietnam War veteran, the association with Agent Orange (2,3,7,8-tetrachlorodibenzo-This case reveals an interesting scenario where a malignant tumor formed after a sternotomy. Usually, when a large mass is found after sternotomy, the differential diagnosis mainly includes infection, granulomas, or pseudoaneurysms. This article proves that the diagnosis of chondrosarcoma should be included in that differential.


Nasopharyngeal masses in the pediatric population are quite rare, and the majority of these are benign. In adolescent boys, there should be a high index of suspicion for juvenile nasopharyngeal angiofibromas. When malignant, the most common lesions encountered are rhabdomyosarcomas, carcinomas, and lymphomas. We report a single case from a tertiary care institution of an adolescent male with an unusual presentation of a benign nasopharyngeal mass and provide a comprehensive review of pediatric nasopharyngeal masses. Whenever possible, radiographic imaging should be obtained, in addition to biopsy, to assist in the diagnosis of pediatric nasopharyngeal masses. Nasopharyngeal masses in the pediatric population are quite rare, and the majority of these are benign. Differential diagnoses of nasopharyngeal masses in the pediatric population include inflammatory lesions, malignant tumors, and congenital masses. The rarity of pediatric nasopharyngeal masses and the diversity of possible pathologies make a clinical diagnosis difficult.An understanding of the anatomy and histology of the nasopharynx is critical in deciphering the etiology of a lesion arising in this region. The nasopharynx is part of  the pharynx, which includes the oropharynx and hypopharynx, and is a part of the upper respiratory tract. Anteriorly it originates at the posterior aspect of the nasal turbinates and is perforated by the posterior nares, forming the choanae. Posteriorly and superiorly it is bordered by the skull base. Inferiorly it extends to the level of the soft palate, where the oropharynx begins. The lateral wall of the nasopharynx contains the ostium of the eustachian tube, which is surrounded by mucosa. The ostium of the eustachian tube is anterior to a pharyngeal recess known as the fossa of Rosenmuller.The nasopharynx is lined by stratified squamous epithelium at the anterior, posterior, and inferior walls and by respiratory epithelium at the roof and nasal choanae. The remaining areas have mixtures of squamous and respiratory or intermediate  epithelium. There is abundant lymphoid tissue present, particularly at the rim of eustachian tube opening, which is functionally equivalent to that of gastrointestinal tract or mucosal-associated lymphoid tissue .We present a case of an adolescent male with a nasopharyngeal mass that presented with physical and radiographic findings concerning a malignancy. Although the pathology in this was benign, this prompted a comprehensive review of pediatric nasopharyngeal masses. Given the anatomical complexity of the region and close proximity to vital structures, a biopsy of the lesion was paramount in ruling out malignancy, as clinical and radiological data would not have sufficed.A 15-year-old male was seen in the head and neck clinic complaining of progressively worsening left-sided nasal airway obstruction for several months. He reported intermittent nonfocal headaches and respiratory infections for the last 3 months. He denied otalgia, epistaxis, epiphora, pain, numbness, purulence, swelling, and anosmia. His past history was significant only for reactive airway disease.On physical examination, an approximately a 2 × 4 cm mass was seen posterior to the left tonsillar fossa. Nasal endoscopy demonstrated a left-sided soft tissue mass emanating from the nasopharynx. A computed tomography (CT) scan of the sinuses demonstrated some extension of soft tissue from the nasopharynx on the left side Figures  and 2. MThe patient was brought to the operating room to evaluate his nasal cavities endoscopically. The mass was then excised via an intraoral approach for diagnostic and therapeutic purposes, given the suspicion of a malignant process, without complication. Histology demonstrated benign lymphoid hyperplasia with no evidence of malignancy Figures  and 5. AThe differential diagnosis of pediatric nasopharyngeal masses includes a broad range of benign and malignant tumors, in addition to congenital lesions. Patient history and physical examination are part of the initial assessment and are the first steps in patient evaluation.Benign masses are the most common abnormalities seen in the pediatric nasopharynx. The most common include adenoidal tissue, antrochoanal polyp (ACP), and juvenile nasopharyngeal angiofibroma (JNA). Benign adenoidal hypertrophy is by far the most common mass in the nasopharynx in children . AdenoidACP is a benign lesion that arises from the mucosa of the maxillary sinus . It growJNA is the most common benign tumor of the nasopharynx and accounts for 0.05% of all neoplasms of the head and neck . The exaLess commonly seen benign nasopharyngeal masses in the pediatric population include fibromas, chordomas, rhabdomyomas, and chondromas. A fibroma is a localized pedunculated mass, usually less than 1 cm in size . PatientCongenital midline nasal masses include nasal dermoids, nasal gliomas, and encephaloceles. These congenital anomalies are estimated to occur in 1 out of  20,000–40,000 births . AlthougNasal gliomas are firm masses which are nonpulsatile and tend to arise from the lateral nasal wall . An impoOther less commonly seen congenital lesions that have been reported in the pediatric nasopharynx include teratomas, craniopharyngiomas, Thornwaldt's cysts, hamartomas, and hemangiomas. Teratomas, also known as hairy polyp of nasopharynx, are derived from pluripotent tissues composed of all 3 germinal layers and are often diagnosed in the neonatal or infancy periods . The lesA craniopharyngioma is a type of pituitary adenoma which is cystic in nature. The cyst contains brownish fluid with refractile cholesterol crystals . It is aMalignancies of the pediatric nasopharynx include rhabdomyosarcomas, nasopharyngeal carcinomas (NPCs), and lymphomas. Malignant tumors of the nasopharynx are rare in this age group, and the histology generally varies with the age of the patient at presentation. Soft tissue sarcomas and lymphomas are more commonly diagnosed in younger children, whereas carcinoma of the nasopharynx has a predilection for adolescents and teenagers . RhabdomNPC accounts for 5% of all pediatric head and neck malignancies and for about one-third of all cancers of the upper airway . It is vManagement of benign tumors is usually surgical, but the approach may vary dramatically depending on the histology and the anatomical pattern of involvement. Malignant tumors are generally treated with radiation therapy and/or chemotherapy. In summary, the necessity of biopsy in the evaluation of pediatric nasopharyngeal masses should be evident given the large spectrum of potential diagnoses in the pediatric nasopharynx. It is the histology which will most influence the physician's choice of therapy.Although the majority of nasopharyngeal masses in the pediatric population are benign, they must be fully evaluated in order to rule out malignancy based on clinical and radiologic findings. A mass in this region can be safely excised only after careful preoperative evaluation.


