Intestinal obstruction secondary to intussusception, occurring simultaneously with complete rectal prolapse, is an unusual entity among young adults. When it occurs the intussusceptum may protrude per anus. Few cases are cited in literature; each with a unique clinical presentation. There is apparently no uniform trend in its clinical and pathological picture.A 38-year-old, African-Ugandan man presented with sudden occurrence of rectal prolapse for one day. He had otherwise been in good health. Symptoms were precipitous. A clinical diagnosis of intussusception of the lower gut with rectal prolapse, and intestinal obstruction, was made. The intussusception was found to have a polyp as the ‘lead point’. He was treated by manual reduction of the intussusception and the prolapse under general anesthesia. Histopathologic examination of the polyp showed it to be an adenoma. Definitive surgical treatment of the patient was not completed due to socioeconomic challenges.Rectal prolapse and intussusception are commonly childhood conditions. Rectal prolapse alone is commoner in the middle-aged and elderly; females in particular. The finding of this combined clinical entity in a young, adult male is therefore a unique condition with an unusual presentation. It is the first case of its kind reported in East Africa. It is also an example of an adenoma constituting a ‘lead point’ for an intussusception at the gastrointestinal tract’s terminus. Even in the presence of a pre-existing adenoma, a relatively common lesion, other differential diagnoses acting as ‘lead points’ ought to be considered in perspective. This characteristic, along with other features described in this case, is useful knowledge for colorectal surgeons, general surgeons, gastrointestinal pathologists, and gastroenterologists given their involvement in the diagnosis and management of anorectal disease of peculiar presentation. Intussusception is the ‘telescoping’ of a segment of the intestine into the lumen of another immediately adjacent segment. Among adults it is relatively rare, constituting less than 5 percent of intussusception cases, with a male to female ratio of 1.8 to 1  and consRectal prolapse is the ‘slipping’ or ‘falling out-of-place’ of the rectum. This may involve its mucosa  or the full thickness of all its layers (complete prolapse). It is also described as ‘revealed’ (overt) or ‘hidden’ (covert). The terminal 12 to 15 cm of gut constitutes the affected area. Typically the condition is commoner in females (80 to 90 percent) compared to males [Generally, intussusception is not usually associated with a demonstrable cause (idiopathic). This particularly is characteristic of childhood intussusception; the commoner type. In adults, there is a demonstrable cause (with a ‘lead point’) in the majority of cases, usually an intra-luminal neoplasm. A number of studies point to a 70 to 90 percent existence of an underlying gut pathological cause . These aOur case features an intussusception with a rectal adenoma and a concurrent complete rectal prolapse protruding per anus. It can also be described as an anal protrusion of an intussusception, alongside a rectal prolapse. This simultaneous occurrence culminated in acute intestinal obstruction. The entire presentation is rare and unique. The commonest lesions constituting a ‘lead point’ are colonic malignant tumors which occur in up to 60 percent of cases . Benign Our patient is a 38-year-old African-Ugandan man, of Bantu ethnicity. He presented to the Emergency Department of Mulago National Referral and Teaching Hospital with complaints of protrusion of a mass per anus, abdominal pain, low-back pain and vomiting for one day. These were associated with passage of loose, bloody and mucoid stool. Moments prior to the onset of these symptoms, he felt the ‘normal’ urge to empty his bowels. Upon bearing down he was unable to empty his bowels normally and found that he had to exert extra effort. He reported a sudden protrusion of a mass consisting of his ‘gut’ through his anus, accompanied by excruciating anal pain. Moments later the protruding mass began exuding a bloody and mucoid discharge. This was followed by severe low-back pain, generalised abdominal pain, vomiting and constipation. Because of these symptoms, he was first taken to the local district hospital from where he was referred to the National Referral Hospital without any intervention. The rest of his medical history was unremarkable.Prior to all these events he was reportedly in good health. He first had overt haematochezia three years ago, and had had three episodes over this period. On each of these occasions there was an accompanying loose, mucoid stool motion. There was also an unusually strong effort exerted to effect voiding of stool. During those periods he also had on and off low back pain, which increased in intensity with each subsequent episode. The pain was relieved with analgesics and bed rest. He partly attributed this pain to his occupation of subsistence fishing; a laborious undertaking. He had no history of protrusion of a mass per anus previously, even on heavy exertion. He has had tenesmus and a feeling of incomplete bowel emptying on a few occasions. However, he had neither a history of abdominal swelling nor one of significant weight loss.On physical examination, he was in pain, alert and maintained a knee-elbow posture. He was afebrile , moderately dehydrated, but was not pale (hemoglobin level was 12.7 gm/dL), and had no significant peripheral lymphadenopathy. His radial pulse was 98 beats/minute, regular and synchronous with other pulses, and his blood pressure was 110/70 mmHg. He had a moderately distended abdomen that moved with respiration. Further examination was done in the operating theatre with the patient under sedation. Blood grouping was also done. An intravenous line was instituted for fluid therapy and antibiotics were administered .Continued physical examination under sedation showed no visible peristalsis and no obvious masses palpable per abdomen. His liver, spleen, and kidneys were not palpable, and a tympanic percussion note was heard throughout the abdomen. On auscultation, his bowel sounds were increased in frequency and pitch. The rectal examination revealed a large, sausage-shaped mass protruding from the anal verge consisting of the anal canal, rectum and apparently, parts of the terminal sigmoid colon. It was 15–20 cm in length, with an anterior convexity. The lower antero-inferior aspect had a sessile, polypoid growth from the mucosa  was taken. Gentle but steady pressure was applied to the mass in a distal-to-proximal direction. Gradually, the mass was reduced completely over a period of about 5 minutes. At the end of the manoeuvre there was passage of flatus per anus and a reduction in abdominal distension. A digital rectal exam confirmed complete reduction of the prolapse and the intussusception, with the polyp palpable in the anterior aspect of the lower third of the rectum . Histopathologic examination of the biopsy revealed an adenoma.We have highlighted that adult intussusception causing intestinal obstruction and occurring simultaneously with complete rectal prolapse is uncommon. But that our patient presented with this combination of disease entities. For the intussusception component, his age of 38 years is particularly uncharacteristic, while his gender is less atypical ,4. IntusTypical intussusception, as seen in childhood, usually presents with a classic triad of symptoms and signs: cramp-like abdominal pain, a palpable sausage-shaped mass (mainly in the right upper quadrant) and currant jelly stools . Adults An explanation of his symptoms is derived from the anatomical abnormalities related to rectal prolapse, and the predisposing pathology associated with intussusception. Anatomical features associated with rectal prolapse include: an ‘intussusception’ per se; a deep rectovesical pouch; absent fixation of the rectum to the sacrum; a redundant rectum and sigmoid colon; weakness of the pelvic floor and/or anal sphincter muscles; and possibly, the presence of a rectocele . One or A history of haematochezia, tenesmus and a feeling of incomplete voiding explains the pathophysiology of an intussusception secondary to an underlying lesion (‘lead point’); in this case, an adenomatous polyp. This polyp caused on-and-off bleeding and a mass effect, presenting as tenesmus and a real or apparent feeling of incomplete rectal emptying upon voiding. A chronic clinical course ensued. The final protrusion of the polyp with the intussusceptum and rectal prolapse upon strongly bearing down happened to all occur in a precipitous fashion causing severe abdominal and low back pain, abdominal distension and complete constipation . This coA biopsy of the polyp was essential to determine its histopathologic characteristics. In this case it was a tubular adenoma Figure , contrarThe diagnosis in our case was largely clinical, and the ‘lead point’ was defined histopathologically. As the patient presented with an acute intestinal obstruction, no other special investigations like plain radiographs, ultrasound scans (USS) or computerized tomography (CT) were done as the nature of his symptoms and signs meant that he could only maintain a knee-elbow position and the immediate priority then was relief of the obstruction by reduction of the intussusception and prolapse. In cases of uncertainty, CT scans are the most efficient diagnostic investigation with an accuracy of 58 to 100 per cent, followed by USS .Management of this patient was aimed at relieving the intestinal obstruction, followed later by definitive treatment of the cause. To relieve the obstruction we first adequately resuscitated him, after which thorough examination and reduction of the mass was done under general anaesthesia . All gutIntussusception and complete rectal prolapse are well described and documented disease entities. While complete rectal prolapse is common among children and older adults, particularly females, it is uncommon among young, adult males. The dividing line between these two entities is clearly defined clinically, but may have simultaneous and overlapping presentation.Complete rectal prolapse is a top differential diagnosis of intussusception with prolapse per anus. It is of paramount importance for one to always assume the presence of an intussusception, even when it may mimic complete rectal prolapse alone, especially with a precipitous presentation as is evidenced in our patient. This is mainly because intussusception causes acute intestinal obstruction whose sequelae may be life threatening, especially gut strangulation with gangrene. This makes it a surgical emergency. On top of this, intussusception in adults is commonly related to other underlying pathology. There is usually a ‘lead point’; an adenoma as in our case. More commonly however the ‘lead point’ is an adenocarcinoma, whose presence paints an even grimmer picture. In contrast, complete rectal prolapse alone tends to have a gradual course of progression. It is largely benign and not associated with a bad outcome in the short run.Written, informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.PAO managed our patient peri-operatively and wrote the manuscript. RLL co-wrote the manuscript, and examined, analysed and described the histopathological slides. Both authors read and approved the final manuscript.


