Primary pancreatic lymphomas (PPL) are rare tumours of the pancreas. Symptoms, imaging and tumour markers can mimic pancreatic adenocarcinoma, but they are much more amenable to treatment. Treatment for PPL remains controversial, particularly the role of surgical resection.Four cases of primary pancreatic lymphoma were identified at Prince of Wales Hospital, Sydney, Australia. A literature review of cases of PPL reported between 1985 and 2005 was conducted, and outcomes were contrasted.All four patients presented with upper abdominal symptoms associated with weight loss. One case was diagnosed without surgery. No patients underwent pancreatectomy. All patients were treated with chemotherapy and radiotherapy, and two of four patients received rituximab. One patient died at 32 months. Three patients are disease free at 15, 25 and 64 months, one after successful retreatment. Literature review identified a further 103 patients in 11 case series. Outcomes in our series and other series of chemotherapy and radiotherapy compared favourably to surgical series.Biopsy of all pancreatic masses is essential, to exclude potentially curable conditions such as PPL, and can be performed without laparotomy. Combined multimodality treatment, utilising chemotherapy and radiotherapy, without surgical resection is advocated but a cooperative prospective study would lead to further improvement in treatment outcomes. Primary pancreatic lymphoma (PPL) is rare, comprising less than 0.5% of pancreatic tumours. To distA retrospective analysis of patients with PPL at our institution was conducted, to examine our treatment outcomes with modern multimodality therapy. We present a case series of four patients – one of the few in the recent literature when newer therapies such as rituximab have become available – and contrast outcomes with previously published case series.An electronic search through the medical oncology department records of Prince of Wales Hospital  identified four cases of PPL among 481 cases of non-Hodgkin's lymphoma between 1990 and 2005. Medical records were reviewed, in regards to age, sex, presenting symptoms, radiological appearance, histological diagnosis and method, staging investigations, treatment regimen and outcome.Staging was assigned by a modification of the Ann Arbor classification ; accordiPreviously reported cases of PPL published between 1985 and 2005 were identified through a Medline search of the English literature using the keywords of "pancreas" and "lymphoma". Further case series were identified through citation review of identified articles. Single case reports, and cases of stage III/IV disease were excluded.Four cases were identified of this rare condition at our institution. All cases were male, and age ranged from 56 to 70. Summary data is presented in table A 64 year old man presented with a five week history of right upper quadrant pain, nausea, and jaundice, with five kilograms of weight loss. Liver function tests demonstrated biliary obstruction. Serum Ca-19.9 tumour marker assay was markedly increased at 500 kU/L . Abdominal CT demonstrated a 6 cm solid mass within the head of the pancreas, invading the portal vein, associated with biliary obstruction, but without lymphadenopathy or hepatic involvement  with concurrent rituximab  was administered with complete response. Chemotherapy was then ceased because of asymptomatic anthracycline-induced decline in ejection fraction. Involved field external beam radiotherapy (36 Gray in 20 fractions) was administered. Fifteen months following diagnosis the patient remains in complete remission.A 70 year old man described a several month history of intermittent vomiting, anorexia and weight loss, on a background of chronic renal failure due to polycystic kidney disease . MRI (magnetic resonance imaging) of the abdomen Figure  demonstrSystemic combination chemotherapy with dose-modified CHOP was administered, after explanation of risk of significant toxicity and treatment-related end-stage renal failure. Dose-limiting toxicity included neutropaenia, cardiotoxicity and sensory neuropathy, which required cessation of treatment after only three of a planned six cycles. Progress CT scan demonstrated over 50% reduction in size of the pancreatic mass. Involved field radiotherapy (45 Gray in 25 fractions) was well tolerated, inducing radiological complete remission.Thirty months after diagnosis end-stage renal failure occurred, requiring hemodialysis. Thirty-two months following diagnosis (23 months following therapy) the patient suffered symptomatic mediastinal and gastric recurrence. Second-line combination chemotherapy  was poorly tolerated, with febrile neutropaenia and grade three gastrointestinal bleeding. The patient had a limited response, and died a short time later.A 56 year old man presented with progressive dyspepsia and 12 kg of weight loss over 18 months. Ultrasound and CT revealed a five cm pancreatic mass involving the transverse colon; without lymphadenopathy, mesenteric vessel or hepatic involvement. At laparotomy for a planned Whipple's procedure, histopathology from open biopsy of the pancreatic mass and adjacent nodes was consistent with CD20-positive grade II  follicular lymphoma. PET scan and bone marrow staging revealed regional nodal involvement. Resection was not performed.Treatment consisted of involved field radiotherapy (36 Gy in 20 fractions), with sequential CVP chemotherapy for five cycles and sequential rituximab for four cycles. Restaging confirmed complete response, and the patient remains in complete remission 25 months following diagnosis.A 61 year old man experienced progressive epigastric and back pain, 12 kg weight loss and then jaundice. Liver function tests were consistent with biliary obstruction. Ca19.9 was within normal range. CT scan revealed a pancreatic mass and peripancreatic lymphadenaopthy, without mesenteric vessel or hepatic involvement. ERCP and insertion of biliary stent relieved obstruction, however biliary brushings and subsequent FNA were non-diagnostic. Laparotomy and open biopsy of the peripancreatic lymph nodes revealed CD20 positive diffuse large B-cell lymphoma, and resection was not performed.nd treatment), the patient remains in remission.Treatment consisted of three cycles of CHOP chemotherapy followed by involved field radiotherapy (42 Gy in 19 fractions). The patient had a complete response to treatment, however relapsed out of field 21 months following diagnosis (17 months after treatment), with complete response to second-line ICE chemotherapy, high-dose BEAM chemotherapy and autologous stem cell transplantation. After 64 months following diagnosis , followed by low grade B lymphomas (15%) and other B cell lymphomas (34%). T cell lymphomas, although very uncommon (4% of cases), carried a dismal prognosis. Our series was comparable to other series with 75% high-grade B cell lymphomas and 5% low grade lymphomas. Histological diagnosis was established by non-operative biopsy in 29 of 105 cases (28%), laparotomy in 69 of 105 cases (65%), and autopsy in 7 of 105 cases (7%). Surgical resection of the tumour was performed in 23 of 107 cases (21%). Chemotherapy was administered in 80 of 107 cases (75%). Radiotherapy was administered in 33 of 105 cases (31%).Comparisons were made between treatment groups. Without any definitive treatment, results were uniformly poor with no patients free of disease and poor long-tem survival. Patients treated with chemotherapy and/or radiotherapy – without resection – did not appear to have worse outcomes than resected patients. Formal statistical comparison was not able to be performed.In our series of non-surgical multimodality therapy, three of four patients were free of disease at time of follow up, with mean survival 34 months, and 2-year survival of 75%.Our case series illustrates that the clinical presentation of PPL can be difficult to differentiate from pancreatic adenocarcinoma without definitive pathological diagnosis. Reliance on symptoms, imaging and tumour markers – in the absence of definitive pathological diagnosis of suspected pancreatic adenocarcinoma – can potentially result in the misdiagnosis of a small minority of potentially curable patients. This is important as the prognosis and management of PPL differs greatly from that of adenocarcinoma.Appearances on CT can be helpful to differentiate the two conditions, but are not definitive,10. Ca19Non-operative evaluation and biopsy of pancreatic masses can avoid the need for invasive surgery, if conditions such as PPL are found. The majority of cases of PPL in our literature review required laparotomy for diagnosis, which may have been avoided with successful non-operative biopsy and modern combined modality treatment. Radiological-guided percutaneous FNA of the pancreas is a very useful technique, which requires experienced radiologists and cytopathologists to obtain a diagnosis on a small amount of tissue. EndoscoTreatment of PPL remains controversial – particularly the role of surgery and radiotherapy – and based on our findings we do not support routine pancreatectomy. All patients in our case series received multimodality treatment with both chemotherapy and radiotherapy, without surgical resection. Experience from our case series and literature review table  indicateIt is pertinent to consider the diminishing role of surgery in other localised extra-nodal lymphoma. Historically, the treatment of localised gastric non-Hodgkin's lymphoma, excluding MALT (mucosa-associated lymphoid tissue)-type lymphomas, was based on surgery – to ensure adequate diagnosis and staging, and maximise survival rates . Three rRadiotherapy has had only limited reported use in the treatment of PPL . We are not aware of its use in other series of PPL, however its use has been reported in gastric lymphoma , and eviBiliary sepsis is a potential problem of multimodality therapy in the setting of PPL, due to both the frequent presence of biliary stents, and the risk of neutropaenia. A study of endobiliary stents in multimodality therapy of pancreatic carcinoma reported low rates of complications, with 15 of 101 cases complicated by occlusion or migration, and no uncontrolled biliary sepsis or stent-related death. AnotherWe have examined for any possible association between polycystic kidney disease and PPL, given the existence of both conditions in case two. Although polycystic kidney disease has been associated with pancreatic cystadenoma and cystadenocarcinoma in a small number of case reports -44, we aThe study of PPL is limited by the rarity of the condition, and the consequent lack of randomised trials or large case series. The comparison of our case series with other series must be interpreted cautiously, given variable follow-up (range two – 108 months), incomplete data in some case series,3,12,45,PPL is a rare but potentially curable pancreatic tumour, and mandates pathological diagnosis of all pancreatic masses, as its treatment and prognosis differs from adenocarcinoma. Non-operative diagnosis may avoid the need for surgery, as outcomes with chemotherapy and radiotherapy – without surgical resection – compare favourably to surgical series. Nevertheless a sizeable cohort of patients are not cured with modern therapy, and we advocate a prospective study to further improve treatment outcomes.The author(s) declare that they have no competing interests.PSG collected the data for the case series and the literature review, and drafted the manuscript. MC also collected data for the case series. MH also drafted the manuscript. DG conceived the study, participated in its design and coordination, and helped to draft the manuscript. All authors read and approved the final manuscript.The pre-publication history for this paper can be accessed here:


Aneurysmal bone cysts are relatively uncommon in the facial skeleton. These usually affect the mandible but origin from the coronoid process is even rarer. To the best of our knowledge, this is the first reported case of a coronoid process aneurysmal bone cyst presenting as temporal fossa swelling.A 17 year old boy presented with a progressively increasing swelling in the left temporal region developed over the previous 8 months. An expansile lytic cystic lesion originating from the coronoid process of the left mandible and extending into the infratemporal and temporal fossa regions was found on CT scan. It was removed by a superior approach to the infratemporal fossa.Aneurysmal bone cyst of the coronoid process can attain enormous dimensions until the temporal region is also involved. A superior approach to the infratemporal fossa is a reasonable approach for such cases, providing wide exposure and access to all parts of the lesion and ensuring better control and complete excision. Aneurysmal bone cyst (ABC) usually affects long bones of the body and its involvement of facial bones is relatively rare. Mandible is usual site of involvement in facial skeleton but usually it originates from the region of body and ramus (in the region of molars) ,2. At feThere are few reports found in the literature describing involvement of coronoid process by ABC -8. MainlWe report a case of ABC involving only coronoid process with infratemporal and temporal fossa extension. It presented to us as temporal fossa mass.It adds one more aspect in diversity of clinical and biological behavior of ABC in maxillofacial region.A 17 years male patient presented to us with the complaint of swelling in the left side temple since 8 months. There was mild pain on pressing the swelling and on opening of mouth. Swelling was progressively increasing in size. There was no other complaint.On examination, there was about 3 × 3 cm bony swelling in the left temporal fossa with facial asymmetry due to lateral bowing of left zygoma. It was immobile, slightly tender with egg shell crackling on pressure. There was no murmur or bruit on auscultation. There was no restriction to mouth opening. Lower part of the swelling could be palpated on digital palpation through mouth along the superior part of anterior border of ramus of left mandible.On aspiration, brownish serous fluid came out. It contained mainly red blood cells.CT scan of head and face Figure  revealedThis cystic mass was removed by superior approach to infratemporal fossa. A "Question mark" shaped incision was given within the hair line, starting 5 cm posterosuperior to lateral end of left supraorbital ridge, curving superiorly and posteriorly and coming down vertically to preauricular area. We went directly up to temporalis fascia and incised it. Then we dissected in the plane deep to it. There was a large cyst with papery thin brownish walls filled with brownish serous fluid Figure . Arch ofHistopathological examination Figure  shows nuABC is a pseudocyst lacking true epithelial lining and usually affecting long bones (50%) and vertebrae 20%) . In faci0% . In faneurysmal cyst which was changed to aneurysmal bone cyst in 1950 [It is being recognized since 1893, but in 1942 Jaffe and Lichtenstein coined the term Aneurysmal bone cyst is most common in those regions of the skeleton where there is both a relatively high venous pressure and high marrow content . This exIt can occur as a primary lesion or secondarily in a preexisting lesion. Martinez V and Sissons HA concluded that most of these cysts occur as a primary lesion . Giant cIn our case, there was no history or evidence of any other associated lesion. So we label it a case of primary ABC of coronoid process of mandible.There are various treatment options suggested in the literature ranging from percutaneous sclerotherapy, diagnostic and therapeutic embolization, curettage, block resection and reconstruction, radiotherapy and systemic calcitonin therapy. Self healing cases have also been reported on long term follow up .Dubois et al  reportedEmbolization of the feeding vessels has been shown to be effective as a preoperative procedure to reduce peroperative bleeding or after surgical failure. It is also employed as a sole treatment modality. But ABC frequently lacks large feeding vessels therefore may require repeated sittings for embolization .Radiation was not considered for this patient looking at his age and surgically accessible tumor.We chose superior approach to infratemporal fossa as it had some distinct advantages in this case. We could approach temporal, infratemporal regions as well as origin of the ABC with single incision. There were minimum chances of injury to facial nerve. With this incision, we could approach infratemporal fossa and coronoid process from laterally also in case of need.There was no excessive bleeding from the cyst in our case.- We presented first reported case of primary ABC from coronoid process of the mandible presenting as temporal swelling.- Superior approach to infratemporal fossa is a reasonable approach while managing such cases providing wide exposure and access to almost all parts of the lesion with flexibility of surgical approach in case of need with the same incision.- We conclude that even after relatively smaller area of bone involvement and without surrounding destruction; ABC of coronoid process can attain enormous dimensions to present as swelling of a distant region which is more important in relatively compact maxillofacial region with regards to symptoms and management.The author(s) declare that they have no competing interests.All five authors1) Have made substantial contributions in management of this case and in conception, design, analysis and interpretation of results of this case report2) Have been involved in drafting the article or revising it critically for important intellectual content.3) Have given final approval to the version to be publishedThe pre-publication history for this paper can be accessed here:


Primary testicular angiosarcomas are extremely rare, and their clinicopathologic features are not well described. Our objective was to further define the clinical features and pathologic spectra of primary testicular angiosarcomas.Six previously reported case reports were identified in the English language medical literature using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews was performed. After excluding 2 cases because they did not involve the testis, we identified 4 previously reported cases of true primary testicular angiosarcoma. We also searched the electronic medical archival records of our institution and identified one additional unreported case of true primary testicular angiosarcomas. Data were extracted on the demographics, predisposing factors, clinical presentation, gross pathology, microscopic pathology, immunophenotype, therapy, and outcomes of each of these 5 cases of true primary testicular angiosarcomas.Primary testicular angiosarcomas were found at a mean age of 43.4 years. None of the cases was associated with exposure to radiation, arsenic, thorium dioxide, or vinyl chloride. However, 1 case was associated with hydrocele. It typically presented with painless mass . Histologically, all showed classic anastomosing channels lined by plump hyperchromatic cells, though most showed epithelioid cytology and some showed solid architectural pattern. One patient had multiple metastatic recurrences but eventual outcome was not available, and 1 patient died a month after diagnosis from stroke but no autopsy was performed. The remaining 3 patients were alive at the time of publication of their respective cases .Primary testicular angiosarcomas are typically rare tumors of men of all ages that appear to segregate into 2 groups; one associated with teratoma and occurring in young people, and the other occurring in the elderly and not associated with germ cell neoplasm, but may be associated with chronic hydrocele. They present with advanced disease and show a wide histologic spectrum. However, their prognosis may be better than previously thought. Angiosarcomas generally occur in the skin and rarely in the deep soft tissue and viscera. Primary angiosarcoma of the testis is an extremely rare tumor. The first bona fide case of true primary testicular angiosarcoma was described in 1991 by Hughes et al . The bioSix prior case reports of angiosarcoma of the testis were found in the English language medical literature in a MEDLINE search. Of these, 2 were excluded from the analysis because they were retroperitoneal  and paraThe histologic diagnosis was based on the morphologic spectra defined by Meis-Kindblom and Kindblom . The arcClinical characteristics of all the reported primary testicular angiosarcomas are summarized in Table Gross, microscopic, and immunohistochemical features of all the reported primary testicular angiosarcomas are summarized in Table The size of the tumors in the 5 cases of primary testicular angiosarcomas ranged from 1.7 to 10 cm in greatest dimension . On gross examination, the vast majority of the testicular angiosarcomas were solid vascular lesions Figure  [80%; 4/All the 5 cases of primary testicular angiosarcomas showed the classic architectural pattern of proliferating anastomosing blood-filled channels Figure . At leasImmunohistochemical staining was performed in all 5 cases, but the panel used varied from case to case. Factor VIII-related antigen was positive in all the cases in which it was used . The other immunohistochemical markers that were positive in all the cases in which they were used were CD34 Figure  100%; 3 100%; 4 100%; 3 . The biologic behavior and prognosis of true primary testicular angiosarcoma may be much better than previously thought. None of the 5 patients died with metastatic disease, though follow-up was incomplete for 2 cases. The remaining 3 patients with complete follow-up were alive and well at the time of their respective publications . Hence, although definitive conclusions cannot be drawn from such a small set of patients without long term 5-year follow-up, the ultimate biologic behavior of true primary testicular angiosarcomas may not be as abysmal as previously thought on comparison with visceral and deep soft tissue angiosarcomas .Visceral angiosarcomas are conventionally regarded as deep soft tissue angiosarcomas because they typically present as hemorrhagic masses . The vasOur further definition of the histopathologic spectrum of testicular angiosarcoma is important from a diagnostic standpoint, especially when faced with small biopsy specimens. Epithelioid cytology and solid architectural patterns are apparently not an uncommon finding in testicular angiosarcomas and may be mistaken in some cases for a poorly differentiated carcinoma, as illustrated by the case report of Sahoo et al  where thThe known angiosarcoma risk factors of antecedent radiation therapy, and prior exposure to arsenic, thorium dioxide, or vinyl chloride -10 were Three of the 5 cases of primary testicular angiosarcoma were associated with the concurrent presence of multilocular cystic areas diagnosed as mature teratoma in the same testicular tumor,3,4. ThePrimary testicular angiosarcoma is an exceedingly rare entity for which the clinical and pathological parameters have yet to be adequately defined. They were not associated with antecedent radiotherapy or prior exposure to arsenic, thorium dioxide, or vinyl chloride. They appear to segregate into 2 groups; one associated with teratoma and occurring in young people, and the other occurring in the elderly and not associated with germ cell neoplasm, but may be associated with chronic hydrocele. These tumors present as sizeable painless hemorrhagic masses typically in the testicular parenchyma and epididymis. They share most of the histopathologic features of other deep soft tissue angiosarcomas, and hence may pose considerable diagnostic difficulty, particularly those with a solid pattern and epithelioid cytologic features. Immunohistochemical studies may be helpful, and more than one endothelial marker and at least one cytokeratin marker should be used to confirm the diagnosis of testicular angiosarcoma. Because of the limited follow-up, the behavior of this entity is uncertain, though the prognosis for these patients may be better than the previously thought generally poor prognosis for deep soft tissue and visceral angiosarcomas.The author(s) declare that they have no competing interests.HBA participated in the histopathological evaluation, performed the literature review, acquired photomicrographs and drafted the manuscript. UNMR and AVP conceived and designed the study, gave the final histopathological diagnosis and revised the manuscript for important intellectual content. All the authors read and approved the final manuscript.


