Most spermatic cord masses are benign, and malignant spermatic cord tumors are uncommon. Spermatic cord metastases originating from hepatocellular carcinoma (HCC) have not been previously reported in the English language literature as determined by a PubMed search. We report a male patient who presented with a painful palpable mass in the right inguinal area. The patient was diagnosed with HCC in 2004 and undertook a nonsurgical approach to control the cancer. A radical orchiectomy was performed, and the pathological report showed metastatic HCC in the spermatic cord. The patient received palliative radiation therapy because of a positive surgical margin. No recurrence was noted after 6 months of followup. Extrahepatic lesions are not uncommon in late-stage HCC patients. These extrahepatic lesions are most commonly found in the lungs, lymph nodes, and bones . There hA 57-year-old man was referred to our urology out-patient department from a gastrointestinal doctor because of a tender inguinal mass that had become progressively larger over the previous one and half months. The patient had a past history of HCC , hepatitis B virus infection, rightside inguinal hernia (status postherniorrhaphy in 2007), a cholecystectomy in 2000, and a vasectomy in the 1990s.He noticed a painful hard mass in the right inguinal area one and half months prior to visiting our department. The mass was progressively enlarging and was nonreducible. Physical examination revealed a 2 × 2 centimeter hard, movable, tender mass in the right inguinal area. An excision biopsy was performed, and the pathological report revealed tumor cells with abundant amphophilic to pale eosinophilic cytoplasm, round nuclei, mitosis, and focal necrosis, and the cells were Hep Par 1 positive as determined by immunohistochemical staining . The diaHCC is the most common primary malignant tumor of the liver. HCC is commonly seen in Taiwanese hospitals due to high incidence of hepatitis B and C in Taiwan. Extrahepatic metastases of hepatocellular carcinoma are not uncommon in late-stage HCC patients. Most documented extrahepatic metastatic lesions have been found in the lungs followed by the lymph nodes, bones, and adrenal glands, but there are cases of distal metastasis to the genitourinary system, such as the kidneys and bladder , 3, 4. SWhen patients present with an inguinal mass, we need to take into consideration several differential diagnoses ranging from benign to malignant lesions . SpermatIn this case, a metastatic mass was highly suspected due to the history of HCC. A metastatic tumor originating from HCC was confirmed after excision biopsy. According to the national comprehensive cancer network clinical practice guideline for metastatic HCC , only so What kind of treatment is suitable for tumor emboli in the surgical margin of the spermatic cord? According to previous reports, palliative radiation therapy has been used to treat HCC with brain and bone metastasis with successful symptom control . RadiatiIn conclusion, we report the first case of metastatic HCC in the spermatic cord, which presented as a painful inguinal mass. Complete tumor excision with clear margins should be performed for spermatic cord tumors. Palliative radiation therapy is a choice for the treatment of metastatic HCC with tumor emboli in the surgical margin.


Onchocerca volvulus, the agent of the so-called “river blindness”, affects about 37 million people globally. Other Onchocerca spp. have been sporadically reported as zoonotic agents. Cases of canine onchocerciasis caused by Onchocerca lupi are on the rise in the United States and Europe. Its zoonotic role has been suspected but only recently ascertained in a single case from Turkey. The present study provides further evidence on the occurrence of O. lupi infesting human eyes in two patients from Turkey (case 1) and Tunisia (case 2). The importance of obtaining a correct sample collection and preparation of nematodes infesting human eyes is highlighted.Among ocular vector-borne pathogens, In both cases the parasites were identified with morpho-anatomical characters at the gross examination, histological analysis and anatomical description and also molecularly in case 1.O. lupi based on their morphology at the gross examination, histological analysis and anatomical description. In the second case, although the diagnostic cuticular characters were not completely developed, other features were congruent with the identification of O. lupi. Furthermore, the morphological identification was also molecularly confirmed in the Turkish case.The nematode from the first case was obviously O. lupi infestation is not an occasional finding but it should be considered in the differential diagnosis of other zoonotic helminths causing eye infestation in humans . Both cases came from areas where no cases of canine onchocerciasis were previously reported in the literature, suggesting that an in depth appraisal of the infestation in canine populations is necessary. Physicians and ophthalmologists are advised on how to preserve nematode samples recovered surgically, to allow a definitive, correct etiological diagnosis.The results of this study suggest that  Onchocerca volvulus , which affects about 37 million people [Brugia spp., Thelazia spp., Dirofilaria spp., and Wuchereria spp.) may infest human eyelids, conjunctival sacs, lachrymal glands and, in some cases, the ocular globe.Many species of helminths may cause human blindness in developed and developing countries, and some of them still represent a major threat for public health . This isn people  in East Wuchereria bancroftiBrugia malayi and Loa loa), others of domestic and wild mammals are regarded as zoonotic agents  [Onchocerca lupi, a parasite described in the periocular tissues of a Caucasian wolf (Canis lupus) in Georgia [O. lupi had been attributed to a technical error (mislabelling of the specimen) or to a case of aberrant infestation, mainly because of the ‘unusual’ finding of a parasitic nematode specific to ungulates in a canid host. However, O. lupi types were re-examined [et al.[O. lupi, a conclusion only recently confirmed molecularly, in two cats [O. lupi induces acute or chronic ocular disease in dogs, characterized by conjunctivitis, photophobia, lacrimation, ocular discharge and exophthalmia [O. lupi have been suspected [Filarioids are parasites that also infest the human eye and, besides these species exclusively infesting humans  -7. The l Georgia  that remexamined ,10, the examined . In dogsexamined -16. Sincexamined , all theexamined -20 were d [et al. argued ttwo cats . O. lupihthalmia . In humauspected  and, recuspected .Onchocerca species are agents of zoonoses. Among the 15 clinical cases of zoonotic onchocerciasis reported worldwide [Onchocerca gutturosa[Onchocerca cervicalis[Onchocerca jakutensis from the European deer [Onchocerca dewitteijaponica from wild boar, reported only in Japan over the last 20 years [O. gutturosa and O. cervicalis presented an ocular localization, whereas other species were detected mostly in the subcutaneous tissues. Information on the ocular zoonosis due to O. lupi remains meagre and whether the human case above was an occasional finding is unclear.Several orldwide ,25, the gutturosa and Onchervicalis, affectiean deer  and, in O. lupi infesting the human eye in two patients, from Turkey and from Tunisia where onchocerciasis caused by O. lupi has never been reported in dogs. In both cases, the nematode was erroneously identified either as Dirofilaria immitis[Dirofilaria sp. , respectively. The main gross morphological and histological features of this parasite have been discussed here as well as the relevance of appropriate sample collection and preparation, instrumental to a correct diagnosis of nematodes infesting the human eye. The parasites were identified morphologically and also molecularly in the Turkish case.The present study describes the occurrence of a immitis or as a Dirofilaria repens.A 26 year old male patient (case 1) was admitted to the Department of Ophthalmology of the Hospital of the Istanbul Faculty of Medicine (Turkey), presenting irritation and itchiness of the right eye. The patient lived in Istanbul  and had never travelled abroad except to Antalya  where he spent ten days of vacation, during the previous summer . The patient experienced a “growing redness” nasally to the right eye for two weeks. The orbital CT at the lesion site showed an increase in density of soft tissues but no foreign bodies. On admission the visual acuity was 10/10 for both eyes with no alterations of the left eye. The right eye showed a normal presentation except for 5x5 mm swelling and a conjunctiva mass in the nasal conjunctiva region. Topical corticosteroid  and antibiotics , 4 drops per day and systemic antibiotics , twice a day were administered, for one week. After one week no clinical improvement was observed and the presence of a foreign body was suspected, thus explorative surgery was undertaken. At the conjunctiva excision Figure  of the fet al.[D. immitis. Briefly, the patient was an 8-year old child referred to the Service d’Ophtalmologie, Farhat Hached, Tunis (Tunisia), the child had been suffering from a fastidious pain of the right eye for four weeks. The patient lived in the region of Kasserine , which is at the centre of an irrigated agricultural area, and had never travelled abroad or to other areas. At the medical examination a subconjunctival mass (0.8 cm in diameter) was observed and immediately surgically excised and delivered to the Laboratoire d’Anatomie et de Cytologie Pathologiques, of the same Hospital for examination. Differently from case 1, along with the parasite, host tissues around the nematode were extracted from subconjunctiva and processed histologically. Following the request of one of the authors (O.B.) the histology of paraffin block samples was made available for further examination and confirmation of the diagnosis (see Results section).In case 2, described in Ziadi et al., the extIn both cases, the presence of dogs living in the same area as that of the patient was reported. In addition, for case 2, cats, donkeys, sheep, goats and cattle were also present in the setting. No history of insect bites was reported. Written informed consent was obtained from the patients for publication of this report and any accompanying images.et al.