Steroid cell tumors of ovary account for less than 0.1% of all ovarian tumors and these tumours may present at any age in association with interesting presentations related to hormonal activities. The subtype, not otherwise specified (NOS), is associated with androgenic changes in 56-77% and Cushing syndrome in 6-10%. Due to the rarity of available data regarding these tumors, little is known about their malignant potential and metastatic behaviour. We hereby report an unusual metastasis of steroid cell ovarian neoplasm presented with both Cushing syndrome and hyperandrogenemia.A 31-year-old woman, who had a past medical history of ovarian tumor resection , presented with hirsutism, hypertension and menstrual disorder. Also, laboratory work-up revealed hypercortisolism and androgen excess. Computerized tomography (CT) of the abdomen showed abdominal paraaortic masses, multiple intrahepatic nodules and retroperitoneal lymph nodes enlargement. Positron emission tomography/computed tomography (PET/CT) scan demonstrated metastatic lesions. Her ovarian tumor sections were re-examined and pathology result was corrected to steroid cell tumor (NOS) associated with active cell growth and necrosis. Subsequent excision of metastatic lesions yielded clinical improvement promptly and metastasis of steroid cell tumor was confirmed by postoperative pathological studies. However, one year after the surgical management of metastasis, recurrence happened while radiotherapy was ineffective. The patient finally died of tumor metastatic recurrence.This case reports a rare coexistence of Cushing syndrome and hyperandrogenemia which occurs based on metastasis of steroid cell ovarian neoplasm. It presents a real diagnostic challenge to both clinicians and pathologists. Therefore, it is very important to establish a final diagnosis by pathological studies along with clinical manifestations and imaging findings. Besides, it is necessary to improve follow-up of patients with this kind of tumors. Steroid cell tumors (SCTs) of the ovary are a rare subgroup of sex cord-stromal tumors (SCSTs), representing less than 0.1% of all ovarian tumors . These tDue to the rarity of available data regarding SCTs, little is known regarding their malignant potential and metastatic behaviour. So far, very few cases have been reported on a late metastatic lesion producing steroid hormones without evidence of recurrence of the primary tumor. Here, we present a rare case of intra-abdominal metastasis of ovarian steroid cell tumor (NOS) which secreted both cortisol and androgen and caused Cushing syndrome and hyperandrogenemia 3 years after the initial tumor was removed.A 31 year-old-woman who had a past history of left oophorectomy was admitted to our hospital with marked hirsutism and menstrual disorder as well as significant hypertension.9/L) with neutrophilia (76.6%), hypokalemia (3.3 mmol/L) and elevated fasting glucose (6.4 mmol/L). Hormonal assays were not conducted. She received left oophorectomy and pathology result was luteinized thecoma of the ovary with focal coagulative necrosis and calcification. Since the patient desired future fertility strongly, she refused total abdominal hysterectomy with contralateral oophorectomy. Postoperatively, the patient’s symptoms of hirsutism and round face were resolved. Her menstrual period and blood pressure returned to normal. Two year later, she gave a natural childbirth and had no difficulty in breast-feeding.Three years and six months before the admission, the patient had irregular menses and excess hair growth on her face, neck, chest, abdomen and thighs. Her face became round. Her skin became thin and bruised easily. Then she turned to a local clinic where physical examination showed that she had a blood pressure of 160/120 mmHg. Meanwhile, gynecologic ultrasonography demonstrated a 25 × 20 × 15 cm left ovarian mass. Routine laboratory workup revealed the following: leukocytosis  beside abdominal aorta was detected by ultrasound. Lab tests showed her tumor markers  and 24-hrs urinary VMA were within normal limits. Antihypertensive drug Doxazosin 4 mg/d was given, but her hypertension was not well controlled. For further diagnosis and treatment, the patient was admitted to our hospital.During physical examination her blood pressure was 195/130 mmHg. She was 147 cm tall and weighed 54 kg (body mass index 25.0). She had hirsutism and her skin was thin with scattered ecchymosis. She had a round and plethoric face, central adiposity, buffalo hump and supraclavicular fat pads. A mass of 7 × 5 cm could be palpated in the left mid-abdomen. There were no purple striae and hyperpigmentation.9/L) with neutrophilia (74.7%), hypokalemia (3.11 mmol/L), impaired glucose tolerance  and alkalosis . Urine potassium level was within the normal range  but was relatively high given her hypokalemia. An endocrinological evaluation revealed elevated morning plasma cortisol , and overnight dexamethasone (1 mg) failed to suppress plasma level of cortisol. Urinary free cortisol was extremely high  and plasma ACTH was relatively low . In addition, her testosterone level rose to 98 ng/dL (ref. range 14–76) with normal LH, FSH, prolactin and estradiol. Pelvic CT scan showed no recurrent or residual mass in the left adnexa location and the right ovary and the uterus were of normal appearance . However, abdominal CT scan demonstrated two masses beside abdominal aorta, multiple intra-hepatic hypodense nodules, and retroperitoneal lymph nodes enlargement  such as hypercortisolism and androgen excess.The most common cause of Cushing syndrome is pituitary or adrenal adenoma. However, in the case of intra-abdominal masses, different etiologies are possible. On the one hand, the patient had some typical clinical features of Cushing syndrome except that she was overweight but thin, which might be caused by underlying devastating malignancy. Her lab tests revealed elevated plasma cortisol which overnight dexamethasone (1 mg) failed to suppress. Thus, Cushing syndrome could be diagnosed. On the other hand, her plasma ACTH was relatively low and the CT images indicated mild adrenal atrophy and intra-abdominal metastatic tumors. As a result, pituitary or adrenal adenoma as well as ectopic ACTH secretion could be ruled out. Based on the above facts and her history of ovarian tumor, paraneoplastic ectopic cortisol secretion was deemed as most likely despite of its rarity. But it was puzzling that ovarian thecoma could not secrete cortisol. Fortunately, her previous ovarian tumor sections were found and the pathological diagnosis of luteinized thecoma was overthrown and corrected to steroid cell tumor (NOS). Ultimately, the patient was diagnosed as malignant steroid cell tumor (NOS) with late intra-abdominal metastasis on the basis of postoperative pathology result. The diagnosis was proved correct by the fact that her symptoms of Cushing syndrome disappeared and her plasma and urinary levels of cortisol decreased significantly after the metastatic lesions were removed. Also in this case no hormone evaluation was performed at the first surgery, but clinical signs strongly suggested co-secretion of cortisol and androgen. Discrepancy between clinics and biology first, and the first result of pathology led to a second pathology reading. Attention should be paid to such a discrepancy by clinicians, in a way that a second reading should have been performed immediately after the first surgery, which should have led to an appropriate and strict follow-up.In addition, the patient also presented with hirsutism, which might result from hyperandrogenemia rather than hypercortisolemia. Routine screening for Cushing syndrome in women presented with hirsutism is a controversial issue. In a recent study, Karaca et al. screened 105 patients with the main complaint of hirsutism for Cushing syndrome irrespective of their suggestive findings of hypercortisolemia. None of the patients was diagnosed as Cushing syndrome by low-dose dexamethasone suppression test. Therefore, they concluded that routine screening for Cushing syndrome in hirsute patients is not required if the patient does not have accompanying clinical stigmata of hypercortisolism .Sex cord-stromal tumors are developed from the sex cord and stromal components of the gonad. Ovarian SCTs are grouped under SCSTs, and they are usually benign, unilateral, and formed by steroid cell proliferation. Between the three subtypes of SCTs, nearly 60% are steroid cell, NOS tumors. Microscopically, steroid cell tumors (NOS) are generally composed of large, round to polyhedral cells with vacuolated cytoplasm as well as smaller cells with eosinophilic granular cytoplasm, which are usually diffusely arranged in nests, clusters, cords or columns resembling adrenal zona glomerulosa and zona fasciculate. In this case, steroid cell tumor (NOS) was misinterpreted as luteinized thecoma, a typical thecoma throughout which clusters of large eosinophilic, lipid-laden lutein cells are scattered. Luteinized thecoma contains plump spindle cells and therefore is distinguishable from steroid cell tumor (NOS). In addition to these microscopic findings, steroid cell tumors would require immunohistochemical markers for accurate diagnosis. Inhibin and calretinin are the most useful markers for the discrimination of sex cord stromal tumors from other tumors . Most ofAlthough most steroid cell tumors (NOS) behave in a benign fashion, malignancy has been reported in as high as 43% of cases . AbsolutSurgical intervention is the most important and hallmark treatment, and complete excision of the tumor could contribute to the regression of symptoms and disappearance of Cushing syndrome. However, young woman are usually conservative towards this solution due to concern of losing fertility. If future fertility is not an issue, hysterectomy, removal of the contralateral ovary, and complete surgical staging are recommended. Age, large tumor size, lymph node involvement, and residual disease are all predictors of poor prognosis . For staThis case reports a rare coexistence of Cushing syndrome and hyperandrogenemia which can occur based on ovarian pathology in females. Metastasis of steroid cell ovarian neoplasm resulting in co-secretion of cortisol and androgen is extremely rare and represents a real diagnostic challenge for both clinicians and pathologists. A correct diagnosis usually involves a multidisciplinary effort and neglect of clinical features only results in misdiagnosis. Therefore, it is very important to establish a final diagnosis by pathological studies along with clinical manifestations and imaging findings. Besides, this patient developed steroid-secreting metastasis three years after the excision of primary tumor, and then one year after surgical management of metastasis, recurrence happened while radiotherapy was ineffective. These features of this case illustrate the necessity for this kind of tumor follow-up.Written informed consent was obtained from the patient’s husband for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.NOS: Not otherwise specified; SCT: Steroid cell tumor; SCST: Sex cord-stromal tumor; ACTH: Adrenocorticotrophic hormone.The authors declare that they have no competing interests.MY led the acquisition of data, review of literature, and drafted the manuscript. MQ gave the concept and design of research paper. MZ reviewed the manuscript critically. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1472-6823/14/12/prepub


