Parapharyngeal liposarcoma is a very rare malignant tumor that often causes nonspecific clinical symptoms, such as progressive dysphagia, globus sensation and/or respiratory disturbances. The combination of radiological imaging techniques and histopathological analysis provides information for diagnosis; however, the pathogenesis is still uncertain.via a transcervical approach, resulting in a complete amelioration of the patient’s symptoms. Interestingly, immunohistochemistry showed that the tumor cells expressed members of the B7 superfamily, including B7-H1, B7-DC and B7-H3. In addition, the expression of TIM-containing molecules, including TIM-3 and TIM-4, was observed.A 30-year-old male patient presented with a pharyngeal cavity mass, which had been present for 2 years. The clinical syndrome included obstructive sleep apnea symptoms  and difficulty swallowing. The radiological examination (CT) demonstrated that there was a low-density irregular solid lesion on the posterior wall of the oropharynx and laryngopharynx, which descended to the superior mediastinum and extended to the left parapharyngeal space and sternocleidomastoid muscle. The boundaries of the lesion were clear, and the lesion’s density was nonuniform. Several septations inside the lesion were observed. The CT values of the lesion at the epiglottis and the vocal folds were 11 HU and minus 30 HU, respectively. After enhanced scanning, there was no apparent enhancement of the lesion: the surrounding tissue and blood vessels were squeezed and shifted, but the neighboring sclerotin of the cervical vertebrae was not invaded. The mass was removed CT and MRI demonstrated that the mass was a parapharyngeal liposarcoma. Furthermore, carcinoma-associated B7 and TIM-containing molecules were observed in the tissue, indicating that these molecules are most likely active in the pathogenesis of this disease.http://www.diagnosticpathology.diagnomx.eu/vs/1907794973876202The virtual slide(s) for this article can be found here:  Parapharyngeal liposarcoma is a very rare malignant tumor that often causes nonspecific clinical symptoms, including progressive dysphagia, globus sensation and/or respiratory disturbances ,2. TheseThe co-inhibitory molecules of the B7 superfamily, including B7-H1 (PD-L1 or CD274), B7-DC (PD-L2 or CD273), B7-H3 CD276), and B7-H4 (B7x or B7S1), have been demonstrated to actively participate in the regulation of T-cell activation . However, and B7-via a transcervical approach; several homogenous, yellow-tan masses were found, the biggest of which was 7×7×6 cm [Figure A 30-year-old male patient who presented with a pharyngeal cavity mass, which had been present for 2 years, was referred to our department. The clinical syndrome included obstructive sleep apnea symptoms  and difficulty swallowing. The radiological examination (CT) revealed a low-density irregular solid lesion on the posterior wall of the oropharynx and laryngopharynx, which descended to the superior mediastinum and extended to the left parapharyngeal space and sternocleidomastoid muscle. The boundaries of the lesion were clear, and the lesion’s density was nonuniform. Several septations inside the lesion were observed. The CT values of the lesion at the epiglottis and the vocal folds were 11 HU and minus 30 HU, respectively. After enhanced scanning, there was no apparent enhancement of the lesion: the surrounding tissue and blood vessels were squeezed and shifted, but the neighboring sclerotin of the cervical vertebrae was not invaded [Figure Fresh or formalin-fixed paraffin-embedded samples were sliced at 3 μm thick, and the sections were prepared for H&E staining, Sultan-III staining and immunohistochemical detection. H&E staining confirmed that the mass exhibited strong malignant changes Figure C. In add2O2, to reduce endogenous peroxidase activity, and the reaction was visualized with DAB.The sections were pressure cooked for 15 min in 10 mM citrate buffer (pH 6.0) for antigen retrieval, after being dewaxed with xylene and rehydrated using a graded series of ethanol. The sections were then incubated in phosphate-buffered saline (PBS) containing horse serum albumin, and the primary antibodies were added for the reaction, which was performed overnight at 4°C. Then, the second antibody was added, and the sections were incubated at 37°C for 30 min. Finally, the sections were treated with 3% HImmunohistochemistry showed that B7-H1-, B7-DC- and B7-H3-positive cells were present in the sample and were distributed throughout the tissue sections. Specifically, these B7 superfamily molecules were found on cell membranes and in the cytoplasm. In addition to being expressed on liposarcoma cells, B7-DC was also found on the capillaries, whereas B7-H4 was absent from the whole tissue section [Figure Parapharyngeal liposarcomas are extremely rare, and only a few cases have been described in the literature. They are very slow-growing tumors and do not cause symptoms until they reach a large size ,3. The mThe mechanisms responsible for liposarcoma development are very complicated and are not fully understood. Expression of co-stimulatory B7-related molecules by cancer tissues has been reported. B7-related molecules can provide positive or negative signals to local T cells and regulate cancer development. The selective enhancement of T cell activation, using the CTLA-4-Ig protein or PD-1 blocking antibodies, has been demonstrated to be a suitable strategy for cancer immunotherapy . The TIMWe presented a case of parapharyngeal liposarcoma, which revealed the presence of carcinoma-associated B7 and TIM-containing molecules within the tumor tissue. Taken altogether, the data further indicated that these proteins likely actively participate in the pathogenesis of this disease.Written informed consent was obtained from the patient for publication of this Case Report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.None of the authors have any conflicts of interest related to this manuscript.HL participated in the discussion for histological diagnosis and manuscript preparation. XZ collected the clinical data and postoperative clinical follow-up of the patient. QR was responsible for CT detection and LW is preparing the manuscript. All authors read and approved the final manuscript.


Testicular tumors are a heterogeneous group of neoplasms exhibiting diverse histopathology and can be classified as seminomatous and non-seminomatous germ cell tumor types. Mixed germ cell tumors contain more than one germ cell component and various combinations have been reported. Here, we present a rare case of a mixed germ cell tumor composed of seminoma, choriocarcinoma and teratoma with a secondary somatic malignancy.A 31-year-old Caucasian man presented with splenic rupture to our hospital. A right-sided testicular swelling had been present for 6 months and his alpha-fetoprotein, beta-human chorionic gonadotropin, and lactose dehydrogenase were increased. An ultrasound of his scrotum revealed an enlarged right testis with heterogeneous echogenicity. Multiple hypervascular lesions were noted in his liver and spleen. He underwent transcatheter embolization therapy of his splenic artery followed by splenectomy and right-sided orchiectomy. A computed tomography scan also showed metastasis to both lungs. During his last follow up after four cycles of cisplatin-based chemotherapy, the level of tumor markers had decreased, decreases in the size of his liver and pulmonary lesions were noted but new sclerotic lesions were evident in his thoracolumbar region raising concern for bony metastasis.Prognosis of testicular tumor depends mainly on the clinical stage, but emergence of a sarcomatous component presents a challenge in the treatment of germ cell tumors and the histological subtype of this component can be used as a guide to specific chemotherapy in these patients. Testicular tumors are a heterogeneous group of neoplasms exhibiting diverse histopathology, variable clinical course and prognosis. Of thesA 31-year-old Caucasian man was referred to our hospital for splenic rupture with active arterial extravasation. During clinical evaluation, he mentioned right testicular swelling; the swelling had been enlarging for 6 months. Transcatheter embolization therapy of the inferior pole of his splenic artery was performed for what was clinically thought to be a hypervascular metastasis in the spleen. Multiple hypervascular lesions were also noted in his liver. An ultrasound of his scrotum revealed an enlarged right testis with heterogeneous echogenicity measuring 5×4×3cm. Serum tumor markers revealed a beta-human chorionic gonadotropin (hCG) of 3804mIU/mL (<5mIU/mL), lactose dehydrogenase (LDH) of 196U/L (100 to 190U/L) and an alpha-fetoprotein (AFP) of 47.7ng/mL (0.0 to 9.0ng/mL). He underwent right-sided orchiectomy and splenectomy. No lymph node dissection was performed.On gross pathological examination, a 5.1×5×2.5cm heterogeneous testicular mass, exhibiting tan-white to yellow to hemorrhagic areas was evident. No gross involvement of his tunica albuginea or spermatic cord was noted.Histopathology showed a mixed GCT, confined to the testis containing seminoma, classic type  for patients having a SC in their GCT when compared to age and stage-matched patients with GCTs without SC[et al. emphasized in their study the importance of histological type of SC as a guide to specific chemotherapy in their patients, for example GCT with rhabdomyosarcoma responds well to a doxorubicin-based regimen whereas GCTs with secondary adenocarcinoma benefit from 5-fluorouracil-based chemotherapy[et al. suggested in their study that the SC confined to the primary testicular GCT may not have as high a risk of mortality as those at a comparable stage without a SC[Development of SC presents a challenge in the treatment of testicular GCT. Malagón thout SC. Many stthout SC-15. Donaotherapy. Guo et out a SC. They fuWe report the first known example of a GCT with an unusual combination of seminoma, choriocarcinoma and teratoma with a somatic malignancy. This case not only reflects the heterogeneity in the biology of GCT of the testis but also highlights the aggressiveness of these tumors and challenges in treatment due to the emergence of a SC in them.Written informed consent was obtained from the patient for publication of this manuscript and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.AFP: Alpha-fetoprotein; GCT: Germ cell tumor; hCG: Human chorionic gonadotropin; LDH: Lactose dehydrogenase; SC: Sarcomatous component.The authors declare that they have no competing interests.AA performed the gross examination of the specimen, AA and SB both conceived the case report, searched the literature and drafted the manuscript. SI performed the histopathological evaluation of the slides and made substantial revisions to the manuscript. JM operated on our patient and made revisions to the manuscript. All authors read and approved the final manuscript.


