Nasopharyngeal cancer commonly manifests with cervical lymphadenopathy, recurrent epistaxis and progressive nasal obstruction. Neuro-ophthalmic and otologic manifestations can also occur. Isolated otologic presentations of nasopharyngeal cancer are rare and the diagnosis of nasopharyngeal cancer may not be foremost in the list of differentials.We present the case of a 29-year-old Nigerian woman with bilateral conductive hearing loss and tinnitus after air travel. There were no other symptoms. The persistence of the symptoms after adequate treatment for otitic barotrauma necessitated re-evaluation, which led to a diagnosis of nasopharyngeal cancer.Isolated otologic manifestations of nasopharyngeal cancer are rare in regions with low incidence of the disease. There is a need for it to be considered as a possible differential in patients presenting with bilateral serous otitis media. The clinical presentations of nasopharyngeal cancer may sometimes be insidious and nonspecific. They are usually related to the local, regional and distant spread or metastasis of the lesion. They may include cervical lymphadenopathy, nasal blockage, epistaxis, hyponasal speech and otologic and neuro-ophthalmic manifestations . The cliThe otological manifestations of this disease entity are commonly unilateral Eustachian tube dysfunction, fluid accumulation within the middle ear, conductive hearing loss, otalgia and tinnitus . HoweverA 29-year-old Nigerian woman, who frequently travels by air, presented with a six-month history of persistent bilateral hearing impairment following a flight. She erstwhile had experienced repeated episodes of this symptom, which occurred each time she flew, but there was always complete resolution after a few days following treatment from an outside health facility. There was associated tinnitus but no otalgia, no ear discharge and no sensation of disequilibrium or vertiginous spells. She did not have any nasal blockage, nasal discharge, epistaxis or postnasal drip. There were no throat or neuro-ophthalmic symptoms. She did not complain of neck swelling. There was no history suggestive of exposure to carcinogens.She had received treatment at peripheral hospitals for barotrauma before presenting to our hospital due to persistence of the symptoms.Examination revealed a young woman with a nevus on the lobule of her right pinna. Both tympanic membranes were dull with a loss of light reflex. The tuning fork test showed evidence of bilateral conductive hearing loss. No evidence of spontaneous nystagmus was noted. A nasal and oropharyngeal examination revealed essentially normal findings. Indirect laryngoscopy findings appeared normal. Her cranial nerves and both eyes were grossly normal. Examination of her other systems did not reveal any abnormalities.A pure tone audiogram confirmed the bilateral conductive hearing loss Figure . ImpedanA diagnosis of bilateral otitic barotrauma was made. She was treated with nasal decongestants, prophylactic antibiotics and asked to perform the Vasalva maneuver frequently. However, her symptoms still persisted after two weeks. This necessitated a re-evaluation; during examination her tympanic membranes were now hyperemic and bulging. A computerized tomographic (CT) scan of her paranasal sinuses was done, which revealed isodense lesions in both fossae of Rosenmüller with complete occlusion of the openings of the Eustachian tubes bilaterally Figure . NasophaThis present study clearly demonstrates a case of bilateral serous otitis media which was the only clinical finding in a patient who was initially thought to have otitic barotrauma. Thorough evaluation after the failure of initial treatment led to a diagnosis of nasopharyngeal cancer. The otologic manifestations of nasopharyngeal cancer are usually unilateral. Bilateral presentation is quite uncommon . BilaterThe otologic manifestations of nasopharyngeal cancer occur as a result of the sheer tumor bulk within the nasopharynx and paranasopharyngeal space extension ,5. Theseet al. of 40 patients with nasopharyngeal carcinoma in north-eastern Nigeria, otologic symptoms were not noted. The clinical profile reported in these patients included cervical lymphadenopathy (72.5%), rhinorrhea (55%) and epistaxis (45%) [et al. of 30 patients in north-western Nigeria reported clinical presentations of deafness and otalgia in 36.3% and 30% of patients, respectively. Other clinical presentations included cervical lymphadenopathy (93.3%), epistaxis (83.3%), nasal obstruction (66.7%), palatal swelling (26.7%), cranial nerve involvement (23.3%) and visual impairment (20%) [et al. of 237 Chinese patients with nasopharyngeal cancer showed that 41% of them had unilateral serous otitis media [et al. reported a steady increase in the disease occurrence over the last two decades in Nigeria [The hidden nature of the nasopharyngeal space poses diagnostic and therapeutic challenges, thus allowing significant spread of the disease before diagnosis . The incis (45%) . Howeverpistaxis 3.3%, nas Nigeria . Inner e Nigeria . In our Otitic barotrauma (barotitis) is a traumatic inflammation of the middle ear occurring as a result of pressure difference between the air in the middle ear and the external atmosphere, developing after ascent or, more usually, descent during air travel. It occurs because of the failure of the Eustachian tube to equilibrate middle ear and atmospheric pressure. It is quite common and presents with ear fullness, otalgia and deafness . Severe In this case report, it is suggested that isolated bilateral otologic symptoms can be the only or initial manifestation of nasopharyngeal cancer even in regions of low disease incidence. It is therefore recommended that, in cases of bilateral serous otitis media or Eustachian tube dysfunction in an adult, nasopharyngeal cancer should be considered.Written informed consent was obtained from the patient for the publication of this case report and any accompanying images. A copy of this consent is available for review by the Editor- in-Chief of this journal.The authors declare that they have no competing interests.DA was the principal investigator, performed the literature search and wrote the manuscript. FAJ assisted in preparing and proofreading the manuscript for intellectual content and gave final approval for the publication. DA and FAJ read and approved the final manuscript and take responsibility for its publication.


Synchronous primary gynecological cancers have been reported to be seen rarely in the literature. In this report, we aimed to describe a 51-year-old patient with the coexistence of villoglandular papillary adenocarcinoma of the cervix uteri and Brenner tumor in the right ovary. She successfully underwent radical hysterectomy, bilateral salphing-oopherectomy and pelvic and para-aortic lymphadenectomy. The coexistence of carcinomas of the cervix and ovary has been reported to be a rare situation in gynecological practice. It is often unclear whether this represents synchronous primary tumors or metastasis that consequently alters staging, management, and expected outcome of these patients. In a study by Kaminski and Norris , the incA 51-year-old female  was admitted to the medical center after experiencing vaginal discharge and spotting for about 2 months. Approximately, 2 years prior to being admitted she had visited a different medical center and a pap smear test had been performed and exfoliated cells resulted in the diagnosis of atypical glandular cells of undetermined significance (AGUS). Following this diagnosis, it was recommended to the patient to undergo colposcopy and biopsy. But the patient failed to return. Her medical history was otherwise unremarkable. Her gynecological examination revealed an approximately 3 × 2 cm vegetative polypoid lesion originating from the endocervical canal and protruding into the vagina. A transvaginal ultrasound revealed an endometrial thickness of 4 mm and a right ovarian solid mass measuring 25 × 21 mm. Laboratory study revealed that cancer antigen (CA) 125 was 9.46 U/mL, CA15.3 was 8.6 U/mL, CA19.9 was 4.75 U/mL, and carcinoembryonic antigen (CEA) was 1.4 U/mL. A cervical punch biopsy was performed and “villoglandular papillary cervical adenocarcinoma” was documented. Following the biopsy procedure, a radical abdominal hysterectomy, a bilateral salpingo-oophorectomy, and a pelvic and para-aortic lymphadenectomy were performed. The hysterectomy specimen revealed an exophytic, polypoid mass with a diameter of 2.5 × 2 × 2 cm occupying the posterior of the cervix and protruding into the vagina. Histologically, the cervical tumor was composed of large cystic glandular and papillary structures surrounded by stroma resembling those of the normal cervix . Mild toSynchronous tumors are defined as two or more tumors occurring in a patient simultaneously. The occurrence rate of synchronous gynecologic malignancies varies between 0.7 and 1.8% in patients with gynecologic tumors. The common occurrence of synchronous ovarian and endometrial adenocarcinoma is especially well known, due to the hormonally active nature of the tumors of the ovary .Villoglandular papillary adenocarcinoma (VPA) is a very rare subtype of adenocarcinoma of the uterine cervix, but a well recognized variant of cervical adenocarcinoma with a favorable prognosis and generally occurring in women of child bearing age . Many ofThe collected data in the present case support that multiple neoplasms with different embryological and histological origin might occur in female genital tract simultaneously. It is important to distinguish multiple primary neoplasms from metastatic disease because of the fact that overall survival as well as treatment would vary considerably. Synchronous primary genital tumors generally have better prognoses than tumors of single metastatic lesions as they tend to be diagnosed at early stages and at low grades . It shouIn our patient, a radical abdominal hysterectomy, a bilateral salpingo-oophorectomy, and a pelvic and para-aortic lymphadenectomy were performed. If Brenner tumors were diagnosed to be malignant tumors, an omentectomy would be added during surgery and chemotherapy and radiation therapy would also be necessary postoperatively.In the literature, there is still a debate about whether concomitant tumors are independent primary tumors or micrometastasis. In a study by Elishaev et al., it was clearly reported that endocervical adenocarcinomas, including some qualifying as microinvasive, can metastasize to the ovaries and simulate primary ovarian surface epithelial neoplasms . TherefoIn our patient, immunoperoxidase staining for HPV types 16 and 18 in the cervical epithelial cells was found negative. Because of the benign nature of the Brenner tumour, we did not perform either immunohistochemistry staining for HPV or PCR in the ovarian tissue.In conclusion, concurrent occurrence of cervical villoglandular adenocarcinoma and Brenner tumor of the ovary is an exceptionally rare situation. To differentiate the nature of the concurrent ovarian tumour, either primary or micrometastasis, from cervical adenocarcinoma, HPV immunohistochemistry or DNA sampling should be performed in a routine manner especially for malignant concurrent ovarian tumours in addition to the pathological examination.