The entity of pituitary (sellar or parasellar) lymphoma includes primary pituitary lymphoma (PPL) and secondary pituitary lymphoma (SPL). The latter has an involvement of systemic lymphoma. Both of these lymphomas are extremely rare. We describe a patient with SPL showing a good prognosis. A 78-year-old woman presented with diplopia, left ptosis, and back pain. Magnetic resonance (MR) imaging revealed a parasellar mass lesion extending to the upper clivus and another mass lesion with compression fracture of the Th3 vertebral body. Transsphenoidal exploration was performed, and it showed diffuse large B-cell lymphoma. Based on the positive tumor cells in the following bone marrow aspiration and hepatosplenomegaly in computed tomography (CT) findings, this patient was diagnosed as having a pituitary involvement of systemic lymphoma. After chemotherapy, she achieved complete remission for 4 years. The entity of pituitary lymphoma is extremely rare. Nineteen cases of PPL and 16 cases of SPL have been reported. Generally, clinical and radiological diagnosis was difficult because there are no specific findings. Therefore, biopsy was necessary in all of the cases. T2 hypointensity of a lesion in MR imaging in addition to an elevated serum level of soluble interleukin-2 receptor (sIL-2R) in a patient with a sellar lesion can be useful clues for the differential diagnosis of this rare disease. According to previous reports, the entity of pituitary (sellar or parasellar) lymphoma includes primary pituitary lymphoma (PPL) and secondary pituitary lymphoma (SPL). SPL includes the involvement of systemic lymphoma. Both of these lymphomas are extremely rare. Nineteen cases of PPL –17 and 1A 78-year-old woman with no remarkable medical history presented with diplopia, left ptosis, and back pain that has persisted for over one month. Her ocular motility improved spontaneously, but 3 months later, fever, polyuria, and polydipsia appeared. Her body temperature rose to between 38° and 39°C every night. Her urinary volume was 4-5 L/day. There was no weight loss or neurological abnormality. So she went to general hospital and performed brain and thoracic magnetic resonance (MR) imaging. These images showed double lesion at sellar region and Th3 vertebral body. Then, she was admitted to our hospital.Laboratory studies showed anemia and a slight elevation of the serum levels of inflammatory markers. Hemoglobin was 10.3 g/dL . C-reactive protein (CRP) was 4.09 g/mL , and the erythrocyte sedimentation rate (ESR) was 20 mm/hr . The lactate dehydrogenase (LDH) level was also high at 535 U/I . The patient's basal levels of anterior pituitary hormones were almost within normal range. Diabetes insipidus was suspected based on the clinical findings. The markers of infections, such as HIV, HBV, and HCV, were all negative. The level of serum interleukin-2 receptor (sIL-2R) was examined because of detected multiple lesions, and it increased to 7526 U/mL .MR imaging of the brain revealed an intra- and parasellar mass lesion extending to the upper clivus, sphenoid sinus, and right cavernous sinus. The intrasellar mass compressed the pituitary gland to the dorsal side. The lesion was isointense on T1-weighted images, iso- to hypointense on T2-weighted images, and inhomogeneously enhancing after contrast injection Figures . No otheWe performed an endoscopic endonasal biopsy. The histological examination revealed hyperplastic tumor cells with a high mitotic index. Immunohistochemically, CD20 (a B-cell marker) was positive, CD3 (a T-cell marker) was negative, and the MIB-1 index was 100%. These findings were consistent with a diffuse large B-cell lymphoma. The bone marrow aspiration revealed positive lymphoma cells. A lumbar puncture showed no tumor cells in the cerebrospinal fluid. Another CT scan of the thorax, abdomen, and pelvis was performed, confirming the presence of hepatosplenomegaly, which seemed to be the consequence of infiltration of the tumor cells. Based on the results of the bone marrow aspiration and CT findings, this patient was diagnosed as having SPL.Postoperatively, the patient underwent five courses of chemotherapy with the R-THP-CVP regimen . It took three weeks for one course of chemotherapy. After this chemotherapy, the follow-up MR imaging demonstrated a significant interval decrease in the size of the suprasellar and vertebral masses . Bone maTo our knowledge, only 19 cases of PPL and 16 cases of SPL have been reported. In these reports, several examinations including whole-body CT, gastrofiberscopy, bone marrow aspiration, and lumbar puncture were recommended to rule out another systemic lesion when a patient has PPL or SPL. This process is also important to differentiate between PPL and SPL. The clinical and radiological features of reported PPL and SPL are summarized in Tables Unfortunately, regardless of PPL or SPL, the differential diagnosis is usually difficult, although prompt diagnosis and the initiation of treatment are crucial to achieve better clinical prognoses in patients with malignant lymphomas. The characteristic findings in MR imaging of pituitary lymphomas are isointense on T1-weighted images and iso- to hypointense on T2-weighted images , 8, 31 aAside from radiological findings, it is well known that the levels of serum soluble interleukin-2 receptor (sIL-2R) are elevated in patients with malignant lymphoma. However, there is only one reported case of systemic pituitary lymphoma in which the sIL-2R level was measured and elevated . In our For the treatment of primary CNS lymphomas, chemotherapy with the combined use ofa cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) regimen, methotrexate (MTx), and/or rituximab has been commonly performed, but over the past decade, high-dose MTx therapy has become more common because of its penetrative tendency through the blood-brain barrier . As showIn conclusion, we have described a case of SPL which was successfully treated with chemotherapy. Transsphenoidal biopsy was required because the differential diagnosis was difficult based on only radiological and clinical findings. However, T2 hypointensity in MR imaging and an elevated sIL-2R level can be useful to diagnose this disease, although these findings are not always specific. Neurosurgeons should be aware of this rare disease in the differential diagnosis of sellar lesion, because prompt diagnosis and treatment are crucial to achieve better clinical outcomes.


Lymphangiomatous cysts are submucosal masses that are rarely found in the gastrointestinal tract and more often in the neck, oral cavity, and skin. These cysts are benign tumors and mostly clinically silent. Symptoms include abdominal pain, diarrhea, and rectal bleeding. Their pathogenesis remains unclear.During a routine ultrasound examination of a Caucasian 25-year-old woman, a structure that raised our suspicions of an ovarian cyst was found. MRI showed a 4.5 cm cystic lesion in the cecal region. Laparoscopic exploration revealed unexpected contact with the ascending colon. The cyst, including its base and of portion of the colon, was resected laparoscopically. The histological examination revealed cystic lymphangioma.Lymphangiomatous cysts of the colon are very rare lesions. Although their pathology is benign, the recommended treatment is resection, which can be performed with minimal invasiveness. Benign cystic lymphangiomas are rare intra-abdominal lesions that may be asymptomatic or may mimic a variety of abdominal symptoms, including an acute abdomen. The gastrointestinal tract, including the involvement of the colon, is an unusual location to find these cysts. Although of benign character, cystic lymphangiomas should be removed surgically to prevent further complications and malignancy. This case report concerns a very rare case of an unexpected cystic lymphangioma of the colon that was resected during minimally invasive surgery.We found a cystic structure in a Caucasian 25-year-old woman incidentally during an ultrasound examination. Our primary clinical suspicions were that it was an ovarian cyst. She had no history of abdominal disease.The lesion was localized in the right lower quadrant. In a control examination a few months later, the finding was described as an extraluminal cystic, septic structure underneath the cecum and in a lateral position in relation to the iliac vessels. For further differentiation, MRI was performed, which revealed a 4.5 cm cystic lesion in the cecal region Figure .The patient's blood chemistry was unremarkable. The indication was for surgical resection because of the size of the cyst, but resection carried the risk of potential complications. However, surgical resection was the wish of the patient. Laparoscopic exploration showed contact of the cyst to the ascending colon Figures  and 3. TThe histopathological examination of the cyst showed a 3.5 cm × 2.7 cm fluid-filled cystic jelly structure with serous content Figure . The masThe patient's recovery was fast and uneventful. Peristaltic activity appeared on day 1 post-operatively, and the patient was discharged within two days after surgery. At her follow-up examination one year after surgery, the patient was symptom-free without recurrence.et al. [The presence of lymphangiomas in the colon is rare. In 1931, Chisholm and Hillkowitz . Geboes There is no difference between men and women with regard to the incidence of these lesions. In Japan, the incidence of colonic lymphatic cysts appears to be higher than in American and European populations. Colonic lymphangiomas in particular, such as that reported in our case, have a peak incidence in the seventh decade of life .The etiology of lymphangiomas remains unclear. As most cases occur during childhood , generalLymphatic cysts show an equal distribution throughout the colon; however, other parts of the body are more often involved. The most frequent localizations are the neck (75%) and the axilla (20%). Within the abdomen, the most common site is the mesentery . Other pIn the largest recent review of patients with lymphatic cysts, a high percentage of the patients showed co-existent lesions. Colorectal carcinomas were found in 7% of the patients, colonic adenomas were found in 16% of the patients .The possible clinical manifestations include abdominal distension, abdominal pain, loss of appetite, nausea, and vomiting. Furthermore, melena and diarrhea are potential symptoms.The most frequent classification system used for lymphangiomas is still that of Wegner , who catet al. [Because of their mainly asymptomatic character, lymphatic cysts are usually found mostly by accident. Fewer cases are found by examination and management of their complications, which include torsion, rupture, intestinal occlusion, and obstruction of nearby organs. Some cases of cysts mimicking other diseases, such as ovarian cysts, pancreatitis, lipomas, adnexal torsion, volvulus, and congenital duplication of the bowel have also been reported. Prakash et al.  reportedNatural regression is very unlikely. Treatment options include endoscopic removal for intraluminal lesions smaller than 2 cm and surgical resection for larger lesions. This could be radical, segmental or wedge resection. The risk of developing diverticular formations caused by weakening of the bowel wall is higher in patients who undergo wedge resection. Surgical excision needs to be as complete as possible to reduce the risk of recurrence.et al. [The first case of laparoscopic excision of a cystic lymphangioma was reported in 1996 by Kenney et al. . This paet al. . Even whLymphangiomatous cysts of the colon are very rare lesions that have been reported with increased frequency in recent years as a result of the development and widespread use of colonoscopy. Although these cysts are benign, resection is essential to prevent complications and to definitively exclude malignancy.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.JH drafted and conceived of the manuscript. AK assisted in the drafting of the manuscript, edited the final version of the manuscript, and reviewed the literature. GS made the histopathological diagnosis. MVF made critical revisions to the manuscript. MZ performed the operation and was involved in the drafting and editing of the manuscript. All authors read and approved the final manuscript.