Although a metastatic presentation of an occult prostatic adenocarcinoma is not uncommon, the majority of these patients present with bone metastasis affecting the axial skeleton. Cranial metastases to the paranasal sinuses are extremely rare. A 56-year-old man presented with loss of vision and numbness of the right side of the face. Computed tomography (CT) scan and cranial magnetic resonance imaging (MRI) revealed a mass invading the sphenoid sinus. The patient underwent surgery to remove the lesion, and the histopathological examination suggested metastasis of an adenocarcinoma, with positive staining to prostatic specific antigen (PSA). However, serum PSA was 4 ng/mL, and the patient did not report any lower urinary tract symptoms or bone pain. Transrectal ultrasound-guided biopsy revealed prostatic adenocarcinomas with a Gleason score of 8 [4 + 4]. The subsequent treatment consisted of radiotherapy and androgen deprivation, followed by first- and second-line chemotherapy (docetaxel and cabazitaxel) when the disease progressed. The patient achieved a good response with the last cycle of cabazitaxel and after a 5-year followup is currently alive. Cranial metastases of prostate adenocarcinoma are rare, and there is currently no standard treatment for these patients. Whenever possible, surgery combined with radiotherapy and hormonotherapy is the recommended option. Metastatic tumors to the paranasal sinuses are rare. Kidney , lung, breast, testis (seminoma), gastrointestinal tract, and thyroid gland are, in order of frequency, the most common locations of the primary tumors that give origin to these metastases –3. The sA 56-year-old male with no previous medical history of interest presented with a chief complaint of gradual right vision loss and numbness of the right side of the face. Computed tomography (CT) scan and cranial magnetic resonance imaging (MRI) revealed a 4.5 × 4.5 × 3 cm mass in the right greater wing of the sphenoid bone invading the anterior pole of the temporal lobe and the sphenoid sinus . A radicPrimary sinusal tumors account for approximately only 0.3% of all tumors . MetastaSome authors estimate that up to 10–20% of prostatic tumors are firstly diagnosed by their metastatic manifestations . The wayThe clinical presentation of the metastases in paranasal sinuses is similar to primary tumors in the same location. Some of the most frequent symptoms are headache, loss of vision, diplopia, facial numbness, loss of hearing, and other symptoms related to cranial pairs affection (II to VIII) , 3, 7. EThe CT scan and the MRI are essential for the diagnosis of metastases within the paranasal sinuses, as they reveal the size of the lesion and its extension to the adjacent structures, such as the orbit or the brain. There is not any specific radiological sign to differentiate metastases from a primary intracranial tumor. For instance, radiological features of a meningioma en plaque could be very similar to those of a retroorbital metastasis of a prostatic adenocarcinoma, as they both share an osteoblastic pattern. Therefore, histopathology study is crucial to reach a correct diagnosis, showing a sinusal mucosa with unspecific glandular architecture and positive to anti-PSA staining .The elective treatment depends on the stage of the disease and the general condition of the patient. Due to the shortage of cases, there is no current standard of care for these patients. On the other hand, the early start of the treatment seems to produce a better control of the symptoms . RadiothThe prognosis of metastatic prostatic disease to paranasal sinuses is not well documented, although it is generally considered to be unfavorable . A revieTo conclude, we want to highlight that prostate cancer should always be considered within the differential diagnosis of any mass appearing in the cranial bones of aged patients with any neurological disorder, even if no urological symptoms are reported. Correct diagnosis is essential, as these patients may achieve prolonged survival with early treatment.


We present a case of sternal steel strut dislodgement and migration in a patient undergoing Ravitch repair for pectus excavatum (PE) 37 years ago. Broken struts perforated the right ventricle and right ventricular outflow tract (RVOT) and additionally migrated into the left upper lobar bronchus.Dislodged sternal struts represent rare complications after surgical repair of patients suffering from pectus excavatum. Reviewing the literature, only five cases of intrapericardial migration of dislodged sternal struts or wires have been reported so far.In our case, the first strut was removed from the airways through a left antero-lateral thoracotomy. Using cardiopulmonary bypass, a second strut was removed via ventriculotomy. These life-threatening sequelae underscore the importance of postoperative follow-up and early removal of osteosynthetic materials used in open PE repair. Accurate preoperative localization of migrated materials and availability of CPB support are crucial for successful surgical removal.The migration of dislodged sternal steel struts or wires into the pericardium and cardiac cavities is a rare but life-threatening complication of open pectus excavatum (PE) repair . Removal A 53-year-old male with known persistent atrial fibrillation was admitted with progressive dyspnea and malaise, aggravated in sitting and stooping position. He had undergone surgical Ravitch repair for PE deformity 37 years ago.Preoperatively, the chest radiograph demonstrated multiple dislodged steel struts and wires. In the lateral view, several struts seemed to be dislocated in the middle mediastinum Figure . CT scanRemoval of the steel struts was undertaken under general anesthesia with endobrochial double lumen tube. After a left antero-lateral thoracotomy, pericardiotomy was performed over the left hilum with preservation of phrenical nerve. Careful dissection exposed a steel component right next to the pulmonary trunk Figure . ObviousAs internal stabilization, metal struts or wires have been widely used in open PE repair. The struts are used to stabilize the sternum at the desired level, reduce the incidence of recurrent sternal depression and prevent paradoxical respiration . Strut rReviewing the literature, in addition to the summary of Tahmassebi et al., only two case reports on intrapericardial migration of sternal wires or struts following open PE repair are presented so far . Cope etThe previously reported intrapericardial migration of sternal struts or wires occurred between nine months and 28 years after initial open PE repair. In the present case, intracardiac migration of steel struts was found even 37 years after initial surgery. It underscores again the importance for early removal of sternal struts or wires, if chest wall stability is sustained. Distinguished from sternal wires, steel struts seemed to be able to migrate deeper and result in perforation into the cardiac cavities, probably due to their size and inflexible character. The presenting symptoms of intrapericardial injury are variable and could be unspecific. However, in three out of six cases, patients were admitted due to sudden chest pain. The intrapericardial injury was mostly described at the right ventricle. Dalrymple-Hay et al. reported a patient with multiple embolic events nine months after Ravitch repair. The useSurgical approaches for removal of migrated materials in pericardium depend on the location of the struts and the injured structures. CPB support or steady state was required in most reported cases and the present case as well. The migrated material could be removed through directional pull followed by defect closure via running sutures. However, aortotomy and atriotomy were required for removal of the thrombus in the left ventricular cavity in the case reported by Dalrymple-Hay et al.  In the pIn summary, intrapericardial migration of sternal struts or wires is a rare, but severe complication of open PE repair. Accurate localization of migrated materials by means of 3-D reconstruction of CT scan images and steady state of CPB are crucial for a successful surgical removal.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.RZ conceived the study, provided the information of the patient, performed literature search, wrote and reviewed the manuscript, CH was the operating surgeon of the patient and participated in the coordination of this study, DB participated in literature search, drafted the manuscript, TB operated the patient and participated in drafting the manuscript, JDS participated in literature search, drafted and reviewed the manuscript, AH participated in drafting the manuscript, supervised and reviewed the manuscript, MK participated in drafting the manuscript, supervised and reviewed the manuscript.All authors read and approved the final manuscript.


Pulmonary blastoma is a rare primary lung neoplasm, in that monophasic variety is far too rare. There are no specific clinical features seen for pulmonary blastoma; computed tomography and histopathology are diagnostic. Surgical excision is the treatment of choice; however, adjuvant chemotherapy and radiotherapy may be required in large and aggressive tumors. Pulmonary blastomas are relatively rare group of primary lung neoplasms with disputed histogenesis and variable biologic behavior that are composed of immature malignant epithelial and/or mesenchymal tissues whose features may resemble early embryological lung tissues. Since the first report by Barrett & Barnard, in 1945, many more cases of pulmonary blastoma have been reported. PulmonaA young healthy female of 18 years presented with left sided chest pain not related to exertion since one month. Examination of respiratory system revealed decreased air entry on left side over mammary and supra-mammary regions. Chest X-ray showed about 10 cm sized rounded opacity on left upper quadrant (See Figure Left postero-lateral thoracotomy through fifth intercostal space was done. Approximately 15×15 cm sized mass occupying entire left upper lobe (See Figure Pulmonary tumors of embryonic origin are rare and among them, pulmonary blastomas are probably the most uncommon. They foApproximately 25% to 40% of patients are asymptomatic at presentation, with incidental diagnosis by chest radiography. Common symptoms are cough, hemoptysis and chest pain while pleural effusion is seen unusually. Present case was having chest pain as a main complaint. The majority of pulmonary blastomas have been reported in adults, occurs mainly in young women. The monRadiography shows a well-demarcated peripheral lesion, well defined homogenous peripherally placed radiopacities with the lesions ranging from 2.5 to 25 cm,6. On ulOn gross pathology, the tumor is rounded, well circumscribed. In some, it may show lobulated appearance and multiple lesions. On immunohistochemistry expression of cytokeratin and vimentin is seen in the blastomatous component, the epithelial elements in blastomas react positively for keratin, carcinoembryonic antigen, epithelial membrane antigen, and milk fat globulin. In the Surgery is the preferred treatment and is mandatory wheneverOverall, survival is 25% at 1 year and 16% at 5 years. Factors that indicate a poor prognosis are tumour recurrence, metastases at initial presentation, tumor greater than 5 cm, and lymph node metastases[Monophasic pulmonary blastomas are rare neoplasms with good overall prognosis. Surgical excision is the treatment of choice and produce better prognosis with adjuvant chemotherapy.Written informed consent was obtained from the patients for publication of this Case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.CT scan: Computed tomogram.The authors declare that they have no competing interests.JHM drafted the manuscript, SBP analyzed and interpreted the patient data, HM performed the performed the surgery and was a major contributor in writing the manuscript, AFP conceptualize the manuscript and PNM involved in the crucial revisions of manuscript. All authors read and approved the final manuscript.