[Both samples were fixed in 10% buffered formalin solution pH 7.4), paraffin embedded and routinely processed for light microscopy. Sections of 5 μm thickness were stained with haematoxylin and eosin (HE) and, in case 2, also with Masson Goldner trichrome for a clearer definition of the parasitological and morphological features. The histological preparations were morphologically examined and photographed. The paraffin blocks were eluted by the heat (case 1) or in xylol (case 2) and nematode fragments were recovered for morphological description , or molecular identification (case 1). The diverse fragments (case 2) recovered from the paraffin block were cleared in lactophenol, observed, drawn and measured as described in Bain .4, parafOnchocerca and its species. Briefly, the cuticle of female body in this genus is composed of two layers, the external with prominent transverse ridges, the internal with transverse striae; the shape of ridges , the distance between ridges, the number of striae per inter-ridge are specific [Onchocerca is not present in the anterior 5 mm but it is formed progressively posteriorly. This feature is acquired during adult growth and in young females ridges are tiny and straight and striae are absent [Onchocerca the buccal cavity is tiny, the vulva is in the oesophageal region, the vagina simple without bends and sphincter; the oesophagus is divided or not, depending on the species [During the study, particular attention was paid to the main diagnostic characters of the genus specific , as wellspecific . The feme absent . Other c species .O. lupi from dogs in Greece  and histological sections of infested sclera from a dog in Hungary  were studied.For comparison with the present worms, specimens of c oxidase subunit 1 (cox1) and 12S genes as described elsewhere [The molecular diagnosis was performed on the specimen from case 2 by genomic extraction and amplification of mitochondrial cytochrome lsewhere .O. lupi. The cuticle was composed of an external layer bearing prominent, transverse ridges, rounded and undulated, spaced from 13 to 30 μm (mean 21.3 μm) and an internal layer with transverse striae .Case 2, from Tunisia (308 JW) was caused by an immature adult female worm. Several coiled fragments (9.6 mm long) were recovered from the paraffin block including one anterior Figure , 2A. The1O. lupi from Greece , two other Onchocerca species, O. volvulus and O. lienalis, which also present two striae per interidge, were also considered [O. volvulus is distinct with wider and thicker lateral chords and more reduced muscles cells [O. lienalis is characterized by ridges and striae developed in correspondence with the posterior extremity of the body only, the cuticle surface is scalloped, the lateral chords are narrow and thick all along the body, and the muscle cells are thicker; in addition, the anterior female region is almost twice as thin [Onchocerca species, namely Onchocerca raillieti, which might be present in local donkeys and possible agent of zoonosis, differs from the nematode identified herein in that the former is characterized by a divided and long oesophagus, as well as cuticle presenting internal striae but no external ridges [All features observed fit previous descriptions ,9,22, annsidered . O. volves cells . O. lien as thin . Anotherl ridges .cox1 and 12S genes of the nematode from case 1, showed a 99-100% nucleotide homology with sequences of O. lupi available in GenBank .In accordance with the morphological identification, the BLAST analysis of O. lupi[O. lupi had never been previously reported. In both cases no information on the occurrence of the infestation in dogs, the proper host for O. lupi, is available indicating that parasitological studies should be carried out to estimate the actual distribution of this parasite circulating in dog populations. Considering the narrow host range of parasites belonging to the Onchocerca genus [O. lupi infects both humans and dogs suggests that, if these two species share the same environment, humans may be at risk of infestation. Unfortunately, up until now infestation by O. lupi was reported only on occasions where dogs presented clinical signs . In contrast, no extensive cognitive surveys based on the diagnosis of microfilariae in skin samples are available, most likely reflecting an underestimation of the actual distribution of this parasitic disease. Definitive evidence that O. lupi may infest humans, and thus the zoonotic role of this nematode, are represented by the fact that the nematode from case 1 was a mature female nematode. This is inferred not only by the measurements of the body fragments and by the morphological characteristics of the cuticle.Following the first evidence of human zoonotic infestation by O. lupi this stuca genus ,30,33, tDirofilaria spp.. Definitively, O. lupi should be considered in the differential etiological diagnosis of nematodes in human eyes. The morphological distinctive characters of female nematodes allow a straightforward identification since O. lupi presents thick cuticle composed of an external layer bearing prominent, undulated annular ridges and an internal layer with transverse striae. These morphological features are not present in the species suspected initially to be agents of these cases; D. repens has longitudinal crests and D. immitis has a smooth cuticle. Without any doubt a proper preparation of samples is pivotal for achieving an etiological diagnosis of parasites extracted from human eyes. Physicians and ophthalmologists should be aware that before treating samples in formalin for further histological analysis or simply for preservation, they should save a small amount of material in ethanol so that it can be processed molecularly. Indeed, a specific molecular assay was useful for case 1 which provided further confirmatory evidence on the etiological diagnosis. Recently, an integrated DNA barcoding of cox1 and 12S mitochondrial markers and morphological approaches were shown to be powerful tools for the taxonomic identification of many filarioid species, including O. lupi[O. lupi infestation in human patients living in, or who have travelled to endemic areas. Indeed, although helminths infesting the human eyes or the surrounding tissues should be removed by surgical procedures, therefore allowing diagnoses to be achieved by means of sample examination, the detection of antibodies against O. lupi could be indicative of the infestation, as in the case of O. volvulus[The results here presented also suggest that this zoonotic infestation is more diffused than previously believed, being probably misdiagnosed with other helminths infesting human eyes such as g O. lupi,24, eveng O. lupi. Additio volvulus.O. lupi needs to be assessed in order to establish their activity as primary definitive hosts and to assess the risk for human infestation in a given area. Accordingly, knowledge on the biological vector of this infestation will contribute to better understanding of the transmission patterns and the seasonality of this zoonotic infestation.The role played by dogs as reservoirs of The authors declare that they have no competing interest.DO, OB conceived the research, contributed with data analysis and interpretation and wrote the first draft of the manuscript and contributed the morphological examination. OB, YM made drawings. FDT contributed with data analysis, interpretation and revision of the manuscript. ZC, BY, NB, OBB, AG, TM collected samples and were responsible for all ophthalmologic aspects and the nematode collection. All authors read and approved the final version of the manuscript.


Prostatic metastasis from a primary bowel adenocarcinoma has been only rarely reported in the medical literature. The case reported here is rare in the fact that the primary tumor was from a right-sided bowel adenocarcinoma. It is unusual because initial immunostaining was not fully conclusive, and so a relatively new method of immunostaining, CDX2, was used to ascertain its histopathology.We describe the case of a 54-year-old Caucasian man who had a right hemicolectomy for a primary cecal adenocarcinoma, which was completely excised. Following the procedure, he received adjuvant chemotherapy. Computed tomography scans showed no evidence of local recurrence or metastatic disease. Then, five years later, he presented to his general practitioner with urinary symptoms. An abnormal prostate was palpated on digital rectal examination. Trans-rectal prostatic biopsies were performed, which showed colorectal metastases within the prostate gland. This was confirmed with CDX2 immunohistochemistry. There was no further evidence of distant metastases on positron emission tomography-computed tomography scans.This case demonstrates a rare isolated hematogenous spread to the prostate from a primary cecal adenocarcinoma, several years after definitive treatment and excision. This highlights the importance of accurate immunohistochemistry and imaging in planning further management and treatment. Prostatic metastasis from a primary bowel adenocarcinoma has been only rarely reported in the medical literature. We describe a case of a rare metastasis from a right-sided primary bowel adenocarcinoma to the prostate gland. It is unusual because a relatively new method of immunostaining, CDX2, was used to ascertain its histopathology.A 54-year-old Caucasian man was admitted to our hospital with an acute history of abdominal pain and vomiting, and a four-month history of changes in bowel habits. A computed tomography (CT) scan of his abdomen and pelvis confirmed a small bowel obstruction secondary to a mass lesion at the cecal pole. A few lymph nodes were identified adjacent to the cecal pole, measuring approximately 1 cm in size.On laparotomy, a mobile cecal tumor was found, with no other evidence of intra-abdominal metastatic disease. A right hemicolectomy was performed. He made an uneventful recovery and was discharged one week after surgery.Histology results showed a moderately differentiated mucinous adenocarcinoma  Dukes C1, with an incidental carcinoid tumor in the appendix. Surgical resection margins were clear. Carcinogenic embryonic antigen (CEA) staining was diffusely positive, cytokeratin 20 (CK20) staining was focally positive, and cytokeratin 7 (CK7) staining was negative. A CK7-negative and CK20-positive profile favors a primary colorectal tumor, although CK20 staining was weakly positive.He underwent adjuvant chemotherapy as part of the QUASAR ('Quick and Simple and Reliable') trial . A post-After five years of follow-up, he was referred by his general practitioner to our urology department with lower urinary tract symptoms and an abnormal prostate on digital rectal examination. There was a large, suspected to be malignant, extrinsic pelvic mass that could not be palpated separately from the prostate. His prostate specific antigen (PSA) level was 1.3 ng/ml .Trans-rectal ultrasound prostate biopsies were taken and results showed normal background prostatic tissue, with all cores infiltrated with mucinous adenocarcinoma. Immunohistochemistry results were positive for CEA staining but negative for PSA, CK7 and CK20 staining.The original bowel specimen was again analyzed and the morphology of the tumor in the bowel specimen was identical to that in the prostate. Additional staining was performed, and both the bowel and prostatic specimens tested positive for CDX2 Figure . It was A fused positron emission tomography-computed tomography (PET-CT) scan was performed to exclude further distant metastatic disease. This demonstrated increased uptake in the prostate with central necrosis, consistent with a metastasis Figures  and 3. TThere have only rarely been other reported cases of prostatic metastasis from primary bowel adenocarcinomas -4. OtherCDX2 is a monoclonal antibody to the intestinal-epithelia-specific nuclear transcription factor, and is a relatively new marker for gastrointestinal tumors. It is expressed in the nuclei of intestinal cells throughout the intestine from duodenum to rectum . Immunohet al. demonstrated 60% positivity for CDX2 in primary colorectal cancers versus 0% expression in primary prostate cancers [Owens  cancers .CDX2's positive nature in the prostate specimens, as well as the original bowel specimens from this case, strongly correlate with a diagnosis of metastatic spread from the original cecal adenocarcinoma to the prostate. One would expect CK20 staining to be positive in the prostatic specimens, but staining on the original bowel tumor was only weakly positive, which may account for the metastatic focus showing a negative CK20 profile. This stresses the importance of advanced immunohistochemical and pathological techniques in differentiating tumor origin in patients with previous malignancy and uncommon sites of metastatic disease. This enables accurate diagnosis and appropriate treatment.This case demonstrates a rare isolated hematogenous spread to the prostate from a primary cecal adenocarcinoma, several years after definitive treatment and excision.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.FRY drafted and wrote the manuscript and summarized pertinent features of the case. LH aided in the summary of the case as a whole and was a major contributor to writing the manuscript. PDM selected and annotated appropriate images from cross-sectional imaging. DRT selected and annotated appropriate images from histopathology slides and reviewed all available histology to ensure an accurate diagnosis was made. RG aided in the summary of the case from a colorectal perspective. MJJ aided in the summary of the case from a urological perspective. All authors read and approved the final manuscript