The aim of this study was to clarify the clinical features of patients with isolated HCC metastases to the heart. A 66-year-old female hospitalized with a hepatocellular carcinoma (HCC) ranging from the right to the left lobe and with a tumor thrombus in the main portal vein, was treated with intraarterial cisplatin, 5-fluouracil, adriamycin and mitomycin. Computed tomography (CT) one month later revealed that the HCC had progressed with multiple lung metastases and moderate ascites. The patient had no symptoms. Magnetic resonance imaging (MRI) and echocardiography revealed a round, movable tumor with a diameter of 2 cm in the right atrium (RA). The patient succumbed to HCC five months later. An autopsy revealed HCC with portal tumor thrombi and metastases to the lungs, inferior vena cava (IVC) and RA. The metastases in the RA and IVC were not continous with the intrahepatic tumor and were histologically attached to the endocardium and endothelium, respectively. An isolated metastasis of a HCC of the RA and IVC is extremely rare. In conclusion, although the majority of isolated metastases of HCC to the heart were diagnosed by echocardiography and were treated with mainly surgery, they had poor prognosis. The echocardiography should be performed for patients with advanced HCC. A novel treatment including molecular targeted drugs is required. Hepatocellular carcinomas (HCCs) frequently invade the vascular system at points such as the portal and hepatic veins. The results of autopsy studies indicate a 2.7–4.1% incidence of atrial metastases of HCC  were as follows: total bilirubin, 0.9 mg/dl (0.2–1.2 mg/dl); aspartate aminotransferase, 115 IU/l (5–40 IU/l); alanine aminotransferase, 105 IU/l (5–47 IU/l); alkaline phosphatase 491 IU/l (111–295 IU/l); and lactate dehydrogenase, 199 IU/l (100–225 IU/l). The patient was positive for the hepatitis B surface antigen and e-antibodies. The serum α-fetoprotein and des-γ-carboxy prothrombin levels were elevated to 687,460 ng/ml  and 1037 mAU/ml , respectively.Abdominal sonography and computed tomography (CT) imaging revealed a large mass reaching from the right to the left lobe and a tumor thrombus in the main portal vein . AngiogrAn enhanced CT in July, 2004, showed that the HCC had progressed and that multiple lung metastases had developed with moderate ascites. Magnetic resonance imaging (MRI) and echocardiography revealed a round, movable tumor with a diameter of 2 cm in the RA , but no An autopsy revealed diffuse-type HCC in the bilateral lobes of the liver that weighed 1,365 g, with a tumor thrombus in the main trunk of the portal vein, bilateral multiple lung metastases and tumor thrombi in the artery of the right upper lung. A yellowish irregular-surfaced mass with a diameter of 10 mm located in the RA, and a similar small independent mass in the IVC , were diThe mechanism of cardiac metastases is as a contiguous extension from the intrahepatic HCC via a tumor thrombus to the IVC or by lymphatic or hematologous spread. The majority of cardiac metastases are direct and contiguous extensions of the intrahepatic HCC. Isolated cardiac metastases that are discontinuous with an intrahepatic HCC are extremely rare. The literature was searched for descriptions of isolated cardiac metastases of HCC and 17 patients were identified  with a met al, RA and left ventricle (LV) in 10 (58.8%), 5 (29.4%) and 2 (11.8%) patients, respectively. A greater number of metastases had invaded the RV than the RA. These findings indicate that the main mechanism is embolism via arterial systemic spread as massive myocardial involvement was described in 4 patients.et al(Surgical  or non-set al reportedet al. However


A case of a 32-year-old female patient with a giant cell tumor originating in the middle part of the left 10th rib is presented. On X-rays and CT, the tumor caused a well-defined osteolysis with nonsclerotic borders. On MRI, it exhibited intermediate signal intensity on T1 sequences and central high signal and peripheral intermediate signal on T2 sequences. On contrast-enhanced MR images both central and peripheral-periosteal enhancement was noted. Thanks to its small size (2 × 1.3 cm), the lesion was easily resected en bloc with a part of the affected rib. The patient is free of recurrence for 3 years after the operation. Giant cell tumor (GCT) is a relatively common, benign, locally aggressive tumor that typically presents in the 3rd and 4th decades of life, more commonly affecting women –3. GCT uA 32-year-old female patient presented with recent onset of left lower chest pain. Her medical history was unremarkable. On the clinical examination, there was a localized tenderness at the middle part of the left tenth rib. She was initially referred for radiographs of the chest and left ribs. A well-circumscribed, ovoid lytic lesion was detected at the middle part of the left tenth rib, causing thinning of the cortex and expansion of the bone . ComputeGiant cell tumors are benign, though aggressive, neoplasms, accounting for 5 to 9 percent of all primary bony tumors . Giant cOn radiographs, GCT of the rib presents as a lytic lesion that may be eccentric and has well-defined, nonsclerotic margins. The lesion causes cortical thinning or even breakthrough and bone expansion . CT cleaSerum acid phosphatase has been found to be increased in many cases of GCTs. It was therefore suggested that serum acid phosphatase could be used as a “tumor marker” for GCT and for monitoring treatment response and disease recurrence. It was also demonstrated that the level of this enzyme is correlated with the size of GCT . In our The remarkable features in our case of GCT are (a) the unusual location of the lesion at the middle part of the rib and (b) the small size of the lesion (in contrast to the majority of the other reported cases), permitting an easy and complete resection with no deformity of the chest wall postoperatively. Despite the multimodality imaging approach, the findings in this case of GCT were atypical and could not establish a preoperative diagnosis. Although remote, the possibility of a giant cell tumor may be included in the differential diagnosis when a relatively small, well-defined lytic lesion of a rib is encountered, even when this lesion is not located at the posterior part of the rib.


Posterior epidural migration of thoracic disc herniation is extremely rare but may occur in the same manner as in the lumbar spine.A 53-year-old Japanese man experienced sudden onset of incomplete paraplegia after lifting a heavy object. Magnetic resonance imaging revealed a posterior epidural mass compressing the spinal cord at the T9-T10 level. The patient underwent emergency surgery consisting of laminectomy at T9-T10 with right medial facetectomy, removal of the mass lesion, and posterior instrumented fusion. Histological examination of the mass lesion yielded findings consistent with sequestered disc material. His symptoms resolved, and he was able to resume walking without a cane 4 weeks after surgery.Pre-operative diagnosis of posterior epidural migration of herniated thoracic disc based on magnetic resonance imaging alone may be overlooked, given the rarity of this pathology. However, this entity should be considered among the differential diagnoses for an enhancing posterior thoracic extradural mass. Posterior epidural migration of a herniated thoracic disc is extremely rare but may occur in the same manner as it does in the lumbar spine. To the best of our knowledge, only five cases of posteriorly migrated thoracic disc herniation have been reported in the English-language literature-5. We prA healthy 53-year-old Japanese man experienced sudden onset of incomplete paraplegia after lifting a heavy object. The physical examination revealed that his motor strength was grade 0/5 in the right lower extremity and 0–2/5 in the left lower extremity. Sensory loss was observed below the level of the umbilicus. Urinary incontinence was also present. Magnetic resonance imaging (MRI) revealed a posterior epidural mass compressing the spinal cord at the T9-T10 level  with posteriorly migrated thoracic disc herniation presented with sudden onset of severe motor weakness-5. GenerHerein we report the case of a patient with an extremely rare presentation of posteriorly migrated thoracic disc herniation. The patient experienced sudden onset of incomplete paraplegia, and surgical treatment relieved the symptoms. Pre-operative diagnosis of posterior epidural migration of a herniated thoracic disc based on MRI alone may be difficult, given the rarity of this pathology. However, this entity should be considered among the differential diagnoses for an enhancing posterior thoracic extradural mass.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.The operation was performed by NM. All authors were involved in the writing of the manuscript and the patient’s clinical care. All authors read and approved the final manuscript.


Coeliac disease is associated with an increased risk of malignancy, not only of intestinal lymphoma but also of small intestinal adenocarcinoma which is 82 times more common in patients with celiac disease than in the normal population. We report three additional cases of a small bowel adenocarcinoma in the setting of coeliac disease in order to underline the epidemiological features, clinicopathological findings, and therapeutic approaches of this entity based on a review of the literature. The three patients underwent a surgical treatment followed by adjuvant chemotherapy based on capecitabine/oxaliplatin regimen, and they have well recovered. Malignant tumors of the small bowel fall in the category of rare neoplasm comprising only 3% of all gastrointestinal malignancies. Primary adenocarcinoma is the most common histological subtype constituting 35–50% of cases , 2. IdenIn this paper, we describe our experience with three cases of SBA in the setting of CD with a brief review of the literature. A. B., a 43-year-old woman had consulted for a 3-month history of epigastric pain and 10 Kg weight loss. Her past medical history included CD diagnosed one year earlier with strict adherence to the gluten-free diet (GFD). Abdominal computed tomography (CT) revealedan irregular duodenal thickening localized to genu inferius portion with proximal stenosis . Upper eM. A., a 46-year-old man had consulted for abdominal pain and weight loss for 6 months duration. Physical examination revealed a 7 × 6 cm sized abdominal mass arising from the left iliac fossa. Abdominal CT demonstrated intestinal tumoral thickening of the left iliac fossa and mesenteric lymph nodes. Upper fibroscopy and colonoscopy were normal. At upper enteroscopy, we found endoscopic appearance suggestive of CD. Biopsies confirmed the diagnosis. The patient underwent laparotomy which revealed the presence of a locally advanced tumor of the ileum involving recto-sigmoid junction with lymphadenopathy without peritoneal implant or liver metastasis. Small bowel resection and sigmoid resection with terminoterminal anastomosis were performed. The diagnosis of poorly differentiated adenocarcinoma of the ileum was retained following immunohistochemical analysis of resected specimen; so the tumor was classified as pT4 N1 M0. Given the lymph node metastasis, adjuvant chemotherapy based on CAPOX regimen was received. Currently, the patient remains well on GFD.B. K., A 37-years-old man was admitted to the emergency room with acute intestinal obstruction. Questioning revealed two months history of vomiting, diarrhea, and abdominal pain. Abdominal CT showed stenosis of the last ileal loop with proximal dilatation. The patient underwent an emergent laparotomy which revealed an obstructive tumor of the ileum; a segmental resection with anastomosis and regional lymphadenectomy were performed. The histological diagnosis was a moderately differentiated adenocarcinoma of the ileum classified as pT2 N1 M0. Diagnosis of CD was made retrospectively 1 month later at upper endoscopy and was confirmed by biopsy and serological tests, and then GFD was started. Adjuvant chemotherapy based on CAPOX regimen was received. Currently, the patient responds to institution of GFD, and he remains well 56 months after surgery.Small bowel malignant tumors are uncommon malignant neoplasms accounting for only 3% of all gastrointestinal malignancies . HoweverSmall intestinal malignancies have been observed to be more common in people with a number of inflammatory bowel diseases and genetic syndrome. Crohn's disease, coeliac disease, Peutz-Jeghers syndrome, familial adenomatous polyposis, and HNPCC are known predisposing factors for SBA , 5. CoelAlthough both etiology and pathogenetic mechanisms of SBA in coeliac setting remain unclear, the following explanations have been suggested: a high turnover of the inflammatory population with mucosal lymphocyte infiltration, an increased permeability to oncogenic factors, a malabsorption of protective substances such as vitamins A and E, or an impaired immune surveillance ; additioUsually, SBA is most commonly located in the duodenum (55%), followed by the jejunum (30%) and the ileum (15%) . HoweverDevelopment of carcinoma is well recognized in association with long-standing gluten enteropathy; nevertheless, it can occur in patients with no history suggestive of a malabsorption syndrome, and the CD is diagnosed until the resection of SBA when the histological analysis of the specimen resection shows villous atrophy in adjacent nonneoplastic mucosa . In our Because of the inaccessibility of the small bowel to routine endoscopy, diagnosis of SBA is usually made at an advanced stage (74% stage III or IV) . SymptomNo consensus on treatment has been yet documented; the only available treatment of SBA is surgery with an overall rate of curative resection of 40–65% . BecauseDoes the gluten-free diet (GFD) protect against recurrence? There is evident data suggesting that GFD has a significant role in reducing risk of CD-related gastrointestinal malignancies, but the protective role of GFD against recurrence in patient previously treated for SB neoplasia is controversial, and there are three reports of CD patients who developed a second metachronous SBA 15, 9, and 2 years after a presentation with a first SBA despite a strict adherence to GFD , 13. TheThese cases confirm that CD is associated with a definite increase in the risk of developing SBA, which represents an entity with specific characteristics. No consensus on treatment is available. As the prognosis is not uniformly poor and some patients are potentially curable by total resection, as in our patients, there is evidence that an early diagnosis is crucial to improve the outcomes of this malignancy; but at present, there are no recommendations for screening in CD patients. In the light of this literature and as clinicians, we must pay attention to CD patients with vague symptoms and bear in mind that this could be related to malignant complication.