Bronchial stump aspergillosis is a rare entity characterized by cough and hemoptysis.We report the case of a 58-year-old Caucasian woman who developed bronchial stump aspergillosis two years after a left upper lobe resection for lung cancer. Bronchial stump aspergillosis was diagnosed as a result of a focus of increased fluorodeoxyglucose activity in a follow-up positron emission tomography and computed tomography scan. She was treated with oral antifungal therapy and presented with good evolution after three months of treatment.Bronchial stump aspergillosis is an unusual complication after pulmonary resection. Clinicians should be aware of it when a local recurrence of cancer around the bronchial stump is suspected based on a positive positron emission tomography and computed tomography finding. Aspergillus species colonization of an endobronchial suture, with the appearance of reacting bronchial granulation tissue, is a rare event that has been associated with the use of silk for sutures  of 10.3 and 8.3, respectively; Figure Aspergillus species are ubiquitous soil-dwelling microorganisms found in organic debris, dust, compost, foods, spices and rotting plants. Colonization is usually as a result of the inhalation of airborne spores from an inanimate reservoir and, if spores reach the peripheral lung, a variety of clinical syndromes may develop [ develop . Althoug develop , few cas develop -3 since  develop . Only fi develop ,3,5 sinc develop  in 1995. develop ,3 but ma develop .PET-CT scans are used to distinguish metabolically active from active lesions because of the biochemical differences, especially in staging cancer . HoweverAlthough it is widely suggested that the removal of persisting sutures is the treatment of choice for BSA -3,5, gooAlthough BSA is rare, clinicians should be aware of this unusual complication after pulmonary resection when local recurrence of cancer around the bronchial stump is suspected based on a positive PET-CT finding. PET-CT can be a useful tool in the monitoring of treatment efficacy in patients with this condition.BSA: bronchial stump aspergillosis; FDG: fluorodeoxyglucose; PET-CT: positron emission tomography and computed tomography; SUVmax: standardized uptake value.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.IG was a major contributor to the manuscript. OR and AF performed the histological examination of the lung. FA, JS and EM performed the bronchoscopy and the follow-up of the patients. All authors contributed to the writing the manuscript and approved the final version.


Primary laryngeal lymphomas are exceedingly rare. Only about a hundred cases have been reported. They consist mainly of non-Hodgkin lymphoma, especially of diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue. We report the first case of a primary laryngeal mantle cell lymphoma.We report a case of a primary mantle cell lymphoma of the larynx in a 70-year-old North African non-smoker male. We present a detailed report of his clinical and paraclinical data as well as treatment options.Mantle cell lymphoma is a very aggressive lymphoma subset associated with poor prognosis. Laryngeal mantle cell lymphoma is exceedingly rare. To the best of our knowledge, this is the first case to ever be reported. Primary laryngeal lymphoma is extremely rare, accounting for less than 1% of all primary laryngeal neoplasms . Among tWe report the case of a 70-year-old North African man who, two months prior to medical examination, presented with mild laryngeal respiratory distress that worsened until he ultimately required a tracheotomy. A direct laryngoscopy had been performed, revealing a subglottic, submucosal lump. A biopsy revealed a mantle cell type B NHL with the following antigen constellation: cluster of differentiation (CD)5+, CD20+, CD23-, cyclin D1+. Importantly, a physical examination and computed tomography (CT) scans showed a lack of lymph node involvement, establishing the larynx as the primary site of the neoplastic lesion.The patient’s history and physical examination found no comorbidities. The patient did not present any B symptoms and had a performance status 3 as measured using the World Health Organization performance scale. During a follow-up four weeks after his initial admission, a physical examination found laterocervical lymph nodes. An investigation into possible tumoral extension by cervical, thoracoabdominal and pelvic CT scans showed a subglottic tumor Figures  and 2 anA bone marrow biopsy did not show any anomalies. A blood workup showed he had normal red blood cell sedimentation rate and lactate dehydrogenase levels.The patient, who has been staged IIEAa (Ann Arbor Classification), received one course of rituximab-cyclophosphamide, doxorubicin, vincristine and prednisone but unfortunately died of unknown causes. No autopsy was performed in compliance with the family’s wish.Extranodal NHL accounts for 20 to 30% of all lymphomas, while MCL represents only 6% of all NHL subtypes, both nodal and extranodal . LaryngeMost lymphomas involving the larynx involve other sites as well, including the salivary glands, the thyroid, the nasopharynx and tonsils . The mosThe age of onset for laryngeal lymphomas varies between 4 and 81 years, with the mean age of occurrence being in the seventh decade (the patient was 70 years old); the distribution between males and females is similar .The symptoms at presentation are common to other laryngeal