NR4A3 rearrangement was present in four of four cEMCs and in none of the MECs. In contrast, three of nine (33 %) MECs and four of five (80 %) cEMCs showed an EWSR1 rearrangement. In summary, MECs and cEMCs share clinical, morphological, immunohistochemical and genetic characteristics. The pathognomic rearrangement of NR4A3 is a useful diagnostic feature in identifying cEMCs.Myoepithelial carcinoma of soft tissue (MEC) and cellular extraskeletal myxoid chondrosarcoma (cEMC) share striking similarities. In this paper, we compare ten MECs with five cEMCs. MEC patients had an equal gender distribution. The age range was 15–76 years . Tumours were located on extremities, pelvic girdle, vulva and neck. Follow-up, available for nine patients, ranged from 4 to 85 months . Five patients were alive without evidence of disease, two were alive with disease and two died 8 months after the initial diagnosis. cEMCs were from three males and two females with an age range of 37–82 years ; they presented in extremities, shoulder and paravertebral/cervical. Follow-up, available for four patients, ranged from 6 to 220 months . All patients were alive, two with recurrences and/or metastases and two without evidence of disease. Morphologically, the distinction between these two entities was difficult since all cases exhibited features typically seen in myoepithelial tumours. Immunohistochemically, MECs expressed pan-keratin (80 %), epithelial membrane antigen , S100 (50 %), alpha-smooth muscle actin , calponin (67 %) and p63 (25 %). S100 and EMA were expressed in 40 % of cEMC cases respectively with additional immunoreactivity for p63, ASMA and glial fibrillary acidic protein in one case. Pan-keratin was negative in all neoplasms.  Myoepithelial tumours of the soft tissue were initially characterised in a series by Kilpatrick et al. . Later oExtraskeletal myxoid chondrosarcomas (EMCs) reveal a cord- or lace-like arrangement of small round to spindle-shaped cells with distinct eosinophilic cytoplasm distributed in a prominent myxoid stroma. In cellular lesions (cEMCs), amounting to one third of the cases, there is a greater morphological diversity –11. AlthNR4A3  located at 9q22 are characteristic for EMC and have never been described in myoepithelial tumours. In contrast, both tumour types harbour EWSR1  rearrangement [Histologically, cEMC can be morphologically similar to myoepithelial tumours but the latter have a broader immunohistochemical profile –4, 12. Gangement –25.In this paper, we report on the morphological, immunohistochemical and molecular overlap of MEC and cEMC.We searched the surgical pathology and referral files of the authors for the diagnoses myoepithelial tumours of soft tissue and EMC. Slides were reviewed and the diagnoses were based on criteria according to the recent WHO classification . MyoepitIn all cases, the tissue was fixed in 4 % buffered formalin, routinely processed and embedded in paraffin; 2–4-μm-thick sections were stained with hematoxylin and eosin. Immunohistochemical methods consisted of the labelled Streptavidin Biotin technique using commercially available antibodies as listed in Table EWSR1, a directly FITC/Rhodamine-labelled break apart-probe  was used [NR4A3 rearrangement (break apart probe) were generated in-house. BAC clones RP11-624K13 (centromeric), RP11-412F16 (centromeric), RP11-121L12 (telomeric) and RP11-467B11 (telomeric) were obtained from the BACPAC Resources Center . Clones were either labelled with biotin or digoxigenin using a nick translation kit, according to manufacturer’s instructions . Copy numbers of chromosome 9 were assessed using a centromere probe (CEP9). A negative control was used for each tumour. A case was considered having a break when at least 10 of 50 counted tumour cells (20 %) showed separation of a red and green signal.Fluorescence in situ hybridisation (FISH) was performed as described earlier. For was used . FISH prn-propanol precipitation. cDNA synthesis was performed in a 24-μl reaction containing 1 μg of RNA, 1 μg of random hexamers (Promega) and 20 nmol dNTPs (Invitrogen) and heated at 65°C for 5 min. Next, 2 μl of RNasin (Promega), 8 μl of ×5 first-strand buffer (Invitrogen), 4 μl of 0.1 M DTT (Invitrogen) and 2 μl of Superscript II (Invitrogen) were added and the sample was heated accordingly: 20°C for 10 min, 42°C for 60 min and 95°C for 3 min.RNA was isolated from formalin-fixed, paraffin-embedded material by proteinase K digestion, followed by phenol/chloroform extraction and EWSR1-NR4A3 and TAF15-NR4A3 fusion products were detected using primers targeting EWSR1 , TAF15 (TAF15 RNA polymerase II)  and NR4A3 .For EMC, most potential translocation-specific n = 4) with one case each on the thigh, calf, forearm and hand. Other sites were vulva (n = 2), gluteal (n = 1), sacral (n = 1) and the neck region (n = 1). In one case, the exact anatomic site was not known. Seven MECs were situated in the deep soft tissue and two subcutaneous. One superficially located tumour of the vulva showed exophytic growth. All patients underwent surgical treatment. Complete resection was reached in seven cases and marginal excision in one case. Tumour-positive margins were reported in two cases. Follow-up, available for nine patients, ranged from 4 to 85 months . Five patients were alive without evidence of disease at 12, 34, 39, 60 and 70 months, respectively. Two patients were alive with disease 4 and 85 months after the initial diagnosis, the latter with a second local recurrence. Two patients died of disease 8 months after the initial diagnosis. Case 10 was the first local recurrence on the hand with secondary bone involvement. Three patients presented with lung metastases. Involvement of regional lymph nodes was additionally observed in two of them, and one patient had bone metastases.Clinical details are summarised in Table n = 4) underwent resection. Tumour free resection margins were reported in three cases . In Case 14, the resection status was not known. Follow-up, available for four patients, ranged from 6 to 220 months . No patient died of the disease so far. Case 15 was the 4th recurrence of a tumour with primarily classical morphological features. This patient had also pulmonary spread. Another patient was known with regional lymph node involvement (Case 13).The five cases of EMC were from three males and two females with an age range of 37–82 years . Two lesions were located in the deep soft tissue of the thigh and one in the subcutis and soft tissue of the shoulder. One tumour each arose at the ankle and the paravertebral/cervical region. All patients but one  and for EMCs 1 to 15 cm . In one EMC, the size was unknown.Histologically, most cases demonstrated a (multi)nodular configuration with expansive margins and an incomplete pseudo-capsule. Infiltrative margins were focally seen. MECs showed in most of the cases varying growth patterns such as trabecular, reticular, nested and solid. A pure trabecular pattern was seen in two cases. Six tumours were composed of different cell types, including spindle, epithelioid, plasmacytoid and/or clear cells. Round cell morphology associated with clear cell features was observed in two cases and a pure epithelioid phenotype in two other cases Fig. . OsteoclAll EMC cases had focally classical features with strands and cords of small, uniform round to spindle-shaped cells set in a prominent myxoid matrix. There were round to oval nuclei and limited deeply eosinophilic cytoplasm. The tumour nodules, separated by fibrous septa, often showed peripheral cell condensation. In the cellular areas, sheets and nests of tumour cells were present. A trabecular and reticular-cystic pattern as well as loosely arranged cells occurred variably. The lesional cells were epithelioid and/or spindle shaped and slightly pleomorphic Figs.  and 4. CImmunohistochemically, eight of ten (80 %) myoepithelial carcinomas were, at least focally, positive for pan-cytokeratin and four of seven 57 %) for epithelial membrane antigen (EMA). The two cases negative for pan-cytokeratin expressed EMA. Two of eight cases showed nuclear immunoreactivity for p63 (25 %). Alpha-smooth muscle actin (ASMA) was detected in six of eight (75 %) and calponin in four of six cases (67 %). A focal expression of S100 was seen in five of ten cases (50 %). Glial fibrillary acidic protein (GFAP), in four cases performed, was negative in all four. In cEMCs, S100 and EMA were each expressed in two of five cases (40 %). One of the mentioned S100-positive cases (Case 11) showed a broader pattern of marker expression with additional immunoreactivity for  exhibited a NR4A3 rearrangement  but a fusion with EWSR1 or TAF15 was not found (RT-PCR). EWSR1-NR4A3 was evident in one neoplasm (Table In three of nine (33 %) MECs, a ent Fig. . One casThe first myoepithelial tumour of the soft tissue was published by Stout and Gorman in 1959 in a series of cutaneous lesions . In the EWSR1 rearrangement in a subset of benign and malignant myoepithelial tumours of skin, soft tissue, bone and visceral locations (lung) [POU5F1, PBX1 and ZNF444 [pleomorphic adenoma gene 1 (PLAG1) rearrangement was discovered in a subset of benign mixed tumours of skin and soft tissues (with well-formed ducts). It seems that this genetic abnormality excludes EWSR1 rearrangement [The occurrence of myoepithelial tumors at different sites possibly reflects an aberrant gene expression pattern during oncogenesis rather than an origin from a specific cell lineage . This iss (lung) , 31. Thed ZNF444 –25. Othed ZNF444 , 32–34. angement . WhetherWhen Enzinger designated extraskeletal myxoid chondrosarcoma as a distinct entity he already mentioned a resemblance with salivary gland type, mixed tumours of deep fascial region of thigh with reference to the report by Dutra in 1960 , 36. ClaBoth tumour types share features such as lobular/multinodular architecture, variable amounts of myxoid stroma and a reticular growth pattern. The stromal component in cellular areas can be poor or even absent. The cells are variably epithelioid, round and spindle shaped. Rhabdoid cytology may also be present in both. Unlike myoepithelial carcinomas, moderate to severe nuclear atypia is much less common in cEMC and can therefore be a helpful discriminating sign. This was also a finding in our series. Ductular structures and metaplastic cartilage or bone, present in not more than 20 % of soft tissue myoepithelial tumours, are not a feature of EMC , 38–41. Immunohistochemically, soft tissue myoepithelial tumours usually show expression of keratins (90–95 %), EMA 60 %), S100 85 %), GFAP (50 %), calponin (90 %), SMA 40 %) and p63 (40 %) 0 % and p. In cont0 %, S1005 %, GFAPSMARCB1/INI1 is also an overlapping feature of the described entities and has been demonstrated in a subset of cases. Whereas underlying genetic alterations (homozygous deletion and frameshift mutation) have been detected in some EMCs, genetic aberrations have not been investigated in myoepithelial carcinomas as yet [NR4A3. The described fusion partners are, in decreasing frequency, EWSR1, TAF15, TCF12 and TGF [Loss of s as yet , 39. Int and TGF –22.NR4A3 rearrangement was found in all of our successfully tested EMCs. In one case, we detected besides a TAF15-NR4A3 fusion an additional EWSR1 rearrangement which is in line with another reported more complex rearranged case [EWSR1 rearrangement, consistent with the results by Antonescu et al. [NR4A3 rearrangement in any of the myoepithelial carcinomas, but as previously reported, changes of chromosome 9 as a recurrent aberration were seen in two of our cases [By RT-PCR or FISH analysis, ged case . Furtheru et al. . We did ur cases , 34.NR4A3 and the general lack of keratin expression identify most cases of cEMC. The seemingly poorer outcome especially in young patients with myoepithelial carcinomas and possible different treatment options make discrimination between these different entities necessary.In summary, myoepithelial carcinoma of soft tissue and cellular extraskeletal myxoid chondrosarcoma share clinical, morphological, immunohistochemical and genetic similarities. The pathognomic rearrangement of