Gastrointestinal stromal tumors (GISTs) in adolescence are far less common than adult GISTs and have varied GIST genotypes that present diagnostic and therapeutic challenges. Here, we discuss a 21-year-old male with diagnosis of unresectable, imatinib-resistant GIST. At initial evaluation, a neoadjuvant treatment approach was recommended. As such, the patient received imatinib over the course of one year. Unfortunately, the GIST increased in size, and a subsequent attempt at surgical resection was aborted fearing infiltration of major vascular structures. The patient was then referred to our institution, at which time imatinib therapy was discontinued. Surgical intervention was again considered and the patient underwent successful resection of massive intra-abdominal GIST with total gastrectomy and Roux-en-Y esophagojejunostomy. Since pediatric GISTs are typically resistant to imatinib, we performed genotype analysis of the operative specimen that revealed KIT mutations associated with imatinib sensitivity and resistance. Given the sequencing data and operative findings, the patient was started postoperatively on sunitinib. This case illustrates the importance of understanding both adult and pediatric GISTs when implementing appropriate treatment regimens. Since the genotype of GISTs dictates phenotypic behavior, mutational analysis is an important component of care especially for adolescents whose disease may mirror the pediatric or adult population. Gastrointestinal stromal tumors (GISTs) represent the most common mesenchymal tumor of the gastrointestinal tract in the adult population, with a reported incidence rate of 6.8 cases per million . These tA 21-year-old African-American male was referred to our tertiary care cancer center with the diagnosis of unresectable, imatinib-resistant GIST. Institutional review board approval was obtained to review his clinical course. He was initially evaluated at another institution approximately one year earlier for a several-month history of gastroesophageal reflux disease, weight loss, and increasing abdominal distension. At the time of this initial presentation, the patient had these nonspecific abdominal complaints but was otherwise healthy. He underwent esophagogastroduodenoscopy (EGD), which demonstrated a large ulcerated tumor near the gastroesophageal junction. Biopsy of the mass revealed a spindle cell lesion and CD117  immunohistochemistry (IHC) staining confirmed the diagnosis of GIST. Computed tomography (CT) imaging demonstrated an 11 × 10 × 8 cm lesion arising from the stomach and no other lesions elsewhere. Due to uncertainty about upfront surgical resectability, the patient was started on imatinib (400 mg/day), a tyrosine kinase inhibitor (TKI) that blocks the constitutively active receptor tyrosine kinase c-KIT, to induce adequate tumor response for potential surgical resection with negative margins –5.On follow-up CT scan nearly seven months later , there wOn our initial examination, the patient had a largely protuberant abdomen with a mass palpable from the costal margin to the pelvic brim. He was no longer able to eat and repeat EGD demonstrated a cystic/solid gastric mass arising from the level of the gastric cardia, leading to extensive extrinsic compression of the entire stomach. The celiac axis could not be visualized on endoscopic ultrasound. Pathologic review of the original biopsy specimen revealed a moderately cellular spindle cell lesion with minimal atypia and no mitosis. IHC revealed that the cells stained strongly positive for CD34 and CD117, again confirming the diagnosis of GIST. Based on our experience with GISTs, we theorized that this patient's increased extent of disease was producing mass effect and compression of surrounding organs rather than infiltrating the surrounding tissues; therefore, our surgical team recommended operative exploration. More than one year following his initial diagnosis, the patient underwent resection of a massive intra-abdominal GIST with total gastrectomy and Roux-en-Y esophagojejunostomy. On our preoperative imaging the GIST measured 30 × 21 × 13 cm , and theAt our institution, genetic testing of the c-KIT gene includes selective assays of exons 8, 9, 11, 13, 17, and 18. These exons are amplified by polymerase chain reaction (PCR) and then undergo Sanger sequencing. Any mutations found are then confirmed via a second independent amplification. Genotype analysis of KIT in the operative specimen revealed an exon 11 deletion in codons 557–559, which is known to confer imatinib sensitivity . An exonGISTs frequently harbor gain-of-function mutations in the KIT or PDGFRA protooncogenes, which are detected in 90% of adult GIST patients , 13. WhiGISTs in the pediatric population are rare with a reported incidence of 0.5–2.7% and a median age at presentation of 13 years . Similarde novo prior to initiation of imatinib therapy, and that resistance may be secondary to the subsequent development of the exon 17 mutation, a phenomenon that has been described in adult patients [de novo, a condition that would have altered the management strategy leading to sunitinib as the initial neoadjuvant agent. Furthermore, knowledge of the mutations beforehand may have provided the rationale to change to sunitinib or plan for surgery earlier in the disease course, when the patient initially failed to demonstrate treatment response to imatinib.Surgical resection with clear microscopic margins is the gold standard treatment for both pediatric and adult GISTs. In our patient, imatinib therapy was used preoperatively in an attempt to decrease disease burden and minimize the extent of surgical intervention , 22. Ultpatients , 7. Howe Upon recovering from surgery, our patient was started on sunitinib. Unlike imatinib, sunitinib has been discovered to be effective in the pediatric GIST population . The gen


Burkitt's lymphoma is a rare non-Hodgkin's lymphoma which can occasionally involve the ovary and may cause confusion for the clinician since its presentation might mimic other much more frequent tumors. We present a case of a 23-year-old woman with sporadic Burkitt's lymphoma presented as advanced ovarian cancer with bilateral ovarian masses, peritoneal carcinomatosis, ascites, and marked elevation of CA-125. Liver involvement and atypical bone lesions, such as the cranial vault and the iliac wing, were also detected without evidence of lymphadenopathy. We describe the MRI and CT findings of simultaneous ovarian and bone lesions, which have never been reported in literature in a patient with Burkitt's lymphoma, before and after one cycle of chemotherapy. In evaluating any ovarian neoplasm in a young woman, Burkitt's lymphoma should be considered as a possibility, particularly if associated with bone lesions. MRI is the most useful tool to characterize the ovarian lesions and suggest the diagnosis before the histopathological results. The updated WHO Classification of Lymphoid Neoplasms 2008) identifies Burkitt's lymphoma (BL) as a highly aggressive and fast growing mature B-cell neoplasm that often presents in extranodal sites or as an acute leukemia. Although the incidence of BL is low, accounting for only 1~2% of all lymphomas in western countries, it is one of the most common types of malignant tumors in children  and immunocompromised hosts [008 ident3/mcL, neutrophils 8.7 × 103/mcL, monocytes 1.6 × 103/mcL, and lymphocytes 2.9 × 103/mcL. Laboratory tests gave the following values: LDH 1774 U/L, ALT 239 U/L, AST 189 U/L, gamma GT 108 U/L, and uric acid 12.7 mg/dL. A pelvic MRI examination with a 1.5 T unit was performed to further characterize the lesions. Multiplanar  conventional T1- and T2-weighted and DWI images were acquired. The exam showed two well-encapsulated masses with predominantly solid appearance, the maximum total size of about 18 cm along the latero-lateral diameter and 7 cm along the anteroposterior diameter, localized in the pouch of Douglas and in the left hypogastrium . Blood count revealed WBC 13.38 × 10gastrium . The masAt this point we performed a full-body CT scan for the staging of the tumor. The exam showed the intraperitoneal pelvic masses seen on MRI and involving both ovaries . The lesA bone scintigraphy showed all the bone lesions seen at MRI and CT as some hyperfixation areas of the osteotropic tracer (Tc99m-MDP).Liver biopsy examination made histological diagnosis of diffuse non-Hodgkin lymphoma with morphological and immunohistochemical features of Burkitt's lymphoma (sporadic). Neoplastic cells were positive for CD20, CD19, CD10, Bcl-6, and MUM-1 and negative for TdT, CD30, CD3, and cytokeratin (clone MNF 116), and the proliferative index assessed by Ki-67 was 100%. A bone marrow aspiration was performed and showed the presence of a hypercellular marrow with 99% of blasts ranging from medium to large size characterized by a basophilic and markedly vacuolated cytoplasm. A lumbar puncture was performed, and it was “negative for malignant cells.”The patient was treated with chemotherapy according to the protocol of AIEOP LNH-97 R4 for B-cell non-Hodgkin's lymphoma and B-ALL (stage IV with LDH ≥ 1000 U/L and negative CNS) and presented considerable clinical improvement after one week of treatment. A follow-up CT scan at 4 weeks showed a remarkable reduction of the adnexal masses and total regression of the neoplastic tissue which infiltrated the liver parenchyma and of the peritoneal carcinosis . HoweverLymphomatous processes in female genital tract are rare although the ovaries are the most common site to be involved . InvolveDiagnosis of intra-abdominal Burkitt's lymphoma is often delayed, as symptoms could be nonspecific or even absent. Abdominal pain, distension, nausea, vomiting, amenorrhea, irregular menses, and osteoarticular aches are the most common findings . After tAt CT ovarian lymphoma presents as hypodense lesions with mild contrast enhancement, without significant necrosis, hemorrhage, or calcifications and with relative structural homogeneity , 7. Although CT scan remains the most important examination for staging and followup after chemotherapy , MRI proFerrozzi et al.  describeMitsumori et al.  and CrawOther neoplasms should be differentiated from NHL involving the ovary such as granulocytic sarcoma, dysgerminoma, adult granulosa cell tumor, and fibrothecoma. Granulocytic sarcoma presents intermediate signal intensity on T1-weighted images and hypointensity on T2-weighted images and is less likely to be bilateral . In our