Adrenocorticotropic hormone-producing extraadrenal paragangliomas are extremely rare. We present a case of severe hypercortisolemia due to ectopic adrenocorticotropic hormone secretion by a nasal paraganglioma.68Ga-DOTA]-TATE-Positron-Emission-Tomography was performed, showing somatostatin-receptor expression of the paranasal lesion but not of the adrenal lesion or the hypophysis. The paranasal tumor was resected and found to be an adrenocorticotropic hormone-producing paraganglioma of low-proliferative rate. Postoperatively the patient became normokaliaemic, normoglycemic and normotensive without further need for medication. Genetic testing showed no mutation of the succinatdehydrogenase subunit B- and D genes, thus excluding hereditary paragangliosis.A 70-year-old Caucasian woman was emergently admitted to our department with supraventricular tachycardia, oedema of face and extremities and hypertensive crisis. Initial laboratory evaluation revealed severe hypokalemia and hyperglycemia without ketoacidosis, although no diabetes mellitus was previously known. Computed tomography revealed a large tumor obliterating the left paranasal sinus and a left-sided adrenal mass. After cardiovascular stabilisation, a thorough hormonal assessment was performed revealing marked adrenocorticotropic hormone-dependent hypercortisolism. Due to the presence of a cardiac pacemaker magnetic resonance imaging of the hypophysis was not possible. [Detection of the adrenocorticotropic hormone source in Cushing’s syndrome can prove extremely challenging, especially when commonly used imaging modalities are unavailable or inconclusive. The present case was further complicated by the simultaneous detection of two tumorous lesions of initially unclear biochemical behaviour. In such cases, novel diagnostic tools - such as somatostatin-receptor imaging - can prove useful in localising hormonally active neuroendocrine tissue. The clinical aspects of the case are discussed and relevant literature is reviewed. Paragangliomas are extra-adrenal neuroendocrine tumors that arise from chromaffin cells in the sympathetic  or parasympathetic  neural paraganglia . Most paA 70-year-old Caucasian woman presented to the emergency department of our hospital with palpitations, facial oedema and hypertensive crisis. The patient reported fatigue, shortness of breath, coarseness of nose and throat and progressive oedema of the face over the past year. She declined weight loss, fever, chills, diarrhoea or further gastrointestinal complaints. Her family doctor had initially suspected an allergic reaction and had treated her with a course of prednisolone (40 mg/d) over two weeks with no improvement of symptoms. Three years ago she had been diagnosed with sick sinus syndrome and had received a cardiac pacemaker. Other than arterial hypertension, treated by ramipril, no further comorbidities were reported. She did not smoke, drink alcohol or have any known allergies. Family history was unremarkable.Clinical examination revealed pitting oedema and redness of face, tachycardia and hypertension. Chest auscultation war normal, her abdomen was tender without signs of distention or peritonism. The neurological examination was unremarkable.+: 1,7 mmol/L), hyperglycemia without ketoacidosis and mild leucocytosis. An electrocardiogram showed supraventricular tachycardia. After haemodynamic stabilisation, a thorough hormonal assessment was performed, which revealed markedly increased serum and urinary cortisole and increased plasma ACTH. Plasma and urine katecholamines and metanephrines as well as the aldosterone/renin ratio were within normal range , computed tomography of the chest was performed, which did not show pulmonary infiltrations. However a left adrenal mass of 1 cm diameter was detected in the lower computed-tomography (CT)-slices. Since no magnetic resonance imaging (MRI) was possible due to the presence of a cardiac pacemaker, pituitary imaging was performed by computed tomography. Brain-CT revealed no pathology of the sella region, however a large tumor obliterating the left paranasal sinus was detected   and D (SDHD) genes thus excluding hereditary paragangliosis. Since no other features of multiple endocrine neoplasia (MEN) syndrome were apparent, we characterised the tumor as sporadic and decided against testing for mutations of the RET-protooncogene.About 75% of paragangliomas are sporadic; the remaining 25% are hereditary and have an increased likelihood of being multiple and of developing at an earlier age. After obtaining written informed consent, we performed genetic testing which revealed no mutation of the succinate dehydrogenase subunit B (68Ga-DOTA]-TATE-PET and was surgically removed, which led to complete remission of the hypercortisolaemic syndrome.Detection of the ACTH source in Cushing’s syndrome can prove extremely challenging, especially when commonly used imaging modalities are unavailable or inconclusive. In such settings, novel diagnostic modalities, such as somatostatin-receptor imaging, can prove useful in localising hormonally active neuroendocrine tissue. In the present case we were confronted by a very rare ACTH-producing nasal paraganglioma. The diagnostic procedure was complicated by the simultaneous discovery of two tumorous lesions and the unavailability of MRI due to the presence of a cardiac pacemaker. The ectopic ACTH-source was eventually localised by [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.ACTH: Adrenocotricotropic hormone; CT: Computed tomography; MRI: Magnetic resonance imaging; PET: Positron emission tomography; SDHB: Succinate dehydrogenase subunit B; SDHD: Succinate dehydrogenase subunit D; MEN: Multiple endocrine neoplasia.The authors declared that they have no competing interests.TT drafted the manuscript and was involved in patient management. SZ aided in drafting the manuscript and was involved in patient management. CT, EJ and MPM supervised patient management and critically read and revised the manuscript. All authors read and approved the final manuscript.TT and SZ are junior physicians training in endocrinology. CT and EJ are senior physicians specialising in endocrinology and gastroenterology. MPM is the chief of the Department of Gastroenterology, Hepatology and Endocrinology of the Hannover Medical School.


Background. Fibrothecomas are benign sex cord-stromal tumors which rarely originate outside of the ovary. To date, two such cases have been reported in the literature. We report the third case of an extraovarian fibrothecoma and the first presenting similarly to a metastatic epithelial ovarian cancer. Clinical History. We describe a 62-year-old woman with history, physical examination, and imaging suggestive of metastatic ovarian cancer. CA-125 was elevated at 1291 U/mL. Paracenteses were negative for malignant cells and core biopsy showed spindle cell proliferation. A primary debulking surgery for a presumed ovarian cancer was planned. Method and Results. At surgery, 6 liters of ascites were drained. The uterus, ovaries, peritoneum, and omentum were normal. An 18 × 11 × 7 cm retroperitoneal mass was found between the left ureter and the sigmoid mesocolon, wrapped with sigmoid colon. Fallopian tubes and ovaries were normal. The mass was resected en bloc with the sigmoid colon, uterus, ovaries, and omentum. Microscopically, there was spindle cell proliferation typical of fibrothecoma. No ovarian tissue was identified in association with the tumor. Conclusion. This third case of extraovarian fibrothecoma highlights the importance of obtaining histologic evidence of malignancy prior to initiating neoadjuvant chemotherapy for a presumed ovarian cancer. Ovarian ectopia and neoplasms arising in them are extremely rare. To date, only two cases of extraovarian fibrothecoma have been reported. We report a third case of extraovarian fibrothecoma found in a patient with a presumed diagnosis of ovarian malignancy. A 62-year-old woman presented with a five-month history of increasing abdominal distention, weight loss, and an abdominal mass. There were no associated gastrointestinal symptoms or vaginal bleeding. On examination, she had gross abdominal distention and shifting dullness, consistent with ascites. There was no associated tenderness. The bowel sounds were normal to auscultation. Pelvic examination revealed a firm nodular mass with minimal mobility. Abdominal and pelvic ultrasound demonstrated a large 15 cm complex and predominantly solid mass with cystic changes in the left adnexa. The right ovary was not seen, and the endometrium was not visualized. A moderate amount of ascites was also noticed around the liver, spleen, and lower quadrants. Computed tomography (CT) also showed a large, mainly solid, complex mass in the lower abdomen/pelvis displacing the uterus to the right side with extensive ascites . There w Laboratory investigations revealed normal complete blood count, electrolytes and liver function tests. The serum CA125 was elevated at 1291 U/mL. Endometrial biopsy showed endometrial hyperplasia with atypia. Given the patient's clinical presentation, imaging findings, and elevated CA-125, there was a provisional diagnosis of metastatic epithelial ovarian cancer. The initial plan for management was to give neoadjuvant chemotherapy followed by interval debulking surgery. For both diagnostic and therapeutic purposes, four paracenteses were done to drain the ascites. Each paracentesis drained approximately 4 L of fluid. However, cytology of the peritoneal fluid was negative for malignant cells on all occasions. A CT-guided core biopsy of the mass was performed in order to get a pathologic diagnosis of malignancy prior to starting chemotherapy. The biopsy was performed in another institution and reported as spindle cell tumor negative for desmin, CD10, pankeratin, low-and high-molecular-weight keratin, CK7, CK20, S100, and CD34 and undetermined for CD117 immunohistochemistry. Ki-67 was immunoreactive in 5% of cells. The patient continued to be symptomatic with rapid accumulation of ascites. The inconclusive results on core biopsy and negative cytology led to the decision to proceed with a laparotomy and primary debulking surgery for a presumed ovarian cancer. At surgery, 6 L of ascites were immediately drained from the peritoneal cavity. Upon exploration of the abdomen, the ovaries and uterus were found to be completely normal. An 18 cm mass was found arising from the retroperitoneum between the left ureter and the mesentery of the sigmoid colon. A significant length of the sigmoid colon was wrapped around the mass. The omentum also looked normal and There was no evidence of peritoneal disease or enlarged para-aortic lymph nodes. An intraoperative frozen section of a portion of the retroperitoneal mass was done and showed spindle cells, with bland histology. The exact origin of the tumor was unclear intraoperatively.  The mass was resected en bloc with the sigmoid colon by a Hartmann's procedure. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, and omentectomy were also performed. The postoperative course was complicated by an ileus and she was discharged on day eight. The main tumor mass measured 18 × 11 × 7 cm and consisted of a firm, solid, well-defined, and light brown lobulated mass with a homogeneous cut surface that was attached through the mesorectum to an 18 cm unremarkable segment of sigmoid rectum . A secon Twenty representative sections from the tumor were submitted. Those revealed a spindle cell proliferation typical of a fibrothecoma. The tumor consisted of fusiform cells arranged in whorls and fascicles alternating with more hypocellular areas with plumped cells . FocallyThe neoplastic cells were diffusely positive for vimentin, calretinin , CD99 anThe neoplastic cells were negative for the following markers: pankeratin and cytokeratin 5/6 ; CD34, CD117, and DOG1 ; desmin, h-caldesmon and smooth muscle actin a (panel used to rule out smooth muscle proliferation); S-100 ; CD10 . The patient's ascites did not reaccumulate after surgery. The serum CA-125 was repeated a month after surgery and it normalized at 19 U/mL. At 3-month followup, the CA-125 was 7 U/mL, and CT scan of the abdomen and pelvis was negative for recurrence or metastasis. The stoma was reversed approximately 7 months after her initial surgery. The patient consented to the case report. Fibrothecomas constitute only 4% of all ovarian tumors and are the most common solid tumors of the ovary –3. Tumor The etiology of how a fibrothecoma develops in an extraovarian location is unknown and its histogenesis remains speculative. There are two types of heterotopic ovarian tissue: (1) the accessory ovary, which is connected to, or lies close to, the orthotopic ovary and arises from the same primordium; (2) the supernumerary ovary, which is a third ovary located at a distance from the bed and arises from a separate anlage . Most su This is the first reported case of an extraovarian fibrothecoma presenting with symptoms and findings similar to a metastatic epithelial ovarian cancer with gross ascites and an elevated CA-125. Ovarian fibrothecomas can be associated with Meigs' syndrome, typically described as the triad of ascites, unilateral hydrothorax, and benign ovarian tumor . Meigs'  The diagnosis of extraovarian fibrothecoma preoperatively would be greatly assisted if characteristic imaging findings existed. The two previously reported cases of extraovarian fibrothecoma were in the 1980s, before the regular usage of modern day imaging modalities. Although sonography has been considered as the imaging modality of choice in detection of ovarian masses, the sonographic features of ovarian fibrothecoma are nonspecific [ Due to the rarity of extraovarian fibrothecoma and the difficulties with making the diagnosis preoperatively, the diagnosis will likely continue to be made after surgery. Ovarian fibrothecomas rarely pose a diagnostic challenge, and designation as extraovarian fibrothecoma can be made only when normal ovaries are confirmed. Preoperatively when diagnostic material is limited to either cytology or core biopsy, reaching a definitive diagnosis is more difficult. Sex cord-stromal tumors should be included in the differential diagnosis of retroperitoneal mass with ascites, and a panel of immunohistochemical markers such as calretinin, CD99, and inhibin can be used as an adjunct diagnostic tool. The sensitivity for sex cord-stromal lineage may vary between markers, and some markers may not be as sensitive in some types of sex cord-stromal tumors compared with other tumors in this spectrum of neoplasms , 18. The Although ovarian fibrothecomas are not unusual, this case represents the third reported case in the literature of an extraovarian fibrothecoma. It is also the first reported extraovarian fibrothecoma to present with symptoms and findings similar to that of metastatic ovarian cancer. This case highlights the importance of obtaining cytologic or pathologic evidence of malignancy prior to initiating neoadjuvant chemotherapy for a presumed ovarian cancer. Despite the benign diagnosis in this case, radical surgery would still be required to remove the tumor.