neoplasms, i.e. hoarseness, dysphonia, dysphagia, stridor, cough and other systemic symptoms, such as weight loss and fever . In someA survey of primary lymphomas of the larynx published from 1996 to 2008 shows that 47% were located in the supraglottic region, 25% in the glottic area, and the remainder were either subglottic or transglottic [Regarding the macroscopic characteristics of the reported cases, 20 of 36 cases (55%) were described either as smooth or submucosal masses, whereas only two cases (5.5%) were ulcerated lesions , a definA wide spectrum of histological subtypes of laryngeal lymphomas has been reported. The great majority of laryngeal NHLs are of B-cell lineage, frequently presenting as diffuse large cell lymphoma; very few are of T-cell lineage ,9. The mWhile there is not a consensus regarding the treatment of MCL, localized stage I and II patients are usually treated with chemotherapy and radiotherapy, while those with disseminated forms (stages III and IV) are treated with chemotherapy only. MCL is an aggressive lymphoma with the poorest long-term survival of any subtype. This might be linked to the fact that they usually are discovered at an advanced stage. Remission induction using intensive chemotherapy regimens appears to increase the time to progression; it does not, however, improve the overall survival ,11. RandLaryngeal MCL is exceedingly rare. After an extensive literature search and to the best of our knowledge, this is the first reported case. MCL is very aggressive and has a poor prognosis. There currently is no consensus for a standardized treatment for this type of lymphoma. New therapeutic protocols, however, are under evaluation and may improve the outcome for patients.Written informed consent was obtained from the patient’s next of kin for publication of this manuscript and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CD, Cluster of differentiation; CT, Computed tomography; MCL, Mantle cell lymphoma; NHL, Non-Hodgkin lymphoma.The authors declare that they do not have any competing interests.SN, AAB-B, MG, HM, SG, LE, MK and HE contributed equally to generating the experimental data. AAB-B performed the tracheotomy, laryngoscopy and laryngeal biopsy. SN was in charge of chemotherapeutic treatment and follow-up of the patient. AAB-B and SN wrote the article and performed the literature review. All authors read and approved the final manuscript.


Background. Necrotizing sialometaplasia (NS) is an uncommon benign reactive necrotizing inflammatory process involving minor salivary gland that often mimics malignancy both clinically and histopathologically. Case Report. We report the case of a healthy 26-year-old man with a painless swelling in the hard palate near the middle raphe, asymptomatic, well limited, and raised edges. The patient was submitted to incisional biopsy and histopathological examination. The histological diagnosis was necrotizing sialometaplasia. Discussion. The clinical and histological similarity between this entity and a malignant lesion implies a risk of unnecessary or mistreatment. Therefore, clinicians and pathologists should be aware of this lesion as to avoid errors in the diagnosis and treatment of this benign pathologic condition. Necrotizing sialometaplasia (NS) was first reported in 1973 by Abrams et al. as reactive necrotizing inflammatory process involving minor salivary gland of hard palate . These lA 26-year-old man presented with a painless swelling in the hard palate. He was referred to dentist that noted in a clinical examination a lesion with 1.5 × 0.5 cm on the posterior aspect of the hard palate near the middle raphe, asymptomatic, well limited, and with raised edges . Thus, aThe histopathological examination revealed a reactive necrotizing inflammatory process involving the minor salivary glands, with pseudoepitheliomatous hyperplasia of the overlying epithelium, but without evidence of dysplasia . In the Since necrotizing sialometaplasia was first described in 1973, by Abrams et al., about 200 cases were reported .Although the exact etiology of NS is not fully resolved, the lesion is believed to be related to a physicochemical or biological injury on the blood vessels that would produce ischemic changes, leading to infarction of the salivary gland acini with posterior necrosis, inflammation and intent of repairing, inducing metaplasia, changes in ducts, and further cicatrization . UsuallyThe ordinary lesion presents itself as a deep-seated palatal ulcer with clinical and histologic findings mimicking those of a malignant neoplasm. Unfortunately, the NS is still easily misdiagnosed as a mucoepidermoid carcinoma or a squamous cell carcinoma. In such cases, the microscopic examination is mandatory, because failure to recognize NS may result in unnecessary radical surgery with undesirable mutilating effects.According to Anneroth and Hansen , the patThe repair of ductal epithelium and acini by squamous metaplasia with accompanying pseudoepitheliomatous hyperplasia can actually be mistaken microscopically with mucoepidermoid carcinoma or squamous cell carcinoma . It shouMost of reported cases of NS have emphasized that an incisional biopsy must be performed on these lesions and careful histopathologic examination should be done to determine the proper treatment and management regimen . HoweverIn conclusion, clinicians and pathologists should be aware of this lesion as to avoid errors in the diagnosis and treatment of this benign pathologic condition.