Post-radiation sarcoma (PRS) is an uncommon disease manifesting as sarcoma in a previously irradiated field, usually with a latent period of 5 years or more. Literature is limited to small series. Optimal management of this disease is unclear. Positive margins are common following attempted curative surgery and outcomes are poor. Radiotherapy is hardly used and its effect on PRS is not known. We described a case of PRS treated with preoperative radiotherapy followed by margin-negative wide excision.The 59-year-old patient presented with a mass in the left supraclavicular fossa and numbness in the arm, six years following radical irradiation of the head and neck for nasopharyngeal carcinoma. Open biopsy showed pleomorphic spindle cell sarcoma. She was treated with pre-operative hyperfractionated radiotherapy followed by margin-negative wide excision and nerve grafting. Cumulative radiation dose to the supraclavicular fossa was 98 Gy. Histological examination of the post-irradiation tumor specimens showed evidence of significant tumor response to re-irradiation. The patient remained free of disease five years after surgery with excellent functional outcome.Role of radiotherapy in PRS is uncertain. We described a case that was successfully managed with preoperative radiotherapy and margin-negative wide excision in terms of tumor control and functional outcomes. The impact of radiotherapy was demonstrated in the post-irradiation resected specimen. Further investigation using re-irradiation and surgery in PRS is warranted. Post-radiation sarcoma (PRS) is an uncommon disease. It occurs in approximately 0.2% of patients treated with radiotherapy that survive at least five years , with a Standard treatment for PRS is radical resection. There is a paucity of chemotherapeutic agents that have shown efficacy in the management of PRS . The rolen bloc resection and nerve grafting, leaving the patient with an excellent functional outcome and free of disease five years following surgery.In this report, we described the case of a patient with PRS involving the supraclavicular fossa and brachial plexus following successful treatment of nasopharyngeal carcinoma with radiotherapy five years earlier. Preoperative accelerated hyperfractionated radiotherapy was used followed by The patient, a 59-year-old Chinese woman, was treated at our centre in 1994 for a T3N0M0 nasopharyngeal carcinoma. She was treated with external beam radiotherapy to 60 Gy in 30 fractions over 6 weeks in accordance to ICRU-50. Two lateral parallel opposed fields were used to treat the nasopharynx and upper neck. The lower neck was irradiated with a direct anterior field with a central cord shield and a lower border just below clavicular heads. The junction of the upper and lower field was at the level of hyoid bone. It was followed by a 10 Gy Iridium-192 brachytherapy boost to the nasopharynx. Treatment was completed in May 1994. She remained well with no further recurrence of the nasopharyngeal carcinoma but developed xerostomia and mild fibrosis of the lower neck.She re-presented in December 1999 with a mass in the left supraclavicular fossa and numbness in the arm, but no motor weakness. The mass was located within the area previously irradiated to 60 Gy. Her disease was staged with our standard protocol at the time with MRI of the neck and shoulder, CT of the chest and functional imaging using thallium-201 scintigraphy . MRI shoen bloc resection. She received an accelerated hyperfractionated course of radiotherapy 48 Gy in 40 fractions (ICRU-50), treating twice a day over four weeks. The radiation field incorporated the brachial plexus and used 6 MV photons in an anterior-weighted, opposed oblique arrangement avoiding the spinal cord. Radiotherapy was completed in February 2000. Restaging investigations showed that the lesion had become more cystic on MRI, but its size had not changed. The degree of thallium-201 scintigraphy uptake had not changed significantly either. The patient proceeded on to surgery four weeks later.The management of her case was discussed in our multi-disciplinary meeting. The agreed treatment plan was preoperative radiotherapy followed by en bloc resection of the tumor along with the left sternomastoid and superior and middle trunks of the brachial plexus. Clavicular osteotomy was necessary to allow dissection of the tumor from the subclavian vessels. Sural nerve grafts were used to restore continuity of nerve supply from C5 and C6 to the suprascapular, musculocutaneous and axillary nerves. A latissimus dorsi myocutaneous flap was raised to close the defect in the neck. The middle third of the clavicle that had been reflected downwards was replaced and secured with a plate and screws.Surgery in March 2000 consisted of Histological examination of the surgical specimen showed evidence of radiotherapy, with large areas of necrosis and hemorrhage seen both macroscopically and microscopically. Comparison was made between the operative specimen and the pre-irradiation biopsy; necrosis was not seen in the pre-irradiation specimen Figures  and 3. SImmediate postoperative recovery was uncomplicated. The patient retained good function in her hand but was left with a flail shoulder and no power in elbow flexion. She thus underwent cable grafting of the musculocutaneous nerve in October 2000, with gradual return of elbow function following this procedure.The patient presented again in December 2001 with pain in the shoulder. X-ray confirmed that there had been non-union of the osteopaenic clavicle, the plate having detached from the lateral end of the clavicle. She returned to theatre and had a bone graft and internal fixation Figure . FollowiThe patient remains well five years after combined therapy for the PRS, with no evidence of recurrence of either her nasopharyngeal carcinoma or the PRS. The patient had no lymphoedema, neurovascular, severe fibrosis or other significant late effect as a result of re-irradiation.This case demonstrates that PRS can be effectively managed with preoperative radiotherapy and surgery, providing excellent long-term control without necessarily sacrificing function. The intent of radiotherapy was to sterilize the microscopic disease at the periphery of the PRS in order to maximize the chance of local control, as the extent of the surgical resection was limited by the anatomy of the supraclavicular fossa.Complete resection rates in PRS are relatively low; approximately 50% of patients undergoing attempted curative resection are left with either macroscopic or microscopic residual disease . In this5/5 tolerance dose of the brachial plexus, which is approximately 62 Gy [The brachial plexus was one of the dose-limiting structures at risk of late effects following re-irradiation. The dose of 48 Gy was delivered in fraction sizes of 1.2 Gy: biologically equivalent to 38 Gy in 2 Gy fractions (using an α:β ratio of 2) and thus a total dose to the brachial plexus of 98 Gy – considerably higher than the TDly 62 Gy . HoweverHistological examination of the surgical specimen showed that there were large areas of necrosis and hemorrhage in the specimen, which were not evident on review of the pre-irradiation open biopsy. Although the response of PRS to radiation has not been widely studied, it is traditionally thought to be relatively insensitive to radiation. This histological finding suggests that the radiotherapy had a significant tumoricidal effect and may have enhanced the surgeon's ability to achieve clear surgical margins despite the difficult location of the tumor. It may also have sterilized subclinical areas of disease adjacent to the tumor, contributing to her long-term disease control.There was non-union of the osteopaenic clavicle following her initial surgery, which culminated in separation of the plate from the lateral end of the clavicle and bearing of the arm's weight by the rotator cuff muscles, which then resulted in pain and spasm. Pathologic fracture and fracture non-union is well described in the post-radiotherapy setting  and is tRole of radiotherapy in PRS is uncertain. We described a case that was successfully managed with preoperative radiotherapy and margin-negative wide excision in terms of tumor control and functional outcomes. The impact of radiotherapy was demonstrated in the post-irradiation resected specimen. Further investigation using re-irradiation and surgery in PRS is warranted.The author(s) declare that they have no competing interests.AT reviewed the literature and prepared the manuscript. SN and PC assisted in manuscript revision and review. All authors read and approved the final manuscript.


Prostatic carcinoma is the second most common cause of cancer-related deaths in males in the West. Approximately 20% of patients present with metastatic disease. We describe the case of a patient with metastatic prostate cancer to the bowel presenting clinically and radiologically as a primary caecal cancer.®) for prostatic cancer diagnosed 6 years previously and was known to have bony metastases. On admission his PSA was 245.4 nmol/ml. The patient underwent a right hemicolectomy. Histology showed a poorly differentiated adenocarcinoma which was PSA positive, confirming metastatic prostatic adenocarcinoma to the caecum. The patient underwent adjuvant chemotherapy and is free from recurrence a year later.A 72 year-old man presented with abdominal discomfort and a clinically palpable caecal mass and a firm nodule on his thigh, the latter behaving clinically and radiologically as a lipoma. Computed tomographic (CT) scan showed a luminally protuberant caecal mass with regional nodal involvement. The patient was being treated (ZoladexMetastasis of prostatic carcinoma to the bowel is a very rare occurrence and presents a challenging diagnosis. The diagnosis is supported by immunohistochemistry for PSA. The treatment for metastatic prostate cancer is mainly palliative. Prostatic carcinoma is the second most common cause of cancer related deaths in males in the West . ApproxiA 72-year-old man presented with abdominal discomfort with small amount of bleeding per rectum and a clinically palpable lump in the right iliac fossa. He also had a large, firm, mobile lump on his left thigh.® injections.He had been treated six years previously for prostatic carcinoma which was Gleason's grade 3+3= 6. Bony metastases had been diagnosed recently. He was on Zoladexth thoracic and 1st lumbar vertebrae and rib cage. In 2003, there was a gradual rise in his PSA levels which had gone upto 233 nmol/ml. Zoladex® injections were then started and his PSA levels fell to 6.3 nmol/ml by January 2004. This however had gradually risen again to 254.4 nmol/ml on admission, inspite of being on Zoladex®.Clinical examination showed a 6 cm hard, mobile, non-tender mass in his right iliac fossa. Digital rectal examination was unremarkable. Rigid sigmoidoscopy revealed radiation proctitis, which would explain his bleeding per rectum. CT scan of abdomen showed a caecal tumour with regional lymphadenopathy and vertebral metastasis Figure . A non-pThe patient underwent a right hemicolectomy for what appeared to be an obvious locally advanced luminally protuberant caecal carcinoma with mesenteric lymph nodal involvement. Histopathological examination of the specimen showed a poorly differentiated adenocarcinoma of the caecum extending throughout the whole bowel wall and breaching the serosa with metastatic involvement of 5 of 15 paracolic lymph nodes Figure . The posProgression of prostate cancer occurs either by direct extension or metastasis through haematogenous or lymphatic routes. The most commonly involved organs are the seminal vesicles, base of the bladder, bones  and lymph nodes. WidesprIt is important to distinguish primary from metastatic colorectal lesions, especially in the presence of a previous history of cancer at another site, in order to facilitate appropriate management. This is best achieved by defining the tumour type on histopathologic grounds and, in this instance, by immunohistochemical staining for PSA,30. The The response of prostatic carcinoma to oestrogen therapy has been well established but patiMetastasis of prostatic carcinoma to the bowel is a very rare occurrence and presents a challenging diagnosis. The mainstay of diagnosis is histopathology supported by immunohistochemistry for PSA.Palliative treatment remains the mainstay of therapy for metastatic prostate cancer and hormonal therapy represents the standard with debulking surgery where feasible.The author(s) declare that they have no competing interests.MAK – research, acquisition of data, acquisition of consent, writing, drafting and revision of manuscriptELD – lead colorectal surgeon involved in this patients management and critical review of manuscriptGM – consultant in charge of imaging and diagnosis, contribution of images, critical review of manuscriptRH – reporting pathologist, involved in discussions leading to manuscript preparation, contribution of data and critical review of manuscriptJM – lead colorectal pathologist, involved in diagnosis, contributed to manuscript conception, involved in collating material, organising manuscript, critical reviews of manuscriptAll authors have read and approved the final manuscript


