Foreign body left after surgery surrounded by a foreign body reaction otherwise known as gossypiboma, have been first described in 1884. Although it occurs rarely, it can lead to various complications which include adhesions, abscess formation and related complications. Intrathoracic gossypiboma is a rare but serious consequence of negligence, mainly during abdominal and cardiothoracic surgery that can lead to severe medical consequences. This paper aims to raise awareness among surgeons and nurses in the operating room to prevent such errors and future complications.A patient with a history of coronary arterial bypass grafting performed 14 years ago, presented with shortness of breath and dry cough. A chest X-ray revealed a large mass in the left hemithorax. The chest CT demonstrated the presence of a heterogeneous density mass of 11 cm and smooth edges in the middle mediastinum, next to the heart and partially intrapericardial. Because clinical and radiologic evidence revealed presence of a mass, we did proceed with CT guided FNA of the mass. The cytology findings confirmed an inflammatory lesion. Based on patient symptomatology and the evidence of a mass, allegedly compressing the cardiopulmonary structures in vicinity, we performed surgical exploration. An old and degraded piece of surgical swap was found and removed through an anterolateral left thoracotomy. The post-operative course was excellent.Forgetting surgical swaps during surgery is a medical fault. To avoid them, surgical units should design and implement a surgical inventory process to account for surgical instruments or surgical swaps. Failure to make a proper diagnosis of cases such as these can lead to further health complications in these patients. The iatrogenic foreign material seen as a mass in the radiologic films had not been previously noticed by other health professionals although the patient had undergone X-ray and cardiac ultrasound examinations in the 14 years following coronary bypass surgery. Once the causative agent was identified and removed the patient returned to normal activity. Gossypiboma (aka Textiloma), described first in 1884, is a mass within the body composed of a cotton matrix, which usually refers to a retained surgical sponge or swap, surrounded by a foreign body reaction . IntrathA 50 year-old male, with a history of coronary arterial bypass grafting 14 years back, presented with shortness of breath and dry cough. An X-ray revealed a large mass in the left hemithorax adjacent to the heart silhouette. A chest CT demonstrated the presence of a mass with smooth edges, in middle mediastinum next to the heart and partially intrapericardial , pelvis (18%) and thorax (11%). Longest reported cases of gossypiboma were found in thorax (43 and 44 years) [%, pelvisCT scanning is the most helpful procedure in diagnosing a gossypiboma, especially if a radiopaque marker is used . Wan et The CT findings change according to type of foreign material, their anatomical location, and type of reactions they generate in the host . .In the.MRI and biopsy can also be helpful. However, MRI features can be confusing, while biopsy is often indecisive , 8.Although rare nowadays, gossypiboma cases do occur. It is clear that leaving unintendedly surgical swaps inside the body during an intervention is a medical liability. Efforts should be made to avoid such iatrogenic errors. Implementing OR checklist pre and post surgery, portable ontable detective rings and strict instrument and sponge count is paramount to prevent such potential catastrophes for patients.In our case, the radiologic image was initially showing a big mass of mediastinum that involved the pericardium and was accompanied by shortness of breath and dry cough. The history of past surgery in this patient additionally suggested that an intrapericardial foreign body could be producing the current symptoms. We decided to perform the exploratory surgery as the best diagnostic and treatment option because it appeared very well circumscribed.Although this error didn’t happen to us, since this case, we have been more cautious in our surgical practice, counting everything at the beginning of the surgery and before chest closure as described by WHO checklist. We don’t close the thorax if something is missing and some time we have used the C-arm to control. We are currently using only surgical swap with radio opaque marker inside.Forgetting surgical swaps inside the body during surgery is a preventable medical error that should be avoid at all cost, even in very difficult cases with a high level of tension in the operating room. They can be prevented by implementing a strict patient safety protocol such as an instrument, needle and surgical swaps count procedure before surgery and before closure. Another lesson learned is that in cases such as ours, surgical exploration is the best treatment. Investigating duration of symptoms, the difficulty to make the correct diagnosis by only routine clinical exam prompts us as surgeons to carefully investigate and intervene in timely fashion. As demonstrated, the iatrogenic foreign material seen as a mass in the radiologic films was the culprit of patient’s severe symptoms. Once the cause was identified and the foreign body removed, patient returned to normal activity.It is inexcusable that this patient for 14 years made a lot of medical tests, including yearly X-rays and cardiac ultrasounds and no one has found anything wrong. This case aims to raise awareness among surgeons and nurses in the operating room and doctors in their everyday work to prevent such errors and future complications that may deteriorate patients’ health.