We report a case of tumor growth over a period of four decades, presenting with large multicentric lytic lesions of the skull and a profound mass effect, without neurological deficits. Clinical and radiological features of a patient with a giant intradiploic epidermoid and its impact on the choice of treatments are discussed.An 81-year-old Caucasian man, who had first noticed a painless subcutaneous swelling over the left frontal scalp about 40 years ago, presented after a short episode of dizziness, which he experienced after treatment of focal retinal detachment. Computed tomography (CT) and magnetic resonance imaging (MRI) examinations revealed an exceptionally large tumor involving major parts of the skull with extensive destruction of the bone and distinct deformation of the brain. Considering his age and the absence of neurological deficits or pain, the patient refused the option of tumor removal and cranioplasty, yet agreed to a biopsy, which confirmed the suspected diagnosis.The course of the disease demonstrates that even patients with large tumors, inducing distinct pathomorphological changes, do not necessarily experience significant impairment of their quality of life without surgery. This is an impressive example of the chance to lead a long and satisfying life without specific medical treatment, avoiding the inherent risks of these procedures. Yet, there is a clear indication for surgery of intradiploic epidermoids in most cases described in the literature. We report the case of a patient with a progressive swelling of the cranial vault and growing lesions of the skull who refused any further diagnostic procedures over a period of 40 years, although it was urgently recommended by his physicians. When he finally agreed to undergo a magnetic resonance imaging (MRI) investigation, large polycystic calvarial defects and extensive deformation of major parts of the brain were detected.The patient had an exceptionally large intradiploic epidermoid cyst with extensive destruction of large areas of the skull and distinct deformations of the brain without neurological deficits.Cranial epidermoid cysts are rare lesions, representing between 0.2% and 1% of all intracranial tumors. Intradiploic epdermoid cysts account for about 25% of these lesions . Most ofAn 81-year-old Caucasian man presented with a large painless swelling of the cranial vault, covering almost the whole left side and also major parts of the right side of the skull.He had first noticed a small swelling over the left frontal region about 40 years ago. During the following four decades the swelling slowly expanded. For more than 15 years his family physician had recommended an MRI for further examination. The patient refused it over a long time period because he felt no pain or other discomfort. He experienced no significant problems during his customary life activities.He finally agreed to further diagnostic procedures after a short period of dizziness following an ambulatory treatment of a focal retinal detachment. After this episode no further clinical symptoms appeared. Neurological examination was entirely normal. No cognitive deficits were found.Examination of the lesion revealed a subcutaneous mass covering most of the left cranial vault and major parts of the right side. Palpation revealed a soft elastic mass, adherent to the surface, without fluctuation.In contrast to the giant size of the cyst, on first sight no severe deformation of the head had developed. The overlying skin did not show pathologic changes, such as defects or inflammatory signs.A spiral computed tomography (CT) (Philips Brilliance 64 CT-scanner) with surface volume rendering technique revealed a large swelling of the scalp, especially over the left hemisphere Figure . ComputeThe MRI scan T2 weighted images (Philips Achieva 1.5 T) revealed an inhomogeneous, mainly hyperintense mass without penetration of the dura. Significant compression of brain hemispheres and ventricles without cerebral edema suggested a slow growing tumor. In diffusion weighted images there was a restriction of diffusion with low signal in the ADC maps Figure . The conIn summary, radiological features were suggestive of the presence of an extradural intradiploic epidermoid cyst.After thorough discussion of the diagnostic findings with the patient, he favored a biopsy to assure the histological classification of the tumor but refused the option of a complete removal of the tumor and subsequent cranioplasty. A biopsy was taken in the left frontal region. Histological examination revealed laminated keratin material, cholesterol crystals, and cellular debris. Pathological findings were consistent with the diagnosis of an epidermoid cyst. Due to a superficial wound infection surgical revision was necessary. His postoperative course was uneventful.Cushing first described an intradiploic epidermoid cyst in 1922 . A totalClinically intradiploic epidermoid cysts commonly present as slowly growing, painless lumps in any part of the scalp. Differential diagnosis of these tumors presenting with lytic bone defects includes dermoid cyst, hemangioma, eosinophilic granuloma, fibrous dysplasia, especially with widening of diploic spaces, and several others .In most cases a tentative diagnosis can be made based on radiological features and history of clinical symptoms but definitive diagnosis requires tissue histomorphology .Extradural epidermoids account for about 25% of cranial epidermoids and are located on the scalp, cranial vault or skull base . The cauTo the best of our knowledge no intradiploic epidermoid of this large size with extensive bony defects and massive deformation of major parts of the brain has been described in the literature. The time interval between first detection of a subcutaneous swelling by the patient and diagnosis, covering a period of about four decades, also is uncommon, yet corresponds to one other case report, in which a slowly progressive, painless proptosis of an eye has been described .The patient experienced no restrictions of his daily life activities or social contacts. According to the patient the subcutaneous swelling only rarely has been addressed. The patient never felt handicapped by the lesion. Although the tumor did not penetrate the dura, CT and MRI examinations revealed a profound mass effect with an extensive deformation of the brain. In spite of additional large skull defects a jolting of the head or rapid movements did not cause headache or other symptoms. The patient also experienced no problems laying down his head on the left or right side for sleep.Taking into consideration the age of the patient, his obviously good quality of life, and the risks of a total removal of this giant tumor with subsequent cranioplasty, in our opinion there was no indication for surgery in this case, even if the patient would have agreed to it.The origin of these tumors is still debated, yet disontogenetic etiology is widely accepted, assuming that the lesion derives from inclusions of ectodermal remnants during closure of the neural tube . In a feAs evident in our case even giant extradural epidermoids with profound deformation of the brain and extensive lytic skull lesions may allow a normal life without any significant restrictions. The course of the disease, covering a period of more than forty years, also demonstrates that an individual risk-benefit calculation is mandatory in the discussion of a specific treatment, which probably also applies to other diseases. There is no evidence that removal of the tumor within the described time interval would have achieved a positive impact on the life of the patient in spite of the distinct anatomical alterations. Notwithstanding, in most cases surgery is clearly indicated due to clinical or pathomorphological features.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interestsWK examined the patient, interpreted the findings and performed the surgery, and was a major contributor in writing the manuscript. AH examined the patient, interpreted the findings, designed and reviewed the manuscript. HH designed and reviewed the manuscript. JM designed and reviewed the manuscript. DF analyzed and interpreted the radiologic examination findings, and was a major contributor in writing the manuscript. All authors read and approved the final manuscript.


Introduction. Malignant transformation in a mature cystic teratoma of the ovary is a rare complication. Squamous cell carcinoma is the most common transformation. We describe a new case of squamous cell carcinoma arising in a mature cystic teratoma. Case Report. A premenopausal 52-year-old female patient is diagnosed with vaginal bleeding. According to examination made on the women and the pelvic scanning, 7 cm mass is found on the right adnexa of the patient. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, pelvic lymph node dissection, and debulking were the treatments completed on the patient. According to histopathological diagnosis, squamous cell carcinoma arising in a mature cystic teratoma is diagnosed as a reason for the mass in the right adnexa of the patient. Conclusion. The prognosis of the malign transformation of MCT depends on surgery stage; however it is extremely poor. The patient should receive chemotherapy regardless of stage. We have decided to administer second cycle carboplatin and paclitaxel treatments on the patient. Mature cystic teratoma (MCT) is the most common germ cell tumor of the ovary. MCT composes 10–20% of all ovarian tumors. MCT diagnosed as bilateral is roughly 9–16%. MCT develops in a woman's fifth to sixth decade of life –6. The pA perimenopausal 52-year-old woman, gravida 3, para3, was admitted by our clinic in April 2012 with a one-month history of vaginal bleeding and progressive abdominal discomfort. She dose not have known medical history, medication, drinking alcohol problem, and family history of cancer. The patient's gynecologic examination and transvaginal ultrasound showed a mass in the right adnexa. Moreover, pelvic magnetic resonance imaging showed mass of about 7 cm in diameter in the right adnexal with coexisting cystic and solid densities. In the Doppler screening, there was a vascularization in solid component, and the value of the Resistance Index (RI) at Doppler scan was 0.71. Serum levels of cancer antigen are CA-125: 57 U/mL (<35). Colonoscopy was normal. Abdominal tomography and pelvic magnetic resonance imaging were negative for any metastasis. The patient underwent total abdominal hysterectomy with bilateral oophorectomy, omentectomy, pelvic lymph node dissection and debulking. Grossly an opened uniloculated cystic mass was received measuring 12 × 7.5 cm in size with 0.2 cm wall thickness. The cyst wall contained a mural nodule measuring 7 × 7 × 2 cm in size. Intraoperative pathology consultation revealed a malignant epithelial tumor consistent with squamous cell carcinoma arising in a mature cystic teratoma. According to the intraoperative pathology consultation, total abdominal hysterectomy, and bilateral salpingo-oophorectomy, omentectomy and pelvic lymph node dissection was made. In permanent sections the cyst wall showed features of a mature cystic teratoma that was composed of ectodermal elements. Sections from the mural nodule showed moderately differentiated squamous cell carcinoma arising from the surface epithelium. A final diagnosis of malignant transformation of moderately differentiated squamous cell carcinoma in a mature cystic teratoma with extensive necrosis was made , also called dermoid cysts, are the most common benign germ cell tumors of the ovary. Malignant transformation of MCT occurring within average frequency of 1%-2% is consistent with previous reports. Moreover, SCC consistent with previous reports and cases is the most diagnosed histologic type –9. TumorAbdominal pain followed by abdominal or pelvic mass is the most common symptoms. Incidentally, it could be observed with an asymptomatic adnexal mass detected on routine pelvic examination or pelvic imaging studies. In some other cases, various symptoms due to invasion of nearby organs are the presenting complaints, such as gastrointestinal symptoms of constipation or diarrhea, rectal bleeding, or urinary frequency . In our Chiang et al. stated that higher concentration of serum CA-125 levels were associated with survival rates . Prior rSome imaging characteristics can be convenient determining malignant transformation. Clinical use of magnetic resonance imaging and computed tomography in preoperative diagnosis remains unclear. Most studies concluded that the Doppler detection method was a more useful indicator than serum SCC antigen levels , 13. As The prognosis of this tumor is often reported to be very poor in the several previous studies. The prognosis heavily depends on the stage of the disease. Other factors effecting diagnosis are tumor grade, tumor dissemination, cyst-wall invasion, rupture, ascites, adhesion, growth pattern, and vascular invasion , 7.The treatment modality choice to an ovarian MCT with malignant transformation is optimal debulking . Chen etMalignant transformation of MCT occurring within average frequency of 1%-2% is consistent with previous reports. The most common malign transformation is SCC which is around 70–80% of malign transformation cases. The prognosis of the malign transformation of MCT depends on surgery stage; however it is extremely poor. The patients should receive chemotherapy regardless of stage.