Teratomas are tumors consisting of tissues derived from more than one germ cell line. Criteria for pulmonary origin are exclusion of a gonadal or other extra-gonadal primary site and origin entirely within the lung. Lung teratomas are rare, and for unknown reasons commonly involve the upper lobe of the left lung. We report a case of intrapulmonary teratoma in a 38-year-old male and review the relevant literature. Mature teratomas are the most common histological type of germ cell tumors. These lesions originate from the third pharyngeal pouch, and may manifest with a variety of clinical and radiological features. Primary lung teratomas have rarely been reported since Mohr's description of this entity in 1839.A 38-year-old male presented with a two year history of intermittent episodes of cough and hemoptysis. He was a non smoker, and had no history of weight loss, fever or expectoration.Clinical examination revealed a well-preserved young male with stable vitals. He was afebrile. Auscultation of the chest revealed coarse crackles over the left upper and middle lobes. The rest of the clinical examination was unremarkable.The chest x-ray showed a well-defined large opacity in the upper lobe of the left lung. Computed tomography (CT) of the thorax showed a well defined solid lesion measuring 7 × 5.2 × 5 cm and occupying the anterior segment of the left upper lobe and the superior segment of the lingular lobe Fig. . It appeCT guided fine needle aspiration cytology (FNAC) showed sheets of degenerating acute inflammatory cells and anucleate squamous cells. A histological evaluation was suggested.Pulmonary function tests showed mild restriction in the forced vital capacity. Routine hematological tests and abdominal sonography was within normal limits. Mantoux test was negative.Thoracotomy and enucleation of the lesion was performed. The lesion, measuring 7.5 × 6 × 5.5 cm, was well circumscribed, encapsulated, and partially cystic and filled with hair and sebaceous material Fig . MicroscMature teratomas are the most common histological type of germ cell tumors, followed by seminomas. Germ cell tumors are predominantly found in the gonads, while the anterior mediastinum is the most common extragonadal site . The firGerm cell tumors in the lung occur typically in the second to fourth decades of life with a slight female preponderance. Patients present with chest pain, hemoptysis, cough and expectoration of hair (trichoptysis); the latter is the most specific symptom .Intrapulmonary teratomas typically range from 2.8 to 3 cm in diameter, and are cystic and multiloculated but may rarely be predominantly solid. In 42% of the cases, the cysts are in continuity with bronchi, and have an endobronchial component resulting in hemoptysis or expectoration of hair or sebum .Clinically, patients with intrapulmonary teratomas present with chest pain (52%), hemoptysis 42%) and cough (39%). The most specific symptom is trichoptysis or expectoration of hair (13%). Bronchiectasis occurs in 16% of cases and may delay the recognition of the pulmonary tumor [2% and coRadiographically, lesions are typically cystic masses often with focal calcification. CT accurately estimates the density of all elements such as soft tissue , fluid (88%), fat (76%), calcification 53%) and teeth [3% and teSurgical resection is the treatment of choice; and radical extirpation leads to a long recurrence-free survival .Intrapulmonary teratomas are rare tumors. They originate from the third pharyngeal pouch and present as cystic lesions in the majority of cases. Histologically, benign teratomas comprise 2 or 3 primordial layers. Patients present with chest pain, cough, hemoptysis and trichoptysis. Complete resection is adequate treatment for patients with a good long term prognosis.


Adrenal crisis after surgical procedure is a rare but potentially catastrophic life-threatening event. Its manifestations, such as hypotension, tachycardia, hypoxia, and fever mimic the other more common postoperative complications. Clinical outcome is dependent upon early recognition of the condition and proper management with exogenous steroid administration.We report a 75-year-old man who presented with shock immediately after surgery for a femoral fracture from lung cancer metastasis. Anemia and severe hyponatremia were detected. Despite adequate fluid resuscitation, nonspecific symptoms including hypotension, tachycardia, hypoxia, fever and confusion occurred. Emergent CT revealed enlarged bilateral adrenal glands. Under the diagnosis of adrenal crisis due to metastatic infiltration of adrenal glands, the patient was treated with appropriate steroid replacement resulting in rapid improvement and recovery.We describe a case of adrenal crisis caused by the lack of adrenal reserve based on metastatic involvement and surgical stress, the first published case of adrenal crisis after surgery for a pathologic fracture from lung cancer metastasis. Surgeons treating pathologic fractures should be aware of this complication and familiar with its appropriate therapy because of increasing opportunity to care patients with metastatic bone tumors due to recent advances in cancer treatment. Adrenal crisis is a potentially lethal entity that is rarely reported after surgical procedures. The clinical manifestations are nonspecific and difficult to distinguish from common symptoms such as fever, hypotension, tachycardia, and electric disturbances that are frequently observed in the postoperative patients. The clinical diagnosis is often empirical, thus requires a high index of suspicion. Further, the administration of corticosteroid replacement can be lifesaving. We report here a patient with acute adrenal crisis, who presented with shock immediately after surgical treatment of metastatic bone tumor of lung cancer origin.In April 2006, a 75-year old man who had been successfully treated for colon cancer 13 years ago, thyroid cancer 12 years ago, and was receiving endocrine therapy for prostate cancer from last year was transferred to our hospital for a fracture of his left proximal femur. The chest radiograph showed a solid mass in the hilum of left lung, and the thallium scintigram demonstrated abnormal uptake at the left proximal thigh and the hilum of left lung. In addition, the screening of tumor markers revealed extremely high level of CEA (1250 ng/ml), slightly high level of NSE (16.6 ng/ml) and SCC (2.2 ng/ml), and low level of thyroglobulin (0.3 ng/ml) and PSA (0.009 ng/ml). Thus the fracture was considered to be a bone metastasis from his fourth primary pulmonary cancer. Physical examination showed no hyperpigmentation of his all body surface. Laboratory investigation revealed serum sodium of 134 mmol/l, potassium 4.7 mmol/l, and plasma glucose 110 mg/dl, with normal renal and liver function.He uneventfully underwent local excision and proximal femur replacement with a mega-prosthesis under general anesthesia. The bone tumor was pathologically diagnosed to be moderately differentiated adenocarcinoma. Later, the immunohistochemical examination revealed that cytokeratin 7 (CK7) and thyroid transcription factor 1 (TTF-1) were distinctly detected but cytokeratin 20 (CK20) never expressed. Recently, Chhieng et al. described that an adenocarcinoma was likely a primary lung tumor when it was of the CK7 positive/CK20 negative and TTF-1 positive phenotype [The estimated intraoperative blood loss was 400 ml. The patient was extubated as usual, but the level of awakening was very poor. Postoperative laboratory examination demonstrated anemia and severe hyponatremia 126 mmol/l), but showed normokalemia (4.3 mmol/l) and normoglycemia (92 mg/dl). Despite the transfusion of blood and the administration of normal saline, the patient subsequently became hypotensive (SBP 60–70 mmHg), tachycardic, hypoxic, febrile (40.5°C) and confused. Re-intubation was required for respiratory distress. A chest radiograph showed diffuse pulmonary edema. We initially suspected this condition of acute pulmonary emboli. Immediately, the patient underwent CT examination from head to abdomen. Enhanced chest CT revealed a left hilar mass suggesting a primary lung cancer but no pulmonary embolus. A ventilation/perfusion scan performed on the next day showed no perfusion defect and confirmed definitely no evidence of pulmonary embolism. Concomitant brain and abdominal CT scan also demonstrated a low density area in the parietal lobe of the left brain and massively enlarged bilateral adrenal glands consistent with metastases  and quality of life (QOL). Recent advances in cancer treatment cause the prolonged survival and increase the prevalence of bone metastasis. Consequently, orthopedic surgeons have been further involved in the diagnosis and treatment of the metastatic bone tumors. To our best knowledge, there have been no reports of adrenal crisis after the surgery for pathologic fracture. However, adrenal glands are commonly involved by metastatic disease from various neoplasms including lung cancer. Besides, prospective study has found that up to one third of patients found to have bilateral adrenal metastases secondary to neoplastic disease will ultimately develop adrenal crisis . AdrenalWe describe a case of adrenal crisis caused by the lack of adrenal reserve based on metastatic involvement and surgical stress, the first published case of adrenal crisis after surgery for a pathologic fracture from lung cancer metastasis. Surgeons treating pathologic fractures should be aware of this complication and familiar with its appropriate therapy because of increasing opportunity to care patients with metastatic bone tumors due to recent advances in cancer treatment.computed tomography, CT; magnetic resonance imaging, MRI.The author(s) declare that they have no competing interests.NN wrote the case report and performed the literature searchST performed the operation and helped in preparing the manuscriptKN performed the operation and helped in preparing the manuscriptYM performed the operation and helped in preparing the manuscriptBC participated in managing the case as an anesthesiologistSS participated in managing the case as an anesthesiologistNH performed the literature search and helped to draft the manuscriptYT performed the pathological diagnosisNA revised the manuscriptAll authors read and approved the final manuscript.