Transverse testicular ectopia (TTE) with fused vas deferens is an extremely rare clinical entity. Herein, we present a case of a 19-year-old patient with persistent left testicular pain lasting for a week. Clinical examination revealed an empty right hemiscrotum, a normal left-sided descended testis, and in close proximity a mass-like structure resembling testicular parenchyma. Laboratory tests were significant for elevated follicle-stimulating hormone (FSH), while sperm count revealed azoospermia. Ultrasound imaging (US) of the scrotum demonstrated the presence of both testes in the same left hemiscrotum with varicocele and no signs of inguinal hernia. Magnetic resonance imaging (MRI) of the penis and scrotum revealed TTE with a single, fused vas deferens, and hypoplastic seminal vesicles. Surgical intervention by means of microsurgical sperm retrieval and transseptal orchidopexy were considered but not performed, primarily owing to the patient's unwillingness and to a lesser extent due to the restriction that the short and fused vas would pose in an attempt to transpose the ectopic testis. Therefore, an annual follow-up was recommended. Transverse testicular ectopia (TTE) is a rare congenital anomaly in which both testes descend on the same inguinal route ultimately lying on the same side of the scrotum. This pathologic condition mostly affects young males with a mean age of 4 years. We present an interesting case of a young adult complaining of constant pain in the left testicle for 7 days. Clinical examination and imaging modalities established the diagnosis.A 19-year-old patient was referred to our department complaining of a week-long history of left testicular pain. His past medical history was remarkable for an absent right testis without any further clinical information. Upon physical examination, the patient presented with an empty and hypoplastic right hemiscrotum. The right testis was not palpable along the ipsilateral inguinal canal. On the contrary, a normal in size and consistency testis was present on the left side. Above that and in close proximity an oval-shaped smooth, parenchymal-like structure of considerably smaller size could be palpated.The patient also complained of intermittent hypersensitivity over the left hemiscrotum, especially on prolonged standing. No signs of infection were present at the time. Clinically, a grade II varicocele with prominent dilated veins above the low lying testis could be detected upon Valsalva maneuver on the left side. Digital rectal examination revealed a small, nonpainful, normal feeling prostate. Laboratory workup was insignificant. Color Doppler US revealed the presence of two testes in the same left hemiscrotum and dilated pampiniform plexus veins. The caudal testis was of normal echogenicity, measuring 32x17x27.2 mm, and the cephalad one was comparatively smaller, approximately 20.4x17.8x11.2 mm, with similar echogenicity . Each teSubsequently, the patient underwent upper and lower abdominal CT with contrast administration which revealed a normal urogenital tract but poorly visualized seminal vesicles. The CT findings excluded urogenital anomalies such as renal agenesis or malformation that are occasionally combined with testicular maldescent and ectopy. It also indirectly excluded the possibility of a right maldescended testis and a supernumerary left testis.