Ectopic liver is a very uncommon developmental anomaly that predisposes to the development of hepatocellular carcinoma. We describe the second documented case of a hepatocellular carcinoma developing in the primary liver of a patient with a rare and uncharacterized genetic symptom complex. Also present was the largest ectopic liver ever reported, measuring 12 cm in diameter which contained a solitary focus of metastatic hepatocellular carcinoma. The primary hepatocellular carcinoma is believed to have arisen in the native liver from a hepatic adenoma that was diagnosed 15 years earlier. The patient's uncharacterised condition featured prominent thick, yellow skin over the dorsum of the fingers, and was associated with follicular hyperkeratosis, abnormal plantar creases, digital clubbing, misshaped ears, a lingua plicata and an angioleiomyolipoma of the right kidney.This unique case of hepatocellular carcinoma arising from liver cell adenoma in a patient with an uncharacterised condition featuring a large ectopic liver invites discussion of the role of local factors in carcinogenesis in the parent liver but not the ectopic liver. It also underlines the imperative ongoing need for clinical autopsies. Ectopic liver (hepar succenturiatum) is a rare developmental anomaly. The incidence was reported as 0.23% in a series of 5500 autopsies in 1940 [Heterotopic liver tissue may be classified either as accessory liver, when it is connected with a thin stalk to the main liver or as a true ectopic liver when no such relationship can be established . Most ofMost ectopic livers are clinically silent. In some cases, recurrent abdominal pain caused by torsion or intraperitoneal bleeding have been reported . EctopicHPGD). HPGD is the main enzyme required for prostaglandin degradation, including prostaglandin E2 [kB ligand or growth factors like Interleukin (IL)-6 are involved in the disease [Pachydermoperiostosis is a rare type of genodermatosis, characterized by pachydermia, digital clubbing and periostosis . Recentlandin E2 ,11. The  disease .A thirty year old Caucasian woman was treated for oligomenorrhoea with Norethisteron for six months. During follow up, a thorough clinical workup for hirsutism, chronic pruritus and abdominal discomfort revealed several nodules in both lobes of the parent liver, and an 18 cm large presumed splenomegaly Figure , both weExternal examination of the body revealed a severe icterus, hirsutism and a malformed left auricle. Additionally, a striking hyperkeratosis, mild acanthosis but no parakeratosis was present on the skin of the soles of the feet. The skin and shape of the fingers was reptile like with mild digital clubbing. There were abnormal skin creases on the palms and soles Figure . ExaminaAfter histological examination, the diagnosis of a dedifferentiated hepatocellular carcinoma (HCC) was established in the parent liver Figure . The tumThe ectopic liver was separated by a fibrous capsule from the adherent spleen. The liver architecture was well preserved Figure  and 1B. Immunohistochemistry for androgen, oestrogen and progesterone receptors was consistently negative in the HCC as well as the parenchyma of the parent liver and the ectopic liver.The right kidney harboured a lipoleiomyoma (diameter 0.8 cm). The hypophysis and adrenal glands were normal in size while both ovaries were atrophic.In this report, we present a rare case of a patient having dedifferentiated HCC in the primary liver along with metastatic lesions in the giant ectopic liver and the lungs (lymphangiosis carcinomatosa). To our knowledge, there is only one other case of a Japanese patient who presented with similar findings, cited in a meta-analysis by Arakawa .The malignant transformation of HA's has been well established in the literature . In factIn our case, the HA persisted in the native liver and eventually underwent malignant transformation. Interestingly, this probably overshadowed the malignancy in the giant ectopic liver. The ectopic liver harboured a 0.9 cm malignant nodule which was interpreted as a metastasis from the HCC in the primary liver. This conclusion was based on the fact that both the lesions shared the same histological characteristics and that, unlike the native liver, no predisposing conditions such as HA or liver cell dysplasia was present in the ectopic liver. The history of portal vein thrombosis and intimal fibrosis of the liver veins suggests an impairment of blood outflow. As far as the carcinogenic process is concerned, both, the ineffective bile reabsorption and the altered blood outflow might have been contributory factors.Generally, ectopic livers are known to form nodules ranging from several millimeters to several centimeters in size ,5. To ouIn addition, the patient presented with a very unusual condition. During her lifetime, the provisional diagnosis of pachydermoperiostosis had been introduced by a consultant dermatologist (P.I.) at the time of detection of the HCC. This rare familial genodermatosis can present variably with the following features such as digital clubbing, swelling of peri-articular tissue, osteoarthropathy, arthralgia and arthritis, congenital heart disease, delayed closure of the fontanels, pachydermia and seborrhoea ,11,17. AIn this case, the patient had digital clubbing, albeit not a very prominent feature. Additionally, she had reptile-like fingers. Other changes included abnormal creases on the hands and feet, thick yellow skin and disseminated follicular hyperkeratosis. It is believed, that the skin changes are due to an activated Wnt signaling pathway with increased dermal fibroblast functions . BecauseHPGD (4q33-q34) that encodes for 15-hydroxyprostaglandin dehydrogenase which is the main enzyme of prostaglandin degradation [The patient also presented with idiopathic hirsutism which can probably be explained by the recently identified mutations in radation . A commoThe patient also had a very distinct auricle malformation. This has not been described in pachydermoperiostosis. In fact, this malformation resembles the case with Auralcephalosyndactyly described by Kurczynski, or the case of long chromosome 4 syndrome, described by Lipson, although the other features of these reports do not fit into the puzzle ,24. WhilLingua plicata has been linked to Down syndrome, Sjörgren syndrome, Melkersson-Rosenthal Syndrome, psoriasis and geographic tongue . HoweverA search in pubmed and OMIM for "heterotopic liver" or "ectopic liver", "pachydermia", "hyperkeratosis", "lingua plicata", "angiolyomyolipoma" and combinations thereof did not reveal any usable hits ,29. TherThe worldwide autopsy rates have declined for many years despite their proven positive impact on various aspects in daily modern medical practice . This caWe report the extremely rare case of a HCC in the parent liver, metastasizing into an ectopic liver in a patient with an association of unique clinical features suggestive of pachydermoperiostosis. It encompasses external malformations, resembling pachydermoperiostosis but cannot be explained by this alone. The internal malformations include a giant ectopic liver, fused with the spleen, a lingua plicata, an enchondroma and an angioleiomyolipoma of the kidney. This association may be either coincidental or represent an undescribed rare genetic syndrome.Written informed consent could not be obtained from the patient. To our knowledge, there are no relatives or next of kin available.The authors declare that they have no competing interests.All authors contributed to this article. All authors read and approved the final manuscript.