Glomangiomas are rare soft tissue tumors originating from the perivascular tissue. The most common localization is in the dermis of the extremities, with a few reports of respiratory tract involvement.We present the case of a 48-year-old Caucasian female patient with a glomangioma in her left lung. It was diagnosed incidentally as a coin lesion in a chest X-ray performed during preoperative work-up for a gastric Roux-en-Y bypass for alimentary obesity. A computed tomography scan of her chest revealed a lesion in her upper left lung lobe 31mm in diameter. After resection, a histopathological examination presented typical signs of a glomangioma, originating from the pulmonary parenchyma.Glomangiomas of the lung are extremely rare. However, whenever incidental lesions in the lung parenchyma are found, glomangioma should be taken into diagnostic consideration. To the best of our knowledge, signs of malignancy have not previously been reported in the literature. In fact, this tumor entity shows benign behavior, with a low potential for recurrence after complete resection. Glomangiomas are rare tumors typically observed in the dermis of the extremities. Very rarely, glomangiomas occur in different organs, including the respiratory system -3. We pr2) and arterial hypertension.A 48-year-old Caucasian female patient presented with an incidental finding of a solitary pulmonary lesion in a chest X-ray during preoperative work-up for a Roux-en-Y gastric bypass for alimentary obesity. Her medical history was unremarkable, except for obesity  scan showed low glucose utilization  and we did not suspect malignancy. A bronchoscopy with alveolar lavage did not show any intraluminal tumor growth and a histopathological examination revealed regular bronchial epithelial cells. Initially, our patient refused an operation to remove the glomangioma. A chest CT scan three months later showed the tumor had not changed. At this time, our patient agreed to a surgical procedure and we performed a pulmonary wedge resection of her upper left lobe via a mini-thoracotomy. Intraoperative frozen sections revealed no signs of malignancy. Exact classification of the entity was not possible from the frozen sections. There were no postoperative complications and our patient was discharged six days postoperatively.A chest computed tomography (CT) scan confirmed the expected lesion in the upper left lobe of her lung, with a maximum diameter of 31mm in the axial plane and contrast enhancement at the outer tumor margin (Figure The histopathological findings presented a classic glomangioma containing highly homogenous glomus cells and partially dilated blood vessels, but no signs of cellular atypia Figure . The tumGlomangioma are derived from glomus bodies, and comprise less than 2% of all soft tissue tumors ,4. GlomuThe histopathological characteristics of glomangiomas are very close to those of glomus bodies. Both are composed of epithelioid smooth-muscle cells, which are typically arranged in sheets and nests around the blood vessels. Within the glomus tumor family, different types of tumors have been described according to their morphological presentation. The glomus tumor itself consists predominantly of glomus cells, whereas the glomangioma presents with an extremely high density of vascularity, including dilated blood vessels or cavernous blood spaces. In a histological examination, the differential diagnosis must exclude several different tumors, such as carcinoid tumors, hemangiopericytomas, sclerosing hemangiomas, leiomyomas and paragangliomas. In general, each tumor entity can be definitively identified by their specific immunohistochemical staining pattern. For example, carcinoid tumors are sometimes mistaken for glomus tumors because they have a comparable conventional histological presentation. However, carcinoid, but not glomus, tumors are positive for cytokeratin and neuroendocrine markers such as chromogranin A and synaptophysin. Hemangiopericytomas are positive for vimentin and CD34, but negative for cytokeratin and smooth muscle markers. Paragangliomas are typically composed of round epithelioid cells with small nuclei, and express neuroendocrine markers and S-100 protein.Primary glomangiomas and glomus tumors of the lung are extremely rare - to the best of our knowledge, only 30 cases have been described in the literature ,6. The mThe biological behavior of glomangiomas seems to be benign according to the follow-up data available. So far, follow-up data is accessible for four patients ranging from 9 to 60 months after surgery, without evidence of a relapse ,3. MaligIn our opinion, due to their uncertain biological behavior, resection of these tumors is warranted once they have been diagnosed. Preoperative imaging and biopsy seldom lead to a conclusive diagnosis, because glomangiomas do not present with specific characteristics in CT or magnetic resonance imaging, and utility of FDG-PET is also of limited value. In none of the reported cases was a correct diagnosis possible preoperatively.Whether an anatomical lobe resection with lymphadenectomy or an atypical wedge resection is the adequate method for surgical treatment of glomangioma remains unanswered. In our opinion, the wedge resection seems to be adequate if no malignancy is seen in the frozen sections. Otherwise oncological surgical standards should be applied similar to the treatment of non-small cell lung cancer.Glomangiomas of the lung are rare tumors, and only a few cases have been reported in the literature. Even though glomangiomas are typically benign, invasive subtypes, such as glomangiosarcoma, have been reported. Glomangiomas of the lung are usually diagnosed incidentally and should always be resected as the behavior cannot be predicted by preoperative examinations. While a complete surgical resection of the tumor is mandatory, a pulmonary wedge resection should be adequate. Even though intraoperative frozen sections may help to determine tumor-free surgical resection margins, a definitive diagnosis from frozen sections is often not possible.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.18Fluorodeoxyglucose positron emission tomography.CT: Computed tomography; FDG-PET: The authors declare that they have no competing interests.FG and YKV prepared the manuscript and performed clinical treatment of and surgery on the patient. AQ performed the conventional histological examination and immunohistochemical staining. JRI reviewed the manuscript and study protocol. All authors read and approved the final manuscript.


Lipomas of the gastrointestinal tract are a rare condition. Only 5% are of gastric origin, and this corresponds to 2% to 3% of all benign tumors of the stomach and less than 1% of all gastric neoplasms. It is our purpose to report an unusual presentation of a giant gastric lipoma in an oligosymptomatic patient and highlight the importance of discussing differential diagnosis in this situation. A review of the literature has shown that this is one of the largest gastric lipomas described.3, and the histological pattern demonstrated well-differentiated mature adipose tissue surrounded by a fibrous capsule, confirming the diagnosis of gastric submucosal lipoma.We describe a rare case of a benign gastric tumor with uncommon features in a 63-year-old Caucasian woman. She was admitted with abdominal discomfort, nausea, and upper abdominal fullness after eating. The lesion was suspicious of malignancy because of its dimension and central contrast enhancement on computed tomography. Conventional upper digestive endoscopy revealed a large bulging mass in the gastric posterior wall and three ulcerated areas. In this procedure, a technical limitation due to the location of the mass in the submucosa prevented an adequate biopsy from being obtained. The fragments obtained from the ulcers revealed nothing but necrotic mucosa. Our patient underwent a subtotal gastrectomy and D1 lymphadenectomy with a Roux-en-Y reconstruction. Macroscopic findings revealed a 12 × 8 × 6cm mass with a volume of 576cmGastric lipoma is a rare benign disease that eventually simulates a malignant tumor. Lipomas of the gastrointestinal tract are rare: only 5% are of gastric origin. They account for 2% to 3% of all benign tumors of the stomach and less than 1% of all gastric neoplasms . We descA 63-year-old Caucasian woman complaining of insidious upper abdominal pain (unrelated to food ingestion) and nausea was admitted after an episode of intense acute abdominal pain. She denied having hematemesis or melena and had a history of untreated dyslipidemia. Her dietary habits included acid fruits, soft drinks, beer, chocolate, and high-fat foods. She was a smoker of 10 or more cigarettes per day and had no family history of cancer. She had systemic arterial hypertension that was being treated with 500mg of methyldopa per day. The results of a physical examination were normal except for a palpable and movable upper abdominal mass. The results of all laboratory tests were normal, and our patient did not have anemia. An abdominal ultrasound was promptly performed, and a large echoic mass compatible with an expansive lesion was found in the gastric antrum. Conventional endoscopy detected a large bulging mass in the posterior gastric wall and three ulcerated areas in the gastric mucosa, but none of them showed signs of recent bleeding (Forrest III). Owing to technical limitations, the procedure did not result in an adequate biopsy of the mass in the submucosa. The fragments acquired from the ulcers revealed nothing but necrotic mucosa. Abdominal contrasted computed tomography revealed a well-defined homogeneous oval mass that was located within the posterior gastric wall and that compressed the second portion of the duodenum. The lesion had fat tissue density and a suspicious central contrast enhancement with trabecular architecture but was noninvasive with respect to the peripheral layers , malignancy was suspected. Our patient then underwent an open laparotomy for abdominal exploration. During surgery, unexpected benign aspects of the mass, such as the envelopment by a fibrous capsule and no signs of lymphadenopathy or local metastasis, surprised the team  was measured. Microscopy revealed a histological pattern of well-differentiated noninvasive mature adipose tissue surrounded by a fibrous capsule , the “cushion sign” (the smooth consistency of the lesion may be flattened and restored), and the “naked fat sign” (fat extrusion is detected after biopsy) ,10-13.Biopsy through conventional endoscopy reveals only a typical mucosa and thus is not able to confirm diagnostic suspicions ,13. In tThe use of endoscopic sonography is useful for identifying the tumor’s primary layer, which is best for describing the lesion’s morphology, and reveals possible invasion of the lymph nodes and peripheral layers. Typically, lipomas are visualized as hyperechoic homogeneous lesions that have regular margins and that arise from the third layer -14. The The best noninvasive exam for large gastrointestinal lipomas is abdominal computed tomography, which is sufficient yet not definitive for diagnosis . ImagingIncidental lipomas should not be treated, as there are no reports of malignant degeneration ,12,13. LGastric lipoma is a rare benign disease that eventually simulates a malignant tumor. This case is presented on account of its rarity and the surprisingly few symptoms reported by the patient given the dimensions and tomographic signs of malignancy. Thus, it is best advised to thoroughly discuss the differential diagnosis for a gastric submucosal mass in order to plan for the best treatment.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.FAFN was the chief surgeon and provided the medical documents for the reported case. MCFF provided the histological examination of the gastric tumor, supervised the study, and performed critical revision of the manuscript for important intellectual content. LCNB provided technical support and performed critical revision of the manuscript for important intellectual content. AAN and WCA revised the patient’s medical records and obtained informed consent. GNC and CAB performed acquisition, analysis, and interpretation of data, drafted the manuscript, and translated the final version. MAMR performed acquisition, analysis, and interpretation of data and drafted the manuscript. All authors read and approved the final manuscript.