In thisreport, we present the first case of extrauterine adenomyoma involving the liverin a 29-year-old woman who presented with a 2-year history of low back pain withrecent worsening and a history of laparoscopic myomectomy 5 yearspreviously. Gross inspection of the specimen revealed a subcapsular mass thathad a well-circumscribed margin with the adjacent liver tissue. Byhistopathologic examination, the multilobular mass was composed of a smoothmuscle component and benign endometrioid glands and stroma. The smooth musclecomponent was focally cellular, and the endometrioid glands had secretoryfeatures. Both the smooth muscle component and endometrioid tissue were positivefor ER and PR. The smooth muscle component was also positive for desmin and SMA,while the endometrioid stroma was positive for CD10. Other extrauterine lesionscomposed of a mixture of smooth muscle tissue and heterotopic endometrioidtissue, including endometriosis with a smooth muscle component,leiomyomatosis/leiomyomas associated with endometriosis and uterus-like masses,should be included in differential diagnoses. The patient was free fromrecurrence 5 months after liver tumor resection. Adenomyomas are benign tumor-like masses composed of smooth muscle tissue and benignendometrioid glands and stroma. These tumors most commonly originate from within theuterine corpus. Adenomyomas in extrauterine sites are extremely rare. To the best ofour knowledge, since it was first reported in 1986, only 14 cases of extrauterineadenomyoma have been reported in the English literature, most often occurring in theovary .In this report we describe the first case of extrauterine adenomyoma of the liver ina 29-year-old woman with a history of laparoscopic myomectomy. This is also thefirst case of a solitary extrauterine adenomyoma arising in an extra-pelvic site. Wealso present four major theories from the literature to explain the pathogenesis ofextrauterine adenomyoma: Müllerian duct fusion defect, sub-coelomic mesenchymetransformation, müllerianosis and endometriosis with prominent smooth musclehyperplasia or metaplasia.A 29-year-old married non-pregnant woman (P0G0), presented with a 2-year historyof low back pain that had worsened over the past 2 months. There were noremarkable findings on physical examination. The patient had a history ofuterine leiomyoma and had undergone laparoscopic myomectomy 5 yearspreviously.Abdominal ultrasonography revealed a 3.6 × 2.5 cmhypoechoic solid mass arising from the posterior right lobe of the liver. Noother abnormality was detected on pelvic or abdominal ultrasonography. Asubsequent CT scan demonstrated a patchy area with slightly lower density in theperipheral zone of the posterior right lobe of the liver. Contrast-enhanced CTshowed heterogeneous enhancement of the lesion in the arterial phaseFigure  . LaboratExploratory laparotomy revealed a solid, firm mass that was located at thesubcapsular region in segment VI of the right liver near the right kidney. Therest of the liver and the other pelvic and abdominal organs appeared normal. Themass was completely removed by liver tumor resection. A frozen section wasperformed and interpreted as a spindle cell tumor, but primary and metastaticsarcoma of the liver could not be excluded. The patient was doing well and wasfree from recurrence 5 months after surgery.Gross inspection of the specimen revealed a3.6 × 2.6x1.8 cm white-gray, irregular mass, which waspartially covered with the hepatic capsule and partially surrounded by normalyellow-gray liver tissue. The mass was firm and solid, with scattered cysts andfoci of congestion and hemorrhage up to 4 mm in diameter according to the immunohistochemical staining (ER/PR positive) and the history ofmyomectomy. However, BML is most frequently involving the lung , and endThe morphologic criteria for distinguishing extrauterine benign Müllerian-typesmooth muscle component from leiomyosarcoma are not well described. The currentapproach is to apply criteria of the corpus uteri to their extrauterinecounterparts, and several potential indicators have been reported to be helpful inthe differentiation -39.The pathogenesis of extrauterine adenomyomas or uterus-like masses remains uncertain.We present four major theories from the literature. he Müllerian duct fusiondefect theory has been proposed to explain the etiology ,4-6. ThiThe preoperative diagnosis of extrauterine adenomyoma is difficult because of itsrarity and non-specific clinical and radiological findings. Primary or metastaticmalignancies should be included in the preoperative differential diagnosis.Moreover, although the malignant transformation of extrauterine adenocarcinomas hasnot been reported, it is an uncommon but possible event in endometriosis,particularly in liver endometrioma. For example, one case of in situ adenocarcinoma,one case of adenosquamous carcinoma and two cases of adenosarcoma were considered toarise in the setting of liver endometrioma -22. A utIn summary, adenomyomas in extrauterine sites are extremely rare. We present thefirst case of an extrauterine adenocarcinoma with a cellular smooth muscle componentinvolving the liver. Such masses could be easily misinterpreted in the preoperativediagnosis and intraoperative frozen diagnosis. The resected tumor specimen shouldfirst be differentiated from other extrauterine lesions, which are composed of amixture of smooth muscle tissue and heterotopic endometrioid tissue. In this case,the sub-coelomic mesenchyme theory was favored for pathogenesis, and we propose thatdifferentiation from sub-coelomic mesenchyme to adenocarcinoma may be induced byhormonal and/or surgical stimulation.To publish this case report and accompanying images, written informed consent wasobtained from the patient’s family.The authors declare that they have no competing interests.HW W was the main author on the paper, took the clinical images, worked up the caseand drafted the manuscript. H Z and XW X conducted the immunohistochemical study. ZHL was the main pathologist involved in the case, made the finally diagnosis and wasthe main editor of the body of the text. All authors read and approved the finalmanuscript.


Low-grade central osteosarcoma is a rare variant of osteosarcoma which comprises less than 1-2% of all osteosarcomas. Most low-grade osteosarcomas involve long bones, most commonly distal femur, and proximal tibia. Histologically this tumor is difficult to diagnose, and an unusual location makes this diagnosis even more challenging. Here we report a case of low-grade osteosarcoma presenting as a chest wall mass involving the left 6th–8th ribs. This unusual site of presentation significantly added to the diagnostic difficulties of this rare tumor with challenging histologic features. To the best of our knowledge, only six cases of low-grade central osteosarcoma of the ribs have been reported in the English literature. Low-grade central osteosarcoma (LGCO) is a very rare variant of osteosarcoma. The most common location is the long bones with the distal femur and proximal tibia being the most common sites .Rare incidence and overlapping pathological and radiological features of this tumor make the diagnosis difficult. We present a rare case of LGCO of the rib in a 33-year-old male with a brief review of the literature and emphasize on diagnostic difficulties, differential diagnosis, and treatment. Six other cases of LGCO of the rib have been previously reported in the English literature , 3.A 33-year-old male presented with a gradually growing mass in his chest wall. The mass was involving the left seventh rib in approximately the mid to anterior axillary line. A chest computed tomography (CT) scan revealed a large left lateral 7th rib mass with an osteoid matrix and sunburst pattern, highly suspicious for a primary osteosarcoma .An enbloc resection of the mass was performed. Gross examination revealed a 4.0 cm hard mass with a gray-white gritty cut surface, predominantly involving the medullary cavity of the seventh rib with extension to the adjacent sixth and eighth ribs . No necrMicroscopic examination showed long sweeping fascicles of moderately atypical fibroblastic cells surrounding streamers of woven bone. No necrosis identified. Rare mitotic figures are noted . The tumLow-grade central osteosarcoma (LGCO) is a very rare tumor and was first described in 1977 by Unni et al. .Most of the patients present with a slow-growing mass with associated pain. Males and females are equally affected.Patients with LGCO are older than those with conventional osteosarcoma with a peak incidence in the third decade of life .The most common site of involvement is the intramedullary cavity of metaphysis or diametaphysis of the long bones . Rare caThis tumor has a significant better prognosis in comparison to conventional osteosarcoma. Treatment with wide local excision and resection essentially is curative versus marginal excision or curettage which almost always results in recurrence with possible transformation into a high-grade dedifferentiated osteosarcoma in about 15% of the cases . TherefoInitial diagnosis of this tumor in a small biopsy can be extremely challenging, and multiple biopsies and sometimes consult with a specialist are required in order to establish a correct diagnosis before a definitive excisional surgery is performed. In a study by Andresen et al., some cases required several biopsies in order to make the diagnosis of LGCO, even in a primary referral. Based on their experience only six of the 11 patients treated primarily had a clear diagnosis of LGCO after the first biopsy .Four radiological patterns have been described for this tumor by Malhas et al. . includiDiagnosis on imaging study might be difficult and can look benign with usually some focal aggressive features .This tumor can be confused radiologically with fibrous dysplasia, desmoplastic fibroma, nonossifying fibroma, osteoblastoma, and aneurysmal bone cysts –10. The main differential diagnoses are fibrous dysplasia and desmoplastic fibroma .The main histologic pattern differentiating this tumor from fibrous dysplasia is a permeative growth pattern encasing preexisting trabecular bone. Besides extension to the adjacent soft tissue is another feature which is not associated with fibrous dysplasia .Any bone formation will exclude desmoplastic fibroma .MDM2 and CDK4 and are due to telomeric deletions [LGCO has a simple karyotype with supernumerary ring chromosomes, which are composed of 12q13-15 clusters containing MDM2 and CDK4 immunostains with a very high specificity and sensitivity. These markers can be used in difficult situation especially in small biopsies to exclude the other fibrous or fibro-osseous benign lesions [In two recent studies by Yoshida et al. and Dujardin et al., it is shown that LGCOs are frequently positive for  lesions , 12.In conclusion, LGCO is a treatable malignant tumor with a high chance of cure with appropriate preoperative diagnosis and adequate surgical treatment. Newly introduced CDK4 and MDM2 immunostains can be helpful especially in the small biopsies. Sometimes multiple biopsies and open surgical biopsies are required for an accurate initial diagnosis. In the event of difficult cases with an unusual site of presentation, like our patient, consult with an expert is necessary in order to make an appropriate diagnosis.