Echinococcosis rates vary in different parts of the world. Italy is regarded as a middle to high risk country with over 1,000 cases requiring surgery each year. Liver (45–75%) and lung (10–50%) are the most frequent locations of this parasitosis.The authors report a clinical case of a 62 year old woman, admitted to hospital with left flank pain. Plain radiographs of the abdomen, ultrasound, CT and MRI scans were performed and the presence of a 3-cm lesion of the left adrenal gland was demonstrated. A diagnosis of hydatid cyst was made. The patient underwent transabdominal laparoscopic left adrenalectomy. Histopathological examination confirmed the presence of a hydatid cyst in the left adrenal gland.A hydatid cyst was correctly diagnosed on the basis of radiologic findings. The uncomplicated cyst was successfully resected using a laparoscopic approach. The pathological features of this case are presented in this paper. Echinococcosis in humans is a zoonotic infection caused by larval stages of cestode species of the Echinococcus genus . The cysEchinococcosis occurs mostly in the northern hemisphere, in geographically limited foci of Europe, Turkey, many areas of the former Soviet Union, Iran, Iraq, China, and Japan ,7. SurveWe report a case of a symptomatic patient with an Echinococcus cyst of the left adrenal gland. Initially observed on a plain abdominal x-ray, the cyst was correctly diagnosed on the basis of computer tomography (CT) scan images and magnetic resonance imaging (MRI) findings and confirmed at surgery. The pathological features of this case are also presented in this paper.A 62-year-old woman was referred to the Department of Surgical Sciences, University of Insubria, Varese, for left flank pain. Physical examination was unremarkable. Complete blood cell count, electrolytes, eosinophil count, serum biochemistry and urinalysis were within normal limits. Plain radiographs of the abdomen showed a small round opacity, about 3-cm in diameter, in the left upper region of the abdomen, with evidence of calcifications. Chest X-ray was normal. The patient underwent ultrasound examination (US) that showed a calcified heterogeneous cystic mass between the superior left kidney pole and spleen in the retroperitoneal area. CT scanning demonstrated the presence of a solitary cystic mass with a calcified wall within the left adrenal gland with no enhancement after iv contrast media injection. No other intra-abdominal or intra-thoracic masses were found, either in the liver, peritoneum or lung. MRI T2-weighted imaging showed a hypointensive lesion with a calcified wall, and a peripheral hyperintensive nodule Figure , 2. CT aThis case is unusual due to the organ involved. Hydatid cysts can occur almost anywhere in the body ,7. The aThe growth of a hydatid cyst is usually slow and asymptomatic. In this case, the patient had nonspecific symptoms. The expansion of a larger cyst, a number of cysts or inflammatory reaction around a cyst with irritation of the adjacent peritoneum may cause pain in the flank. Occasionally, a palpable mass may be found -6. The mA variety of tumors can be located in the adrenal gland and must be considered in the differential diagnosis . Cysts oSerological tests are based on the reaction and precipitation between the test antigen and the circulating antibodies in the host -7. The sAntihelmintic agents have been used in the treatment of systemic echinoccosis in endemic areas . There aSurgery remains the mainstay for the treatment of hydatid cysts -6.The rapid development of laparoscopic techniques has encouraged surgeons to replicate principles of conventional hydatid surgery using a minimally invasive approach. This case reports the use of a transabdominal laparoscopic adrenalectomy. The benefits of endoscopic surgery compared to open traditional surgery are well known and include less surgical scarring, less operative pain and shorter length of postoperative stay . In geneThe author(s) declare that they have no competing interests.GD acquisition of dataFS, LB, FR, GC, CR: drafting of manuscriptCR critical revision and supervision


Desmoid tumors that present as a part of Gardener's syndrome can present very difficult management problems.We report a case of intra-abdominal desmoid tumor causing distal small bowel obstruction that complicated the management of a more proximal enterocutaneous fistula from the jejunum. After failure of more conventional management options including imatinib, the patient's disease responded to doxorubicin and ifosfamide. The response resolved the bowel obstruction and allowed small intestinal resection to resolve the enterocutaneous fistula.Systemic cytotoxic therapy with doxorubicin and ifosfamide can be useful for patients with complications from intra-abdominal desmoid tumor. Gardner's syndrome includes familial adenomatous polyposis with extracolonic manifestations, such as osteomas, supernumerary teeth, fibrous dysplasia of the skull, fibromas, and epidermoid cysts. Desmoid tumors, also known as "aggressive fibromatosis", can have a significant impact on the morbidity and mortality of patients with Gardner's syndrome. Locally aggressive desmoid tumors can impinge on surrounding structures, causing symptoms and organ impairment. When treatment is indicated, these tumors should be surgically resected with wide margins. However, resection is often followed by recurrence, and resection of some lesions would cause unacceptable deficits. Although desmoid tumors have no metastatic potential, systemic therapy with a variety of agents can be helpful. Cytotoxic chemotherapy is effective in some cases refractory to more conservative treatment options. We report a case of a 40 year-old woman with Gardner's Syndrome and desmoid tumors causing an enterocutaneous fistula with distal small bowel obstruction, successfully managed with a strategy that included cytotoxic chemotherapy.A 40-year-old-woman with a history of Gardner's Syndrome status post prophylactic colectomy presented with an enterocutaneous fistula and for reevaluation of desmoid tumors. She had three previous abdominal operations including a prophylactic colectomy in 1992. In 1995, she had a resection of small intestine and marginal resection of associated desmoid tumors causing small bowel obstruction. In 2002, an emergent resection of small intestine and desmoid tumors for intestinal perforation removed only a portion of the diffuse mesenteric disease. This operation was complicated by an enterocutaneous fistula from the jejunum that subsequently healed with conservative care. She had no adjuvant radiation therapy at any point. Estrogen and progesterone receptor expression of the tumors was never assessed.2 IV on days 1, 2, and 3 and ifosfamide 2 g/m2 daily for 3 days of a 21 day cycle. She received five cycles of this regimen, with a clear radiographic response on CT scan  declare that they have no competing interests.GD, LB and RC treated the patient clinically and designed the study, PB and RC drafted the manuscript. PB, RC, LB and GD all contributed to critical revisions and intellectual content. All authors read and approved the final manuscript.


We report the first case of a posterior mediastinal granular cell tumor initially diagnosed on cytologic material obtained via endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) in a 51-year-old male with a prior history of colon cancer. Aspirates obtained were cellular and composed of polygonal cells with abundant granular cytoplasm and small, round dark nuclei. An immunoperoxidase stain performed on the cell block for antibodies to S-100 protein showed strong, diffuse staining of the cytoplasmic granules. Electron microscopy performed on the cell block revealed numerous cytoplasmic lysosomes. This is the first case report in the English literature of a definitive preoperative diagnosis of a mediastinal granular cell tumor utilizing material obtained via EUS-FNA. Granular cell tumors are uncommon generally benign soft tissue neoplasms first described in 1926 by Abrikossoff . MediastA 4.9 × 3.1 cm peritracheal posterior mediastinal mass was incidentally discovered on a computed tomography (CT) scan of a 51 year-old gentleman status-post left hemicolectomy in October 2003 for a T3N1Mx, stage III colon cancer. He had moderate gastroesophageal reflux and mild dysphagia. A barium swallow revealed compression and displacement of the proximal thoracic esophagus. Esophageal mucosal ulcerations were not observed. Integrated positron emission tomography(PET)/CT scan using fluorodeoxyglucose (FDG) showed no uptake in the lesion. Due to the benign clinical features of the mass, the differential diagnosis included benign neoplasms such as a leiomyoma or a benign thyroid neoplasm. The possibility of lymphadenopathy secondary to metastatic adenocarcinoma was remote. Endoscopic ultrasound was performed using the UC-30P  and revealed a 52- × 39-mm hypoechoic, well-circumscribed posterior mediastinal mass in the thoracic inlet approximately 22 cm from the incisor , CD68 , thyroglobulin , and TTF-1 . Electron miscroscopy was performed on the paraffin-embedded cytologic cell block.The Institutional Review Board at the University of Alabama at Birmingham approved the use of human tissue in this case report.The cytologic smears revealed a cellular aspirate composed of loosely cohesive groups of polygonal cells and single cells Figure . The celRepresentative sections of the resected mass were submitted for histologic evaluation. The tumor consisted of sheets of cells similar to those seen in the fine needle aspiration Figure . Frozen Mediastinal granular cell tumors are very rare tumors. Prior to this report, only seven mediastinal granular cell tumors have been described in the literature . The aveIn 1997, Smith et al. described fine needle aspiration cytology findings of a mediastinal granular cell tumor in a 53 year-old woman . While tIn the past, mediastinal lesions were sampled utilizing mediastinoscopy, transtracheal biopsy, or CT-guided biopsy. Superior and posterior mediastinal masses have been difficult to approach using those sampling modalities. EUS-FNA, however, has emerged as a safe, effective method of tissue sampling in the mediastinum . Here weThe cytologic findings of granular cell tumors have been previously described in organs such as esophagus, breast, tongue, bronchus, and skin -15. TypiThis is the first case report of a preoperative diagnosis of a mediastinal granular cell tumor by EUS-FNA. While granular cell tumors are rare tumors, it is important to include it in the differential diagnosis of mediastinal lesions. The preoperative diagnosis of a granular cell tumor by EUS-FNA will tremendously benefit the patient by preventing further tissue sampling procedures and possibly even surgical resection.The author(s) declare that they have no competing interests.All authors made substantial contributions to the intellectual content and/or presentation of the manuscript. SMB (cytopathology fellow) is the first author, and she wrote the intial version of the manuscript under the guidance of DJ (cytopathologist) who is a senior author. DJ and IE (cytopathologists), diagnosed the cytopathological aspects of the case. ME is the endoscopist who performed the EUS-FNA and revised the manuscript. RC is the surgeon who resected the mass and revised the manuscript.