Chondrosarcomas are the most frequently occurring primary malignant chest wall tumors. Furthermore, the lungs serve as the most frequent sites for metastases. Pulmonary metastases from sarcomas usually appear as round nodules of varying sizes on roentgenograms. Here, we report an unusual clinical and radiographic presentation of pulmonary metastasis from a costal chondrosarcoma. Bilateral pulmonary metastases developed soon after wide surgical resection. Thoracic computed tomography revealed unusual radiological findings: consolidation accompanied with ground-glass opacity. To confirm the metastasis, we recommend a transbronchial biopsy in cases where unusual pulmonary findings are detected. Chondrosarcomas are the second most frequent primary malignant bone tumors, after osteosarcomas ,2. They On roentgenograms, pulmonary metastases usually appear as multiple peripheral, round nodules of varying sizes. Here, we describe an atypical presentation of pulmonary metastasis occurring soon after wide surgical resection of a costal chondrosarcoma. In this case, a thoracic computed tomography (CT) scan showed consolidation, predominantly in both the lower lobes, surrounded by ground-glass opacities and air bronchograms, mimicking serious pneumonia.® . Histological examination revealed a grade II chondrosarcoma with increased cellularity and myxoid stroma  in the right eighth rib. The mass was hard with an unclear border, no mobility, redness, or local heat, but it was tender. An X-ray revealed a mass with coarse calcification located on the right eighth rib, expanding beyond the irregular cortex. Thoracic CT revealed a 70 × 60 × 30 mm low-density mass  along the right eighth rib; it arose at the bone-cartilage border and destroyed these tissues Figure . No pulm.5 cm in 9 cells/L  and 3.7 mg/dL  respectively. Findings of other biochemical and serologic tests were normal. The chest roentgenogram showed air-space consolidation accompanied with an air bronchogram in the right upper and left lower lung fields and C-reactive protein (CRP) level were 8.2 × 10s Figure . Bronchos Figure . The broChondrosarcomas are classified on the basis of their aggressiveness into 3 grades according to their cellular density, degree of anisokaryosis, and nuclear hyperchromatism . The hisThe pulmonary metastasis in this case was atypical in the following ways: (1) The radiological features mimicked those of pneumonia. Thoracic CT revealed pulmonary non-segmental consolidation, predominantly in the peripheral lung field, surrounded by ground-glass opacities. This indicated interstitial pneumonia such as cryptogenic organizing pneumonia. 2) Although the operation involved only the right side, bilateral pulmonary metastases developed after the resection. Time taken for metastasis to develop has been reported to be an average of 20 months [ AlthoughTransbronchial biopsy, endobronchial biopsy, or surgical lung biopsy can be performed to obtain tissue specimens. Surgical lung biopsy includes video-assisted thoracic surgery (VATS) and open lung biopsy. The procedure chosen is based on clinical judgment, which entails weighing the yield versus the risk to the patient. In particular, transbronchial biopsy is usually the procedure of choice for the initial examination due to its high yield and relatively low risk , and theSoon after the curative surgery is performed, to confirm the pulmonary metastasis, we recommend that transbronchial biopsy should be performed in cases where unusual clinical and radiological pulmonary findings are detected.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.ME: assisted in the writing of the manuscript and in the orthopedic workup of the patient; KH: assisted in the drafting of the manuscript and in the orthopedic workup of the patient; TK: assisted in the writing of the manuscript and performed the radiological evaluation; KN: performed the radiological evaluation; YT: performed the pathological evaluation; NN: assisted in the orthopedic workup of the patient; NA: evaluated critically the manuscript and gave final approval for the manuscript to be published. All authors read and approved the final manuscript.The authors declare that they have no competing interests.


Atresia of the colon is among the rare types of all gastrointestinal atresias. Ascending colon is the rarest site of all the colonic atresias. The authors report a case of a 4-day-old male baby who presented with the features of distal intestinal obstruction. At laparotomy type I atresia of the ascending colon, just distal to cecum, was found. Primary ceco-colic anastomosis along with a covering ileostomy was performed. Ileostomy was reversed 3 weeks later.  Atresia of the ascending colon is one of the rarest causes of neonatal intestinal obstruction. Reported incidence of colonic atresia is 1 in 20,000 live births of which ascending colon is the rarest [Due to rarity of the disease large series have not been reported in the literature. Colonic atresia occurs in descending order of frequency at sigmoid, splenic flexure, hepatic flexure and ascending colon respectively .It may be associated with anomalies of other systems. Mortality is usually high when treatment is delayed for more than 72 hours. However prognosis is satisfactory with early diagnosis and proper management . Rarity A 4-day-old term male baby was born through cesarean section, to an otherwise healthy primigravida, at a peripheral private clinic. No prenatal problem was detected on routine antenatal visits.The baby did not pass meconium till 4th day when he developed marked abdominal distension along with other features of intestinal obstruction. At the time of admission to our hospital along with distension mild dehydration was also present. There was no other apparent associated anomaly. Rectal stimulation was inconclusive.Plain x-ray of the abdomen in erect posture showed multiple air fluid levels suggestive of distal small bowel obstruction. A diagnosis of distal small bowel atresia was made. Baby was optimized by fluid and electrolytes replacement. Parenteral antibiotics along with vitamin K were administered.Laparotomy was performed through a right upper transverse incision. Operative finding was type I atresia of the ascending colon just distal to hugely distended cecum . A ceco-colic anastomosis was performed by opening the lumens of dilated cecum and micro ascending colon through a longitudinal incision on their anterior walls. Intervening mucosal septum was excised and the defect closed transversely with covering ileostomy.The recovery was smooth and patient discharged on 7th postoperative day. After 3 weeks a contrast radiograph through ileostomy was performed to confirm the distal patency before ileostomy reversal .Colonic atresia accounts for 1.8-15% of intestinal atresias . AscendiDelayed recognition of symptoms increases the risk of complications like perforation and sepsis. Etiology of this anomaly is still debated. Commonly accepted theory is that of in-utero vascular accidents in the early gestation . ColonicColonic atresia in babies with gastroschisis seems to result from the bowel compression with narrowing of abdominal defect .Association of small bowel atresia and Hirschsprung’s disease is of paramount imporatance . Rectal Uncomplicated right colonic atresia can be treated with primary anastomosis with little morbidity whereas staged reconstruction with proximal diversion is advised in sigmoid and left colonic atresia to avoid the complications of anastomosis .Preservation of ileocecal valve is desired for future growth of the child. Due to hugely dilated cecum and the enormous disparity between the cecum and atretic ascending colon in the reported case primary cecocolic anastomosis with covering ileostomy seems appropriate. However the operative strategy depends on the clinical state of the patient and the safety of the procedure should always be a priority. In the case presented staged procedure was adopted and it resulted in early recovery and discharge of the patient. Stoma care is an issue in these cases especially with ileostomy where effluent is more fluid in nature. To address this issue an early reversal was performed in our patient. It thus appears to be an appropriate approach in a set up where stoma care may be an issue.Source of Support: NilConflict of Interest: None declared


A ten-year-old male standard dachshund was presented with a history of neck pain and progressive gait disturbances. Following a neurological examination and diagnostic imaging, including CT, a neoplastic lesion involving the third and fourth cervical vertebrae was suspected. The lesion included an extradural mass on the right side of the spinal canal causing a local compression of the cervical cord. Surgery, using a modified dorsal laminectomy procedure, was performed in order to decompress the cervical spinal cord. Histopathological examination of the extradural mass indicated that the tumour was a chondroid chordoma. Following discharge, the quality of life for the dog was very good for a sustained period, but clinical signs recurred at 22 months. The dog was euthanased 25 months post-surgery. On post-mortem examination, a regrowth of neoplastic tissue was found to have infiltrated the bone and spinal cord at C3-C4. This is the first report to show that palliative surgery can offer successful long-lasting treatment of chondroid chordoma of the cervical spine in the dog. Chordomas are slow-growing, locally destructive neoplasms arising in the cerebrospinal axis from remnants or derivatives of the notochord. These tumours are uncommon in humans and in animals, and only a few cases have been reported in dogs ,2, cats By clinicopathologic, immunohistochemical and DNA flow cytometric studies, three subtypes of chordomas are recognized in humans: 1) conventional chordoma, 2) chondroid chordoma and 3) chordoma with a malignant spindle cell component -12. DistTo the authors' knowledge, eight cases of chordomas or probable chordomas in dogs have been reported previously, and the locations of the tumours varied. Two were located in the cranial region ,14, two This is the first report describing surgical treatment and follow-up of a chordoma in the cervical spine of a dog.A ten-year-old, male, longhaired standard dachshund of 11 kg, presenting with lethargy and ataxia was referred to the Norwegian School of Veterinary Science for evaluation and treatment. It had a three month history of gradual behavioural change, reluctance to exercise and increasing stumbling on the right thoracic limb. A week before admission, the referring veterinarian also identified neck pain, a slight degree of general muscular atrophy and tetraparesis with an absence of the proprioceptive positioning response in all four limbs. The referring veterinarian then prescribed 10 mg prednisolone orally once daily for seven days.On referral, the dog was bright and alert. Clinical examination revealed a reduced body condition including general muscular atrophy. The claws of the third and fourth digits were worn on all four limbs, especially on the right thoracic limb.The dog was walked on a leash and ataxia and weakness of all four limbs were observed. The dog was able to walk up and down stairs and stumbling on a smooth floor was not observed. Signs of discomfort or spontaneous pain were not registered.versus the left thoracic limb. On flexion