Relapsing lymphoma involving the trachea causing tracheal obstruction is exceedingly uncommon. Despite its rarity, it should be considered in the differential diagnosis when a patient with known lymphoma presents with signs of airway obstruction such as stridor. We report an unusual case of relapsing non-Hodgkin's lymphoma with tracheal involvement in a 57-year-old female and review the relevant literature. It is highly unusual for relapsing lymphoma to involve the trachea causing tracheal obstruction. Despite its rarity, it can present with life-threatening airway obstruction which may be rapidly progressive requiring immediate surgical intervention such as tracheostomy. The most common etiologies of acute upper airway obstruction include infection, neoplasms, trauma, foreign bodies, and angioedema. Tracheal neoplasm accounts for less than 0.1% of all malignancies . AlthougIn non-Hodgkin's lymphoma, diffuse large B-cell lymphoma is the most common subtype which accounts for approximately 25% of cases . The incWe report the first case in the literature of relapsing non-Hodgkin's lymphoma in the trachea who presented with an acutely threatened airway requiring immediate surgical tracheostomy.A 57-year-old female with a background of B-cell non-Hodgkin's lymphoma in remission presented to the emergency department with rapidly progressive noisy breathing and dyspnoea that was worse when supine over the last 7 days. There were associated symptoms of cough and hemoptysis as well as constitutional symptoms including fever, night sweats, and weight loss over this period. She denied voice changes, dysphagia, odynophagia, or trismus.The patient was originally diagnosed with B-cell non-Hodgkin's lymphoma 6 months prior and had undergone six cycles of chemotherapy according to the German Multicenter Study Group for Adult Acute Lymphoblastic Leukemia (GMALL) 2002 protocol.On review, she was afebrile with audible biphasic stridor. Both the stridor and dyspnea were significantly exacerbated in the supine position. On nasal endoscopy, there was a large round subglottic mass causing near-complete (>80%) luminal obstruction of the airway . Chest XHer airway was urgently secured with an awake tracheostomy in the operating theatre, followed by microlaryngoscopy, esophagoscopy, and bronchoscopy. The large flesh-colored subglottic mass was biopsied and debulked with a laryngeal Skimmer Blade . There were no other synchronous tracheal tumors or upper esophageal lesions. Pathology from the tracheal mass revealed cells staining positive for CD 20  and MUM Six days later, a second operation was performed for endoscopic evaluation and change of tracheostomy tube. Intraoperatively, the debulked tumor was noted to have rapidly regrown, reaching its original size, with evidence of distal extension, confirming its aggressive nature. The patient underwent palliative chemotherapy  and radiotherapy with clinical and radiological remission at 4 months. Whilst she met all decannulation parameters, she remained tracheostomized due the aggressive nature of the disease and high risk of recurrence.Even though extranodal lymphoma is reasonably common , a primary presentation of extranodal lymphoma involving the trachea is extremely unusual. Seven series with a total of 425 tracheal tumors between 1930 and 1989 reported only 1 case of non-Hodgkin's lymphoma  . RelapsiTracheal lymphoma can cause symptoms and signs of upper airway obstruction , mucosal ulceration, and irritation . Given the submucosal location of tracheal lymphomas, hemoptysis is uncommon. Airway obstruction has been reported in 87% of these patients, with half of them requiring emergency intervention .Chest X-ray is commonly obtained as part of the initial radiological investigation but is rarely diagnostic, and tumors may be easily missed. CT is the most useful imaging modality for the assessment of tracheal tumors and is the gold standard imaging technique for the diagnosis and evaluation of tumor extent. Bronchoscopy is usually performed to diagnose and stage tracheal tumors as well as obtaining tissue samples.Reports in the literature of relapsing lymphoma involving the trachea are exceedingly rare. To date there have been 4 published cases –9 summarIt is highly unusual for relapsing lymphoma to involve the trachea causing tracheal obstruction. Despite its rarity, it can present with life-threatening airway obstruction which may be rapidly progressive requiring immediate surgical intervention such as tracheostomy. Clinicians should be aware of this possibility, as our case necessitated prompt securing of the patient's airway.


Cystic meningiomas are rare variants of meningiomas; they can pose a radiological diagnostic dilemma.We present a rare case of a 30-year-old Chinese woman with a histopathological diagnosis of infratentorial cystic meningioma  in which the features in imaging modalities were suggestive of a hemangioblastoma. Intraoperatively, however, the gross macroscopic features were more in keeping with a pilocytic astrocytoma.In benign cystic meningiomas, particularly the infratentorial variety, radiological findings utilizing the various imaging modalities and intraoperative impressions may not be reflective of or in keeping with the final histopathological diagnosis. Meningiomas are the most common primary non-glial intracranial tumors. They arA 30-year-old Chinese woman with a childhood history of acute lymphoblastic leukemia, currently in remission, presented to our Neurology clinic with a 3-week history of worsening headaches, giddiness and vomiting. The neurological examination revealed positive cerebellar signs in keeping with an infratentorial space-occupying lesion.A computed tomography (CT) head scan revealed a large 4.0cm×4.1cm×4.8cm) posterior fossa cystic mass with an eccentrically located enhancing nodule measuring 1.5cm×1.9cm  confirmed the cystic mass in the posterior fossa with an eccentrically located nodule lying adjacent to the vein of Galen. This nodule was hyperintense on T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences, hypointense on T1 weighted and showed avid enhancement with gadolinium Figure  also shoThe tumor was approached via the infratentorial supracerebellar corridor with the patient in a prone position. Frameless neuronavigation using a fine-cut (1.2mm) contrast-enhanced CT brain scan was utilized in planning the surgical access. Gross total excision of the tumor was achieved under microscope assistance. The cystic component of the lesion contained xanthochromic fluid. The well-circumscribed solid component was a greyish, soft vascular lesion attached to the undersurface of the tentorium cerebelli. The wall of the cyst was smooth with no nodular lesion, suggestive of a pilocytic astrocytoma. The wall was not excised. The deep venous complex was visualized and preserved. A blood transfusion was not needed. The symptomatology improved post-operatively. She was discharged on the 4th post-operative day.A pathological examination revealed that the tumor tissue was composed of meningothelial cells in sheets and rudimentary whorls Figures  and5. Tet al.[A different classification of cystic meningioma has been used to define the site of cystic cavity in relation to tumoral lesion. According to Nauta et al. cystic m1. Centrally located intratumoral cysts2. Peripherally located intratumoral cysts3. Peritumoral cysts in the adjacent parenchyma and4. Peritumoral cysts between the tumor and the adjacent parenchymaVarious pathophysiological mechanisms of cyst formation in meningiomas have been postulated. Peritumoral cyst and intratumoral cyst formation have different pathophysiological mechanisms. Peritumoral cysts may be caused by reactive gliosis, fibroblastic proliferation, the final stage of the intense peritumoral edema, a widening of the subarachnoid space, or mechanical trapping of the cerebrospinal fluid spaces. ConversIn solid meningiomas the sensitivity of a CT brain scan is virtually 100% and the specificity is 90%, however, with cystic meningiomas, the diagnosis is made pre-operatively in less than 38% of cases. The preClassically the MR imaging characteristics of a cystic meningioma have been described as an extra-axial lesion with enhancement of solid component and presence of a dural tail, which iAn MRS of the enhancing solid component in this cystic meningioma did not help in differentiating between cystic meningioma and hemangioblastoma as both tumors showed an almost similar reduced N-acetylaspartate peak with increased choline-to-creatine ratio. It has been reported that the presence of high mobile lipid on proton MRS is more suggestive of hemangioblastoma, which was not seen in this case. However, the lipid peak on the MRS is non-specific and is also observed in other high-grade tumors such as high-grade gliomas, metastatic tumor, and anaplastic meningioma.In benign cystic meningiomas pertaining to the infratentorial variety, radiological findings utilizing the various MRI sequences and MRS and intraoperative impressions may not be reflective or in keeping with the final histopathological diagnosis.Written informed consent was obtained from our patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.TKG was the major contributor in writing the manuscript. DP analyzed and interpreted the patient’s data. KHT performed the histological slide preparation and interpretation. GK reviewed and interpreted the biomedical imaging results. HC and DG are the senior authors who performed the surgery, contributed and supervised the writing of the manuscript. All authors read and approved the final manuscript.