−1, luteinizing hormone (LH) 7.9 mIU ml−1, alpha-fetoprotein (a-FP) 2.0 ng ml−1, beta-human chorionic gonadotropin (b-HCG) <1.2 mIU ml−1, and follicle-stimulating hormone (FSH) 26.22 mIU ml−1. Hormonal screening excluded androgen deficiency, but his elevated FSH implied primary testicular damage.An MRI of the pelvis and scrotum was additionally performed, which allowed to assess the seminal vesicles, the spermatic vasculature and the length and anatomy of the vas in further detail. The MRI findings demonstrated TTE with a single, fused vas deferens and bilaterally present but hypoplastic seminal vesicles . A spermThe patient refused any further investigation with testicular biopsy, as fertility was not his primary concern at the time. On the other hand, reconstruction and transseptal transfer of the ectopic testis to the contralateral side were deemed technically demanding, due to the presence of a single fused vas that would restrict testicular dissection and mobilization. Furthermore, the clinical benefit on his sperm count would be doubtful. Follow-up assessments were planned on a yearly basis with clinical examination, scrotal US, and hormonal screening since no orchidopexy was performed. This was an arbitrary follow-up scheme since there are no guideline recommendations or expert opinions in the literature.Testicular ectopia is an abnormally positioned testis that can lie anywhere along its theoretical descent route from the retroperitoneum down to the scrotum. In crossed or transverse ectopia the testis crosses the midline to land on the contralateral hemiscrotum –3. This Based on the associated developmental anomalies, a classification system was proposed in 1982 replacing a former one that relied on the etiology of this pathologic entity , 11. AccDespite the fact that the two testes, in most cases, hang over a separate and distinct vas deferens, it has been described the variation of a common vas either as a single duct or an early fusion of two separates. This means that the single vas either originates from a common mesonephric duct, or from two separate counterparts that fused early during development after the crossing over of one of the two to the contralateral side. Gray and Skandalakis postulated that in cases where two distinct vasa deferentia exist, the testes develop from two separate ipsilateral urogenital ridges and the crossing over occurs during testicular migration . AccordiThe investigation of crossed testicular ectopia includes transabdominal ultrasound, MRI of abdomen and pelvis and contrast-enhanced CT to look for associated anomalies , 5, 14. To our knowledge, Akin M et al. and Yıldız A et al. have reported the two largest case-series of TTE so far, each one with six patients , 15. MosIn any case the aforementioned algorithm applied to young patients, whose testes were still developing and could be favored by a reconstruction. In our case, the patient was an adult without associated anomalies to be treated and although we considered repairing the varicocele, the patient did not opt for concomitant varicocelectomy and testicular transfer for two reasons: firstly, because the fused vas would necessitate excessive dissection and mobilization of its common trunk distorting the anatomy of the native testis; secondly, a paucity of data in the literature favouring such a management for restoring fertility in adults. Our patient also refused considering microTESE and cryopreservation at the time reserving this option for later on. Interestingly, it is uncertain whether TTE poses an independent risk factor for testicular malignancy but has been linked to seminomatous, nonseminomatous germ cell tumours, and teratomas in published literature , 20.In summary, TTE is a rare congenital anomaly which should be included in the differential diagnosis of every adult patient with an absent testis and fertility problems. In this setting, physicians should maintain a high index of clinical suspicion in every adult with symptoms of persistent testicular pain. If diagnosed, a thorough investigation should be employed for associated anomalies, taking into consideration the wide spectrum of associated conditions. Finally, it should be noted that the current classification system does not discriminate between a solitary and two distinct vasa deferentia, which might radically affect the therapeutic approach applied on patients exactly like the one presented here.