of the neck, the dog showed signs of cervical pain.On neurological examination, cranial nerve function was normal except for a bilateral absence of the menace response that was explained by presence of keratitis and cataract in both eyes. The proprioceptive positioning response was absent in all four limbs. The hopping reaction and the tactile placing response were delayed in the right The results of clinical biochemistry and a complete blood count were within the normal reference ranges for the laboratory.The dog was premedicated with an im administration of 0·1 mg/kg acepromazine  and 0·2 mg/kg methadone hydrochloride  and lateral and ventrodorsal radiographs of the cervical and thoracic vertebral column were taken. In the ventrodorsal view, a smoothly marginated expansive bone lesion was seen on the right side of the vertebral arch of C3 Figure . A moder® Lipuro B; Braun), maintained with 1·6 to 2 per cent isoflurane  in oxygen and air delivered by means of a circle patient breathing system. Computed tomography (CT) of the cervical spine was performed with the dog in ventral recumbency. The CT machine was a GE highspeed Fxi single slice helical scanner , the voltage and milliampere-seconds used were 120 kV and 150 mAs respectively and the slice thickness was 3 mm and 1 mm in both soft tissue  and bone  algorithms.General anaesthesia was induced by an iv injection of 4 mg/kg propofol  on the right side of the neck was also observed.CT revealed an extradural heterogenous and amorphous mass to the right of the vertebral canal just above the C3-C4 disc extending from mid C3 to mid C4 Figure  and 3A. Six days after the CT scan, surgery was performed. The dog was premedicated and anaesthesia inducted and maintained with the same procedure as presented in the Diagnostic Imaging section (above). For antimicrobial prophylaxis 40 mg/kg cephalothin  was administered iv. Analgesic therapy included transdermal administration of 4·5 μg/kg/h fentanyl .The dog was placed in ventral recumbency with the head and neck gently flexed in a neutral position. A dorsal midline skin incision extending from the occipital protuberance to the dorsal spinous process of C6 was made. The laminae of vertebrae C2- C5 was exposed through an approach with lateral retraction of epaxial musculature to the level of the articular processes . PeriostAfter surgery, the dog was monitored in the intensive care unit and given an iv injection of 4 mg/kg carprofen . An im administration of 0·2 mg/kg methadone hydrochloride  was repeated q4h in the immediate 12 hour-postoperative period. Anti-inflammatory treatment was continued by giving 2 mg/kg of carprofen po twice daily for two weeks. Two days post-surgery the dog was ambulatory with moderate ataxia and weakness in all four limbs. Spontaneous pain was not registered and urination was normal. The fentanyl patch was removed three days post-surgery.The dog was discharged after six days. At that time, neurological function was similar to that observed preoperatively. Exercise restriction was advised, and the use of harness instead of a collar was recommended.The formalin fixed biopsy measuring 0·5 × 0·5 × 2 cm was decalcified and sectioned for histopathological examination. The morphological features together with immunohistochemical examinations indicated a chondroid chordoma.The owner was questioned by telephone about the dog's condition twelve months after surgery. She reported that the dog was doing well without observable gait disturbances. The claws of the third and fourth digits of the right thoracic limb were normal. However, the owner commented that the dog tended to lie down when patted on his back and she presumed this behaviour was to avoid discomfort.Twenty-five months following surgery, the owner telephoned and reported recurrence of clinical signs with gait disturbances and cervical pain. The dog had become progressively worse over the last three months, and despite treatment with carprofen, the owner felt the dog now was suffering. On readmission to the Norwegian School of Veterinary Science, the dog was depressed and reluctant to walk. Worn claws were seen on both thoracic limbs. When walked on a lead, the dog showed weakness (tetraparesis) and a mild lameness in the right thoracic limb. Hands-on examination of the head and neck revealed signs of pain, including attempts to bite the examiner. A systolic murmur was heard on both sides of the thorax.The dog was sedated and a second radiographic study of the cervical vertebral column was performed. A mixed (destructive and productive) bone lesion was identified at C3 and C4, and included the body and arch of both vertebrae Figure . Areas oCT of the cervical spine was repeated with the dog in ventral recumbency. The slice thickness was 2 mm and 1 mm in both soft tissue and bone algorithms. The studies were performed before and after iv injection of 300 mg I/ml iohexol  at a dose of 1·8 ml/kg bodyweight.The laminectomy of C3 and C4 was visible. The CT examination revealed a large mass to the right of the vertebral canal involving the C3 and C4 vertebral arches and the C4 vertebral body Figure  and 3B. Owing to the size, location and history of the cervical mass, the prognosis was considered to be poor and with the owner's consent the now twelve-year-old dog was euthanased and submitted for post-mortem examination.At post-mortem examination of the vertebral column, gross pathological findings were observed between cervical vertebrae C3 and C4  tissue, the decompression can be classified as palliative. However, following treatment the dog lived for two more years, including 22 months with a very good quality of life. This case shows that surgical treatment of a chondroid chordoma significantly increased the life span of a dog despite incomplete resection of the tumour. This finding indicates that surgical treatment of chondroid chordomas in the dog is warranted.Surgical excision is considered to be the most important mode of treatment for chordomas in humans. Radiation therapy and chemotherapy are used as additional treatment modalities ,23, but versus in 36 patients with conventional chordomas. In dogs, distinct subtypes of chordomas have not been described and, to the authors' knowledge, no reports have included a follow-up after surgical treatment of canine chordomas. Therefore, the prognostic importance of the chondroid component in the chordoma presented herein is unknown.In 55 cases of spheno-occipital chordomas in humans, Heffelfinger and others  found thIn higher vertebrates, the nucleus pulposus is believed to be the only derivative of notochordal tissue . HoweverIn chondrodystrophoid dogs, intervertebral disc degeneration is characterized by an early initiated and very accelerated metamorphosis of the nucleus pulposus from a mucoid to a chondroid type . From thBesides physaliferous cells and the nest formation of chordoid tumour cells, in chordomas and not in cartilaginous tumours, immunohistochemical labelling is an important tool for differentiating these two tumour types and to confirm the diagnosis . As was In dogs, chondroid chordomas may arise in the cervical spine and cause clinical signs from extradural masses that compress the spinal cord and spinal nerves. Both cervical vertebrae and discs may be subjected to neoplastic tissue. Sclerotic and lytic lesions could be identified by diagnostic imaging. Chondroid chordomas localized in the vertebral column may not be possible to remove by complete surgical excision. However, an incomplete resection including decompression of the spinal cord, may subsequently give a dog an excellent quality of life for almost two years.Written informed consent was obtained from the owner 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.ØS carried out the clinical examination, performed the surgery and is the main author of the paper. NO carried out the diagnostic imaging. HG and CPÅ carried out the gross pathological, histopathological and immunohistochemical examinations. All authors read and approved the final manuscript.


Primary small cell carcinoma of the ureter is an extremely rare disease, only several cases have been reported worldwide so far. We report a 70-year-old woman who was examined with intravenous urography and abdominal computed tomography and was diagnosed as small cell carcinoma confirmed by pathology. We describe and discuss the urography and computed tomography findings of this case. Small cell carcinoma (SCC) is usually found in the lungs, but its extrapulmonary counterpart is rarely encountered . AccordiA 70-year-old woman presented with left-sided flank pain without symptoms of bladder irritation and gross hematuria. The pain was accompanied by nausea. The patient had no smoking history. The ultrasonography revealed a 6mm diameter calculus in the distal region of the left ureter with hydronephrosis. Then she was examined using intravenous urography (IVU), abdominopelvic CT, and chest radiography.The IVU showed the amputated end of the left ureter with hydroureterosis  A. CT inThe patient underwent left nephroureterectomy. Macroscopically, a 1.6×1.2×0.5 cm grayish white lesion was identified between the bladder and end 3.5 cm of the ureteral specimen. Histological and immunohistochemical staining examination confirmed PSCC of the ureter . The abdThere are two hypotheses regarding the histopathogenesis of urinary tract SCC. One indicates that it originates from intrinsic neuroendocrine cells within the normal genitourinary tract derived from the neural crest during embryogenesis . WhereasBecause of high mitotic activity , 8-10 anIn this case, the enhancement of the lesion increased equal to 29.5 HU from the corticomedullary phase to the nephrographic phase. It indicates PSCC of the ureter has slight-midrange enhancement. The extent of slight-midrange enhancement at CT reflects the underlying extent of nodular angiogenesis . The attThe boundary between the mass and the ureteral wall could not be discerned. This characteristic may be due to the tumor mass infiltrating the ureteral wall completely, even encasing it leading to an early and extensive metastasis in PSCC. We did not find the metastasis in other organs prior to or during surgical operation. However, 9 months after the surgical procedure we found extensive metastases scattered over the liver and lung and numerous lymphadenopathies around the retroperitoneum and para-aorta, as well as metastatic implantation in the bladder. This PSCC of the ureter in particular impacts the ureterostoma and causes hydroperinephrosis; so metastasis to the psoas major also occurs.It may be difficult to differentiate PSCC of the ureter from transitional cell carcinoma (TCC) that account for 5% to 7% of all urinary tract tumors . But theLimitations of our report include only one case of PSCC of the ureter and no plain and excretory phase of abdominal CT; nevertheless, PSCC of the ureter is such a rare disease that no report has described the IVU and CT appearances of PSCC of the ureter in detail. Diagnosis by cystoscopy may cause trauma of the ureteral wall and increase possibility of metastasis. So describing and discussing the urography and CT features of this PSCC may also aid in the clinical diagnosis and promote timely treatment for affected patients, however nephroureterectomy is the optimal method by which PSCC of the ureter is diagnosed and managed.