Extrarenal angiomyolipomas (ERAMLs) are rare tumors that present as incidentalomas upon imaging for other conditions. Retroperitoneal ERAMLs present a unique challenge from a diagnostic and treatment standpoint as they can mimic other benign and malignant retroperitoneal tumors. We present a case of a 39-year-old female with a 19.3 cm × 13.5 cm × 10.7 cm left extrarenal retroperitoneal mass. Histopathologic examination and HMB-45 staining revealed the mass to be a retroperitoneal ERAML. Our case report provides a comprehensive literature review and an evidence-based algorithm for taking care of patients with ERAMLs.  Extrarenal angiomyolipomas (ERAMLs) represent a very rare subset of tumors that often present as incidentalomas upon imaging for other conditions. Lesions located in the retroperitoneum present a unique challenge from a diagnostic and treatment standpoint as they can mimic other malignant retroperitoneal lesions. We present a case of a 39-year-old female with a 19.3 cm × 13.5 cm × 10.7 cm left extrarenal retroperitoneal mass detected on a follow-up chest computerized tomography, obtained after prolonged treatment for pneumonia. Histopathologic examination and HMB-45 staining demonstrated the mass to be an extrarenal angiomyolipoma. A comprehensive literature review of ERAMLs as well as evidence-based care algorithm is provided.Angiomyolipomas (AMLs) are common primary renal parenchymal tumors which comprise about 1% of all renal masses. In contrast, extrarenal angiomyolipomas (ERAMLs) represent extremely rare tumors with less than 60 reported cases since they were first described by Friis and Hjortrup in 1982  iastinum, , 34 duodRetroperitoneal ERAMLs present a unique diagnostic challenge since they must be distinguished from other retroperitoneal masses including retroperitoneal sarcomas, atypical lipomas, adrenal adenocarcinomas, leiomyomas with fatty change, and renal cell carcinomas. Although the majority of ERAMLs are benign, 2 cases of metastatic and recurrent ERAMLs have been reported. Gupta et al.  describe3 to 7980 cm3 and weighing between <1 kg and 11 kgs. The majority of cases (69%) were managed surgically via en bloc radical nephrectomies and in 4 cases a renal-sparing resection was performed. One case was managed with embolization without resection.To date, only 16 cases of retroperitoneal ERAMLs (including our case) have been reported, making the retroperitoneum the second most common extrarenal location of AMLs . Among pen bloc nephrectomy with distal metastasis to liver and bone 12 months postoperatively [en bloc radical nephrectomy, these lesions should be followed closely with CT imaging during the first year after resection, with continued yearly followup for 5 years or dictated by symptoms.56% of patients had follow-up evaluation ranging from 2 to 60 months after surgical resection. Outside the context of tuberous sclerosis, the only reported recurrence to date happened after a radical ratively . All othen bloc nephrectomy. Since the longest documented follow-up duration for an ERAML is 5 years, it is recommended that patients undergo serial CT imaging for the first year and be followed for 5 years based on symptoms. The ERAML reported in this paper measured 23 cm × 14 cm × 9 cm (2898 cm3), weighed 1.7 kgs (Extrarenal angiomyolipomas are rare and occur most commonly in the liver; however, the retroperitoneum is the second most common location. Lesions in the retroperitoneum present a unique diagnostic challenge since they can mimic other retroperitoneal benign and malignant tumors, which must be differentiated. CT scans and MRIs are the diagnostic imaging modalities of choice and are useful in delineating the anatomical relationship of these lesions to the kidney and its vasculature. Hemodynamically stable patients should undergo surgical resection, while unstable patients may benefit from emergent tumor embolization and a subsequently staged surgical resection. Once the pathology specimen is obtained, immunoreactivity to an HMB-45 stain is a useful tool to differentiate ERAMLs from other retroperitoneal tumors. ERAMLs should be analyzed from mitotic index and the presence of epitheloid variant as these characteristics may be associated with distal metastasis and disease recurrence. To date, only one case of disease recurrence and distal metastasis has been documented in a patient with tuberous sclerosis. This occurred in an 80-year-old female one year after a radical  1.7 kgs , and did


Tufted angioma, first recognized in Japanese literature as “Angioblastoma of Nagakawa”, is a rare benign vascular tumour with a variable clinical presentation. It commonly manifests as a macule, papule or nodule in infancy or childhood in the region of the upper trunk and neck. Here in we report two cases of this rare progressive angioma as lesions of the eyelid in adults. Tufted angioma has a classical “cannon ball” like appearance of vascular tufts on histopathology. Immunohistochemical staining with actin highlights the spindly stromal cells surrounding the capillaries. Complete physical examination and haematological work up is recommended in patients with tufted angioma to exclude rare association of port wine stain and Kasabach-Merritt syndrome with this rare entity. To the best of our knowledge, our cases illustrate the first case report of tufted angioma presenting as an eyelid lesion.http://www.diagnosticpathology.diagnomx.eu/vs/1230909536950947.The virtual slide(s) for this article can be found here:  Tufted angioma, first recognized in Japanese literature as “Angioblastoma of Nagakawa”, is a benign progressive angioma, with a variable clinical presentation . Wilson-A 17-year-old male presented with a history of a gradually progressive, painless swelling of right lower eyelid of 6 years’ duration. There was no history of trauma, previous ocular surgery or presence of similar lesion elsewhere in the body. Clinical examination revealed a right lower eyelid soft tissue mass not fixed to the underlying tarsus. It was non-tender and spongy to firm on palpation. On eversion of the eyelid the lower palpebral conjunctiva was unremarkable. Rest of the ocular examination was normal. A vascular lesion, probably a lymphangioma, was suspected. Systemic examination and complete blood picture was normal and a biopsy was planned. At biopsy under local anesthesia, the mass appeared o be reddish and ill-defined with intermingling soft and firm areas. Piecemeal excision of the lesion was done and the excised tissue was submitted for histological examination.A 44-year-old female presented with a slowly progressive painless swelling of the right upper eyelid of about 3 years’ duration. There was no other significant ocular or systemic history. Clinical examination revealed a mobile, non-tender, and cystic to firm nodular mass, not fixed to the tarsus. Systemic evaluation and complete blood picture was normal. Clinically, the lesion was thought to be an epidermal cyst. At biopsy under local anesthesia, a nodular, well delineated, non-encapsulated, firm mass about 15 mm in its maximum dimension was excised in Toto and submitted for histological evaluation.Histopathology sections from case 1 showed multiple fragments of stromal tissue of eyelid with adnexal structures, striated muscle bundles of orbicularis oculi Figure . CapillaHistopathology sections from case 2 showed a single fragment of stromal tissue of eyelid with striated muscle bundles of orbicularis oculi and closely set relatively circumscribed ovoid to round angiomatous aggregates. These angiomatous aggregates were composed of blood less capillaries with slit like or mildly dilated lumina, lined by plump bland endothelial cells Figure . These cImmunohistochemical staining in both cases showed similar findings. Staining with actin revealed a prominent pericytic component Figure . CD34 stComplete physical examination by a dermatologist was normal. Both cases have not had a recurrence in over 12 months of follow up.Tufted angioma is a rare, slowly progressive benign vascular tumor which can have variable clinical morphology. It can present as red or bluish to violaceous papule, plaque or nodule over neck, upper back and proximal part of limbs typically in childhood and infancy. These lesions may also develop in adults or the elderly . There iTufted angioma in childhood needs differentiation from strawberry nevi and kaposiform hemangioendothelioma . AngiomaAs evident by this paper, TA although not described in the eyelid before, should be considered as a differential diagnosis of benign eyelid vascular lesions. TA can have variable clinical presentation and can present at any age. They can be ill defined to circumscribed, and should morphologically be distinguished from other commoner benign and malignant vascular eyelid lesions. A complete physical examination and hematological work up has been recommended in patients with classical TA to exclude rare association of Port wine stain and KMS. Whether a significant risk of the KMS exists in patients of eyelid TA is not currently known. Considering the smaller size of eyelid lesions this risk is less likely. Nonetheless it may be prudent to get a detailed physical and hematological work up done in all cases diagnosed with eyelid TA.Written informed consent was obtained from the patient for publication of this Case Report and accompanying images. A copy of the written consent is available for review by the Editor-in-chief of this journal.TA: Tufted angioma; KMS: Kasabach-Merritt syndrome.The authors declare that they have no competing interests.RM carried out the histological and immunohistochemical work up, compiled the cases and drafted the manuscript. DT carried out the clinical work up, provided the clinical information, participated in designing the manuscript and edited the manuscript. Both the authors read and approved the final manuscript.