Ovarian granulosa cell tumors (GCTs) are divided into adult GCT (AGCT) and juvenile GCT (JGCT). The AGCT is more common type, conversely, less than 5% of tumors are the JGCT and occur in mainly premenarchal girls and in women younger than 30 years. Although JGCT have different histologic features compared to AGCT, the two types have similar imaging features because they have similar gross appearance. Therefore, it is difficult to distinguish two types by radiologic findings. In addition, it has not been described about the growth rate of JGCTs in past literatures. The aims of this report were to describe a case of rapidly growing JGCT arising in adult with difficulty in diagnosing and to review the literatures.A 38-year-old woman, presented with abdominal distension and frequent urination, was found to have a pelvic mass measuring approximately 12 cm on ultrasonography. On magnetic resonance imaging (MRI), right ovarian multiloculated cystic mass accompanied with hemorrhagic foci was demonstrated. Although the presumptive diagnosis of GCT was made based on MR findings, the intraoperative differential diagnoses included GCT, yolk sac tumor or malignant mucinous tumor due to cytologic atypia and lack of the typical findings for AGCT such as nuclear grooves and Call-Exner bodies. As a result, abdominal simple total hysterectomy, bilateral oophoro-salpingectomy, partial omentectomy and appendectomy were performed. Moreover, she had a history of laparoscopic uterine myomectomy about one year before, and during that surgery bilateral ovaries were found to be macrospically normal. Therefore, it was suspected the tumor became enlarged within the short period of time.Even though it is difficult to distinguish two types of GCT by imaging findings, in some cases without typical findings for AGCT pathologically, MRI could provide useful information in accurately diagnosing JGCT. Moreover, in this case, the tumor growth rate seemed to be rapid regardless of its borderline malignant potential. It may be related with nuclear atypia and high mitotic rate of the tumor. Granulosa cell tumors (GCTs) are rare sex cord-stromal tumors, encompassing 1–5% of all ovarian tumors . The AGCA 38-year-old Japanese woman, gravida 0, presented with abdominal distension and frequent urination, was found to have a pelvic mass on radiologic examinations. She had a history of laparoscopic uterine myomectomy about a year before the onset, where the bilateral ovaries were macroscopically normal Fig. . She hadThe MR showed a large, multiloculated cystic mass with numerous septations in the pelvis measuring approximately 10 × 10 × 12 cm. On T2-weighted images, fluid-fluid levels were demonstrated in several cystic components , solid 27.8%) or solid and cystic 41.7%) , while J7.8% or s.7% , whiIn this case, the tumor showed multiloculated cystic mass with intracystic hemorrhage on the MRI. On diffusion-weighted images (DWIs), the septations of tumor demonstrated high signal intensities (SIs). The feature was consistent with the previous result reporting that DWIs showed high SIs in the solid component of the GCT . AlthougThe most common presenting symptoms of both AGCT and JGCT are abdominal pain and increasing abdominal girth . The proAnother striking feature in this case was the growth rate of the tumor regardless of its borderline malignant potential. Any macroscopic abnormalities in the bilateral ovaries in the previous surgery about one year before were not observed. Therefore, it was suspected the tumor became enlarged within the short period of time. It has not been described about the growth rate of JGCTs in past literatures. It may be related with nuclear atypia and high mitotic rate of the tumor.In conclusion, even though JGCT in adult patients could be correctly diagnosed as GCT preoperatively, intraoperative pathological diagnosis may be difficult. MRI could provide useful information in accurately diagnosing JGCT. Moreover, it was suspected the tumor became enlarged within the short period of time in this case. It may be related with nuclear atypia and high mitotic rate of the tumor.