Testicular neuroendocrine tumors (NET) or carcinoid tumors are rare neoplasms which represent 1% of all testicular tumors and can be divided into 3 subgroups: pure primary testicular NET, primary testicular NET associated with a teratoma, and NET metastases to the testis. We report an unusual presentation of a primary testicular neuroendocrine tumor in a 39-year-old male who presented after a physical altercation during a soccer game. Histology showed a diffuse infiltrating tumor with extensive involvement of the tunica albuginea and tunica vaginalis. Immunohistochemical expression of CD56, synaptophysin, and chromogranin A was strongly positive in the tumor cells. Foci of tumor cell necrosis and occasional mitotic figures as well as extensive lymph-vascular invasion were also identified. A review of the literature reveals differing opinions on the prognostic significance of primary tumor size, mitotic index, tumor necrosis, and nuclear atypia. In our patient, the increased mitotic rate (3–5 mitotic figures per 10 hpf and a Ki-67 index of 5%), foci of necrosis, and mild to moderate nuclear atypia warranted a diagnosis of neuroendocrine tumor grade 2, formerly atypical carcinoid. Long term surveillance in these patients is essential as metastasis occurs in up to 15% of cases. At the 6-month followup, the patient remains symptom free. Testicular neuroendocrine tumors (carcinoid tumors) are rare neoplasms which represent 1% of all testicular tumors . We repoUpon presentation to the emergency room, the patient reported multiple blows to the face and eye. Initial evaluation preoperatively revealed an eyelid laceration for which the patient was taken urgently to the OR for repair. Intraoperative exam revealed a previously unnoticed massively enlarged left hemiscrotum without ecchymosis. Upon further questioning of the patient's wife regarding the history, it was noted that the patient had an at least 15-year history of asymptomatic enlarged scrotum on the left side which had not been previously evaluated. Ultrasound showed a diffuse, heterogeneous, and predominately hypoechoic enlargement of both testicles without increased vasculature and scattered lobulated anechoic cystic foci of various sizes, as well as an irregular contour in the left testicle . The ultIntraoperative bilateral scrotal exploration was performed. There was a small hydrocele in the right hemiscrotum, and inspection of the right testicle was unremarkable. The left hemiscrotum contained a large left hematocele which was drained, revealing a markedly enlarged left testicle with a thickened, firm tunica albuginea containing multiple irregular nodules. A left orchiectomy was performed due to the suspicious operative findings. Gross examination revealed several small firm nodules on the tunica albuginea; the largest had heaped borders and measured 4.2 cm in greatest dimension. Sections of the testicle showed a heterogeneous yellow tan mass infiltrating the testicular parenchyma. Also, within the testicular parenchyma was a multiloculated hemorrhagic cystic nodule measuring approximately 3.2 cm. The remaining testicular parenchyma was distorted, congested, and edematous with a large, gelatinous hemorrhagic clot.  Histology showed a diffuse infiltrating tumor with extensive involvement of the tunica albuginea and tunica vaginalis. The tumor was composed of moderately pleomorphic cells in a pseudoglandular and nested pattern. The tumor cell nuclei had coarsely granular chromatin, surrounded by abundant eosinophilic cytoplasm . ImmunohClinically, the patient denied all symptoms and signs of a carcinoid syndrome. Urinary 5-hydroxyindoleacetic acid levels were not elevated, 2.4 mg/L and 7.6 mg/24 hour. However, chromogranin A was moderately elevated at 9 nmol/L. Postoperative CT of the chest, abdomen, and pelvis was normal except for borderline enlarged retroperitoneal lymph nodes on the left side, which measured up to 1 cm. The tumor was diagnosed as a primary neuroendocrine tumor grade 2 (G2) of the testis.At the 6-month followup, the patient remains symptom free. 5-HIAA 24 hr urine, CBC, CEA, CA 19–9, and chromogranin A levels were all normal. Scrotal ultrasound demonstrated a normal right testis. Repeat abdomen and pelvis CT showed stable lymphadenopathy with no signs of recurrence. Testicular neuroendocrine tumors (NET) or carcinoid tumors of the testis are rare, comprising less than 1% of all testicular tumors, and they can be divided into 3 sub-groups: pure primary testicular NET, primary testicular NET associated with a teratoma, and NET metastases to the testis –4. They While it is noted that the prognosis of testicular NET arising within a teratoma is better than pure testicular NET , determiThe management of localized well-differentiated testicular neuroendocrine tumor is complete surgical excision. The role of adjuvant treatment such as conventional chemotherapy or radiotherapy for higher grade tumors is not well defined. Somatostatin analogues have shown to be effective in improving prognosis . In our patient, initial chromogranin A was slightly elevated, but follow-up levels were normal. Plasma levels of chromogranin A have been used as a marker for recurrence in patients with midgut neuroendocrine tumors with fairly good sensitivity . MarkedlLong term surveillance and followup in these patients are essential as metastasis occurs in up to 15% of cases, and late disease recurrence has been reported many years after orchiectomy, with at least one reported case in the literature of a recurrence after 17 years , 11. The


Dentigerous cysts are the most common developmental cysts of the jaws, most frequently associated with impacted mandibular third molar teeth and impacted canines. Bilateral dentigerous cysts are rare and occur typically in association with a developmental syndrome. The occurrence of bilateral dentigerous cysts is rare and, to date, only 21 cases have been reported in literature till 2009. Here, we report a case of bilateral dentigerous cysts in maxillae of non-syndromic 10-year-old patient with brief review of literature. A dentigerous cyst is an epithelial-lined developmental cavity that encloses the crown of an unerupted tooth at the cementoenamel junction. DentigerA 10-year-old boy was referred to the department of oral surgery at Dr D.Y. Patil Dental College and Hospital for the evaluation of an asymptomatic, cystic lesion in the right maxilla Figures . IntraorFNAC of right side showed cholesterol crystals with inflammatory cells.Histologically both the specimens were similar and showed a thin fibrous cystic wall lined by a 2-3 cell layers thick non-keratinized stratified squamous epithelium. Retepegs were absent and the connective tissue showed inflammatory cell infiltrate. Subepithelial layers showed parallel bundles of collagen fibers observed at periphery. These findings confirmed diagnosis of dentigerous cysts Figures  and 11.et al, reported an incidence rate of 0.1-0.6%, whereas Shear found the incidence to be 1.5%.911125911911The age range for the reported cases varies widely, from 5 to 57 years of age. Eight of the cysts occurred in children under the age of 12 as with our case. All but three cases 6, 12, 13 were identified at ages corresponding to the normal eruption times of the affected teeth. As with our case, these three cases occurred in asymptomatic individuals, which accounts for the delayed diagnosis. All but three of the 21 cases 2, 16, 18 have been associated with mandibular molar teeth. But bilateral dentigerous cyst s affecting premolar and canine are rare as with our case. There have been no reported cases of non-syndromic, bilateral dentigerous cysts occurring in all four dental quadrants. Since cysts can attain considerable size with minimal or no symptoms, early detection and removal of the cysts is important to reduce morbidity. Moreover, all but three of 21 reported cases, presented without pain. And, all but one was discovered during investigation of asymptomatic slow growing swellings. It is therefore important to perform radiographical examination of all unerupted teeth. While bite-wing and periapical radiography is typically performed in the routine examination of patients with a healthy dentition, this series of radiographs may occasionally fail to delineate the full extent of a lesion if present. A panoramic radiograph supplemented with skull series or more advanced imaging such as tomography may permit a better delineation of the extent of the lesion and its relationship to adjacent anatomical structures.[Removal of associated tooth and enucleation of soft tissue component is definative therapy in most instances. In case Bilateral dentigerous cyst is a very rare entity. In case of multiple dentigerous cyst, a through clinical and systematic examination should be done to rule out any associated syndrome.


Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing's sarcoma in as adult patient. Since its first description by James Ewing in 1921, Ewing's sarcoma represented a strange kind of malignancy. Poorly differentiated tumor of uncertain histogenesis with a variable biologic behavior, it presents heterogeneity inside. It is common in Caucasians and rarely arises in individuals of African and Asian ancestry. Currently, we talk about the Ewing family of tumors which comprises Ewing's sarcoma (EWS), extraskeletal EWS, primitive neuroectodermal tumor (PNET) of bone and soft tissue, and chest wall tumor (Askin tumor) . The tra2), and cyclophosphamide (1.2 g/m2) alternating with etoposide (100 mg/m2/5 days) and ifosfamide (1.8 g/m2/day/5 days) every 3 weeks. Five cycles of vincristine, doxorubicin, and cyclophosphamide (VDC) and 4 cycles of vincristine, cyclophosphamide (VC) alternating with ifosfamide, etoposide (IE) (8 cycles), and associated to mesna rescue were administered for a total of 17 cycles. Unfortunately, after 27 months from surgery, the patient was admitted again to our hospital for progressive paraparesis. MRI disclosed a massive intradural dissemination of the lesion with nodules seeding from D8 up to S2. The patient was operated again for decompression at the level of D8 where the mass was more consistent. The histologic examination confirmed the diagnosis of EWS, and the patient died few weeks later.A 58-year-old man with no significant medical history came to our attention after a 3-month history of a progressive worsening, nocturnal, bilateral buttock cramping associated to a left predominant back pain. Paresthesias with tingling were also present on the left leg. No abnormalities were found on neurological examination, beside a diffuse tenderness in the lower back and a restriction of the active flexion-extension motion. The passive straight leg-raising test was positive at more than 45°. He had a low-grade fever (37.4°C). Laboratory tests revealed a mild leukocytosis (8700/L). Erythrocyte sedimentation rate and C-reactive protein level were elevated. All other parameters were normal. Lumbosacral MRI revealed a large, enhancing mass occupying the left hemivertebra, pedicle, and hemilamina of L2 with paravertebral soft tissue extension . The theEWS remains for several aspects an anomalous tumor with bizarre behavior. Hense et al. described an unexplained, intrinsic major aggressive behavior of axial tumors . They po