We report a case of a female patient who was operated at the third relapse of an atrial myxoma caused by Carney complex. The difficult operation was performed without any complications despite extensive adhesions caused by the previous operations. The further inpatient course went without complications and the patient was discharged to the consecutive treatment on the 9th postoperative day. The echocardiographic finding postoperative showed no abnormalities. In 1998 a 45 year old female patient presented with complete left sided hemiparesis after a cerebral embolism. While focusing on the reason for this clinical presentation a large mass lesion was found in the atrium via echocardiography whereby suspicion of atrial myxoma was raised. The patient was referred to our department and operative resection of the large myxoma was performed which was predominant prolapsed into the left ventricle at the distal roof of the left atrium above the mitral valve. Both intraoperative and inpatient course progressed without complications and the patient was rapidly discharge for early neurological rehabilitation.Three years later, a left atrial tumor was seen in a routine cardiological echocardiography follow up and the suspicion of a relapse of the atrial myxoma was raised. A resection of the two-hazelnut-sized myxoma at the posterior wall of the left atrium was performed again. The operation and inpatient course again proceeded without complications and the patient was rapidly discharged again.After a period of four years an echopenic structure with freely moving component parts was found at the left side during a transesophageal echocardiography and the suspicion of a second relapse was raised. Making additionally there were found sutures of the previous operations at the septum. But making a clear differentiation between a thrombus at the previous suture line and a myxoma relapse was not possible; therefore a three months oral anticoagulation with phenprocoumon was started.With a positive family history for myxomas (the patients's brother and grandfather) and multiple lentigines an evaluation of the presence of Carney complex, an autosomal dominant disorder, was conducted. After genetic analysis the diagnosis of Carney complex type I with a deletion mutation in the PRKAR1A gene was detected. Carney complex type I is characterized by recurrent atrial myxomas, skin, conjunctiva and lips lentigines, subclinical hypercortisolism and nodular thyroid changes.After three months of effective oral anticoagulation the structure at the atrial septum was not increased in size. A conservative approach was agreed while maintaining oral anticoagulation and regular echocardiographic follow up. Three years later an echocardiographic study revealed a new tumor, however this time the location was the right atrium. The left sided structure was unchanged. An operative resection was carried out again via re-sternotomy with an oscillating saw after preoperative evaluation (computed tomography (CT) scan of the thorax, transthoracic doppler echocardiogram, doppler examination of the femoral vessels, and lower limb arteries). The cardiopulmonary bypass with systemic 32°C mild hypothermia was established via ascending aortic and bicaval cannulation. The right atrial tumor (approximately 3 cm in diameter) was located at the confluence of inferior vena cava and the lateral atrial wall. An atrial myxoma was confirmed histologically with a tumour-free resection margin. The postoperative course was again without complication. During the last echocardiographic follow up a progressive left atrial structure was seen and the decision was made to resect the fourth atrial myxoma Figure . Again tAs with every other organ the heart can be affected by tumor and its relapses. Primary tumors of the heart are rare. The incidence varies between 0.0017 and 0.19% in unselected autopsy studies. Three quarters of primary heart tumors are benign and half of them are atrial myxomas. Other benign tumors are lipoma, papillary fibroelastoma, rhabdomyoma and fibroma .Myxomas are mesenchymal tumors, which can occur at any age, however, they mainly exist between the 30th and 60th year of life. This disease is approximately two to three times more prevalent in women than in men -3. AboutThe clinical examination, chest X-ray and ECG are inefficient and non-specific. Often the diagnosis is an incidental finding detected by echocardiography measurements in the context of peripheral tumor emboli diagnosis. However, transthoracic or transesophageal echocardiography can show exactly the tumor's position and dimension, alternatives are cardiac CT and cardiac MRI. Differential diagnosis to consider is always a thrombus giving the same clinical picture. The preferred treatment is always surgical removal indicating a curative approach. Both short- and long-term results are excellent -3.An operation is always indicated because the outcome and clinical course without intervention cannot be predicted. Due to the high rate for embolisms and the danger of the sudden cardiac death the operation should be performed as soon as possible following diagnosis -3. The rAs with sporadically occurring myxomas a resection of the cardiac tumor and its relapses should be performed in patients with Carney complex. Serious surgical problems are especially peri- and epicardiac adhesions from previous operations which increase after every relapse resection. This can lead to a situation where the surgical risk is higher than the risk of death due to complications of the myxoma. Therefore a careful consideration of the complication-benefit analysis should be made especially for patients with Carney complex. In individual cases a non-operative approach has been described . In the Normally echocardiographic follow up should be performed each year to detect myxoma relapses early. Patients with known Carney complex should have this examination every six month if they have already had a surgical resection ,9. AfterThe Carney complex is an infrequent congenital disease where the relapse rate of cardiac myxomas is manifestly increased. With close follow up with echocardiography relapses can be detected early and adequately treated. Concerning postoperative anticoagulation we considered it appropriate that our patient received a platelet aggregation inhibitor in light of present of inconspicuous echocardiographic findings.The authors declare that they have no competing interests.CB and AP performed data, and wrote the paper. ME, MF, and TT are members of surgical teams. HS was the anaesthetist involved in theatre and in intensive care unit. BT an FS added import comments to the paper. TT co-wrote the manuscript. All authors have read and approved the final manuscriptWritten informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.


Schwannomas are slow-growing typically encapsulated tumors composed of differentiated Schwann cells, the primary class of peripheral glial cells. Complete excision is the treatment of choice for orbital schwannomas that cause pain, disfigurement, diplopia, or optic neuropathy. The presence of multiple schwannomas in a single patient suggests possible association with neurofibromatosis type 2 (NF2) or schwannomatosis.We present 2 patients who experienced recurrent orbital schwannoma without evidence for neurofibromatosis. The recurrence in one patient, a 59-year old man, occurred 6 years after complete excision of the initial tumor. This recurrence consisted of 2 independent tumors in the same orbit. The recurrence in the second patient, a 5 year-old girl, occurred multiple times within days to weeks of partial excisions until eventually a complete excision was performed.The clinical history, histopathologic features and particularly the intraoperative findings suggest that the 59 year old man suffers from orbital schwannomatosis, while the rapid recurrence in the second patient correlated with the cellular features of her plexiform schwannoma. Hence, the recurrence in each patient is linked to a different etiology, with implications for treatment and patient counseling given the difficulty in treating orbital schwannomatosis. To our knowledge, this is the first description of isolated orbital schwannomatosis. Schwannomas are slow-growing typically encapsulated tumors composed of differentiated Schwann cells, the primary class of peripheral glial cells. They can occur in any location, but occur infrequently in the orbit, representing only 1% of orbital tumors . SchwannComplete excision is the treatment of choice for orbital schwannomas that cause pain, disfigurement, diplopia, or optic neuropathy -8. HistoNF2 tumor suppressor gene germline mutation. Schwannomatosis is characterized by multiple pathologically proven schwannomas without vestibular tumors diagnostic of NF2. Although most schwannomatosis cases are sporadic, instances of autosomal dominant transmission with incomplete penetrance have also been reported [The presence of multiple schwannomas in a single patient suggests possible association with neurofibromatosis type 2 (NF2) or schwannomatosis. The hallmark of NF2 is bilateral vestibular schwannomas resulting from reported , though reported -15.de novo, contrasting with a 5-year old girl with a case of a recurrent isolated orbital schwannomas who underwent 2 surgical excisions with recurrence of the tumor within 1 month of each surgery.Accurate characterization of schwannomatosis is important as it is a prognostic factor that helps guide treatment. Here we present two cases, a 59-year old man with clinically recurrent orbital schwannomatosis, manifesting as multiple independent schwannomas that likely arose A 59-year old man with a past medical and surgical history significant for diabetes mellitus, hypertension, sleep apnea, and thyroid lobectomy for goiter first presented in September 2004 complaining of intermittent vertical diplopia. On examination, there was a palpable mass of the superomedial orbit. An MRI demonstrated this to be a dense, well-circumscribed lesion, and a 27 x 10 x 10 mm mass was completely excised via a left anterior orbitotomy. Histopathologic exam revealed a schwannoma with intense positivity for S-100 protein . She underwent excision of this mass at an outside facility with histopathology suggesting schwannoma. The mass recurred within 1 month of excision and the patient underwent a second excision 4 months after the first surgery, with pathology again suggesting schwannoma. Within 1 month of excision, the mass again began to enlarge and by 1 year had returned to its original size. At this point the patient was referred to our clinic for further evaluation and treatment.At our initial evaluation, the patient’s ALL was in remission. She was otherwise healthy and developmentally normal without evidence of other abnormal masses or signs of neurofibromatosis. On ophthalmologic examination, the patient’s visual acuity was 20/20 bilaterally with normal pupil examination and extraocular motility. External examination revealed a mobile 9 x 10 mm left medial canthal mass resting on the underlying orbital bone with an overlying scar. The remainder of her examination was unremarkable. An MRI scan revealed a well-circumscribed, contrast enhancing mass without posterior extension and possible bone involvement. The pathology slides from the specimens obtained during the two previous excisions were reviewed, revealing plexiform cellular schwannoma.The patient underwent left medial anterior orbitotomy with complete excision of the 15 x 11 x 5 mm canthal mass with uninvolved margins Figure A. Histopde novo recurrence in the case of the 59-year old man (leading to late recurrence).Our cases demonstrate different mechanisms for schwannoma recurrence: residual tumor in the case of the 5-year old girl (leading to rapid recurrence), and likely Although orbital schwannomatosis is a rare entity, our 59-year old male patient case 1) fits previously proposed diagnostic criteria, with multiple independent histologically confirmed schwannomas in his left orbit and no evidence of NF2 ,17. Alth fits preInterestingly, at the time our 5-year old girl (case 2) was originally diagnosed with orbital schwannoma, she was undergoing chemotherapy treatment for ALL. There is one case in the Japanese literature of a patient diagnosed with a thoracic schwannoma while in remission from acute lymphoblastic leukemia, although with only two known cases, a causal relationship between ALL and schwannomas cannot be concluded . PatientWhile surgery is the mainstay of treatment for orbital schwannomas, radiation therapy is an alternative treatment is some cases. However, even with vestibular schwannomas, the efficacy of radiation therapy is unclear . FurtherIn summary, we report 2 cases of recurrent orbital schwannomas, demonstrating the challenge of treating this tumor. In both cases, the patients should be carefully monitored for further recurrences. In cases of schwannomatosis, patients should be advised that the goal of therapy may be mostly palliative, and that close monitoring would be required in order to facilitate early intervention of further recurrences, before the tumor size becomes problematic. In the case of plexiform schwannomas, the patient and family should be advised of the risk of early recurrence.Written informed consent was obtained from the patient or patient’s guardian for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.NF2: Neurofibromatosis type 2; ALL: Acute lymphocytic leukemia.The authors declare they have no competing interests.MK, BB and AK drafted the manuscript. AK conceived of the case series, performed surgeries, and coordinated the specimen analysis. JM assisted in analysis of the pathology specimens and summary of histopathologic findings. SA performed surgery and assessed eye movement. All authors read and approved the final manuscript.AK and SA are practicing ophthalmologists, specializing in oculoplastics and pediatrics, respectively. JM is a practicing pathologist. BB is a pediatric ophthalmology fellow and MK is a transitional year resident.The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-2415/12/44/prepub