Adenoid cystic carcinomas of the Bartholin's gland are extremely rare and are often misdiagnosed. There are currently no definite treatment guidelines. This article describes the case of a 33-year-old female who was managed at our centre for adenoid cystic carcinoma of the Bartholin's gland. She presented with a prolonged history of a vulvar lesion which was eventually diagnosed as adenoid cystic carcinoma of the Bartholin's gland. She was subsequently treated with wide local excision of the primary and inguinal lymph node dissection followed by adjuvant radiotherapy and chemotherapy. She had gross perineural invasion on MRI imaging. The present case highlights the diagnostic dilemma in this extremely rare cancer and the literature further explores the natural history and treatment options. Primary Bartholin's gland cancer (BGC) is rare and consists of 0.001% of all female genital cancers . AdenoidA 33-year-old, ECOG 0 female was referred to our centre for opinion and management of postoperative locally invasive adenoid cystic carcinoma of the Bartholin's gland (ACCBG). She was nulliparous on an oral contraceptive, with menarche at the age of 15. Gynaecological and family history was otherwise unremarkable.She first presented to a hospital in 2009 for a right vulvar lesion. Biopsy of this lesion was benign, and the lesion resolved without intervention. In 2013, the patient noticed a new right perineal lesion. CT, MRI, and bone scans were organized by her gynaecologist. Investigations were unremarkable except for a lesion in the ischial tuberosity on MRI. The lesion was deemed benign after review by orthopedic surgeons. 4 years later, the patient began to experience dyspareunia associated with a burning sensation. An MRI performed in June 2018 showed soft tissue swelling in the perineal region and a 14x13x13mm rounded soft tissue mass at the right posterolateral margin on the vaginal introitus consistent with a Bartholin's cyst. The lesion appeared to infiltrate the vaginal wall in the anteromedial margin, but this did not cross the midline .A biopsy was performed with histological features consistent with adenoid cystic carcinoma followed by wide local excision of the right vulva. The specimen spanned 43x25x32mm, and the tumour involved the excision margins. On histology, the specimen had foci of perineural invasion and invaded fibrous tissue, fat, and skeletal muscles . One monPET scan 2 months after surgery showed FDG uptake consistent with postsurgical changes and uptake in a right axillary node that was likely inflammatory rather than a distant metastasis. Further MRI contrast scans to assess extent of perineural invasion showed linear enhancement along the course of the perineal branch of the right pudendal nerve, terminating before Alcock's canal .2 cisplatin chemotherapy which she tolerated well. The ovaries were spared to prevent premature ovarian dysfunction. At her 3 month posttreatment follow-up, there was clinically and radiologically no evidence of locoregional recurrence. The patient is planned for close surveillance.The consensus of the Gynaecologic Oncology Multidisciplinary Tumour Board was to proceed with adjuvant chemoradiation therapy. The patient received radiotherapy postoperatively to the tumour bed to a total dose of 66 Gy and to the right pudendal nerve to 59.4 Gy in 33 fractions by a VMAT technique with concurrent weekly 40 mg/mThe etiology of ACCBG is unknown, though a series of ACCBG cases by Copeland has suggested that pregnancy may be an independent risk factor . ACCBGs Histologically, most ACCBG occurs in a ‘cribriform' pattern  and is dACCBG's are slow growing but are locally aggressive and have a high recurrence rate . UsuallyThere are no standard treatment guidelines for these extremely rare tumours. Surgery is currently the mainstay of treatment. Surgical options reported include wide local excisions, hemivulvectomy, simple vulvectomy, and radical vulvectomy with and without inguinal and/or femoral lymphadenectomy , 19, 20.Adjuvant radiation is also used to treat ACCBGs with a range of doses though there is no consensus regarding the role, extent, or total dose of radiation . It is rThere has been one report which stated a similar overall survival with primary radiation or chemoradiation compared to other surgical series , but to There is less evidence for chemotherapy in ACCBG compared to adjuvant radiotherapy. A variety of chemotherapy regimens used include drugs such as paclitaxel, cyclophosphamide, cisplatin, methotrexate, doxorubicin, and 5-fluorouracil , 33, 34.Adenoid cystic carcinoma of the Bartholin's gland is an extremely rare tumour with no established diagnostic or therapeutic pathway. We present a case of a 33-year-old woman with adenoid cystic carcinoma of the Bartholin's gland treated with surgery followed by adjuvant concurrent chemoradiotherapy, preserving ovarian function. Our case interestingly showed invasion of the pudendal nerve and the patient in fact had prolonged signs and symptoms for a few years before the eventual discovery of adenoid cystic carcinoma. This rare tumour often poses substantial diagnostic and therapeutic dilemmas. Case reports and multi-institutional pooled studies are imperative in contributing to future diagnostic and treatment guidelines of this extremely rare malignancy.