Neurofibromatosis (NF) is divided into two types, NF type 1 and NF type 2. Optic nerve gliomas have a high degree of association with NF type 1. NF 2, less commonly seen, is a complex of cutaneous and deep neural tumors. It is an autosomal dominant familial disorder in which CNS is affected in about 15% of the cases. Bilateral acoustic neuromas are pathognomonic of NF type 2 which may be associated with meningiomas or ependymomas. Typical clinical manifestations of neurofibromatosis are cafe-au-lait spots and multiple cutaneous tumors. There is bone involvement as scoliosis, pseudoarthrosis of long bones, scalloping of vertebral bodies, abnormal rib tubulation and defective ossification of the skull. Extraskeletal manifestations of neurofibromatosis include optic nerve gliomas, pheochromocytoma, aneurysms of cerebral and renal arteries, acoustic neurilemmoma and superficial skin nodular neurofibromas.Here, we intend to present images of several cases of neurofibromatosis with different patterns of body involvement. Neuro fibromatosis (NF) is often noticed at birth or soon later. This manifests in several different ways. Typical clinical manifestations of NF are cafe-au-lait spots and multiple cutaneous tumors . NF is d1. NF type 1 or Von Recklinghausen's disease: It affects 1 in 4000 of the population. It is an autosomal dominant familial disorder associated with a defect in the long arm of chromosome 17. The CNS is affected in about 15% of cases and the skull and spine are also frequently involved. CNS involvement of NF1 is associated with glial tumors, including optic nerve gliomas and cerebral and brainstem astrocytoma and also with anomalies of migration resulting in various types of dysplasia and heterotopia in the white matter. Hamartomas with no mass effect are frequently present, leading to areas of high signal on T2 MR studies. Generalized or focal cerebral atrophy may also be seen associated with mental retardation. Vascular dysplasias involving the terminal internal carotid or anterior or middle cerebral origins have also been described in NF1, as has the resulting moyamoya disease [2. NF type 2: It is much less commonly seen, affecting only 1 in 50000 of the population. It is also inherited as an autosomal dominant condition, but is due to a defect of chromosome 22. The CNS lesions are quite different from those of NF1 and consist of bilateral acoustic neuromas which may be associated with schwannomas of other cranial nerves and meningiomas. In the spine, bony abnormalities are absent, but large bilateral neurofibromas may be seen. Spinal meningiomas as well as ependymoma of the cord or filum also occur [Skeletal manifestations of NF1 include scoliosis, pseudoarthrosis of long bones (particularly the tibia) and hemihypertrophy. Scoliosis, often of short segment distribution, frequently occurs in the thoracic spine. In addition, numerous abnormalities arise related to the neural canal, including generalized dilatation with scalloping of vertebral bodies, internal meningoceles and dysraphic anomalies. Abnormal rib tubulation results in a ribbon-shaped appearance. A particularly common abnormality is defective ossification of the posterior superior wall of the orbit. Pseudarthrosis of the tibia is characterized by marked absorption of the fracture margins, so that they become pointed. Similar lesions may occur in the radius or clavicle .Many extraskeletal manifestations of NF occur because of the neuroectodermal and mesodermal derivation of the tissue . GliomasStudy of neurofibromatosis imaging also has a significant role in prevention. As mentioned previously, neurofibromatosis is an inheritable disease. Therefore, early and correct diagnosis of the disease may be helpful in preventing disabilities in the next generation. Because sometimes the clinical manifestations of the disease are shown late or difficult to lead the clinician to the correct diagnosis, imaging may be a complementary limb in early diagnosis. For example, bilateral optic gliomas in an infant or an orbital defect in a neonate may guide the clinician and radiologist to the probable neurofibromatosis and early disease detection may prevent future disabilities of the patient in later life and subsequently transmitting the disease to the siblings.Here, we intend to present images of several cases of NF with different patterns of body involvement.This case is a 20-year old girl with progressive loss of vision and cafe-au-lait spots on her skin.In the axial T1-weighted MR image of the orbits, bilateral thickened optic nerves which were isointense with brain parenchyma were detected . In the NF1 with bilateral optic nerve gliomas was the diagnosis. Histologically, optic nerve gliomas are juvenile pilocytic astrocytomas seen in up to 30% of all NF patients. 10% of all optic nerve gliomas are associated with NF.She was a 23-year old girl with sensory-neural hearing loss.Axial  and sagiThe masses were proved to be multiple acoustic neurinomas. One of them extended into the right internal acoustic canal as is shown on axial FLAIR image . BilaterA 24-year old girl presented with bouts of headache and sensory-neural hearing loss.Axial T1-weighted MR images of the brain revealed an isosignal intensity meningioma in the left frontal lobe and another meningioma in the left parietal lobe with mid-line shift  & B. AxiAxial T1-weighted MR images without contrast from CP-angles also show bilateral CP-angle masses as low signal intensity . Axial TAxial and coronal post-contrast T1-weighted MR images demonstrate severe homogeneous enhancement in meningiomas including dural tail  & L.As previously noted, bilateral acoustic neurinomas are diagnostic for neurofibromatosis. In NF2, there are some associations as meningiomas and ependymomas, the so-called MISME . This is A 42-year old man with painful skin lumps on his body and weakness in his upper limbs.Coronal T1-weighted MR image of the cervical cord demonstrated multiple ovoid subcutaneous tumors as low signal intensity . These aHamartomas may be detected in up to 75 to 90 percent of the cases and are probably dysmyelinating lesions. They are located in the pons, basal ganglia , thalamus and cerebellar white matter [A 25-year old man presented with weakness and paresthesia in his upper limbs.Axial T2-weighted MR image of the cervical spinal column  revealedThe above findings are compatible with neurofibromatosis type one.A 16-year old boy presented with deafness and skull deformity.Axial post contrast CT scan image of the brain showed multiple extra-axial coarse calcifications which were proved to be meningiomas including intraventricular meningiomas  & B. CorBilateral acoustic neurinomas and multiple meningiomas are diagnostic for NF2.A 30-year old woman complained of abdominal pain.CT scan of the abdominopelvis with IV and oral contrast revealed a large retroperitoneal lobulated