Meningiomas represent the most common benign intracranial neoplasms, and may spread by direct extension into nearby venous sinuses, but gross spread to the extracranial venous system is uncommon. We report the case of a patient with extracranial intraluminal spread of meningioma within the internal jugular vein to level III of the neck. Review of the preoperative assessment and management is also presented. Meningiomas are the most common benign intracranial neoplasms and are typically well controlled with complete surgical resection. However, atypical meningiomas (WHO grade II) are associated with an increased risk of recurrence and mortality. Extracranial extension of meningioma is seen in less than 2% of cases and usually occurs by direct extension through the skull, perineural spaces, or vascular channels. Though meningiomas may invade dural venous sinuses, extension of the tumor through the sinuses into the extracranial venous system is uncommon .We present a case of extracranial spread of an atypical meningioma extending into the internal jugular vein and its branches. A review of the literature and discussion of extracranial meningiomas are also presented. A 79-year-old male with a recurrent right posterior fossa meningioma, treated previously with surgical resection, gamma knife radiosurgery, reresection, fractionated radiation therapy, and chemotherapy with temozolomide and bevacizumab over a 7-year period, presented for evaluation with an exophytic postauricular mass. At the time of initial assessment, the patient complained of retroauricular pain, tinnitus, and right-sided hearing loss. On physical exam, a 5 × 6 cm fungating mass was seen at the postauricular crease. Cranial nerves II through XII were grossly intact. CT of the head showed extracranial growth of tumor abutting the posterior and middle cranial fossa with mass effect on the cerebellar hemisphere and the fourth ventricle. MRI of the brain demonstrated the heterogeneous mass to have intracranial and extracranial components involving both the supra- and infratentorial compartments Figures . The extThe patient underwent a single-stage surgical resection of the meningioma that involved the temporal bone, suboccipital and jugular foramen, and extracranial components. The resection required removal of the external ear and temporomandibular joint and included mastoidectomy, partial labyrinthectomy, parotidectomy, and modified radical neck dissection with sacrifice of right internal jugular vein (IJV) . Of noteAtypical (WHO grade II) meningiomas represent approximately 20–35% of all meningiomas and are associated with an increased risk of recurrence and mortality [As intracranial meningiomas grow, they typically displace but do not invade adjacent neural tissues yet may invade bone through haversian canals. The tumor may infiltrate the dura and adjacent venous sinuses, but it is far less likely for the tumor to extend into the extracranial draining vein. Meningiomas involving the jugular foramen are uncommon and can present surgical challenges that may require a staged approach to surgical management. Although the treatment choice is surgery, resection can be associated with compromise of multiple cranial nerves (in particular CN VII and X) and is also associated with an increased local recurrence rate . Althoug Recurrent locally invasive presentations of meningioma, in particular those involving the jugular foramen, frequently require presurgical evaluation utilizing CT, MRI, and MRA/MRV. CT scan may demonstrate a dural-based lesion with smooth contours and can be useful for identifying hemorrhage or the permeative-sclerotic appearance consistent with bony erosion. On T1 weighted-image (WI) MRI, meningioma is a hypo- to isointense mass with the absence of high-velocity flow voids. In contrast, dense uniform enhancement is noted spreading centrifugally along dural surfaces. On T2 WI, atypical meningiomas may vary being hypo- to hyperintense dependent upon the degree of cellularity associated with the tumor. MRV can be used to confirm patency of adjacent dural sinuses prior to surgery [Extracranial extension of meningioma is uncommon, and direct intraluminal spread within the internal jugular vein to the neck is a rare finding. Recurrent cases with atypical pathology may represent a scenario with a higher likelihood for this presentation. Appropriate preoperative imaging and treatment planning are essential in these patients.


Osteoarthritic subchondral cysts within or around the hip joint can sometimes be difficult to distinguish from primary osseous and soft tissue tumors due to their radiological appearance and uncommon location.We report the case of a 74-year-old Turkish man with a subchondral cyst arising from the hip joint, eroding the acetabulum and located on the medial side of the iliac bone, which imitated a soft tissue tumor. This cystic lesion was resected and the results of histopathological analysis of tissue samples were found to be consistent with an osteoarthritic cyst.The present case illustrates how an osteoarthritic subchondral cyst can grow into the soft tissue planes in the presence of destruction of the articular cartilage and subchondral bone continuity, and present as an apparent soft tissue tumor. Osteoarthritis (OA) is a degenerative joint disease with some radiological characteristics including the presence of multiple, small cysts (geodes) with subchondral sclerosis, marginal osteophytes, intra-articular osteochondral bodies and narrowing or loss of joint space ,2. OsteoIn the present work, we report the case of a patient with an atypically located osteoarthritic cyst arising from the hip joint, eroding the acetabulum and located on the medial side of the iliac bone, which imitated a soft tissue tumor on radiological imaging studies.A 74-year-old Turkish man was referred to our out-patient clinic because of right hip pain without a history of trauma. He had experienced severe pain in the groin on weight bearing, with variable degree of pain at rest, over the last 45 days. He was able to walk only with the assistance of crutches. His medical history was unremarkable.On physical examination, he was afebrile and had a blood pressure of 120/80mmHg. His active ranges of motion (ROM) for both hips were restricted in all directions and the passive ROM of right hip was painful in flexion, abduction and internal rotation. The result of a Thomas test was positive for the right hip.3/μL) counts. The results of functional tests for the kidney and liver, as well as other biochemical blood analyses, were normal.Antero-posterior and frog-leg pelvis radiographs Figure  showed lSurgery for excision of the cystic lesion was recommended, and was performed under general anesthesia. Our patient lay in a supine position and an anterior ilioinguinal incision was made in the right hip. The interval between the tensor fascia lata and sartorius muscle was identified. The lateral femoral cutaneous nerve was retracted laterally. The dissection was extended proximally to expose the medial surface of the iliac bone. The rectus femoris muscle was not incised from its attachment to the upper part of the acetabular rim, but was instead retracted laterally. The iliacus muscle was identified and stripped from the medial surface of the iliac bone. The cystic lesion was identified. There were no adhesions between the cyst membrane and surrounding soft tissue. However, it was associated with the antero-medial acetabular wall and had eroded the adjacent acetabulum. The cystic lesion was resected en bloc and examined in the operating room on the surgical table. Calcified necrotic material was exposed in the cyst.Our patient had an uneventful post-operative course with no complications. Post-operative prophylaxis of intravenous antibiotics, consisting of a first-generation cephalosporin  and an aminoglycoside (gentamycin 5mg/kg/day) were continued 48 hours after the surgery and low-molecular-weight heparin prophylaxis was administered for 10 days. Active and passive hip ROM exercises were started on the second day post-operatively with mobilizing on crutches, and he was discharged from hospital on the third post-operative day. Full weight bearing without support was allowed at the third post-operative week. The histopathological results of tissue samples were found to be consistent with an osteoarthritic cyst that contained degenerative calcified and necrotic chondroid tissue and bone trabeculae Figure .There were no complications such as infection and skin necrosis during the follow-up period. At the final follow-up (4 months post-operatively), our patient was assessed clinically. The active ROM for both hips were restricted as had been the case pre-operatively. However, he had only slight pain in his right hip and was able to walk without support. Primary total hip replacement surgery for both hips was recommended in view of the radiological findings.Tumors and tumor-like lesions located in the pelvis have some specific differences from those tumors arising in other parts of the body. The main difference is that pelvic tumors are located deep in the body, while tumors located in the extremities are relatively superficial. Patients with pelvic tumors are usually older and their tumors larger relative to patients diagnosed as having tumors in the extremities. The clinical presentation is therefore often several years later compared to the same tumor type located in an extremity . The numSubchondral cysts in degenerative osteoarthritis are often multiple and variable in size, whereas solitary and large cysts are unusual. It has been shown previously that nearly two-thirds of osteoarthritic cysts completely disappear radiographically or become smaller with no additional treatment . HoweverSome theories have been described to explain the pathophysiology of subchondral cysts in OA. Intrusion of synovial fluid through the articular cartilage secondary to elevated pressure of intra-articular fluid, which results in hydraulic destruction of subchondral bone, has been proposed as a possible etiology . This thOur patient’s case illustrates how an osteoarthritic subchondral cyst can grow into the soft tissue planes in the presence of destruction of the articular cartilage and subchondral bone continuity, and present as a soft tissue tumor.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.The authors declare that they have no competing interests.MG and TÖ contributed to the conception and design, and carried out the literature research, manuscript preparation and manuscript review. TÖ, OK and FÖ were involved in the literature review and helped draft part of the manuscript. FA contributed to the conception and design. MG, TÖ and OK revised the manuscript. All authors read and approved the final manuscript.