The perivascular epithelioid cell tumor (PEComa) is rare in young man and rarely occurs in the large intestine.The clinical characteristics, diagnosis, and managements in a 28-year-old boy who presented with sudden onset of cramping and abdominal pain and intermittent melena with a blood pressure of 74/39 mm Hg was retrospectively reviewed. CT scan of the abdomen revealed a 8.9 × 7.2 cm mass in the pelvic floor.Given the difficulty of obtaining a diagnostic specimen, surgical resection was performed. The pathology report of lower anterior resection was malignant PEComa of the rectum in 2006.Treatment consisted of surgical resection only without additional adjuvant therapy. Over the next 49 months (until 2010) after surgery, abdominal CT showed a 0.6-cm hypodense mass over the liver with suspected liver metastasis. He refused any further evaluation and treatment. After 4 years (2014), abdominal CT showed that the original mass had increased from 0.6 to 1.5 cm and the number of tumors had increased from 1 to 3. In August 2014, he underwent a metastatic hepatectomy without additional chemotherapy or radiotherapy.We noted that the metastatic progression was slow in the 4 years after the first operation. At 28 months after metastatic hepatectomy, the patient was doing well. There was also no recurrence of the PEComa of the rectum at the 120-month follow-up in 2016.To the best of our knowledge, this is the first report of a PEComa of the rectum with liver metastases treated with only surgical resection. At approximately 8.8 cm, this is the largest PEComa of the rectum reported in the recent literature. The same authors coined the term “benign clear cell (sugar) tumor” of the lung in 1971. The name refers to the clear cytoplasm of the cells, which is rich in glycogen. The cell of origin and different types of clear cell tumors of the lung  have been enigmatic and controversial for the last 3 decades. Zamboni et al asserted that clear cell tumors of the lung arise from the “perivascular epithelioid cell” (PEC) and noted that similar cells have been identified in AML and lymphangiomyomas. Tazelaar et al reported a case described as primary extrapulmonary sugar tumors (PESTs) of the vulva. The modifier “primary extrapulmonary” was used to emphasize that these tumors are not restricted to the lung.The perivascular epithelioid cell (PEC) is a cell type that is histologically and immunohistochemically present in a group of tumors, including renal angiomyolipoma (AML), clear-cell sugar tumor (CCST) lymphangioleiomyomatosis (LAM), and rare clear-cell tumors of other anatomical sites. in 1992. The term PEComa was introduced by Zamboni et al in 1996 to describe this rare family of morphological, immunohistochemical, and ultrastructural features. The World Health Organization defines PEComa as unusual mesenchymal tumors composed of histologically and immunohistochemically distinctive PECs. Currently, PEComa is a widely known and accepted entity. We present a case of rectum PEComa in a young male who initially presented with an area of intratumoral hemorrhage.There have been increasing numbers of reports of different views on and insights into clear cell tumors in recent years. The existence of PECs was first reported by Bonetti et al23, hemoglobin level of 7.4 g/dL, hematocrit of 23.9%, and platelet count of 162,000/mm3. Other biochemical tests were normal. The serum carcinoembryonic antigen level was normal.A 28-year-old male was transferred to our hospital in November 2006 because of a sudden onset of cramping and abdominal pain and intermittent melena for 4 days. Initially, he exhibited symptoms including generalized weakness, dizziness, and massive bloody stool passage. There was no significant past medical history of recent infection, inflammatory bowel disease, bleeding disorders, changes in bowel habits, significant weight loss, or tuberous sclerosis complex. The patient had no previous surgeries, and the family history was unremarkable. The patient's height was 180.2 cm, weight was 78.5 kg, blood pressure was 74/39 mm Hg, pulse rate was 122 beats/min, and temperature was 36.4°C. The physical examination revealed moderate distention and tenderness in the left lower quadrant with associated defense. On digital rectal examination, there was some gross blood mixed with soft stool and an empty ampulla. Laboratory data showed a