Congenital nevi are present in approximately 2–3% of neonates. These lesions are present at birth. They are characterized by pigmented lesions with regular margins, smooth or lobular surfaces and occasionally have long coarse hair. The risk of melanoma development is proportional to the size, especially if it involves over 5% of the body surface, or is > 20 cm in adolescents . The risk of malignant change ranges from 5–40%. We present a case of a congenital melanocytic nevus in a six-year-old female child which was surgically treated. Genetic mechanisms may account for familial aggregation. These lesions, when large, make a formidable undertaking in view of the lack of suitable donor sites and multiple procedures involved in its treatment. Melanocytic nevus of the face is a particular challenge to a plastic surgeon not only because of a very high standard of skill required but also for the patient and parental concern for cosmetic results. We present a case of melanocytic nevus in a six-year-old female child which was surgically treated.Melanocytic nevus of the face is a unique form of congenital nevus, fortunately rare and typically hair-bearing. These lesions have a relatively high risk of becoming malignant. They develop probably between 40 days of gestation and six months A six-year-old female patient was referred to the Department of Oral and Maxillofacial surgery with a chief complaint of swelling on the right upper eyelid from past two years. Examination of the patient revealed soft, well-defined swelling over the right upper eyelid Figure  and b. SCongenital pigmented nevi are present in approximately 2–3% of neonates. Fewer than 10% of these lesions are larger than 3 or 4 cm, a size cut-off below which the designation small is usually given.2 The opin8Large pigmented nevi of the face present a major deformity for the child and his or her parents. It is also a major challenge for the treating plastic surgeon to plan and achieve the best cosmetic results. There is complete consensus that the percentage risk of malignancy in these cases is fairly high. Complete early prophylactic excision of these lesions is warranted and should be accomplished in infancy or early childhood. The most common complications of eyelid reconstruction causing true functional impairment are ptosis, or the creation of a droopy upper eyelid and lagophthalmos, or tissue shortage preventing adequate closure. Manageme10


Myoepithelioma is a rare neoplasm of the salivary glands, generally occurring in the parotid gland and less often in the mi-nor accessory salivary glands of the oral cavity. It is known to be a rare entity occurring at unusual locations, which makes it difficult to diagnose. Such a rare case of myoepithelioma in salivary glands present at an unusual location around the left orbit is presented here. Two years later, Bauer called them adenomyoepitheliomas.4 Following the original description, numerous reports were published discussing whether myoepitheliomas originated de novo or as part of a mixed tumor. Such a rare case of myoepithelioma at an unusual location is presented here. These types of cases create a dilemma in the mind of the clinician.Myoepitheliomas of salivary glands are extremely rare, comprising approximately only 1–1.5% of all salivary gland tumors.,A 22-year-old male came with a complaint of a swelling on the left side of left eye since 1 year. The swelling was a small quiescent nodule approximately 1 year back and had gradually increased to attain the present size. There was no history of any trauma in this area with no history of any paresthesia or recent weight loss. Extra-oral examination revealed a well defined oval swelling approximately 4 cm in maximum diameter antero-posteriorly, present 1 cm lateral to outer canthus of left eye and ending approximately 6 cm in front of external auditory meatus, and measuring about 5 cm superoinferiorly. The overlying skin was smooth and normal in color. On palpation, the swelling was firm in consistency and was non-tender. It was not fixed to the underlying tissues or to the overlying skin. No lymph nodes were palpable. No radiThe swelling was surgically excised and sent for histopathological examination, where it was diagnosed as myoepithelioma. The pat5 The age and gender distribution of myoepithelioma is similar to that of mixed tumors; mostly occurring in 4th to 6thdecade of life.5 The most common locations of myoepithelioma of the head and neck are the parotid gland (40%) and the palate (21%).6 Myoepitheliomas have been known to occur at various uncommon locations like maxillary sinus,7 and sinonasal cavity.8 Up until the present report, no similar case has been reported in the literature, making it a unique case owing to its uncommon location. There are no distinctive clinical features and, similar to most of the other salivary gland tumors, myoepitheliomas present as asymptomatic, slowly growing masses. Parotid lesions never cause facial nerve dysfunction or cervical lymphadenopathy, and those of the palate rarely ulcerate.Myoepitheliomas account for less than 1.5% of all salivary gland tumors. A review of the literature through 1993 yielded approximately 100 cases. The number of case reports is increasing as pathologists have become more aware of its existence.10 The appearance of myoepithelioma on computed tomography (CT) imaging, as a well-defined enhancing mass, is not specific.11Microscopically, several growth patterns occur: solid, the most common, mixoid (pleomorphic adenoma like), reticular, and mixed. Cells can vary in histological appearance being spindle-shaped, plasmacytoid, and epitheloid; occasionally, epitheloid cells have clear cytoplasm. The different cellular compositions have no correlation to prognosis.1 Such myoepitheliomas can arise either de novo or develop ex-pleomorphic adenoma or ex-benign myoepitheliomas.13 These can be differentiated on the basis of radiographic or sonographic changes, which were absent in the present case. The treatment of a myoepithelioma is generally considered as complete surgical excision.1The differential diagnoses of myoepitheliomas include pleomorphic adenoma and other mesenchymal neoplasms, nasal fibrosarcoma, fibromatosis of the sinonasal tract, neoplasms of smooth muscles, sinonasal myxoma, and embryonal rhabdomyosarcoma. Malignant myoepitheliomas have been described that infiltrate the surrounding tissues and even metastasize.


Although both appendiceal tumor and intestinal endometriosis have been reported as rare causes of abdominal pain, the coexistence of appendiceal mucinous neoplasm and ileal endometriosis has not previously been reported.A 41-year-old Japanese woman presented with a positive fecal occult blood test and a 3-year history of menstruation-related lower abdominal pain. A colonoscopy demonstrated extrinsic compression of the cecum, suggesting a mass arising from the appendix or adjacent structures. Abdominal imaging showed a 6-cm cystic mass with intraluminal thick fluids originating from the appendix. At ileocecal resection for an appendiceal tumor, a 2-cm mass in the terminal ileum was incidentally found, which was included in the surgical specimen. Microscopic examination confirmed a diagnosis of a mucinous neoplasm of the appendix with endometriosis of the terminal ileum.To avoid urgent surgery for subsequent serious events associated with disease progression, appendiceal tumor and intestinal endometriosis should be ruled out in patients with chronic abdominal pain. Appendiceal tumor and intestinal endometriosis have been reported as rare causes of chronic abdominal pain. In a 10-year-old boy, chronic right lower abdominal pain every 3 to 6 weeks for the past year prior to presentation was attributed to a mucinous neoplasm of the appendix. Of the A 41-year-old nulliparous Japanese woman has suffered from intermittent right lower abdominal pain for 3 years, which was sometimes related to her menstrual periods and associated with diarrhea, but settled without medical interventions. She received a colonoscopy because of a positive fecal occult blood test at a colorectal cancer screening. Colonoscopy did not detect any mucosal lesion in the colon and rectum, but demonstrated extrinsic compression of the cecum, suggesting a mass arising from the appendix or adjacent structures. Abdominal computed tomography showed a 6-cm cystic mass originating from the appendix with intraluminal thick fluids, the features of which were compatible with those of a mucinous neoplasm of the appendix Figure. There wApproximately 25% of appendiceal mucinous neoplasms are asymptomatic and found incidentally on abdominal imaging or at surgery. Rare clIntestinal endometriosis may be more frequent than expected, but the involvement of the terminal ileum is rare. There were 163 cases of bowel endometriosis (5.4%) among 3037 laparotomies for endometriosis, and resections of the ileum and/or cecum were performed in 11 cases, whereas a colectomy was needed in 30 cases. The claBoth appendiceal tumor and intestinal endometriosis can cause chronic abdominal pain, which may be an omen of subsequent severe events associated with disease progression. Careful history taking and relevant abdominal imaging are needed when encountering a patient complaining of chronic abdominal pain.Written informed consent was obtained from the patient for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.We declare that we have no competing interests.TK and TF drafted the manuscript. NI, KE and MO equally contributed to data collection and KY supervised manuscript writing. All authors read and approved the final manuscript.


Colonic adenocarcinoma revealed by metastatic anorectal fistula is rare, with few cases in the literature. Such lesions can be taken for the more common manifestation of a benign perianal abscess or fistula. Once diagnosed, the management of such conditions remains controversial. We herein report two cases with perianal fistula that were subsequently found to have developed perianal adenocarcinoma on biopsy. Further colonic investigation revealed a rectosigmoid adenocarcinoma. Histology and immunohistochemical staining was identical in both primary and metastatic tumors. Preoperative chemoradiation with further rectal low anterior resection and local excision of metastatic anal fistula was performed. There is no recurrence after 3 years of follow-up. Distal implantation by a proximal adenocarcinoma into an anal fistula remains a rare condition. It has been previously reported that exfoliated colorectal cancer cells can implant in distal locations such as the staple lines  and hemoWe herein present two cases of patients diagnosed with rectosigmoid adenocarcinoma revealed by metastatic perineal fistula, and treated with neoadjuvant chemotherapy followed by low anterior resection and local excision of the perineal tumor.A 55-year-old man presented with a 10-year history of recurrent anal fistula. He had no history of inflammatory bowel disease. Physical examination revealed several external openings, in the right and left perianal regions. The internal opening was located posteriorly in proctoscopy. The patient underwent fistulectomy with seton placement in fistula tracts. The pathology of the fistula excision did not show any malignancy. After 3 months of follow-up, a discharging nodule appeared in the fistula tract wound. Rectal examination did not reveal any abnormalities in the anal canal or the rectum. The nodule was biopsied, and adenocarcinoma was found on histology review. Further colonic investigation revealed a rectosigmoid tumor.Histology of both the primary tumor and the perianal nodule revealed a well-differentiated adenocarcinoma with cytokeratin 20 (CK20)+ and cytokeratin 7 (CK7)- staining. Magnetic resonance imaging (MRI) of the pelvis Figure  and endoA 68-year-old man presented to the emergency room with a perianal abscess. He had no history of anal fistula. Examination under anesthesia was performed confirming the perianal abscess with an internal opening in the anal dentate line, proving the origin was a cryptoglandular fistula. Simple drainage was performed. Because the cavity of the abscess had an atypically indurated border, biopsy was done. Histology revealed a well-differentiated adenocarcinoma. Further investigation, including rectosigmoidoscopy, MRI of the pelvis and a CT scan, showed a large carcinoma 26 cm from the anal verge and a perianal mass extending to the anal sphincter, which was confirmed by endoanal ultrasound.No distant metastasis was noticed. Histology of the primary tumor was the same as the perianal mass. Immunohistochemistry for CK7 and CK20 was performed on tissues to distinguish colorectal adenocarcinoma from anal gland carcinoma. Both the colorectal cancer and the perianal tumor were CK7- and CK20+ Figure . The modet al. have previously described that anal glands were strongly immunoreactive to antibodies against CK7 but not to CK20 [Cancer of the colon and rectum can be spread by direct extension, transperitoneal, lymphatic, and hematogenous spread. Spreading by implantation has been also described . In 1907to CK20 . In our Controversies remain in the management of metastatic anal fistula. It is difficult to define the best treatment from the literature, as some authors have performed APR, while others have chosen local resection.After discussing the modality of treatment  with our patients, we opted for preoperative chemoradiation (45 Gy), followed by anterior resection of the rectum and local excision of perianal metastasis after 6 weeks. Our choice was guided by the age of patients, differentiation and immunohistochemical characteristics of the primary and metastatic tumors, evidence of sphincter involvement on MRI and endoanal ultrasound and the absence of distant metastasis on abdominal CT. Furthermore, perineal masses in both cases shrank dramatically after chemoradiation. Consistent with that, the pathology report showed a complete response to chemoradiotherapy in both cases. The primary tumors were without lymph node metastases. Chemoradiation has been indicated as an adjuvant therapy in some previous reports ,20,25, bin ano either before or after surgery  and not to hesitate to perform biopsy in order to exclude any malignancies that may be the visible part of the iceberg. Sphincter-saving surgery is an option that should be discussed with the patient.It is strongly recommended to be aware of any unusual changes in the clinical features of the fistula Written informed consent was obtained from the patients 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.BEB has made substantial contributions to conception, bibliography and drafting the manuscript. AS has been involved in drafting the manuscript. CL has been involved in bibliography research and conception of pathology figures. IM and KM have been involved in or revising it critically for important intellectual content. KA has given final approval of the version to be published. All authors read and approved the final manuscript.