nonenhanced and homogeneous mass with encasement of the aorta and extension into the neural foramina. There is also neural foraminal widening of the lumbar vertebra. The para-spinal muscles contained multiple small non-enhanced, homogeneous masses. The masses were proved to be neurofibromas from the neural foramina to the retroperitoneal space (plexiform neurofibromatosis- type one) and from the para-spinal muscles . On the A 19-year-old man complained of a 6-month pain on the left side of the thoraco-lumbar area.Axial CT scan without IV contrast showed several ovoid contiguous masses inside the left-side quadratus lumborum muscle. These masses extend downward and may be detected in two axial consecutive sections. The masses were isodense to the adjacent muscle & B.After contrast injection, the axial CT scan at the level of the kidneys reveals the mentioned masses, which are again isodense to the adjacent muscles .The pathological examination demonstrated that the resected masses were neurofibromas. After scrupulous physical examination, the patient had cafe-au-lait spots on his skin (NF1).A 13-year old boy with apparented deformity of the spinal column and recent dyspnea.In the PA chest X-ray, there is scoliosis in the thoracic spinal column and a soft tissue mass in the upper posterior mediastinum  which waA fifteen-year old boy presented with swelling of the right lower neck.The postero-anterior chest radiograph revealed a subcutaneous soft tissue mass in the right supraclavicular area with extension to the lower neck. The mass had pressure effect on the trachea. There is also short-length scoliosis of the upper thoracic spinal column . The masA 20-year old boy presented with deformity of the chest.The plain PA chest radiograph showed tubulation of at least four ribs in the right lower thoracic area . Rib tubAn 18-year-old man presented with apparent leg deformity and cafe-au-lait spots.AP and lateral views of the leg showed pseudarthrosis of the tibia  indicatiA 12-year-old girl presented with cafe-au-lait spots and apparent deformity in the forearm.PA and lateral views of the forearm and hand showed severe pseudarthrosis of the radius and ulna resulting in marked absorption of the fracture margins . The surA 40-year-old man presented with swelling and pain in the left ankle.Lateral view of the ankle showed a large-sized tumor at the back of the ankle which was proved to be neurofibroma. Other smaller soft tissue neurofibromas may also be detected. Pseudoarthrosis of the talo-calcaneal joint is also seen . AnotherA 17-year old girl presented with chronic left foot swelling.AP and oblique X ray views of the foot showed a large soft tissue swelling on her left foot similar to previous case including deformity of tubular bones of metatarsals and phalanges . The masAn infant presented with hand deformity.AP plain radiograph of the hand revealed deformity as local gigantism of first and second fingers  which isA 25-year-old woman presented with swelling of fingers.The AP and oblique views of the left hand revealed soft tissue swelling of the thumb and index finger  due to mNeurofibromatosis has various imaging manifestations which are different in each of type one and two neurofibromatoses. All imaging modalities such as plain radiographies, CT scan and MRI may demonstrate various aspects of neurofibromatosis imaging and in a position to initially suspect NF, the radiologist should be aware of the manifestations. One should also be aware of the accompanying involvements of NF such as optic glioma, meningiomas, ependymomas and pheochromocytomas.NF may involve different parts of the body such as the brain, spinal cord and exiting nerves, bones, soft tissue, kidneys and even the mediastinum. Therefore, evaluation of other parts, if in doubt, is necessary. Involvement of each part of the body may also have different imaging manifestations; for example bone involvement in NF may be in the form of scoliosis, pseudarthrosis, bony defect, posterior scalloping of the vertebral bodies and tubulation of the ribs.


Chondroblastoma is a mostly benign bone neoplasm that typically affects the second decade of life and exhibits a lytic lesion in the epiphysis of long bones. We report an extreme case of massive, destructive chondroblastoma of the proximal humerus in a 9-year-old girl. It was difficult to differentiate using imaging information the lesion from malignant bone tumors such as osteosarcoma. Histopathological examination from biopsy proved chondroblastoma. The tumor was resected after preoperative transcatheter embolization. Reconstructive procedure for the proximal humerus was not performed due to the local destruction. The present case demonstrates clinical and radiological differentiations of the massive chondroblastoma from the other lesions and histopathological understandings for this lesion. Chondroblastoma is a mostly benign bone neoplasm that typically affects the second decade of life and exhibits a lytic lesion in the epiphysis of long bones , 2. PainThe patient and her family were informed that data concerning her case would be submitted for publication, and they provided written consent.A 9-year-old girl who had been treated for adjustment disorder was referred to our hospital for a massive lesion of the left shoulder with severe limitation of joint mobility over approximately one year. The left shoulder exhibited local warmth and varicoses. Neither motor, sensory, nor circulatory disorder was seen distal to the shoulder joint. Laboratory tests showed moderate increases in C-reactive protein  and alkaline phosphatase (817 U/L). Plain radiographs showed a 14 × 15 cm poorly-demarcated mass with irregular calcification . ComputeAs malignant bone tumor such as osteosarcoma was a plausible diagnosis, incisional biopsy was performed. The lesion had a thin membrane but not a firm cortical bone structure. The specimen grossly consisted of a white, solid component and a grey, soft fibrocartilaginous element. Histological examination revealed homogeneous spread of round- to polygonal-shaped mononuclear cells having an eosinophilic cytoplasm and a round- to ovoid-shaped nucleus . These hen bloc tumor resection via an extended deltopectoral incision was completed. The resected specimen measured 18 × 15 × 12 cm and weighed 2.4 kg, containing fibrocartilaginous, calcified, and hemorrhagic components. Total amount of bleeding during operation was approximately 2,300 mL. Since all muscles inserting the proximal humerus and the articular surface of the glenoid were completely compromised, shoulder joint reconstruction was not performed. The axillary and radial nerves were released from the tumor, although both had been stretched by expansion of the tumor. The humeral insertion of the thinned deltoid and the long head of triceps were preserved, and the short head of biceps, which was detached at the