She made an uneventful postoperative recovery. Histopathology revealed a bronchopulmonary foregut malformation with pulmonary sequestration. This developmental anomaly of the foregut typically occurs in the thoracic cavity; however, it can occur below the diaphragm. Herein we report a case and a detailed review of the embryology, clinical features, and management of these extremely rare clinical entities.We report a case of a lady who presented with epigastric discomfort. Physical examination revealed a large left upper quadrant mass filling the left upper quadrant. Following extensive preoperative evaluation, she underwent resection of this 9 × 10 × 11 centimeter mass with  Cysts and duplications are common anomalies of development. A bronchopulmonary sequestration is a rare congenital malformation of the lower respiratory tract that consists of a nonfunctioning mass of lung tissue that lacks normal communication with the tracheobronchial tree . DependiThe term bronchopulmonary foregut malformation is used to broadly encompass a spectrum of embryological lesions that include bronchopulmonary sequestration, tracheal stenosis, bronchogenic cysts, congenital cystic adenomatoid malformations, bronchial atresia/stenosis, and congenital lobar emphysema. The relative incidence of bronchopulmonary sequestration within the spectrum of bronchopulmonary foregut malformations is 27% .Retroperitoneal bronchogenic cysts despite their rarity occur with equal frequency among genders across a wide age range. The reported incidence of such extralobar, subdiaphragmatic malformations is 1 per 10000  with theThe majority of reported cases are incidental findings on imaging modalities and measure less than 5 cm in diameter. The commonest presenting symptom is epigastric or back pain similar to the current case, but these occurred in cysts greater than 7 cm. The largest reported case in the medical literature claims a maximum dimension of 10 cm [A 48-year-old female presented to the emergency department with a two-month history of worsening epigastric discomfort. She had no associated symptoms. Clinical examination revealed a firm, tender mass in the left upper quadrant. It was nonpulsatile, dull to percussion, and moved inferiorly with respiration. It was not possible to palpate a superior edge while the lower margin was palpable. She had previously undergone a right ovarian cystectomy for a benign dermoid cyst. Initial haematological and biochemical indices were all investigated and within normal parameters.Ultrasound revealed a large (9 cm × 10 cm × 11 cm), poorly vascularised mass, separate from the spleen, containing mixed solid and cystic elements. Internal septations were also apparent. CT imaging  demonstrMR imaging  showed hen bloc following aspiration of 700 millilitres of viscid fluid. An intercostal drainage tube was placed in the left hemithorax attached to an underwater seal and the diaphragmatic defect was closed with 2/0 polydioxanone. She made a favourable postoperative recovery and was discharged home on the 6th postoperative day. She remained well at outpatient followup.Since the mass was causing significant discomfort and of uncertain malignant potential, a decision was made to excise this mass. The patient was placed in a supine position. An upper midline incision facilitated adequate access to the left upper quadrant. The cystic mass was adherent to the membranous portion of the left hemidiaphragm. This was excised Haematoxylin and Eosin analysis revealed that the multiloculated structure was lined by ciliated respiratory-type epithelium with a florid granulomatous reaction . DepositGerle et al. in 1968 first used the term “bronchopulmonary foregut malformation” and described 13 cases of pulmonary sequestration communicating with the gastrointestinal tract [It is generally accepted that foregut malformations arise from a supernumerary lung bud that arises caudally to the normal lung bud and migrates caudally along with the developing oesophagus. If the accessory lung bud arises before the development of the pleura, it becomes invested in the adjacent normal lung forming an intralobar sequestration. Conversely, if it develops later, after the formation of the pleura, it grows separate from the adjacent lung surrounded by its own pleura, becoming an extralobar sequestration. This process occurs during the 3rd–7th weeks of development . The exaThe differential diagnosis of a left upper quadrant mass is extensive and beyond the confines of this case report but the differential of a retroperitoneal cyst lined with pseudostratified, ciliated columnar epithelium includes cystic teratoma (only six reported cases) , bronchoPresentation is entirely variable and depends upon the type, size, and location. They are often detected on antenatal ultrasonography. Hydrops occasionally occurs, presumably as a result of vascular compression. In a series of 41 pregnancies whereby extralobar sequestration was detected antenatally , the lesThe commonest sites of communication of these retroperitoneal lesions are in the left para-adrenal region with lower oesophageal and cardiooesophageal junctions (83%) . Blood sSurgical resection is the standard treatment to alleviate symptoms and in case if concern exists regarding its malignant potential , 12. SurOwing to the paucity of the published literature on this topic, little is known about the outcome following resection. Patients with intrathoracic sequestrations did very well without significant complications. The majority morbidity was recurrent respiratory tract infections .In conclusion, extrathoracic bronchopulmonary malformations are rare benign pathological entities. These can reach a substantial size prior to presenting with nonspecific symptoms. Despite detailed preoperative imaging, surgical extirpation is essential to alleviate symptomatology and elucidate its malignant potential.


Langerhans Cell Histiocytosis (LCH) is a rare neoplasm characterized by abnormal proliferation of histiocytic cells. In this case report, we describe a unique case of a 13-year-old boy who presented to the clinic with an insidious onset of mid-clavicular pain. The provisional radiologic diagnosis of Langerhans Cell Histiocytosis of the clavicle was confirmed by an incisional biopsy of the left mid-clavicle lesion. The patient's lesion was treated by curettage, bone grafting, and internal fixation, due to the presence of pathologic fracture. At the 2-year followup, the patient was asymptomatic, and the lesion showed complete resolution without recurrence. The case report highlights the characteristic features of Langerhans Cell Histiocytosis in an unusual location, the knowledge of which would help avoid delayed or missed diagnosis in the future. Langerhans Cell Histiocytosis (LCH) is the current nomenclature for disorders previously known as histiocytosis X, which grouped eosinophilic granuloma, Hand-Schuller-Christian disease, and Letterer-Siwe disease –4. LCH iA 13-year-old Caucasian male presented to clinic with an insidious onset of nondominant, left-sided, mid-clavicular pain for six weeks of duration. The mother described the texture of this area as “sand-like.” There was no history of trauma, fever, chills, night sweats, weight loss, joint pain, or swelling. On physical examination, the left clavicle demonstrated an isolated, tender, firm, nonerythematous, nonfluctuating, soft tissue swelling directly over the mid-clavicle without a noticeable deformity. Skin changes were absent. Laboratory examination showed total blood count of 8.9 K/UL (4.5–13.5) with normal differential. Erythrocyte sedimentation rate (ESR) was 14 mm/hour (0–10) and C-reactive protein (CRP) was 1.3 mg/dL (<1.0).Conventional radiograph of the left clavicle and a Tc-99m-MDP bone scan were performed. Subsequently CT scan and MRI were performed. An incisional biopsy of left mid-clavicle was performed in the operating room and the fragments of soft red-tan tissue were sent for histopathologic examination. It showed a histiocytic neoplasm admixed with eosinophils, confirming the diagnosis of Langerhans Cell Histiocytosis (LCH). Cultures were negative. The lesion was treated by curettage, bone grafting, and internal fixation with a 3.5 mm reconstruction plate due to the presence of pathologic fracture. At 2 years of followup, patient was asymptomatic and the lesion showed complete resolution without recurrence.The radiographic appearance of typical solitary LCH within a long bone is a lytic, medullary based, metaphyseal, or diaphyseal lesion, with or without periosteal reaction . The perThe most common causes of nontraumatic lesions of clavicle are neoplasm, infection, or developmental abnormalities . MajoritBiopsy is performed to confirm the diagnosis of LCH. On histology, sections show a histiocytic neoplasm admixed with eosinophils expanding and destroying bone . The tum


Ectopic hormone secretion is a well-recognised phenomenon; however, ectopic prolactin secretion is exceptionally rare. Hoffman and colleagues reported the first ever well-documented case of ectopic prolactin secretion secondary to a gonadoblastoma. We report a lady who presented with galactorrhoea and a large ovarian tumour that was found to secrete high levels of prolactin.Aim of this case report is to highlight the occurrence of this condition.Lack of awareness can often lead to a diagnostic conundrum. We report this case to make clinicians aware of this rare condition A 27-year-old lady presented with 8-week history of hot flushes, headaches, galactorrhoea and delayed periods. She denied any problems with vision or fluctuation in weight. Her medical history was asthma and irritable bowel disease. She was an ex-smoker of 5 years with a two packs per year smoking history and drank alcohol occasionally. On examination, she was noted to have galactorrhoea on expression. She had normal visual fields tested by confrontation method. Systemic examination revealed a 10 cm supra-pubic mass on palpation.2) 83 pmol/l. She had normal TSH 1.8 mIU/l (0.30–5.00), thyroxine 12 pmol/l (9–25 pmol/l) and normal cortisol response to short synacthen test . Her tumour markers showed CA 125 antigen 67 kU/l (0–35), alphafetoprotein 83 kU/l (0–10), CA19-9 antigen 19 kU/l (0–10) and carcino-embryonic antigen <2 (0–5). Her repeat prolactin level was 36 066 mIU/l.Her initial prolactin level returned at 24 642 mIU/l  with suppressed LH <0.5 IU/l, FSH 2.7 IU/l and 17β-oestradiol  of the abdomen showed a right adnexal mass measuring 18×14×18 cm with retroperitoneal lymphadenopathy .2 355 pmol/l.Therapy with cabergoline was initiated but discontinued due to intolerance. She was then started on bromocriptine 2.5 mg. She underwent right salpingo-oophorectomy with para-aortic node clearance. Three weeks post-operatively, her prolactin dropped to undetectable levels. Bromocriptine was discontinued. Her prolactin levels remained normal  at 367 aPost-operative histopathology, immunohistochemistry results under haematoxylin and eosin stain showed that the ovary was replaced by diffuse sheets of undifferentiated, highly atypical cells possessing enlarged nuclei. Tumour cells were positive for synaptophysin, p53 and epithelial membrane antigen (EMA), and showed negative expression for chromogranin, CK7, desmin, inhibin, melanA, PLAP and CD56. Following immunohistochemistry, the tumour was categorised as ovarian malignant small cell tumour of pulmonary type. Immunohistochemical staining for prolactin showed patchy but intense cytoplasmic, peri-nuclear dot-like positivity, which was reported to perhaps represent localisation of prolactin in Golgi complex .This patient had adjuvant chemotherapy following her surgery. Her PET FDG scan performed 10 weeks post-operatively showed right oophorectomy, unremarkable upper abdominal viscera, clear lung fields and no evidence of mediastinal, abdominal or pelvic adenopathy. She had no disease recurrence on her 8-month follow-up scan. Her prolactin levels remained normal.Our case demonstrates ectopic prolactin secretion secondary to an ovarian malignant small cell tumour. Normalisation of prolactin levels soon after surgical removal of the tumour, positive immunohistochemistry for prolactin and normal pituitary gland on imaging strongly support the diagnosis. We report this case to highlight and make clinicians aware of this rare condition.Written informed consent was obtained from the patient/patient’s mother for publication of this case report.Co-authors have contributed towards patient care, management and also towards writing and finalising the draft.