Lymphoceles are usually related to trauma or surgery, and its spontaneous development is rare. The aim of this paper is to report an uncommon case of idiopathic lymphocele located on the right infraclavicular region, in a female patient with no previous local trauma or surgery and unremarkable medical history. The diagnose was suggested by the tomographic aspect of the lesion and confirmed by ultrasound-guided fluid aspiration and cytological analysis. The authors also provide a brief review of the most important thing diagnostic features and treatment options for this condition. For the practicing clinician, the most important is to achieve an accurate diagnose and to choose the proper therapeutic method according to each clinical scenario. A lymphocele, also referred to as chylous pseudocysts, cystic lymphangiomas, and lymphatic duct hygromas, is defined as a circumscribed collection of protein-rich lymphatic fluid without an epithelial lining that develops in anatomic compartments as the result of trauma or interruption to the lymphatic system –4. Lymphocele formation is a described complication of surgery in and around the inguinal and femoral vessels , cardiot Currently, no diagnostic algorithm is in place for the evaluation of a suspected lymphocele, but numerous radiographic modalities when used in combination with clinical history and presentation will aid in the diagnose . If imag There is scarce information about the natural history of spontaneous lymphoceles, but it is established that those related to trauma/surgical procedures, when small, are usually asymptomatic and can reabsorb spontaneously , 8; on t The aim of this paper is to report a case of idiopathic infraclavicular lymphocele and review the most important diagnosis features and treatment options for this condition. Female, 54 years old, without previous noticed diseases, went to the dermatologist complaining about a “node” at the infraclavicular right region. The patient noted the lesions about 2 months before, denied systemic symptoms but referred antiaesthetic complains and local discomfort, caused by a “mass” effect. She also denied previous traumas or surgical procedures nearby the mentioned topography. A soft tissue ultrasound revealed a cystic lesion attached to the subclavian vessels; the patient was referred to the vascular surgery team. A more detailed physical exam revealed a visible, well limited mass, painless, firmly fixed at the right infraclavicular region, without fremitus nor murmur, presenting a discreet pulsation (giving the impression of being transmitted from the subclavian artery). A computerized tomography showed a cystic lesion, under the pectoral muscles, measuring approximately 8 × 3.8 cm, without enhance after intravascular contrast material was administered, on intimate contact to the subclavian vessels trough it's upper border Figures , 2 and 3 A fine-needle aspiration, guided by ultrasound was performed. On the application of suction, a clear yellow fluid was obtained. About 20 cc was removed. In spite of the fact that ultrasound examination pointed a remaining volume of approximately 5 cc, because of the proximity to the subclavian vessels and pleura  and the Cytologic analysis of the fluid showed 100% of normal lymphocytes, confirming the diagnose of lymphocele. A post-procedure thorax X-ray was obtained and documented no pneumo/hemothorax.After the procedure the patient complained no more about the “mass” sensation and discomfort referred before the treatment.  After one month the patient remained without complaints and no mass was palpable at the infraclavicular right region. Because of the patient's lack of symptoms, she elected to forgo additional interventional procedures and be followed up clinically. The appearance of idiopathic lymphocele is uncommon and can be only speculated to be associated to lymphatic leakage due to a miss diagnosed minor trauma or repetitive effort injury; its diagnose, evolution, and management are, for this reason, thought to be similar to those that occur after traumatic injuries or surgical procedures. Because lymphatic fluid is protein rich and devoid of platelets or clotting factors, a transected lymphatic channel cannot clot and is therefore prone to leakage . A lymph Lymphoceles constitute a diagnostic challenge , especia Currently, no diagnostic algorithm is in place for the evaluation of a suspected lymphocele . The dia Findings on ultrasound include a thin-walled collection  with the The typical presentation on a chest radiograph includes a smooth focal mediastinal mass or mediastinal widening. These abnormalities typically prompt further evaluation with a chest computerized tomography (CT) scan . CT scan features classically described for lymphoceles include a smooth, oval, or tubular mass with a thin wall that does not enhance after intravascular contrast material is administered, homogeneous attenuation, typically in the range of water and no infiltration of adjacent structures –3, 9, 12 Magnetic resonance imaging (MRI) features depend on the fluid chemical composition . Lymphoc Although some authors find MRI useful , others  In obscure cases, additional investigations may be found helpful, and lymphangiography and lymphoscintigraphy can be considered . Lymphos If the radiographic findings do not correlate or the patient is symptomatic, however, it may be necessary to perform needle aspiration of the fluid and cytologic analysis . Fluid i Management varies with size, symptoms, and anatomical location. Options include observation, needle aspiration, surgical resection, internal drainage via open or laparoscopic/thoracoscopic marsupialization, and percutaneous external drainage with or without the addition of sclerosing agents , 7, 13.  Simple aspiration, preferentially under CT or ultrasound guidance, has been used successfully to treat lymphoceles and relieve pressure symptoms , 3, 13.  Percutaneous catheter drainage has a mean duration of 14.5 days , and for Surgery has been considered the treatment of choice because of the rates of recurrence after percutaneous aspiration or drainage . Surgica Idiopathic lymphoceles are infrequently. If even diagnostic algorithm is not currently established, the same is applied for the therapeutic strategies. For the practicing clinician, the most important is the suspicion to achieve an accurate diagnose and to choose the proper therapeutic method according to each clinical scenario.


Background. Lymphangioma is a rare venolymphatic lesion, characterized by dilation of lymphatic vessels. It may occur as an isolated lesion or, more often, represent the surface component of a deep orbital lymphangioma. Case. We report a case of a conjunctival lymphangioma on a 58-year-old male that had simultaneously an upper respiratory tract infection. Excision and biopsy confirmed the nature of the lesion, and there has been no relapse to date. Conclusion. Conjunctival lymphangioma is a rare condition in which the diagnose, must be kept in mind in patients with a red eye resistance to topical therapy and in association with an upper respiratory tract infection. Finally, it is also necessary to be aware of possible recurrence of the lesion. Lymphangioma is an uncommon venolymphatic lesion, also defined as vascular hamartoma of lymphatic origin , 2. The Etiology of lymphangioma of the conjunctiva is not yet clear. It is usually an unilateral sporadic occurrence unless associated with the Turner syndrome or the Nonne-Milroy-Meige disease . Most frThe lymphangioma can be exacerbated by intralesion hemorrhage resulting in large “chocolate cysts” or by episodes of upper airway infections, resulting in lymphoid hyperplasia , 5.This paper describes a clinical case of a patient with an isolated conjunctival lymphangioma and its ophthalmological implications.A 55-year-old man presented a lush conjunctival hyperaemia and chemosis of the inferior bulbar conjunctiva in the left eye accompanied with dry eye symptoms, on March 2011. This lesion has been described for the first time three months earlier, and it had been the reason for five emergency hospital visits. In this period the patient has been treated with different drops and ointments, including topical steroids, anti-inflammatory, antibiotics, and eye lubricants, but none has proven any efficacy.On March 2011, examination of the left eye disclosed an unilateral swollen and hyperemic inferior bulbar conjunctiva extending to both canthi; this was a light-pink-coloured lesion, painless, compressible and with an underlying transparent content  . RootmanOrbital lymphangiomas often present with proptosis, ptosis, and impaired extraocular movements. Conjunctival lymphangiomas generally appear as a visible mass without affecting vision, or the globe , as occuLymphangiectasia can lead to irritation and redness from desiccation of the overlying conjunctival epithelium, epiphora, if the lacrimal puncta become functionally occluded by overhanging conjunctiva, blurred vision, and pain .As described by Duke-Elder , conjuncIn a review of 13 cases of orbital-adnexal lymphangiomas, Rootman et al.  classifiBiopsy with a histopathological report has an essential role in the diagnosis. Histopathologically, lymphangioma is a nonencapsulated, irregular mass, lined by endothelial cells, composed of numerous cyst-like channels that contain clear fluid, blood, or a combination of both. Separating the channels is loose connective tissue that contains aggregates of small lymphocytes . In our Treatment of superficial lymphangioma by surgical excision is often curative , 13, 14 Alternative treatments for conjunctival lymphangiectasia have been described. In a case report by Jordan and coworkers , a carboWe did not find any correlation between Alport's syndrome and conjunctival lymphangioma in the literature , 21.This paper presents a rare case of a lymphangioma isolated to the bulbar conjunctiva that was a differential diagnosis of red eye resistant to therapy. The upper respiratory infection may have exacerbated the signs of the conjunctival lesion. These lesions are a diagnostic challenge, and the biopsy has an essential role in the accurate diagnosis. Surgical excision is one of the treatment options, and it was effective in our patient. It ishowever necessary to be aware of possible recurrence of the lesion.