Synovial cyst occurs secondary to traumatic, degenerative, or inflammatory conditions. Synovial cysts represent abnormal distension of bursae, which communicate with the joint. Giant synovial cysts are typically due to rheumatoid arthritis, other causes being trauma and synovial pseudoarthrosis. A 33-year-old male presented to an outpatient clinic with a massive swelling on his posterolateral aspect of right thigh extending from upper one-third to the knee joint which had been increasing in size over the past six months. This was associated with dull aching pain. All laboratory investigations were within normal parameters. Even FNAC was inconclusive. With time, swelling was increasing in size. Ultrasound revealed the cystic nature of swelling. MRI showed large cystic lesion 24 × 10 × 12 cm in posterolateral aspect of thigh extending up to knee joint. Following the MRI, an excision was planned. After excision, histological examination confirmed the synovial nature of the cyst, which had a collagenous wall and dense chronic inflammatory cells. As the disease is extremely rare and asymptomatic, precise diagnosis is difficult and often delayed. We consider that open surgical excision should be reserved for cases of large synovial cysts because it can provide a complete resection of the lesion and minimize the risk of recurrence. Synovial cyst occurs secondary to traumatic, degenerative, or inflammatory conditions. Synovial cysts represent abnormal distension of bursae, which communicate with the joint . The pop A 33-year-old male presented to an outpatient clinic with a massive swelling on his posterolateral aspect of right thigh extending from upper one-third to the knee joint which had been increasing in size over the past six months. This was associated with dull aching pain. The patient also felt the mass to be aesthetically displeasing. Pain was aggravated by movement and alleviated to some extent by rest. There was no history of trauma, no history of any joint pain, and no personal and family history of gout, rheumatoid disease, or other arthritis.Physical examination revealed 30 × 20 cm swelling, nontender, and the mass was cystic in consistency and transilluminated with well-defined margin . Flexion3, differential leukocyte count of n61l34e03 m02, erythrocyte sedimentation rateof 25 mm/hr, total protein of 4.4 g/dL, albumin of 2.1 g/dL, blood urea nitrogen of 13 mg/dL, and random blood sugar of 133 mg/dL. Aspiration produced rusty coloured fluid. Synovial fluid protein and glucose were 5000 mg/dL, and 82 mg/dL respectively, rheumatoid factor was negative, C-reactive protein was normal, and serum uric acid was 4.2 mg/dL. Bacteriological cultures failed to grow any organisms. Cytology showed acute inflammatory pathology. Laboratory examination revealed haemoglobin of 14.4 gm%, total leukocyte count of 8500/mmAn ultrasound scan of the mass revealed the cystic nature of swelling. An MRI of the right lower limb was obtained which revealed a large cystic lesion 24 × 10 × 12 cm in posterolateral aspect of thigh extending up to knee joint. The lesion was closely related to the biceps femoris and hamstring muscle . FollowiOur case was a large extra articular synovial cyst and this was the reason we believed that arthroscopic intervention cannot provide a complete resection of the cyst. In such cases, the possibility of leaving even a small piece of wall lining poses a high potential risk of recurrence. Therefore, an open surgical procedure was necessary.As the skin over the swelling was so tense, aspiration was done before the surgical incision, and approximately 500 mL of fluid was aspirated and was found to contain thin, serous material . Intraoperatively, the cyst measuring 24 × 13 cm was identified, which was adherent to the sciatic nerve posteriorly and popliteal vessels were displaced anterolaterally. Cyst was originating from the surface of hamstring and biceps femoris tendon sheath and adjacent posterior surface of femur. Cyst was extending proximally in thigh and no communication was found between the joint space and the cyst. The cyst was removed totally and the base of the cyst was cauterized Figures . The basHistopathological examination confirmed the synovial nature of the cyst, which had a collagenous wall and dense chronic inflammatory cells .Postoperatively, the patient was pain free and showed almost normal strength and sensation in the right thigh. The patient had uneventful recovery with complete resolution of all symptoms. No recurrence has been observed for three years. Synovial cysts can originate from joints, bursae, or tendon sheaths. It is difficult to discriminate between cysts from bursae and those from tendon sheaths, because both share a close anatomic relationship and a similar histological appearance. Synovial cyst constitutes para-articular fluid collection lined by synovial membrane, which may or may not communicate with the adjacent joint. Ganglion cysts may have a definable connection to the joint which seems, however, to be the most important mechanism for the development of ganglion cysts. The degree of intra-articular pressure is an important factor as well, which causes the enlargement of the ganglion cyst. Furthermore, a valve mechanism supports the development of these cysts , 5. TypiFor any of these reasons, a shift of synovial fluid from regions of high to low pressure causing a progressive mobilization of the synovial tissue and its eventual outgrowth outside the capsule. Once this phenomenon occurs, the liquid may organize or move freely from the joint to the cyst through a channel with permeability that can be permanent, episodic, or short lasting. The displacement of fluid in one direction or another can increase or decrease in the size of the cavity , 9. Synovial cysts may cause pain or limitation of joint mobility. Uncommonly, they may cause compression of the neighbouring neurovascular structures. Van Mourik et al. reported a case of synovial cyst of hip joint presenting as deep venous thrombosis, secondary to compression of the femoral/iliac vein . Acute rThere is various treatment modalities available in form of aspiration and steroid administration, arthroscopic removal, and surgical excision described in the literature. Ultrasound-guided aspiration of symptomatic ganglion cysts is a valuable therapeutic modality because incomplete aspiration often results in cyst recurrence. Ultrasound guidance is especially useful for nonpalpable cysts, those near neurovascular bundles, and those with loculations and internal septations . The dra Arthroscopic treatment is very useful when there is intra-articular communication and associated joint disorder which lead to the concept that these lesions should also be healed to obliterate these cysts and prevent their recurrence . However Surgical excision is the definitive treatment but procedures have been tested including needle aspiration of intracystic content and administration of fibrin-based substances that affect the final sealing of the walls .Extra-articular giant synovial cysts may enlarge considerably to manifest as mass lesions at locations away from the joint and may rupture leading to compartment syndrome, infection, or compress the neurovascular structures. As the disease is extremely rare and asymptomatic, precise diagnosis is difficult and often delayed. More attentions should be paid because of its severe complications.We consider that open surgical excision should be reserved for cases of large synovial cysts because it can provide a complete resection of the lesion and minimize the risk of recurrence. On the other hand, arthroscopic treatment is more suitable for small lesions that lay strictly within the synovium.


A case of two fibrotic lesions of the oral mucosa in a 17-month-old African-American female is reported. Both lesions occurred on the anterior maxilla, one lesion pedunculated on the buccal attached gingiva and the other lesion sessile on the palate. Histological examination characterized the buccal lesion as focal fibrous hyperplasia (FFH) and the palatal lesion as a giant cell fibroma (GCF). A case is made for continuing the consideration of GCF as a histologically distinct entity from FFH but that no difference in clinical impact between the two lesions exists. Focal fibrous hyperplasia or fibroma is considered the most common benign soft tissue growth in the oral cavity , 2. ThisA 17-month-old African-American girl was referred from her private dentist for evaluation of two papillomatous lesions in the anterior maxilla . HistoryThe lesion on the buccal mucosa was about 1 centimeter in size, pink in color, stippled and attached via a peduncle to the attached gingiva opposite tooth no. 51 and tooth no. 52. The lesion blanched slightly with digital pressure.The lesion of the palate was about 0.75 cm in size, circular, pink in color, stippled, sessile, and adjacent to the incisive papilla between tooth no. 51 and tooth no. 52. No blanching was noted with slight digital pressure.A radiograph of the area revealed no bony involvement .Due to the age of the patient and the relative complexity of the procedure, it was decided to perform excisional biopsy of both lesions under general anesthesia. The lesions were excised utilizing a number 11 scalpel blade. The excised areas were then cauterized. On one-week followup both areas were healing well; no pain or discomfort and no difficulty in eating were reported by the mother .Buccal lesion—dense fibrous connective tissue surfaced by stratified squamous epithelium with a normal maturation pattern. No evidence of malignancy. Diagnosis of focal fibrous hyperplasia .Palatal lesion—dense fibrous connective tissue surfaced by stratified squamous epithelium with a normal maturation pattern. Many stellate fibroblasts and long thin rete pegs are present. Diagnosis of giant cell fibroma .The pathology report also suggested that since there were only subtle histological differences between the two lesions, they might have simply been in different stages of maturation. The report also advised that the child be assessed now and in the future for additional lesions and to consider and rule out fibromatosis syndrome.As the most common nonneoplastic growth in the oral cavity, much has been written about the fibroma (FFH). The identification of a GCF as a separate entity by Weathers and Callihan added a new dimension to the discussion , 4. The Multiple fibrotic lesions can concurrently occur in young children.GCF as a separate entity from FFH can be established histologically.Treatment of the FFH and GCF is identical.