Malignant mixed mullerian tumors (MMMT) are rare biphasic malignant neoplasm. The commonest site of their occurrence in female genital tract is body of the uterus. MMMT of the cervix is extremely rare.We report the clinical, pathological and immunohistochemical profile and diagnostic difficulties in a case of giant MMMT of the cervix in a postmenopausal woman who presented with a large cervical mass. On microscopic examination, initially tumor appeared to be endometrial stromal sarcoma, however, immunohistochemical examination revealed the biphasic nature of the tumor. The malignant epithelial component was basaloid squamous carcinoma with homologous sarcomatous component. The patient was treated with surgery. However, she experienced vaginal vault recurrence four months after the initial treatment, which was successfully treated with pelvic radiotherapy.Accurate diagnosis of cervical MMMT is important for appropriate treatment of the patient. Malignant mixed mullerian tumors (MMMT) are rare biphasic malignant neoplasm. The commonest site of occurrence of female genital tract MMMT is the uterine corpus. MMMT of the cervix is extremely rare. It was first described by Ferriera in 1951 .We report the clinical, pathological and immunohistochemical profile and diagnostic difficulties in a case of giant MMMT of the cervix where malignant epithelial component resembled endometrial stromal sarcoma.A 60-year-old postmenopausal woman presented with lump in lower abdomen of 2 months duration. Abdominal examination revealed a large, irregular, firm supra-pubic mass. On pelvic examination, the same mass was felt in the vagina. Cervix and uterus were not felt separately from the mass. Computed tomography (CT) scan showed a large, well defined heterogeneous abdomino-pelvic mass. Serum CA-125 level was 52.3 U/mL. Exploratory laparotomy was performed, which revealed a large, bosselated mass arising from the cervix. Body of the uterus, bilateral ovaries, tubes and other abdomino-pelvic organs were within normal limits. Total abdominal hysterectomy with bilateral salpingo-oophorectomy with complete excision of the mass was done after identifying the ureters from pelvic brim to their entry into the urinary bladder. Her postoperative recovery was uneventful.The patient was kept under close observation after initial treatment. Four months after surgery, on routine follow-up, vaginal cytology showed the malignant squamous component of MMMT. The recurrence was confirmed by colposcopy-guided biopsy from the vaginal vault. Physical examination and CT-scan did not reveal any evidence of disease elsewhere. This recurrence was treated with radical pelvic radiotherapy (RT) . The patient is free of disease at eighteen months after the treatment of recurrence.Gross examination of the surgical specimen revealed a 28 × 20 × 15 cm bosselated, mass arising from the cervix. The cervical canal was distorted because of the mass. On cut surface, the mass was fleshy, solid with cystic areas.On hematomylin and eosin staining, the entire tumor showed cellular whorls dispersed amidst pale sarcomatous stroma. The cells in whorls were round to short spindle shaped and had moderate mitotic activity figure . AlthougCervical MMMT usually occur in post-menopausal women with mean age at diagnosis of 61 to 69 years although the age range in the literature varies from 12 to 93 years [MMMT are biphasic tumors that consist of an admixture of malignant epithelial and mesenchymal components. The epithelial component represents a variety of different histological sub-types, alone or in combination, which include squamous cell carcinoma, basaloid squamous carcinoma, adenocarcinoma, adeno-squamous carcinoma, adenoid-basal carcinoma, adenoid-cystic carcinoma and undifferentiated carcinoma -5. The sThese tumors must be differentiated from other tumors on histopathology. Our case illustrates the varied pattern of MMMT and its tendency to mimic ESS. The islands of squamous differentiation were the clue to the diagnosis, as they are not seen in ESS. Extrauterine ESS arising in the cervix is extremely rare with only three reported cases available in the English literature . These tet al., [Grayson et al.,  studied et al., [Clement et al.,  reportedet al., . In our Cervical malignant mixed mullerian tumors are rare malignancies that may at times present diagnostic difficulties to the clinician. They are best treated by surgery with or without adjuvant radiation and/or chemotherapy. Accurate diagnosis is essential for appropriate treatment and prognosticating the disease.The author(s) declare that they have no competing interests.AM conceived the idea and drafted the manuscript, SG participated in the patients management and help in preperation of the manuscript. TS, RW did the literature search and helped in drafting the manuscript. HG did the overall supervision, and edited the final manuscript. All authors read and approved the final manuscript.


Hitherto uncommon, the incidence of extracranial metastases of primary brain malignancies may increase, with improved treatment methods and longer patient survival. Fine needle aspiration biopsy is a simple, safe and reliable method to diagnose metastatic malignancy. It has definite advantages over tissue biopsy, which is more invasive and is of higher risk to the patient. Ours is a case of glioblastoma multiforme, which metastasized to the scalp and was diagnosed on fine needle aspiration biopsy. Only a few articles document the cytological features of extracranial glioblastoma multiforme, diagnosed by fine needle aspiration biopsy.We report the case of an elderly female who presented with focal neurological symptoms. She was diagnosed radiologically with an intracranial lesion in the left temporal region, which was subsequently resected. Histology revealed a glioblastoma multiforme confirmed by immunohistochemistry. The tumor recurred subsequently and the patient was treated with chemotherapy, intraoperatively. At a later stage, she presented with a scalp mass on which fine needle aspiration biopsy was performed. The cytomorphological features aided by immunohistochemistry supported a diagnosis of metastatic glioblastoma multiforme. The mass was later resected and histology confirmed the fine needle aspiration diagnosis of glioblastoma multiforme.Reports of extracranial metastases of primary brain tumors are few. When they do occur, the primary cause is implantation during surgery or biopsy. However, spontaneous metastases to other organs do occur rarely. We believe fine needle aspiration biopsy to be very useful in the diagnosis of metastatic glioblastoma multiforme. The ability to use a cellblock for immunohistochemical studies is greatly advantageous and helpful in differentiating this tumor, from other malignancies that can occur in the scalp. A detailed discussion of the material obtained from fine needle aspiration biopsy of metastatic glioblastoma multiforme is presented, as well as a review of previous accounts in the literature. Extracranial metastasis of primary brain malignancies is an uncommon event . With inA 74-year-old female with no significant past medical history presented with complaints of headache in conjunction with speech and memory problems for approximately one month. Magnetic resonance imaging (MRI) revealed an enhancing cystic lesion in the left temporal lobe. A partial resection of the left temporal mass, revealed a malignant neoplasm on frozen section and on final pathological evaluation, a diagnosis of GBM was made. Immunohistochemical results showed positive staining for glial fibrillary acidic protein (GFAP) and no evidence of positive staining for cytokeratin. The patient received adjuvant external beam radiation therapy.Approximately nine months later the patient returned with both speech and short-term memory problems. MRI revealed tumor recurrence in the left temporal area. Stereotactic biopsy demonstrated a necrotic tumor consistent with GBM on frozen section, a diagnosis confirmed on permanent sections. The stereotactic biopsy was followed by intraoperative injection of the chemotherapeutic agent DTI-015 . Three m®); the 95% ethanol fixed smears were stained with the Papanicolaou stain.FNA biopsy of the palpable left parietal subcutaneous scalp mass was performed by a pathologist, using a 23 gauge needle. Four smears and one cell block were prepared. The air dried smears were stained with a modified Wright stain  is not described in all cases. A GFAP stain may be helpful in highlighting this feature. Thick-walled capillaries with endothelial proliferation are documented in one case study , but thiFine needle aspiration (FNA)Glial fibrillary acidic protein (GFAP)Glioblastoma multiforme (GBM)The author(s) declare that they have no competing interests.SS (since deceased) carried out the initial literature review and first draft of manuscript. GP assisted with preparation of the manuscript and photography. NW assisted with the cytologic evaluation and description. MC provided clinical information. SR carried out additional literature review and subsequent drafts of manuscript. SM participated in the coordination of the study and preparation of the final draft of the manuscript.


We report on a 14 year old boy who presented with the symptoms abdominal pain, fever and proteinuria. A hematoma in the region of the right pararenal space was diagnosed. Prothrombin time and activated partial thromboplastin time were prolonged, lupus anticoagulant and anticardiolipin antibodies were positive and serum cortisol was normal. Ten days after admission the boy suddenly suffered generalized seizures due to low serum sodium. As well, the patient developed hemolytic anemia, acute elevated liver enzymes, hematuria and increased proteinuria. At this time a second hemorrhage of the left adrenal gland was documented. Adrenal function tests revealed adrenal insufficiency. We suspected microthromboses in the adrenals and secondary bleeding and treated the boy with hydrocortisone, fludrocortisone and phenprocoumon.Adrenal failure is a rare complication of APS in children with only five cases reported to date. As shown in our patient, this syndrome can manifest in a diverse set of simultaneously occurring symptoms. The antiphospholipid syndrome is characterized by clinical evidence of arterial or venous thrombosis and repeated presence of antiphospholipid autoantibodies. The disease, first described by Hughes in 1983 [The autoantibodies, of which the most important are lupus anticoagulant and cardiolipin-antibodies, comprise a heterogeneous group which are mainly directed against complexes of anionic phospholipid with some phospholipid-binding proteins such as beta-2-glycoprotein I and human prothrombin. In a proportion of individuals the circulation of the antibodies may induce thrombosis of virtually any vein or artery. The most common are deep vein thrombosis, pulmonary, or cerebrovascular embolisms. In addition, other complications such as cardiomyopathy, hepatitis, hemolytic anemia, bleeding, vasculitis and renal failure have been reported .The complexity of manifestations and the risk of severe complications, led us to conclude that this syndrome is an important differential diagnosis in patients with the described symptoms.In September 2001, a previously healthy, Caucasian, 14-year-old boy was admitted to our hospital. Two weeks prior to his arrival, he had developed abdominal pain, a recurring fever of up to 39°C, and an intermittent cough. These symptoms were worsening and his general condition was deteriorating.On examination he had diffuse abdominal pain located primarily in the upper abdomen, and his temperature was 38.5°C. His skin and the further physical examination was normal. Pertinent laboratory investigations are listed in Table The initial abdominal ultrasound revealed a tumor in the right pararenal space. However, a clear anatomical relation to the adrenal gland could not be established  declare that they have no competing interests.Table 2Click here for fileFigure 1: scanned ultrasound photographyClick here for fileFigure 2: scanned ultrasound photographyClick here for fileFigure 3: scanned photography of computer tomographyClick here for file