white blood cell count of 17,120/mmThe gastroscopic evaluation was normal. The full colonoscopy revealed a greater than 4.0 cm in diameter, large ulceration with an easy bleeding mass of the rectum lying beneath the mucosa but protruding into the lumen 15 cm from the anal verge Fig. . A technGiven the difficulty of obtaining a diagnostic specimen, surgical resection and lower anterior resection were performed. On gross examination, the resected specimen was an 8.8 x 5.5 x 4.5 cm, tan, gray-white, soft, and well-circumscribed transmural mass of the rectum, mainly involving the muscularis propria and protruding into the tunica adventitia. The mucosa and submucosa were intact Fig. . The tumUnfortunately, the patient did not complete the follow-up in the outpatient department after surgery because he relocated to another city to seek work. At the 49th month (2010) postoperatively, the patient returned to the clinic upon our scheduled request. An abdominal CT scan showed a 0.6 cm hypodense mass over the liver. He refused further evaluation and treatment. In July 2014, during the course of a required physical health check, he received an abdominal CT that showed that the original mass had increased from 0.6 to 1.5 cm and that the number of tumors had increased from 1 to 3 compared with the previous CT image in 2010. We noted that the distant liver metastasis progressed very slowly during the 4 years of follow-up. The liver function and the other serum tumor markers were normal upon examination. In August 2014, he underwent segmental hepatectomy  combined with wedge resection. The pathology report of segmental hepatectomy was metastatic PEComa.This patient underwent 2 different surgical resections at 2 different times. He underwent the lower anterior resection of the PEComa of the rectum in 2006 and received the segmental hepatectomy in 2014. He is currently undergoing regular surveillance and has remained free of disease 28 months after the second operation. At the follow-up examination, the patient felt well, and the general clinical examination, subsequent colonoscopies, and abdominal CT scan once every 3 months revealed no significant findings after second operation in 2014. Since the primary surgery in 2006, there was also no recurrence of the PEComa of the rectum according to the general clinical examination, subsequent colonoscopies, and abdominal CT scan at the 120-month follow-up of the very first instance back in 2006.3,8,9 This rare neoplasm seems to arise most commonly at retroperitoneal, visceral, abdominal, and pelvic sites but has been reported at almost every body site, and the growing list of reported sites include gastrointestinal, gynecologic, and genitourinary sites, the extremities, somatic soft tissue, and the skin; among these sites are the falciform ligament, uterus, uterine cervix, liver, kidney, lung, breast, cardiac septum, pancreas, prostate, thigh, and gastrointestinal tract.,9–13 These tumors all share a distinctive cell type, the PEC, which has no known normal tissue counterpart.PEComa was introduced to describe a family of tumors, including AML, pulmonary and extrapulmonary CCST, LAM, and similar lesions arising at a variety of visceral and soft tissue sites, all of which are characterized by the same morphological, immunohistochemical, and ultrastructural features. A 7-year-old boy presented with a 3.7-cm PEComa in the ascending colon and first underwent adjuvant interferon-α 2b therapy. The other patient was a 23-year-old male who received adjuvant chemotherapy with doxorubicin and ifosfamide. However, the latter patient had evidence of local recurrence and liver metastasis at 4 months postoperatively and died of the tumor after 23 months.We reviewed the literature on gastrointestinal tract PEComa over the past 20 years, finding that malignant PEComa of the colon is extremely rare. Given the relative rarity of this group of tumors and that it was not delineated until the mid-1990s, it has not been possible to fully define criteria for malignancy in PEComas. The clinicopathological features of a total of 32 sporadic cases are summarized in Tables –17 3 of whom underwent re-resection of their tumors. The 2-year disease-free survival rate of the 12 patients was 91.7% (11/12). Two patients died of their disease. One patient died 38 months after the first operation, and the other patient died 23 