Hydatid disease is endemic in Tunisia and has been considered as one of the most common surgical pathology. Several localizations have been described, but hydatidosis of the liver is the most frequent clinical entity. Primary hydatid cyst of the gallbladder is very rare. We report in this observation a new case of primary hydatid cyst of the gallbladder diagnosed by Magnetic Resonance Imaging (MRI). Human hydatid disease is a particularly frequent in Tunisia where echinococcosis is endemic. It is a zoonotic infection caused by Echinococcus granulosus for more than 95% of cases while Echinococcus multilocularis is found in fewer than 5% of cases. The main species pathogenic for humans in mediterranean and southern european countries is Echinococcus granulosus. Hydatid cyst of the liver; witch is the most frequent location is easily diagnosed by ultrasound examination. The other abdominal cysts need generally more investigations. CT abdominal scan is usually performed to characterize cystic abdominal masses. For hydatid cyst; it allows the diagnosis in up to 90% of cases. However magnetic resonance imaging (MRI) is rarely needed. Primary hydatid cyst of the gallbladder is an unusual and very rare localization of hydatid disease and must be segregated from gallbladder daughter cysts secondary to liver primary hydatidosis.The aim of this case report is to highlight the diagnostic features of this rare clinical entity.A 32-year-old woman was admitted suffering from constant mild pain in the right hypochondrium reflecting to the epigastrium; and often accompanied by nausea during the previous 6 months. There was no history of fever or jaundice. The physical examination didn't show abnormal abdominal signs except tenderness in the right upper quadrant of the abdomen. Routine blood tests proved unremarkable. Plain abdominal X-rays was normal. The ultrasound examination showed that her gallbladder was deformed with a localised thickening of its wall . There wTo eliminate gallbladder neoplasm; a CT abdominal scan was performed showing inflammatory gallbladder wall which is free of suspect lesions . AgainstAntiechinococcal antibodies were not found in serum. The diagnosis of primary hydatid cyst of gallbladder was supported and surgery has been decided. The patient underwent right subcostal laparotomy. Intraoperatively it has been found a cyst of the body of the gallbladder which is inflammatory and deformed. No other cysts were found in the liver and peritoneal cavity. A cholecystectomy was performed. It permitted a total removal of the cyst without rupture. A preoperative cholangiography searching daughter cysts in the common bile duct proved unremarkable . The patHydatidosis is a human disease caused by the larval form of Taenia echinococcus. This parasite is endemic in many sheep- and cattle-raising countries , 2. HydaThose manifestations lead habitually to ultrasound examination which is the most important investigation. It supports the diagnosis in up to 90% of cases in hepatic hydrated cysts. This examination can divide hydrated cysts into five groups depending on age of cyst. This classification; established by Garb et al; is always used in the Tunisian literature since 1981Surgery is always required for the treatment , 10. AccPrimary hydatid cyst of the gallbladder is a very rare clinical entity. Preoperative diagnosis seems to be possible with a specific imaging finding which can be offered by CT scan or RMN such as in our observation. The gallbladder primary hydatid cyst may have different spread routs of parasite embryos. Prognosis is better than hepatic localisations due to earlier diagnosis and the possibility and the safety of total pericystectomy.


In the current study, a case of recurrent desmoplastic small round cell tumor (DSRCT) is presented, which was successfully treated by repetitive debulking surgery. In May 2010, a 39-year-old male, with a history of surgical resection of intra-abdominal DSRCT, visited the Ibaraki Medical Center, Tokyo Medical University Hospital  with severe lower abdominal discomfort. Abdominal computed tomography revealed a large tumor in the pouch of Douglas with a small number of nodules in the abdominal cavity. The recurrent DSRCT was diagnosed and removed via lower anterior resection; however, complete resection was impossible due to multiple peritoneal metastases. One year later, the patient developed pain in the right groin due to the growth of metastasized tumor cells in the groin lymph nodes. The affected lymph nodes were removed utilizing an extra-peritoneal approach. At the time of writing, the patient continues to survive without any symptoms 60 months since the initial surgery. In conclusion, surgical debulking is a significant procedure for relieving patient symptoms as well as improving the survival time of patients with metastatic and recurrent DSRCT. Desmoplastic small round cell tumor (DSRCT), which was first described by Gerald and Rosai , is a raIn May 2010, 39-year-old Iranian male visited the Ibaraki Medical Center, Tokyo Medical University Hospital  presenting with lower abdominal and pelvic pain together with constipation. His medical records revealed that he had undergone palliative surgery at another hospital for a large intra-abdominal tumor two years previously. Histological diagnosis of the resected specimen indicated DSRCT. One year after the first surgery, the residual tumor had grown rapidly and he had received anticancer medications. However, the residual tumor appeared to be worsening and the patient complained of debilitating symptoms. Physical examinations on the individuals’ admission to the Ibaraki Medical Center, Tokyo Medical University Hospital revealed lower abdominal tenderness without a palpable mass. The majority of the laboratory assessment results were normal, with the exception of a slight liver function disorder. Furthermore, numerous tumorigenic factors, including carcinoembryonic antigen, carbohydrate antigen (CA) 125, CA 19-9 and neuron specific enolase (NSE) were within the normal range. Abdominal enhanced computed tomography (CT) revealed a large mass  with slight heterogeneous enhanced areas in the pouch of Douglas, coupled with nodules in the abdominal cavity . In magnAmong the several treatment strategies available, the patient opted for surgical excision of the tumor. A laparotomy was performed to relieve the patient’s symptoms, during which a voluminous mass was identified that had occupied the pouch of Douglas. Furthermore, numerous small nodules were observed on the omentum, which were coupled with diffuse peritoneal seeding on the surface of the diaphragm. The large mass had penetrated the anterior wall of the rectum, however, the bladder and the urethra were not affected. The tumor was removed via a low anterior resection and a covering ileostomy was constructed. However, complete elimination of the tumor appeared to be difficult due to multiple peritoneal metastases, spreading to the nodules, diaphragm and spleen.Macroscopically, the tumor, which measured 18×11×7 cm, had an uneven surface and when cut, necrotic areas were observed. Histological examinations of the excised tumor revealed that the tumor cells had focally invaded the muscularis propria of the rectum . InvasivThe patient was followed up and one year later, complained of intermittent pain in the right lower limbs. A follow-up examination by CT scan revealed metastasis and growth of tumor cells in the inguinal lymph nodes. A right inguinal lymphadenectomy was performed and five days later, the patient was discharged with no postoperative complications. The excised lymph nodes measured 4.5×3.0 and 5.0×2.0 cm. In the immunohistochemical examinations of the excised lymph nodes, CK AE1/AE3, CK CAM5.2, EMA, CD99, desmin, NSE, CK 7, c-kit and CA 125 were positive, however, CK 34βE12, CK 5/6, CK 20, CD34, S-100 protein, neurofilament, synaptophysin and α-SMA were negative. The MIB-1 index was 70% and these pathological findings of the lymph nodes were compatible with the metastatic DSRCT. At the present time, 60 months following the first diagnosis of DSRCT, the patient continues to be symptom free, without any local progression of the tumor.DSRCT is a rare, but aggressive type of tumor with a poor prognosis and a high prevalence in young males, with the peak age at diagnosis ranging between 16 and 26 years ,6. In thDSRCT is a member of the family of malignant small round cell tumors. They are characterized by small, round, relatively undifferentiated cells and generally include Ewing’s sarcoma, peripheral neuroectodermal tumor, rhabdomyosarcoma, synovial sarcoma, non-Hodgkin’s lymphoma, retinoblastoma, neuroblastoma, hepatoblastoma, and nephroblastoma or Wilms tumor. Differential diagnosis of small round cell tumors is particularly difficult due to their undifferentiated or primitive features . Histopaet al , VAC , IVA , P6  and PAVEP , have been attempted with a certain degree of chemosensitivity and improved survival rates –22. HoweIn conclusion, this case report presents a potential treatment strategy for patients who develop recurrence of intra-abdominal DSRCT as a solitary mass with multiple seeding of the peritoneum.