A 52 year-old man who had previously undergone a laryngectomy for squamous cell carcinoma, presented with a 1.2 cm nodule in the right upper pulmonary lobe. Three months later a new CT scan found that the nodule had slightly increased in size and also detected two new smaller nodules in the middle lobe. A PET/CT scan showed metabolic hyperactivity of all nodules. Since needle aspiration of the upper one revealed malignant cells, the patient was considered to be suffering from metastatic cancer and started on chemotherapy. At follow-up both CT and PET scans found a significant reduction in volume and activity of the lower nodules but no change in the upper one. At diagnostic thoracoscopy, histology demonstrated that the upper nodule was an adenocarcinoma while the lower ones were inflammatory. An upper lobectomy and systematic nodal dissection were therefore performed. Histology established a diagnosis of upper pulmonary adenocarcinoma and sarcoidosis. Our report suggests that in complicated oncologic cases in which non-invasive diagnostic tools yield incongruous results surgery should be considered without delay.The aim of this paper is to focus attention on complex cases of lung disease that may benefit from being managed outside formal guidelines.  We report the case of a patient with three right pulmonary nodules and a previous advanced metastatic carcinoma of the pharynx. Follow-up and treatment were managed by a team of surgeons and oncologists according to the American College of Chest Physicians (ACCP) evidence-based clinical practice guidelines . The comA 52 year-old male who was a heavy smoker presented with a single right upper lobe pulmonary nodule 1.2 cm in diameter. In May 2007 he had developed a tongue carcinoma which had been treated by a supraglottic laryngectomy extended to the tongue base, temporary tracheotomy and elective neck nodal dissection. Histology was pharyngeal squamous cell carcinoma, pT2N2M0G2 with minimal invasion of the deep tongue surgical edge. The advanced tumor stage had required adjuvant radiotherapy (65 Gy) and chemotherapy (two cycles of cisplatin). In September 2008 a CT scan revealed the 1.2 cm irregular nodule in the upper pulmonary lobe. The radiologic report stated it was suggestive of scarring tissue. Bronchoscopy and bronchoalveolar lavage cytology were negative. The fully informed patient chose a non-aggressive management approach. Therefore, according to the ACCP evidence-based clinical practice guidelines, a serial CT scan observation was adopted  and it rThe hypothesis of multiple lung metastases of pharyngeal carcinoma seemed the most likely and the patient underwent chemotherapy with cisplatin and vinorelbine. After three chemotherapy cycles a CT scan was repeated. It showed that the right upper lobe nodule was unchanged and that the two middle lobe nodules were significantly reduced. A PET/CT scan confirmed this pattern of evolution, showing that the activity of the upper lobe nodule was stable (SUV = 9 g/ml versus 10.6 g/ml) whereas the activity of the middle lobe nodules was in one case significantly reduced (SUV = 2.6 g/ml versus 5.1 g/ml) and in the other almost completely absent (Figure Over the last few years a number of international scientific societies like the ACCP have developed evidence-based guidelines for the diagnosis and management of lung cancer. They certainly have a great value in assisting physicians throughout the decision-making process and as such are being increasingly used in clinical practice. Abidance to the guidelines means better outcomes for patients, a standardised approach to a disease of worldwide importance and also legal shielding in the case of alleged malpractice. However, the case we report shows there are unusual and complex situations in which the physician's clinical judgment still plays a key role and that must be addressed in a more flexible manner, even by adopting less conventional approaches.In our patient the clinical and instrumental data - previous history of neoplasia, metabolic hyperactivity of the three pulmonary nodules and positive cytology of the upper nodule - strongly pointed to metastatic pharyngeal carcinoma but intra-operative diagnostics painted a different picture altogether. In fact only one nodule was truly neoplastic and indeed it was not a metastasis but an early-stage primary lung cancer. This information changed the prognosis and the therapeutic strategy. Thus, correct diagnosis and management were possible only thanks to a surgical approach that is commonly inappropriate in such context but was justified by unexpected findings on post-chemotherapy CT and PET imaging.In the current era of thoracoscopy, when non-invasive diagnostic tools yield incongruous results surgery can give an important contribution to the diagnosis of lung diseases. The invasiveness of endoscopic surgery is minimal and risks are more than outweighed by benefits, namely saving time and avoiding excessive instrumental exams, misdiagnoses and inappropriate treatments.Written informed consent was obtained from the patient for publication of this case report and accompanying images.The authors declare that they have no competing interests.UC participated in the design of the study and drafted the manuscript, FR participated to the surgical operation and the patient's management, MDS participated in the design of the study, VV participated to the patient's management, MMC participated in the design of the study, AB performed the surgical operation, participated to the patient's management, participated in the design of the study and drafted the manuscript. All the Authors read and approved the final manuscript.


Background. Small cell carcinoma (SCC) of the gallbladder is a rare entity and is often seen in elderly women. SCC of gallbladder is typically a nonsecretory carcinoid tumor without overt clinical symptoms and is often discovered at advanced stages. SCC of gallbladder carries a dismal prognosis as compared to SCC of lung and adenocarcinoma of gallbladder. To date, only 73 case reports have been published in the world literature. Case Presentation. Herein, we report a case of a 73-year-old Saudi woman who presented with one week history of right upper quadrant abdominal pain and obstructive jaundice and was found to be a case of locally advanced, metastatic SCC of gallbladder cT4N1M1 . The patient was treated with neoadjuvant etoposide and cisplatin (EP) chemotherapy three cycles after biliary stenting followed by radical cholecystectomy, lymphadenectomy, and adjuvant EP chemotherapy and then one year later developed distal humerus osseous metastasis. Conclusion. SCC of the gallbladder is very rare entity and is often seen at advanced stages. Osseous metastases of peripheral skeleton from SCC gallbladder are rarely reported. Surgery is curative option but only for early stage tumors. Incorporation of chemotherapy along with radical resection increases the survival. Gallbladder carcinomas are the fifth most common gastrointestinal malignancies . Most coHerein, we report a case of a 73-year-old Saudi woman with locally advanced, metastatic SCC of gladder cT4N1 M1  who was treated with multimodality regimen (neoadjuvant and adjuvant chemotherapy followed by surgery).μmol/L), alkaline phosphatase 197 U/L ↑, and ALT 91 ↑); however, hematology and electrolytes were within normal limits. Ultrasonography revealed a gallbladder mass of size 1.5 cm in diameter along with dilatation of the intrahepatic biliary ducts with two hepatic masses of size 4.20 × 3.6 cm located in segment I of the liver and another hepatic mass measuring about 6.5 × 5 cm in segment IV of the liver with two regional enlarged lymph nodes. Endoscopic retrograde cholangiopancreaticography (ERCP) guided biopsy and stenting were performed. Biopsy came out as high grade small cell carcinoma. Abdomenal computed tomography (CT) showed a large 7.9 × 5.1 cm heterogeneously enhancing mass involving the gallbladder and the surrounding liver segments, namely, segments VI, V, and IVB with well-defined irregular outlines. There were associated enlarged lymph nodes: the largest one is seen at the portocaval area measuring about 4.2 × 4.7 × 7.2 cm in AP and transverse and craniocaudal diameters, respectively. A left paraaortic lymph node (PALN) was seen of size 2.9 × 4.7 × 4.7 cm. Another enlarged lymph node is seen at the aortocaval group measuring 1.8 × 1.7 cm (2 D1 and etoposide 100 mg/m2 (EP) three cycles. Postchemotherapy CT imaging showed significant response (35–45% regression)  but was not relieved. On physical examination, she was found to have good performance scale (ECOG-0) and severe icteric without any pallor or palpable lymphadenopathy. On abdominal examination, there was deep tenderness in right hypochondrium. The rest of systemic examination was found unremarkable. Baseline liver function tests were markedly deranged  , and patression) . Patientression) . CT-posiression) . Biopsy Small cell carcinoma (SCC) of gallbladder is a rare disease, with an incidence of approximately 0.5–3.5% of all gallbladder malignancies . Median Due to the scarcity and advanced stage at time of diagnosis, there are no consensus guidelines available for SCC gallbladder management. However, surgery is the best curative option for early stage tumors. For locally advanced unresectable SCC gallbladder, multi-modality approach by neoadjuvant or adjuvant chemoradiation is alternate option with better median survival rates ranging from 3.5–13 months as our patient was treated and she was alive at 15 months after initial diagnosis .In conclusion, SCC of the gallbladder is very rare entity and is often seen at advanced stages. Osseous metastases of peripheral skeleton from SCC gallbladder are rarely reported. Surgery is curative option but only for early stage tumors. Incorporation of chemotherapy along with radical resection increases.