beginning, was repositioned. The entire specimen was sliced parallel in the coronal plane, and the whole of the largest slice was examined thoroughly. Histopathological findings of the resected specimen were consistent with those of the previous biopsy findings. No atypical cells, which indicate malignant transformation of the tumor, were found anywhere. The specimens were further examined by a consultant specialist of sarcoma pathology, and the definitive diagnosis of chondroblastoma was confirmed. Follow-up CT revealed multiple nodular lesions over the bilateral lungs, which could indicate spontaneous transformation to aggressive or malignant chondroblastoma with the potential to metastasize , 5. ThesTwo years after surgery, the patient had readjusted to school life, despite complete loss of shoulder mobility. No recurrence or metastasis has been detected . The pat en bloc resection of an intra-articular giant tumor arising from the scapula, which measured 39 × 30 × 18 cm  and weighed 14.44 kg. Chondroblastoma is recognized as a benign, cartilaginous lesion that characteristically arises in the epiphysis of long bones, particularly the humerus, tibia, and femur  and thatIn the present case, the rotator cuff and the articular surface of the glenoid were completely destroyed, which made functional reconstruction of the shoulder joint difficult. The distal half of the humerus is, then, floating with intact wrist and hand functions. Suspension arthroplasty of the shoulder using a vascularized fibula graft should be one of the reconstructions following a massive defect of the proximal humerus. To date, the patient has not received reconstructive procedures, considering her psychiatric background, and neither the patient nor her family is open to discussing reconstructive surgery. The radiological feature of chondroblastoma is typically a lytic lesion arising in the epiphysis with an eccentric location, mostly without periosteal reaction –3. The lIn the present case, the pulmonary lesions were concluded to be nodular septic embolisms but not metastatic lesions. Pulmonary metastases are common in osteosarcoma. It would be rare for such a huge primary lesion not to show any pulmonary metastases if osteosarcoma had been present over approximately one year. Pulmonary metastases have been reported even in the cases of chondroblastoma, and these metastases were generally observed after operative managements of the primary lesion –5, 9. An


A 33-year-old man presented with a pulsatile mass in the left temporal region; about 1 year before the current presentation, the swelling had arisen on the upper lateral border of the orbital arch and increased in recent months. His medical history was negative for accidental or iatrogenic head injury. Color echo Doppler and angio-computed tomography demonstrated a fusiform aneurysm of the zygomatic orbital artery, a branch of the superficial temporal artery. Blood tests were negative for human immunodeficiency virus (HIV), hepatitis C (HCV), and hepatitis B (HBV) markers. Aneurysmectomy under local anesthesia was performed. Histology of the surgical specimen confirmed the diagnosis of a true aneurysm measuring 8.4 × 5.7 mm, which showed no atherosclerotic degeneration of the vessel walls; the lumen was filled by a recent thrombus but without inflammatory cells. Surgical treatment is indicated for the prevention of rupture, the relief of pain when present, and the removal of facial defects. To the authors' knowledge, this is the first case in the literature of a true aneurysm of the zygomatic orbital artery. The superficial temporal artery (STA) is one of the terminal branches of the external carotid artery. At the zygomatic process, it divides into a frontal (anteriorly) and a parietal (posteriorly) branch. Just before its bifurcation, the transversal facial artery, the anterior auricular arteries, and the zygomatic orbital artery branch out of it. The zygomatic orbital artery runs parallel to the zygomatic arch and anastomizes with the palpebral artery and the lacrimal artery, both branches of the ophthalmic artery. In 22% of cases, the zygomatic orbital artery is absent .Aneurysms of the STA account for nearly all posttraumatic pseudoaneurysms  among the over 400 cases described in the literature . In contA 33-year-old man, in otherwise good health, was seen because of a subacute enlargement of a pulsatile mass in the left temporal region. His medical history was negative for accidental and iatrogenic head injury, headache, and cardiovascular risk factors. Physical examination revealed a small pulsatile mass on the upper lateral border of the left orbital arch ; the sweTo date, no cases have been reported of spontaneous aneurysm of the zygomatic orbital artery, one of the branches of the STA. True aneurysms of the STA are fairly rare, whereas those from injury are much more common (about 90% of cases)  because Spontaneous or nontraumatic aneurysms may be caused by atherosclerosis , congeniThe differential diagnosis includes hematoma, cysts, reactive lymph node, lipoma, and arteriovenous fistula. On palpation, the aneurysm can be appreciated as a pulsatile mass; pulsation of the swelling will diminish or disappear when the artery is compressed proximal to the mass. The presumptive diagnosis can be confirmed by color echo Doppler, which shows triphasic blood flow within the lesion. Second level diagnostic imaging studies (CT scan and magnetic resonance angiography) are useful for confirming the diagnosis and to detect possibly associated intra- and extracranial aneurysms .Scrupulous history taking ruled out previous head injury  in our patient. Histology demonstrated that the aneurysm involved all three vessel wall layers , withoutOwing to its low incidence, the natural history of true aneurysms of the STA is unclear, whereas pseudoaneurysms are known to tend to progress to acute thrombosis or rupture . The litTrue spontaneous STA aneurysms are a rare occurrence and are nearly always posttraumatic pseudoaneurysms. Careful history taking and clinicodiagnostic assessment are key to establishing a correct differential diagnosis.Therapeutic treatment is indicated for preventing rupture, relieving pain, and restoring cosmesis in particular. Surgery is the first choice of treatment for STA aneurysms, given the low risk and high success rates, whereas selective embolization may offer an alternative surgical option in some cases. In our opinion, however, especially in cases such as ours, the zygomatic orbital artery is not amenable to endovascular treatment because of its extremely small caliber (±1 mm) and because of the high risk of failure to remove the facial defect caused by the aneurysm.This case holds interest because, to the authors' knowledge, it is the first in the literature of a spontaneous aneurysm of the zygomatic orbital artery without traumatic origin.