Gastric duplication cysts comprise 2-7% of gastrointestinal duplications, rare congenital malformations that can be present at almost any part of the alimentary tract. They mostly present with gastrointestinal obstruction symptoms, ulceration, and painless hemorrhage. Symptoms include nausea, vomiting, and fullness sensation. Gastric duplications are mostly cystic in shape. Herein, we present a 58-year-old man with a gastric duplication cyst, 70×30×35 mm in size, with the initial presentation of abdominal pain, icterus, and elevated liver enzymes. The patient provided informed consent for this report.  Gastric duplications, the least common among all duplications, constitute 2-7% of GI duplications and mostly present with GI obstruction symptoms, ulceration, and painless hemorrhage, mostly in early ages.2 Most cases of gastric duplication cysts suffer from nausea, vomiting, and fullness sensation as the semi-obstruction symptoms. Gastric duplications are mostly cystic as shown by a conclusive study done by Holcomb et al.3 who reviewed 96 patients with 101 duplications over 37 years and observed that 75 of the duplications were cystic and 26 were tubular. Duplications are mostly located in the greater curvature of the stomach and do not communicate with the gastric lumen.2,4,5 We describe a patient presenting with a gastric duplication cyst and the initial presentation of icterus. It is deserving of note that the cyst was positioned in the proximity of the gastric lesser curvature and as such exerted pressure on the portal vein and caused jaundice. Our literature review showed a paucity of data on the alimentary tract duplications initially presenting with icterus and elevated liver enzymes.Congenital alimentary tract malformations are rare developmental errors that can be present at almost any part of the gastrointestinal (GI) tract.2 blockers with moderate improvement of the symptoms. Recently, he had developed on-and-off icterus, right upper quadrant abdominal pain, fever, nausea, and vomiting. He had previous abdominal ultrasound evaluations, which were unremarkable. No significant history was noted except exposure to chemical weapons during the Iran-Iraq war 24 years previously. On physical examination, the vital signs were normal and stable. The epigastric area was mildly distended, and a mass was only just palpable. Physical examination was otherwise normal. Laboratory work-up was remarkable for elevated liver enzymes and serum bilirubin, which were checked twice at a 24-hour interval:A 58-year-old man presented with long-standing postprandial abdominal pain (epigastric area) for 25 years. The pain had been misdiagnosed and managed as peptic ulcers with proton-pump inhibitors and H● Serum glutamic oxaloacetic transaminase (SGOT): 135 and then 148● Serum glutamic pyruvic transaminase (SGPT): 187 and then 173● Alkaline phosphatase: 564 and then 520● Total bilirubin: 7.8 and then 7.9● Direct bilirubin: 3.4 and then 3.8The patient’s plain abdominal flat and upright X-ray were normal. Abdominal sonography revealed a 5-cm ovoid cystic mass arising from the lesser curvature (near the antrum) of the stomach distending toward the portal vein. Color Doppler sonography of the common and proper hepatic artery and the portal vein was performed to evaluate the possibility of the luminal invasion of a cholangiocarcinoma or adenocarcinoma of the pancreas as differential diagnoses, which revealed reduced blood flow of the common hepatic artery and proper hepatic artery without any intraluminal lesion. Computed tomography (CT) scan of the lesion was compatible with the sonographic findings and showed a 70×30×35 mm mass with liquid density and thin calcification in the walls in the posterior aspect of the gastric antrum and pylorus in the vicinity of the posterior wall of the stomach . The panThe patient underwent exploratory laparotomy and excision of the duplication cyst. The cyst, as the abdominal CT scan reported, was located in the lesser curvature of the stomach, adherent to the stomach wall without any communication with the gastric lumen. The cyst stretched toward the portal vein, with obvious signs of inflammation in the area that caused a tension effect on the portal vein, resulting in the narrowing and flow impairment of the hepatic artery and common bile duct. The duplication cyst was excised successfully  and 3.The sample sent to the pathology lab was a small portion of the stomach, creamy-brown in color and measuring 7.5×3.5 cm in size, with a blind tip. Pathological diagnosis was gastric duplication as we had expected. The sample showed mucosal flattening with focal erosion, chronic inflammation with lymphoid follicle formation, and focal calcification of the wall. Surgical margins showed mild chronic inflammation with vascular congestion. Fortunately, there was no evidence of dysplasia or malignancy in the specimen. The patient had a normal uneventful postoperative recovery and was discharged 3 days after the operation with favorable results in one year of follow-up sessions without any of the symptoms with which he had initially referred.The patient provided informed consent for this case report. 3 study with 101 cases of duplication, 21 duplications were confined to the thorax, 3 were thoracoabdominal, and 77 were abdominal.3Duplications are believed to be rare congenital malformations that can eventually occur at any part of the GI tract. In the Holcomb6 in 1937. Since such anomalies are very rare, the current literature merely consists of few case reports. Among all abdominal duplications, gastric duplications are the least common (approximately 5%). They are more prevalent in men than in women, and patients present at a mean age of 3 years  .Gastric duplications are cystic structures located along the greater curvature or posterior of the stomach. They typically do not communicate with the stomach cavity.2,7 Ultrasonography, CT, and myelography are helpful diagnostic tools.3These malformations were first introduced and termed as GI duplications by LaddOur literature review demonstrated that the duplication of the alimentary tract has many different forms; therefore, the application of a single embryologic theory does not seem to be valid. This has led to the suggestion of some different theories to explain the embryologic events that result in duplications. 8 who presented 9 cases of gastric duplication, only one single case was adult.Gastric duplications are rarely detected in adults since they present in the first years of life. In a study performed by Kremer et al.2 Also, abrupt hemorrhage with hemodynamic instability can be seen when a cyst which is lined with the gastric mucosa ulcerates the adjacent organs or vessels.9Many duplications are incidentally diagnosed. However, most patients present with a combination of pain and obstructive symptoms. The symptoms may be the direct effects of the distention of the duplication or may be caused by the compression of the adjacent organs or blood supplies.5 Some rare presentations include  hematemesis, gastrointestinal bleeding, recurrent pancreatitis, and perforation with peritonitis.10,11 In a case report by Kayastha et al.1 a gastric duplication presented as acute abdomen.Obstructive presentations of gastric duplications are mostly epigastric postprandial pain and discomfort, nausea, vomiting, and abdominal mass.There is a paucity of information in the existing literature on the alimentary tract duplications initially presenting with icterus and elevated liver enzymes. What was also extremely rare as regards our patient was the fact that his cyst was located in the proximity of the gastric lesser curvature, creating a pressure effect on the portal vein and giving rise to jaundice.2 In the Holcomb3 study, management was based on the patient’s age and condition, the location of the lesion, whether it communicated with the intestinal lumen, and the number of anatomic locations to which it was extended. It is essential that sufficient attention be paid to vital structures such as bile ducts and vessels during the excision of the cyst.Patients with undetected duplications may present with acute bowel obstruction or severe GI hemorrhage (in cases of ulcerating gastric mucosa within a duplication cyst). In these incidental situations, duplications should be surgically approached to avoid further complications. Surgical treatment of the alimentary tract duplications depends on the specific anatomical location of the lesion and its relation to the adjacent organs and vessels.The position of the duplication cyst in our patient was rare insofar as it was located in the proximity of the gastric lesser curvature. This uncommon position of the cyst had created a pressure effect on the hepatic vein and caused the incomplete obliteration of the common bile duct and the hepatic artery. As a result, the patient had developed icterus and elevated liver enzymes, which are deemed rare signs and symptoms of the initial presentation of the alimentary tract duplications. Furthermore, our patient had elevated alkaline phosphatase, SGOT, and SGPT as the late complications of a long-standing untreated gastric duplication. The post-hepatic signs and symptoms can be regarded as the complications of a gastric duplication cyst.


Evaluation of palpable neck masses may be a diagnostic problem in pediatric patients, with differential diagnosis including congenital, inflammatory, tumoral and traumatic lesions. Ultrasonography is usually a satisfactory method to make a correct pre-operative evaluation of neck masses, although diagnosis is often challenging for the surgeon and the radiologist and sometimes only possible after a histopathological examination of the resected lesion.We report an 8-month-old patient with a cervical, anterior midline mass. Ultrasonographic images showed features suggesting a partly cystic lesion, with a preoperative suspect of thyroglossal duct cyst. Histological examination, performed after surgical removal of the mass, led to a diagnosis of lymph node angiomyomatous hamartoma (AH).AH, a rarely occurring benign lymph node lesion, has been reported in the neck lateral region only twice. This case, presenting as a palpable neck midline mass, is the first reported case occurring in infancy. Although rare, AH should be included in the differential diagnosis of head and neck masses. Palpable neck masses are a common clinical concern in pediatric patients, and differential diagnosis includes a wide range of pathologies, such as congenital, inflammatory, tumoral and traumatic lesions. Malformative neck masses in childhood are branchial cleft cysts, ectopic thymus, teratomas, cystic lymphangioma, aberrant thyroid tissue and others-6. ThyroUltrasonography (US) is an accurate, cost-effective, non-invasive preoperative analysis, usually sufficient to make correct preoperative diagnoses, even if the accuracy of this imaging method may have limitations, because of the variability of the sonographic appearances,13. CompThe authors herein describe an unusual case of AH presenting as a palpable mass in the anterior midline neck region of an infant. US preoperative evaluation of the lesion appeared compatible with a partly cystic mass, and, due to the peculiar location, a thyroglossal duct cyst was suspected or, secondly, a dermoid cyst, a small teratoma or an enlarged reactive lymph node.An 8-month-old Philippine female baby without other health problems presented with a palpable neck mass of 4 months’ duration. The patient did not suffer from dysphagia nor dysphonia. The past medical history was unremarkable. On physical examination, a midline anterior neck mass, tender and mobile when swallowing, contiguous to the hyoid bone was observed. No enlarged lymph nodes were detected in the lateral neck region. Laboratory values were in normal range. At US, a lesion largely hypoechoic measuring 20 x 14 mm in diameter, with internal heterogeneous echoes, with a well defined periphery (FigureOn gross examination, the excised mass measured 20 mm in largest diameter; a central fibrous areas was detected, without evidence of cyst. The tissue was fixed in 10% buffered formalin, routinely processed and paraffin embedded. Histological examination revealed a preserved lymph node structure at the periphery, with reactive follicles and dilated sinuses, whereas, in the region of the hilum, the nodal parenchyma was completely replaced by a fibrous tissue Figure. On immuNeck lesions more commonly encountered in pediatric population consist of congenital, inflammatory or neoplastic diseases,5. In adAH is a rare, benign form of lymph node hilar modification characterized by parenchymal replacement by fibrous tissue containing vessels and smooth-muscle proliferation. It prevalently involves inguinal lymph nodes, appearently starting in the hilum and extending towards the cortex. This entity was first described by Chan et al. in 1992, and, so far, 28 cases have been reported in the literature,14-23. TIn our infant patient, AH occurred in a midline neck lymph node. To our knowledge, only two cases, both in adults, have been documented in the lateral neck region. The ultrasound image of the lump yielded a suspected cystic lesion, for the variable tissue density and for the presence of an internal fibro-vascular area contrasting with the intact lymph node periphery and providing the hypothesis of thyroglossal duct cyst. The histological examination, performed after excision, allowed the correct diagnosis.The pathogenesis of this lesion is unclear. A possible explanation is that AH represents a vascular and smooth muscle proliferative response to chronic impairment of nodal lymphatic flow or to previous nodal inflammation,16.Notwithstanding the rarity, AH should be included in the differential diagnosis of neck masses. The treatment of choice for this benign disease is surgical excision. The Sistrunk procedure, performed in our case, is a safe and successful technique with low complications.Written informed consent was obtained from the parents for publication of this case report and any accompanying images.The authors declare that they have no competing interests.VDC and AC conceived the study and drafted the manuscript. VDC participated in surgery. LL has been involved in drafting and critical revision of the manuscript. CM did surgery and revised the manuscript. MN was involved in preparation of figures and manuscript revision. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2431/12/172/prepub