Gastrointestinal duplication is a rare congenital disease which affected more commonly the ileum, while the stomach is rarely involved. Generally diagnosed in paediatric or young age, it could be difficult to suspect a gastrointestinal duplication in adults. Herein, we report a 55-year-old male with a gastric duplication cyst found on routinely checkup for chronic hepatitis and first misdiagnosed as a gastrointestinal stromal tumor (GIST); we also discuss its embryology. Gastrointestinal duplication is a rare congenital disease defined as a spherical hallow structure with a smooth muscle coat, lined by a mucus membrane and attached to any part of the gastrointestinal tract from the base of the tongue to the anus; because these malformations are formed before differentiation of the lining epithelium, they are named for the organs with which they are associated . Most coHerein, we report a gastric duplication cyst in a 55-year-old man first misdiagnosed as a gastrointestinal stromal tumor (GIST) and discuss its embryological origin.A 55-year-old man suffering from B type chronic hepatitis treated with antiretroviral drugs was found to have a hypoechoic round-shaped mass sized 4.7 cm, with regular margins, during an abdominal ultrasonography performed for scheduled checkup. The mass was located between the left liver lobe and the anterior surface of the pancreatic body.The patient did not complain of any symptoms about gastrointestinal tract.To better evaluate the mass, patient underwent MR imaging that confirmed the presence of a cystic mass with complex content located forward to the gastroesophageal junction. A subsequently endoscopic ultrasonography showed a hypoechoic mass with a slightly heterogeneous internal echo and regular margins located just below the gastroesophageal junction; the lesion measured about 4.5 cm and seemed to be contiguous to the fourth wall layer (muscularis propria) .On the basis of a diagnosis suspicious for GIST involving the upper part of the gastric wall, a diagnostic confirmation through an EUS-guided fine needle aspiration with a 22 G needle was taken; it was aspirated mucoid material. Cytological smears showed, in a mucoid background, scattered histiocytes, gastric and oesophageal mucosal cells, and few groups of ciliated columnar cells .However, a surgical excision was performed.Resected specimen measured about 5 × 3 × 3 cm and was sutured on one site with mechanical clips; on cut-section, it was cystic with smooth wall and filled with mucus-like fluid.Histologically, the wall consisted of mucosa, subepithelial connective tissue, a layer of smooth muscle, and an outer fibrous capsule. Focally, the mucosa was lined by gastric foveolar epithelium with cardial glands . Most ofA diagnosis of gastric duplication cyst lined by pseudostratified columnar ciliated epithelium was made.Intestinal duplications or enterogenous cysts are spherical or tubular structures possessing the basic pattern of the enteric wall; they can be located in any part of the alimentary tract even if usually duplications are intraabdominal with the small intestine being the most commonly involved, particularly the distal ileum.About foregut duplications, those associated with oesophagus are more common, while gastric duplications are rare and usually located on the greater curvature being twice more common in female. A variety of mechanisms have been invoked about their origin like failure of recanalization of the bowel lumen following the so-called solid-epithelial phase of the intestinal development, persistence of epithelial outpunching described in embryonic intestine, intestinal ischemia in early intrauterine life, impaired separation of the notochord from intestinal endoderm, and the formation of neurenteric bands with embryonic growth, producing traction diverticula .Gastric duplication cysts are usually diagnosed in a younger age due to their position and mass effect; in adults, diagnosis may be difficult because of the wide range of symptoms and signs reported . Moreover, radiological findings can overlap with some other pathologies like in our case in which it was misdiagnosed, on MR and endoscopic ultrasonography, with a cystic GIST. However, when it can be performed, cytology could aid in the differential diagnosis; in this case, it did not show malignant cells but a mucoid background containing normal cells.More commonly, gastrointestinal duplication cysts are lined by typical gastric mucosa even if in some cases it can be a ciliated pseudostratified epithelium like in our case. The presence of respiratory epithelium could suggest an explanation to its origin because some authors have demonstrated expression of TTF-1 and surfactant .Early in the 3rd week, the respiratory diverticulum appears along the ventral wall of the pharyngeal gut. In this phase, the dorsal portion, which becomes the oesophagus, and the ventral portion, which becomes the trachea, are both lined by a pseudostratified columnar ciliated epithelium. Subsequently, the oesophageal mucosa undergoes squamous metaplasia . Khoury and Rivera describeThe expression of MUC1 is explained since MUC1 is normally found on surface respiratory cells .In our case two hypothesis are available about duplication cyst's origin: formation before 11 weeks' gestation or from the most dorsal portion of foregut, so further studies needed to better understanding the embryogenesis of intestinal duplication cysts. However, whatever could be the origin, it is important for the practitioner physician to know that this unusual embryological malformation can also occur in adult people, and it must be considered in the differential diagnosis of thoracoabdominal masses.


We herein report a rare case of giant intramuscular lipoma of the tongue. A 75-year-old Italian male presented at our department with a large tumor at the tip of the tongue that had been present for over 30 years. Clinical examination revealed a yellowish lesion, measuring 10 cm in maximum diameter, protruding from lingual surface. Histological examination showed an unencapsulated lipomatous tumor composed of mature adipocytes, uniform in size and shape, diffusely infiltrating striated muscle fibers of the tongue. The patient is well with no local recurrence after a 15-month follow-up period. Histoloe fibers -6]. We . We infith postoperative day. The specimens were fixed in 10% buffered formalin, and embedded in paraffin for routine histological examination.A 75-year-old Italian male presented at our department with a large tumor at the tip of the tongue that had been present for over 30 years. The patient denied neurosensory disturbance, including dysgeusia. His speech was not very clear due to the bulkiness of the mass and he had difficulties for swallowing. Clinical examination revealed a yellowish lesion, measuring 10 cm in maximum diameter, protruding from lingual surface and covered by mucosa rich in vessels  showed an unencapsulated lipomatous tumor composed of mature adipocytes, uniform in size and shape, diffusely infiltrating striated muscle fibers of the tongue Figure . LipoblaWorld Health organization (WHO) classification of benign lipomatous tumors recognizes conventional lipoma, fibrolipoma, angiolipoma, spindle cell/pleomorphic lipoma, myxolipoma, chondroid lipoma, osteolipoma, myolipoma, lipomatosis, lipomatosis of nerve, lipoblastoma/lipoblastomatosis, and hibernoma . LipomasIntramuscular (infiltrating) lipoma is a rare variant of lipoma first defined by Regan and his colleagues in 1946 -12]. We. We12]. CT: Computer tomography; WHO: World Health organization.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.GC and PB were major contributors in surgical excision of the lesion. GM and RC were major contributors in the histological diagnosis. GMV and PA were major contributors in writing the manuscript. All authors read and approved the final manuscript.


Objective. To present a case of otic Langerhans' cell histiocytosis in an adult. Also included the diagnosis and management of the condition and a review of the relevant literature. Case Report. We report a case of a 41-year-old man with a history of persistent unilateral ear discharge associated with an aural polyp. Radiological imaging showed bony lesions of the skull and a soft-tissue mass within the middle ear. Histological analysis of the polyp demonstrated Langerhans' cell histiocytosis. His otological symptoms were completely resolved with the systemic therapy. Conclusions. Otic Langerhans' cell histiocytosis can present in adults. Persistent ear symptoms along with evidence of soft-tissue masses within the ear and bony lesions of the skull or elsewhere should prompt the otolaryngologists to include Langerhans' cell histiocytosis in their differential diagnosis. Management should be with systemic therapy rather than local surgical treatment. Langerhans' cell histiocytosis (LCH) is a rare disease that is characterised by the idiopathic proliferation of abnormal histiocytes, either in one organ or as part of a systemic disease –3. LCH iA 41-year-old self-employed brewery engineer was referred to the ENT department with a four-week history of persistent left-sided mucopurulent ear discharge, despite topical antibiotic therapy including gentamicin. He did not have any other otological symptoms, nor any rhinological or upper aerodigestive tract symptoms. His hearing was within normal limits. He was a smoker of 30 cigarettes per day, had a past medical history of hypertension, and had been recently diagnosed with Langerhans' cell histiocytosis involving the skull, for which he was under the care of Haematology. His medication list included azathioprine for LCH, antihypertensives, and a statin.On examination, his left external auditory canal was very oedematous and was filled with an inflammatory polyp. Cranial nerve examination was unremarkable.He had a PET-CT as part of his LCH workup, which showed increased fludeoxyglucose (FDG) uptake in the frontal bone, left parietal bone, right occipital bone, and right sphenoid wing, as well as a 1.7 cm mass within the left middle ear. He also subsequently had a CT of his petrous temporal bones, as represented in The patient underwent an examination under anaesthesia of his left ear which demonstrated a polyp arising from the bulging posterior extent of the external auditory canal. The polyp was excised and sent for histological analysis. The stroma contained granulation tissue and a dense mixed inflammatory cell infiltrate which included abundant eosinophils and numerous Langerhans' cells. Immunohistochemically, CD68 highlighted numerous macrophages and Langerhans' cells. Langerhans' cells are also labelled with S100 and CD1a . These aAlthough he was asymptomatic with regards to his skull lesions, the persistence of otorrhoea from the affected ear indicated continued LCH activity. The dose of azathioprine was therefore doubled from 50 mg to 100 mg, following which his otorrhoea resolved. After his ear symptoms remained under control for more than six months, he was discharged from ENT followup and remained under the care of Haematology for regular monitoring of the LCH. He has not required any further ontological input.LCH is predominantly a disease of childhood and has been well documented in the paediatric population, with a mean age at presentation of three years , 5, 6. MEar and temporal bone involvement occurs in 14 to 61% of children with LCH , but onlThis case demonstrates that persistent ear discharge in adults can be due to LCH. In such a patient presenting to the otolaryngologist, with radiological evidence of a soft-tissue mass within the middle ear and external ear canal with or without bony erosion and other skull lesions, the diagnosis of LCH should be considered. CT is useful in delineating bony involvement and shows bony destruction of petrous apex, and MRI gives information about the extent of any soft-tissue involvement . Bone scTreatment of LCH depends on the pattern of the disease. Localised disease may be treated with surgical excision and intralesional steroids , 2, 5, 6in toto with the concomitant risks of serious complications such as conductive and sensorineural hearing loss, facial nerve paralysis, postoperative fistula, and persistent activity of the disease [The majority of patients presenting with ear involvement, as discussed above, suffer from systemic LCH. It is our recommendation that otic LCH should not be managed surgically. This is due to the extreme difficulty in resecting diseased tissue  disease , 9. Although Langerhans' cell histiocytosis is a disease known to predominantly affect children, adults can also be affected, including within the ear and temporal bone. Nonresolving ear symptoms along with evidence of soft-tissue masses within the ear and bony lesions of the skull or elsewhere should prompt the otolaryngologists to include LCH in their differential diagnosis. Once tissue diagnosis has been confirmed, treatment should be systemic rather than surgical.