Malignant transformation of adenomyosis is a very rare event. Only about 30 cases of this occurrence have been documented till now.The patient was a 57-year-old woman with a slightly enlarged uterus, who underwent total hysterectomy and unilateral adnexectomy. On gross inspection, the uterine wall displayed a single nodule measuring 5 cm and several small gelatinous lesions. Microscopic examination revealed a common leiomyoma and multiple adenomyotic foci. A few of these glands were transformed into a moderately differentiated adenocarcinoma. The endometrium was completely examined and tumor free. The carcinoma was, therefore, considered to be an endometrioid adenocarcinoma arising from adenomyosis. Four months later, an ultrasound scan revealed enlarged pelvic lymph nodes: a cytological diagnosis of metastatic adenocarcinoma was made.Immunohistochemical studies showed an enhanced positivity of the tumor site together with the neighbouring adenomyotic foci for estrogen receptors, aromatase, p53 and COX-2 expression when compared to the distant adenomyotic glands and the endometrium. We therefore postulate that the neoplastic transformation of adenomyosis implies an early carcinogenic event involving p53 and COX-2; further tumor growth is sustained by an autocrine-paracrine loop, based on a modulation of hormone receptors as well as aromatase and COX-2 local expression.Adenocarcinoma in adenomyosis may be affected by local hormonal influence and, despite its small size, may metastasize. Leiomyoma and adenomyosis are both commonly encountered in hysterectomy surgical specimens, often as incidental findings. A frequent association of adenomyosis with other hormone-dependent uterine lesions, both benign and malignant, such as endometrial hyperplasia, endometrial carcinoma and leiomyoma has already been described in the literature ,2. Howev1) evidence of pre-existing endometriosis at the site of the supposed malignant lesion;2) exclusion of the possibility of the carcinoma representing an invasion or metastasis from another location;3) evidence of transitions between the benign and malignant glandular structures; and4) both glands and stroma must be present to constitute genuine adenomyosis .At present, only about 30 cases of adenocarcinoma arising from adenomyosis without endometrial malignancy have been reported in the English literature -12. In aWe report on the clinico-pathological and immunohistochemical findings of a small endometrioid adenocarcinoma arising from adenomyosis, incidentally discovered around a leiomyoma after extensive sampling. To the best of our knowledge, this is the single smallest reported instance of such an occurrence with a distinct immunohistochemical profile. The possible connection between malignant transformation of adenomyosis and benign, hormone-dependent disease is discussed.The patient was a 57-year-old nulliparous, asymptomatic woman of normal weight. She was in follow-up for a fibromatous uterus. On palpation, a uterine softening was noted. An ultrasound scan displayed the enlargement of a pre-existent leiomyomatous nodule and multiple minute focal lesions throughout the uterine wall, diagnosed as a likely adenomyosis. Menopause had commenced at the age of 54. Her past medical history was significant for papillary serous cystoadenoma of the left ovary which was removed when she was 33 years of age. For the last few years, the patient was being treated with an antihypertensive therapy, while, nine years previously, for a period of two years, she had taken symptomatic therapy for perimenopausal symptoms including progesterone, and, during the last year, a non-hormonal therapy with a hyperprolactinemic effect to reduce hot flushes.Because of the recent appearance of focal lesions in the uterine corpus in an already enlarged uterus (as seen on the ultrasound scan), a total hysterectomy and unilateral adnexectomy were performed. Four months later, an ultrasound scan revealed an enlargement of the left external iliac chain nodes and a thickening of the lateral pelvic wall up to the inguinal ring. A second-look surgical inspection as well as an extensive clinical work-up, which included a thoraco-abdominal CT scan, excluded the presence of other primary tumors.The tissues were fixed in Bouin solution and embedded in paraffin for histologic processing. Tissue sections were stained with hematoxylin and eosin for conventional histology.Immunohistochemical stainings were performed using a combination of the avidin-biotin complex peroxidase method  and microwave antigen retrieval with the following antibodies: ER ; PR ; C-erb B2 ; Cyclooxygenase-2 (COX-2) ; p53  and CA125 .The sections were also stained for aromatase as follows: sections were dewaxed in xylene and taken through a graded series of ethanol. Epitope retrieval was performed by enzymatic digestion with trypsin for 20 minutes at 37°C . After incubation with 0.3% hydrogen and protein block solution (DAKO) for 5 minutes, the sections were treated with polyclonal antiaromatase antibody  for 15 minutes at room temperature following dilution to 1:1,000 in buffer. After incubation with the primary antibody, streptavidin-biotin complex and streptavidin-peroxidase complex were applied for 15 minutes each at room temperature. Colour was developed by incubation with 3,3-diaminobenzidine-tetrahydrochloride for 1 min, followed by counterstaining with hematoxylin.Negative controls included sections incubated with normal rabbit serum instead of the primary antibody. In addition, an immunoabsorption test was done as follows: a complex of 1 μl of anti-aromatase antibody and 1.5 μl of microsome (at a concentration of 4 μg/μl), extracted from placental tissue, was stirred in phosphate buffer saline containing 1% bovine serum albumin for 12 hours at 4°C, and the resulting precipitate was then centrifuged . Instead of the primary antibody, the supernatant was used for immunostaining.On gross examination, the uterus measured 7 × 4 × 9 cm and the right ovary measured 3 cm. On cut section, the myometrium of the fundus presented a subserosal whorled leiomyomatous nodule which measured 5 cm, was well circumscribed, solid and surrounded by gelatinous foci. The endometrium appeared thinned with no superficial irregularities. It was totally examined, and only atrophic-cystic changes with focal proliferative activity were seen. There were several adenomyotic foci in the inner half of the myometrium, while some others, in the outer half, were pushed out by the leiomyoma and were located proximally to the serosa and parametrium. A cluster of these glands harboured a focal malignant transformation of the epithelial component, diagnosed as moderately differentiated endometrioid adenocarcinoma measuring 0,8 cm Figures  and 1B aThe enlarged iliac lymph nodes, detected 4 months after surgery, were biopsied by fine-needle aspiration during a second-look procedure, and were diagnosed as metastatic adenocarcinoma Figure .Immunohistochemically, the primary tumor displayed strong positivity for ER, PR, COX-2, CA125 and focal positivity for p53 and aromatase Figures . The leiper se a rare event, but the development of metastatic lymph nodal deposits from a very small primary adenomyotic adenocarcinoma is quite exceptional.The case that we present is a single, small, moderately differentiated endometrioid adenocarcinoma which developed into adenomyosis with subsequent metastatic spread to the iliac lymph nodes. The primary tumor was positive for ER, PR, COX-2, CA125, p53 and focally weak-positive for aromatase. Malignant transformation of adenomyotic foci is The diagnosis of malignant tumors in adenomyosis is usually made in an advanced stage of disease when the neoplasm has grown in nodules within the uterine wall or has involved the endometrium causing abnormal uterine bleeding and/or has spread out of the uterus ,7-9,12.In 1925, Sampson introduced the histological criteria to define an adenocarcinoma arising in external endometriosis, which can be adapted to adenomyosis .In the literature, the clinico-pathological findings of 8 cases of adenocarcinoma arising in adenomyosis were presented by Hernandez and Woodruff in 1980, but these were all associated with endometrial adenocarcinomas. They also evaluated 16 cases previously reported by other authors in the literature, and found that only 8 of these were not associated with endometrial adenocarcinomas . In the It is well known that gynaecological malignancies, including breast, ovarian and uterine carcinomas, are influenced by hormonal stimulation. In the uterus, a peculiar hormonal microenvironment may explain different pathologic conditions, both benign and malignant. The complex inter-relationships between adenomyosis, endometriosis, leiomyomata, endometrial hyperplasia and carcinoma arising in the endometrium or in adenomyosis have been illustrated together with their hormonal dependency ,2,13, anIn postmenopausal women, estrogen formation is ensured by extraglandular tissues, namely adipose tissue and skin. Endometriosis and leiomyomas constitute two added sources of aberrant estrogen biosynthesis, primarily by aromatase enzymatic activity, promoting their self-stimulation ,16. AromIn endometriosis, aromatase activity is stimulated by prostaglandin E(2) wich, in turn, is up-regulated by increased levels of the enzyme cyclo-oxygenase-2 (COX-2) .Finally, besides aberrant aromatase expression, a local estrogen overproduction in endometriosis may also be a result of a deficiency of 17β-hydroxysteroid dehydrogenase, resulting in failure to metabolize 17β-Estradiol .In three studies, adenocarcinomas in adenomyosis were reported as all negative for ER and fairly negative for PR ,9,12. ThCOX-2 overexpression has been described in ectopic endometriosis implants when compared with eutopic endometrium , in endoMoreover, COX-2 has been suggested to be involved in multiple steps in endometrial cancer: besides tumor progression, the enzyme has been implicated in the earlier phases of neoplastic transformation, in endometrial hyperplasia  and in eAccordingly, we found an enhanced positivity for COX-2 both in the tumor and in the neighbouring adenomyotic foci, when compared to the distant adenomyosis and the endometrium.P53 has been showed to be expressed in endometrial carcinoma with and without adenomyosis , in otheOur case is quite peculiar for the early metastatic spread to the iliac lymph nodes, confirmed by cytological fine-needle aspiration performed during the second-look surgical inspection and by an extensive clinical work-up to exclude other sites of primaries. The origin of the metastasis from adenocarcinoma in adenomyosis was also confirmed by its immunocytochemical profile. A possible explanation for such early metastatic spreading is that this tumor arose in the outer myometrium, near to the richly vascularized parametrium.In conclusion, considering that no evidence of systemic exogenous or endogenous hyperestrogenism are on record in our case, it is arguable that the presence of a leiomyoma, close to a pool of adenomyotic foci, along with the overexpression of estrogen receptors and enzymes involved in steroidogenesis, represent a hyperestrogenic microenvironment with converging sources of estrogen biosynthesis. Therefore, the same local estrogen excess, besides sustaining adenomyosis and leiomyoma persistence, may also promote growth in tumors having adequate ER/PR expression. This local hormonal overproduction most likely interacts with other events, such as p53 mutations and COX-2 increased activity, in the pathogenesis of adenocarcinoma in adenomyosis.ER: estrogen receptors; PR: progesterone receptors.The author(s) declare that they have no competing interests.GP conceived and coordinated the study and drafted the manuscript. MS was responsible for the immunohistochemical staining with aromatase and revised the manuscript for important intellectual content. TP was responsible for the sampling of the surgical specimen. KN was responsible for the immunohistochemical staining with aromatase. AG was responsible for the immunohistochemical staining with ER, PR, C-erb B2 and COX-2. EC was responsible for performeance of all the surgical interventions. VC participated in the coordination of the study and revised the manuscript for important intellectual content. All the authors approved the final manuscript.The pre-publication history for this paper can be accessed here:


It is potentially aggressive, capable of frequent recurrences and loco-regional and distant metastases.Clear cell odontogenic carcinoma is a rare odontogenic tumor occurring in the anterior region of the mandible in 5A 45- year- old woman presented with a radiolucent left mandibular swelling associated with loss of teeth. Left cervical lymph nodes were enlarged on palpation. The patient underwent resection of the tumor but consequent to resected margins being positive for tumor cells underwent left hemimandibulectomy with ipsilateral functional neck dissection and was free of recurrence at 8 months follow-up.Clear cell odontogenic carcinoma should be considered in the differential diagnosis of jaw tumors with conspicuous clear cell component. Curettage or conservative resection inevitably results in recurrences and/or metastasis and more radical resection is warranted in these tumors, especially when they are large and show soft tissue invasion. Clear cell odontogenic carcinoma (CCOC) is a rare odontogenic tumor arising from the anterior region of mandible and has a predilection for females with 43 cases reported in literature. We report a case of CCOC in the mandible of a 45- year- old female with lymph node metastasisA 45-year-old woman presented with a painful swelling on the left side of her jaw of 5 months duration. The swelling had recently started to grow rapidly. There were no associated sensory symptoms. In addition, a few teeth adjacent to the swelling had become loose, carious and had fallen off. Local physical examination showed a 4.0 × 3.2 × 3.0 cm, tender, firm, irregular lump that appeared to arise from the left mandible. Intra orally, the tumor could be seen as a pink, bulging and fleshy mass. Two lymph nodes were palpable, one in left submaxillary region measuring approximately 1 × 1 cm and the other in the left cervical region and was barely palpable. Remainder of the physical examination was not contributory and no other swelling was noticed. Orthopantomogram (OPG) of the jaw showed a radiolucent lesion with irregular margins, centered in and destroying a portion of the left mandibular body . The reconstruction was done using ipsilateral 12th rib and the soft tissue coverage was given by pectoralis major flap. Postoperative histopathological examination showed tumor morphology similar to incisional biopsy and 1/11 lymph nodes (submaxillary) was positive for the tumor metastasis without any extranodal spread. The tumor was extending into the soft tissue and the left bony margin was positive for the tumor cells. Due to the positive margin, the patient was referred for adjuvant radiotherapy for which she refused to give consent. At the time of discharge the patient was accepting soft diet and the suture line on the neck and chest were healthy. Subsequent to the discharge, the patient was lost to follow-up only to reappear after 8 months and at that time had no signs of local or regional spread. Since then patient is again lost to follow-up.et al., reported a locally aggressive odontogenic neoplasm, and named it clear cell odontogenic tumor [In 1985, Hansen ic tumor . Subsequic tumor -7.The classic clinical presentation of CCOC is a painful anterior mandibular swelling in an elderly woman. There may be loosening of adjacent teeth, and a roentgenogram shows an irregular radiolucent mass, most often in a pre-molar location. Histopathologically, CCOCs may show one or more of three architectural patterns: biphasic, monophasic, and ameloblastomatous. The most common biphasic pattern of tumor growth comprises of nests of cells with clear cytoplasm admixed with cells containing eosinophilic cytoplasm. The monophasic pattern comprises only of clear cells, while the ameloblastomatous pattern resembles the growth pattern of ameloblastoma with nests of cells showing central cystic change and squamous differentiation, and peripheral nuclear palisading with reverse polarity . The difA recent review of literature of the 43 cases reported so far  showed tCCOC although rare, should be considered in differential diagnosis of jaw tumors with prominent clear cell component. The limited number of reported cases precludes a definitive assessment of prognostic factors or efficacy of therapy in CCOC. Currently, treatment is aimed at achieving wide surgical resection with tumor-free margins, and loco-regional control by lymph node resection and local radiation in cases with extensive soft tissue invasion, perineural spread, lymph node metastasis with extranodal involvement or in those where tumor-free margins are not possible. A long-term follow-up is essential as these tumors may recur locally or present with late distant metastases.The author(s) declare that they have no competing interests.SA participated in histopathological diagnosis, reviewed the literature and partly wrote the manuscript. DR reviewed the slides, did special stains, took pictures and contributed suggestions in drafting the manuscript. AB clinically managed the patient and helped in preparing the draft manuscript. All the authors have read and approved the final version of the manuscript.


Epithelial ovarian neoplasms are an important cause of morbidity and mortality in women. The surgical management of ovarian neoplasms depends on their correct categorization as benign, borderline or malignant. This study was undertaken to evaluate the accuracy of intra-operative frozen section in the diagnosis of various categories of ovarian neoplasms.Intraoperative frozen section diagnosis was retrospectively evaluated in 217 patients with suspected ovarian neoplasms who underwent surgery as primary line of therapy at our institution. This was compared with the final histopathologic diagnosis on paraffin sections.In 7 patients (3.2%) no opinion on frozen section was possible. In the remaining 210 patients frozen section report had a sensitivity of 100%, 93.5% and 45.5% for benign, malignant and borderline tumors. The corresponding specificities were 93.2%, 98.3% and 98.5% respectively. The overall accuracy of frozen section diagnosis was 91.2%. The majority of cases of disagreement were in the mucinous and borderline tumors.Intraoperative frozen section has high accuracy in the diagnosis of suspected ovarian neoplasms. It is a valuable tool to guide the surgical management of these patients and should be routinely used in all major oncology centers. Epithelial ovarian neoplasms are an important cause of morbidity and mortality in women. The three main categories of ovarian neoplasms are benign, borderline and malignant, which differ with respect to their biologic characteristics, management and prognosis. Tumors of 'borderline malignancy' are an important group characterized by some histologic features of malignancy  but lack stromal invasion. They have an excellent long-term outcome even after conservative surgery. It is extremely helpful and sometimes critical to know intraoperatively the category of tumor one is dealing with, primarily to decide the extent of surgery. Benign and borderline tumors can be adequately treated with conservative surgery, which may involve preservation of fertility in younger women . In contPreoperative imaging and tumor markers have only limited value in differentiating between these tumor categories ,3. IntraCase records of 241 patients with ovarian neoplasms who underwent frozen section diagnosis between 1997 and 2001 were retrospectively reviewed. During this time period, only patients with the following indications received intraoperative frozen section diagnosis:1. Clinically benign looking tumors (on preoperative radiology or intraoperative inspection) with raised CA- 125.2. Adnexal mass in a patient with a past history of malignancy at another site.3. Young patients with ovarian neoplasms in whom fertility sparing surgery was planned.The procedure for carrying out the frozen section diagnosis was as follows: A laparotomy was performed and the tumor was first removed. The unfixed tumor was immediately delivered to the frozen section laboratory situated in the operation theatre complex with all the clinical details of the patient. After gross examination of the tumor, sections were obtained from representative areas at the discretion of the pathologist. The number of sections frozen ranged from 1 to 4 and depended on the type and size of the tumor. In all tumors diagnosed as borderline at least 2 sections were frozen. This was done in the cryostat instrument. Seven to 8 μm sections were obtained and stained with hematoxylin-eosin and toluidine blue. All the sections were studied microscopically under low and high power by two pathologists. The frozen section diagnosis was conveyed to the surgical team who then proceeded with the appropriate surgery. The average time taken in the entire procedure (sending the sample to obtaining the result) was approximately 10 minutes. The frozen section diagnosis was categorized as one of the following: primary epithelial ovarian neoplasm – benign, borderline or malignant; primary ovarian germ cell tumor, metastatic carcinoma to ovary, benign non-neoplastic conditions and no definite opinion possible. Frozen section diagnosis was compared to the final paraffin section diagnosis.The overall accuracy was defined as the total number of agreements between the frozen section and the final diagnosis divided by the total number of tests performed. For the purposes of this study the final histopathologic diagnoses were assumed to be correct. The sensitivity and specificity and predictive values of frozen section for the diagnosis of various categories of neoplasms were calculated using the standard 2 × 2 method shown in Table Two hundred and forty one patients of ovarian neoplasms underwent frozen section diagnosis. The median age of the patients was 44 years (10–80 years). Twenty four patients had their frozen sections performed on tissues other than ovarian and were excluded from further analysis. Of the remaining 217 patients, the frozen section diagnosis agreed with the final histopathology as to the classification of benign, borderline or malignant in 198 cases (91.2%) and disagreed in 12 cases (5.5%). In 7 cases (3.2%) no definite opinion was possible on frozen section. The reasons for deferment of frozen section diagnoses were as follows: 1) Infarcted and necrotic lesions, and 2) Cystic lesions devoid of lining epithelium.Seven deferred cases were excluded from the further analysis which was performed on the remaining 210 cases. Table Table Correct intraoperative histologic assessment of an ovarian mass is crucial to select an appropriate surgical procedure and avoid under- and over-treatment of the patient. The results of the present study show that frozen section analysis has a high overall accuracy for the diagnosis of ovarian neoplasms (91.2%). Most studies have reported the accuracy of frozen section from 90% to 97% ,5-8. ThiFurther analysis shows that the test fared very well on all 4 conventional indices  in benign conditions and malignant tumors. It is evident that frozen section does not miss the diagnosis of benign and malignant ovarian tumors in the vast majority of patients (high sensitivity) and furthermore frozen section diagnosis of benign and malignant ovarian tumors are correct in the vast majority . Thus only in 3 patients out of the 114 called benign by frozen section, the diagnosis was changed to malignant tumor on the final histopathology . All these were low grade, mucinous tumors on final histology. In only 2 patients out of the 88 patients diagnosed as malignant on frozen section, the final diagnosis was changed to borderline while none was changed to benign . A recently published meta-analysis of eighteen studies comparing frozen section diagnosis of ovarian pathology with the final histopathology showed the sensitivity of frozen section for benign and malignant lesions to vary from 65% to 97% and 71% to 100% respectively [However the situation is different in borderline tumors. The sensitivity and the positive predictive valve of frozen section were 45.5% and 62.5% respectively in the current study. The reported sensitivity of frozen section in borderline tumors varies from 0–87% in various studies -12. VariA recently published systematic review of 14 studies on the accuracy of frozen section in the diagnosis of ovarian tumors also concluded that frozen section has high accuracy rates for the diagnosis of benign and malignant ovarian tumors but the accuracy rates in borderline tumors remains relatively low [We conclude that frozen section is an accurate and useful test in the intraoperative evaluation of patients with suspected ovarian neoplasms. Its results can be used to guide the type and extent of surgery, especially in institutions with experienced pathologists. The accuracy of frozen section is very high for benign and malignant tumors but lower for borderline tumors. Every effort should be made to establish this procedure in institutions that treat large numbers of patients with ovarian tumors.The author(s) declare that they have no competing interests.Dr. Maheshwari conceived the study and was the involved in its planning, data collection analysis and writing. Dr. Gupta was involved with the statistical analysis and writing the study. Drs. Kane, Kulkarni, Goyal and Tongaonkar critically reviewed the paper and were involved in the preparation of the final manuscript.