Malignant acanthosis nigricans (AN) is a rare paraneoplastic syndrome seen primarily in adults with an underlying diagnosis of gastrointestinal adenocarcinoma. Malignant AN is characterized by hyperpigmentation and velvety hyperplasia of the epidermis. This condition is generally not associated with tumors in pediatric populations or in the adrenal gland. We present a case of malignant AN in a pediatric patient with a nonmalignant, functional adrenocortical tumor. Childhood adrenocortical tumors (ACTs) generally present during the first 5 years of life, with a second, smaller peak noted during adolescence –4. MajorThe diagnosis of ACTs in pediatric patients is generally made within 5–8 months of the first presenting signs and symptoms , 4, 5; hMalignant acanthosis nigricans (AN) is a rare paraneoplastic syndrome seen mainly in adults with adenocarcinomas, primarily of gastric origin , 8. MaliA previously healthy 33-month-old Caucasian male presented with a three-month history of rapid virilization and a neck rash. Height and weight remained unchanged over the preceding months at the 50th percentile. Blood pressure at the time of diagnosis was 123/65 mmHg and heart rate 112 bpm. Physical examination was notable for coarse facial features, facial acne, anterior and posterior cervical acanthosis nigricans, Tanner 2 pubic hair, and pubertal phallus (8 cm in length and 2 cm in diameter), but prepubertal testes at 3 mL volume bilaterally. Laboratory evaluation revealed an elevated nonfasting insulin level with normal values for glucose, random cortisol 19.6 mcg/dL , ACTH 8.35 pg/mL , and hemoglobin A1C 4.7% . Abnormal laboratory findings for age included elevated testosterone, androstenedione, dehydroepiandrosterone sulfate (DHEAS), all within Tanner 2-3 range. IGF-I level was increased for age. Gonadotropins were Tanner stage 1, confirming a peripheral source for androgen production. Serum electrolytes, beta-HCG, and alpha-fetoprotein levels were all within normal limits. MRI of the abdomen revealed a 2.3 × 3 cm mass within the right adrenal cortex, absent retroperitoneal lymphadenopathy, and lack of tumor thrombus within the IVC. The patient underwent a successful right adrenalectomy and retroperitoneal lymph node dissection without any complications. Postoperative screening morning cortisol was undetectable (<1.0 mcg/dL), indicating suppression of contralateral unaffected adrenal gland. He was treated with stress-dose hydrocortisone and subsequently transitioned to and slowly weaned off of physiologic replacement postoperatively. Follow-up random cortisol level one year postoperatively was normal at 7.1 mcg/dL . Pathology review revealed a 12.5-gram encapsulated tumor without hemorrhage, necrosis, or capsular invasion . Tumor cACT peaks during the first and fourth decades of life . The incAcanthosis nigricans (AN), a common cutaneous finding, is characterized by hyperpigmentation and velvety hyperplasia of the epidermis . In geneThe pathophysiology of malignant AN is not well understood. Elevated levels of growth factors released from tumors, such as transforming growth factor alpha , can bind to epidermal growth factor receptors (EGFR) in the epidermis and stimulate keratinocyte growth , 12, 17.Our patient's AN likely resulted from excess glucocorticoid production by the adrenal tumor, albeit not captured in preoperative laboratory studies. His elevated IGF-1 driven by increased adrenal androgens may have also played a role. Contralateral adrenal cortical suppression with undetectable cortisol and suppressed ACTH after surgery points to excess glucocorticoid hormone secretion by the adrenal mass in addition to the noted elevated adrenal androgens. The excess glucocorticoid production likely contributed to glucocorticoid-induced insulin resistance, resulting in AN. This case represents the first reported association of malignant AN with a nonmalignant, functional cortical adrenal mass in a pediatric patient and an important clue to a possible underlying cortical adrenal tumor as a contributing cause for this unusual phenomenon.