The incidence of ectopic pregnancy after hysterectomy is extremely rare with only 56 cases reported in the medical literature. Due to its rare occurrence, this diagnosis may not be initially considered when such a patient presents with abdominopelvic pain. It is an important diagnosis to keep in mind since a delay in diagnosis may lead to death. The case presented below describes this extremely unusual diagnosis of an ectopic pregnancy which occurred six years after a supracervical hysterectomy. Posthysterectomy ectopic pregnancy cases can be classified as early (preexisting pregnancy) or late occurring based on the presence or absence of an unrecognized pregnancy at the time of hysterectomy . Among tA 36-year-old Latin American woman, G3 P3, had initially presented to the emergency department with a two-week history of bilateral lower quadrant pain at an outside hospital from which she was referred to out institution after initial testing. The pain had started 2 weeks ago coinciding with mild vaginal bleeding. She denied any other symptomatology. She had undergone a cesarean section for fetal distress during her third pregnancy (G3) which was followed by cesarean hysterectomy due to uncontrollable bleeding. A supracervical hysterectomy was performed at that time which was approximately 6 years ago. Physical examination revealed normal vital signs. Her abdomen was soft and mildly tender to palpation. Recent laboratory studies from an outside lab showed a beta-HCG level of 10,587 mIU/mL . Initially, an ultrasound was obtained which was followed by an MRI examination for further characterization.On transabdominal ultrasound, uterus was absent. A 10 cm heterogeneous mass was present in the midline pelvis without any significant Doppler flow. Additionally, there was a 5 cm complex heterogeneous left adnexal mass without any Doppler flow. Transvaginal ultrasound also demonstrated the large heterogeneous mass measuring 10 cm × 6.5 cm × 8.4 cm in the central pelvis with no color flow on Doppler examination later confirmed with MRI Figures . It was MRI depicted postsurgical changes from supracervical hysterectomy with a nonenhancing T1 hyperintense and T2 hypointense mass in the midpelvis measuring approximately 11 cm × 10 cm compatible with blood products Figures . AdditioPatient was taken to the operating room for laparoscopic surgery. Hemoperitoneum was evacuated, and omental adhesions to the anterior abdominal wall were dissected free. Multiple adhesions were noted in the left pelvis, with enlarged left fallopian tube and ovary surrounded by clots. The right adnexa was not visualized and appeared to be surgically absent. The left adnexa was adhered to the vaginal cuff. The adhesions were dissected, the pedicle was excised, and the specimen of the tubo-ovarian complex was obtained and sent to pathology. Patient had an uncomplicated postoperative course. She was discharged from the hospital on postoperative day 1 with appropriate instructions. At discharge, her beta-HCG was noted to have decreased to 4401 mIU/mL from 10,587 mIU/mL. On pathologic examination of surgical specimens, midpelvic tissue sample was identified to be fat and organized blood. Left ovary showed chorionic villi and blood. The diagnosis was made of a tubo-ovarian complex with ectopic pregnancy within the left ovary.On post-operative follow-up visit one month later, a repeat measurement of Quantitative beta-HCG showed a normal level of <3.0 mIU/mL .Preexisting or early presenting ectopic pregnancy after hysterectomy can occur after virtually every kind of hysterectomy . Theorie There have been 25 reported cases of late-presentation ectopic pregnancies after hysterectomy occurring as late as 12 years after hysterectomy. It is believed that late-presenting posthysterectomy ectopic pregnancy occurs when sperm gains access to an ovulated ovum through a fistulous tract between the vagina and the peritoneal cavity. This tract can often be diagnosed by fistulography , 8 or MRIn subtotal hysterectomy, chances of a fistulous tract formation may be increased by leaving a remnant of cervix (as in our case) or the epithelialization of a much larger vaginal cuff closure area due to cervical dilation at the time of cesarean hysterectomy , 10. It In conclusion, it is imperative to maintain a high index of suspicion for pregnancy in a posthysterectomy patient presenting with acute abdominal pain if the ovaries are in situ. MRI can help not only in making the diagnosis of an ectopic after hysterectomy but also in diagnosing a vaginal cuff fistula, adhered adnexal structures to the vaginal cuff, and in diagnosing a prominent cervical remnant.


Objective. Histopathology of parotid gland tumors is extremely varied and complex due to heterogeneous cellular composition. Preoperative diagnostic tools include fine needle aspiration cytology, the role of which remains controversial. The aim of this paper is to evaluate the usefulness and accuracy of fine needle aspiration cytology (FNAC) in the diagnosis of parotid gland tumors. Methods. We retrospectively reviewed charts of 129 patients who underwent parotidectomy for parotid lesions at Aga Khan University Hospital from 2002 to 2010. We compared the results of preoperative FNAC with final histopathological diagnosis. Results. Concordance with histological results was observed in 86%, specificity was 98%, sensitivity was 84%, and diagnostic accuracy was 94%. Conclusion. Our results demonstrate that preoperative cytology in parotid lesions is fairly accurate and useful in diagnosing benign from malignant and in planning appropriate approach for treatment.  A lump in the salivary gland region often presents as a diagnostic challenge with regards to its site of origin, benign or malignant. Most of these occur in the parotid glands; small percentages occur in the submandibular, sublingual, and minor salivary glands. Parotid gland lesions are a histologically diverse group. Tumors of this region comprise 3% of all head and neck and 0.6% of all tumors of human body .The history of fine needle aspiration cytology (FNAC) goes back to the 1920s where it came into use simultaneously in Europe and the United states . FNAC isFNAC is an easily done procedure with minimal incidence of complication and no risk of implantation of tumor cells (<1%). The complications are rare and bleeding or inflammatory reaction in the region of the puncture. The impairment of the involved nerves has been reported as a very rare complication , 8. FNACBecause of the distinct morphology of parotid gland, the effectiveness of FNAC in its interpretation is still considered controversial. However, the use of FNAC helps to distinguish between reactive inflammatory process, which may not require surgery, and neoplastic lesions, between benign and malignant thus allowing proper planning before embarking any treatment. Surgical planning is dependent on clinical and radiological investigations; however, if the pathology is known preoperatively it is easy to counsel the patient and plan surgery. It is here where FNAC is of help specially for malignant lesions. In this article we describe our experience of using FNAC in the preoperative evaluation of parotid lesions.A retrospective review was carried out of medical records of patients that underwent parotidectomy for various pathologies between 2002 to 2010. A total of 205 parotid surgeries were performed during this period. Of these, only 129 patients were included in this study in which preoperative FNAC was done at our institution exclusively. Rest of the patients, in which we only performed surgery but FNAC was not carried out at our centre, were excluded.FNAC was performed at our pathology department using a 22-gauge needle attached to a 10 mL syringe holder by a free hand technique. A minimum of two needle passes were made in each case. The specimens were expelled onto two or three slides, and thin smears were prepared between two slides and immediately fixed. The slides were generally stained with Papanicolaou and occasionally with May-Grunwald Giemsa methods. None of the FNAC was carried out with ultrasonography.We classified our FNAC results into the following categories: true-negative , true-positive , false-negative , and false-positive . We also compared the histopathology of the surgical specimens with the preoperative cytology of the FNAC specimens and evaluated the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and overall accuracy of FNAC to differentiate between benign and malignant disease.There were 73 males and 56 females patients. The age range was 15 to 78 years with a mean age of 44 years. The final histological diagnoses of the included cases are listed in There were 98 benign lesions (89 neoplastic and 9 nonneoplastic) while 31 (24%) were malignant tumors . PleomorAn overall of eighty-five percent (110/129) concordance between FNAC and final histological diagnosis was established, 78% with malignant and 88% with benign cases on break up . The cytA statistical analysis of 129 cases was carried out to assess the diagnostic accuracy of parotid FNAC compared with that of histological results . A sensiLocal inflammation was observed in only two cases after performing FNAC; however, no complication such as hematoma, infection, or facial nerve damage was observed.The FNAC has been widely used as a diagnostic tool for the management of various head and neck lesions . Many auThe diagnostic sensitivity of cytopathology in detecting malignant disease was 84% in this study. This result implicates that if FNAC is used as a screening tool in this patient group, 12% of the malignant lesions would have been missed. These values fall within wide range of sensitivity reported in various studies, from as low as 27% up to 97% –21. The Specificity in our study was 98%. Specificity reported in the literature has been similarly high, in the range of 84% to 100% . The falIn the recent literature, the accuracy has ranges from 84% to 97% , 22. In Because of the varied, complex and overlapping morphological features, the diagnosis of pleomorphic, monomorphic adenoma, and adenoid cystic carcinoma can be sometimes problematic. We had one case of monomorphic adenoma which was reported pleomorphic on FNAC. Similarly a case of carcinosarcoma was labelled as Warthin's tumor by FNAC . The ratWe correctly typed pleomorphic adenoma in 73 of 77 95%) cases, better than the reported ranges from 82 to 94% in the literature 5% cases,. MucoepiAccording to the experience of Shaha et al.  the diagStill, to some authors, role of FNAC in the diagnosis of parotid lesions has not been well taken . The onlThe most important questions to be answered by this study is whether results gained from FNAC can be useful in the clinical management of patient with parotid lesions. Our experience has demonstrated a variety of circumstances in which such data may be valuable. The usual recommendation for the neoplastic lesions regardless of the preoperative cytological diagnosis is excision but recognition of benign lesion, beforehand like in case of Warthin's tumor, in poor risk patients may be of benefit in avoiding inappropriate surgery.Our study shows that preoperative FNAC plays an important role in the accurate diagnosis of parotid tumors. It is a safe and effective modality for the treatment of patients with parotid lesion. This office based procedure is reliable, well tolerated, easy to perform and cost effective. Moreover preoperative differentiation of tumors may help prepare both the surgeon and patient for an appropriate surgical procedure.