A diagnosis of primary osseous HL was made. The patient received systemic chemotherapy and local radiotherapy, and was on regular follow-up for 24 months. There was no sign of recurrence of tumor and lymph node or bone marrow involvement. Because there is a possibility of secondary bone involvement by systemic HL, strict histological analysis and thorough radiographic examination are suggested to be necessary for accurately diagnosing this tumor when it presents as a solitary bone lesion.Hodgkin lymphoma (HL) typically presents as nodal lesion and may involve extranodal sites during the progression of the disease. Primary osseous HL without any lymph node association is extremely rare and only a few such cases have been described in the literature. We present a case of unusual primary HL in rib occurring in a middle-aged female patient. Computed tomography (CT) scan revealed an osteolytic lesion was located at the right second rib and was associated with a large soft tissue mass. There was no regional lymph node involvement. CT scan of neck and abdomen was performed and showed no pathologic findings, particularly no lymphadenopathy and organomegaly could be observed. Histologically, typical binucleated Reed-Sternberg (RS) cells and lacunar cells were scattered in the background of reactive inflammation with infiltration of lymphocytes, histiocytes and eosinophilic granulocytes. By immunohistochemistry, RS cells and lacunar cells were positive for CD15 and CD30 with typical membrane and paranuclear dot-like staining pattern. However, these cells were negative for Epstein-Barr virus detection by The virtual slide(s) for this article can be found here:http://www.diagnosticpathology.diagnomx.eu/vs/2846916171507084 Hodgkin lymphoma (HL) most commonly occurs in cervical lymph nodes, and the extranodal forms are rare accounting for less than 1% . TypicalA 38-year-old Chinese female presented with pain at the right upper side of the chest and adjacent soft tissue swelling for 3 months. A week before admission to our hospital, she was suffering from a gradually severe pain at the chest and fever. As a result, the patient was referred to our hospital for examination and treatment. Physical examination showed the patient had a mild soft tissues edema at her right upper lateral chest wall and severe pain was elicited upon chest pressure. The patient had a low grade fever of 37.4°C. There was no weight loss and no palpable lymphadenopathy or organomegaly. The laboratory results, including blood count, differential, liver and renal function, were within the normal range. A computed tomography (CT) scan of the chest revealed a single osteolytic lesion in the right second rib with associated soft tissue mass, measuring 6.0 × 5.0 × 5.0 cm in size. The most of rib was observed to be destroyed and the involvement of adjacent pleura and right upper lobe of lung were also noted Figure . The lesin situ hybridization for EBERs according to the manufacturer’s instruction . The antibodies were obtained from Dako Cytomation, Santa Cruz Biotechnology  and Novocastra laboratories LTD .The surgical biopsy was routinely fixed in 10% neutral buffered formalin. The tissues were embedded in paraffin. Four micrometer-thick sections were stained with H&E. Immunohistochemical analyses were performed using the ChemMate Envision/HRP Kit . The antibodies used in this study included a broad panel of antibodies against CD1a, CD3, CD4, CD8, CD15, CD20, CD30, CD38, CD43, CD45,CD56, CD79a, ALK, Pax-5, BOB-1, OCT-2, S-100 protein, pan-cytokeratin and epithelial membrane antigen (EMA). Epstein-Barr virus (EBV) infection was also assessed by Under microscopic examination, the lesions were characterized by a fibrosis developing in bone marrow spaces, which was infiltrated with diffuse lymphocytes, plasma cells, histiocytes, and eosinophilic and neutrophilic granulocytes. Typical morphology of Reed-Sternberg (RS) cells, with large cytoplasms and prominent eosinophilic nucleoli, were scattered in the background of reactive inflammation. Lacunar cells, mummified cells and small foci of acidophilic necrosis were also found in the tissue. The immunohistochemistry staining of the RS population confirmed the expression of PAX-5, CD30 and CD15. However, these cells did not express CD1a, CD3, CD4, CD8, CD20, CD38, CD43, CD45, CD56, CD79a, ALK, BOB-1, OCT-2, S-100 protein, pan-cytokeratin and EMA  are well established lymphoproliferative neoplasms of the lymph nodes with bimodal age curve. The extranodal HLs are rare and account for less than 1%. The most common site of extranodal HLs is gastrointestinal tract, followed by the pulmonary system, thyroid, skin, genitourinary system, and central nervous system. Although osseous involvement could be observed in the late stages of HLs, it is extremely rare for patients to present with primary HLs of the bone. To our knowledge, so far primary osseous HL has been reported in approximately 30 patients in the literature. Some of the cases have been associated with extra-osseous involvement. Primary osseous HL confined to the bone is so rare that approximately 19 cases have been reported worldwide to date, only 12 patients with primary solitary osseous HL -14 and 7d-glucose positron emission tomography/computed tomography (18 F-FDG-PET/CT) enables systemic HL with secondary bone invasion to be distinguished from primitive osseous HL. This technique is highly specific in demonstrating the isolated osseous localization of the tumor and should be recommended in all patients with putative osseous lymphoma [Since cases of primary osseous HL are so rare, the diagnosis should be only made by strict histological and clinical manifestation. According to the WHO criteria, a diagnosis of primary bone lymphoma must be (1) a single skeletal tumor, with or without regional lymph node involvement, and (2) multiple bone lesions without visceral or lymph node involvement . Many prlymphoma .Regarding the cellular origin of RS cells in HLs, especially for those in extranodal lesions, it remains to be determined whether RS cells are clonal B cells that have lost their B cell phenotype due to destructive somatic mutation . Althougin situ hybridization although it presented typical histological and immunohistochemical profile of classical HL. Therefore, detection of EBV-encoded products in tumor is not a definitive point to confirm the diagnosis of HL. In addition, classical HL has to be distinguished from anaplastic large cell lymphoma (ALCL) in such an unusual extranodal location because both tumors show CD30 immunoreactivity with typical membrane and paranuclear dot-like staining pattern [Osseous HLs usually show osteolysis in radiological examination, it is often misdiagnosed as osteomyelitis ,16,29. TIn conclusion, only a few cases of primary osseous HL have been reported in the literature. Our additive case is also presented for its rarity of site. It is the first case of primary osseous HL occurring in Chinese. The diagnosis of primary osseous HL is difficult and should be made cautiously. Besides confirmation by strict histopathological and immunohistochemical analysis, radiographic examination is helpful for demonstrating potential extra-osseous lesion to distinguish systemic HL with secondary bone invasion from primary osseous HL. In clinical, an immediate diagnosis at the early stage of disease and timely treatment with systemic chemotherapy and local radiotherapy are required because the patients with primary osseous HL appear to have a good long-term prognosis when the lesion is restricted in bone without systemic dissemination.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 we have no competing interests.YL and XB W made contributions to acquisition of clinical data, and analysis of the histological features by H&E staining they are joint first co-authors and made an equal contribution to this work. XYT drafted the manuscript. ZL revised manuscript critically for important intellectual content and had given final approval of the version to be published. BL carried out the immunoassays. All authors read and approved the final manuscript.