months after the first operation.Follow-up data were available for only 25 of the 32 patients, and the median follow-up duration was 28.8 months (range 3–180 months) after surgery. Four cases, including our presented case, had recurrence,3.1 reported that the incidence of colon neoplasms was less than 0.1%. Folpe et al asserted that the peak incidence was in the fourth decade of life. In addition, there was a marked female preponderance.,12,13 In 2016, Chen et al reported in a review study of gastrointestinal PEComa that the gastrointestinal tract is the second most frequent site of PEComa, with the most frequent site being the gynecological tract. They further indicated that gastrointestinal PEComas of the colon and rectum were most common. In addition, there were several cases of PEComa in the gastrointestinal tract that were reported in both the pediatric,14,20–25 and adult populations,17,26–30 over the last 2 decades. In 2000, Prasad et al reported the first child with PEComa involving the appendix. In 2008, Pisharody et al reported the first child with metastatic PEComa involving the sigmoid colon. In the study by Folpe et al, follow-up information on 26 patients (median follow-up period of 30 months) revealed local recurrence and metastases in 13% and 21% of patients, respectively. The most common sites of metastasis included the liver, lung, and bone. Until more cases of this extremely rare neoplasm are evaluated in a systemic review, definitive criteria for malignancy will remain uncertain.PEComas can arise in patients of almost any age, but the precise incidence of gastrointestinal PEComas is not known. In 1999, Domoto et al3.2 Birkhaeuser et al suggest CT image evaluation at follow-up due to the unclear biological behavior of these tumors and the possibility of the rather aggressive behavior of these tumors at other locations. Most gastrointestinal PEComas manifest as a well-demarcated mass with homogeneous density in plain CT and show heterogeneous or homogenous enhancement in contrast-enhancement CT. The lesions are hypointense to isointense on T1-weighted imaging and heterogeneously hyperintense on T2-weighted imaging when applying magnetic resonance imaging (MRI). Ultrasonography may reveal a highly vascularized heterogeneous mass. However, abdominal CT, MRI, and ultrasonography are not sufficiently sensitive to enable the diagnosis of PEComas because of their nonspecific imaging characteristics.Given the present situation, close follow-up, including imaging studies and colonoscopy, is mandatory after surgical resection of gastrointestinal PEComa, especially in patients with high-grade malignancy. reported a case in which the longest period of follow-up for a colonic PEComa was 180 months. The patient, who underwent radical excision with a favorable outcome, was considered as a successful example of management. Pisharody et al presented a surveillance of a patient with physical examination and CT scans every 6 months, simultaneously, and a yearly endoscopy was also recommended. It is important that closed and long-term follow-up be accompanied by endoscopy and imaging for the purpose of ruling out local recurrence or distant metastasis of the tumor, especially in patients with high-grade malignancy. Close and long-term follow-up clinically and by CT scan is recommended. Endoscopy can help detect the lesions, which include a polypoid tumor or fungating mass protruding into the lumen with an ulcerated or smooth surface that has no specific sign.Freeman and Webber3.3 These tumors all share a distinctive cell type, the PEC, which has no known normal tissue counterpart.PEComa has been applied to an expanding family of tumors that are presumed to originate from microscopically and phenotypically unique PECs, although there is no known normal cellular counterpart to these cells. The unifying concept proposed for this family of PEComas evolved over the past century and was developed by identifying unique clear cell features as well as the smooth muscle and melanocytic characteristics of the tumor cells. The surface of the neoplasm may be tan to gray and solid, firm, or even myxoid, and areas of hemorrhage or necrosis with a white-tan to gray-red color may be grossly observed. The overall cellularity is low to moderate, but some cases are highly cellular. PEComas are comprised of the following: plump, epithelioid cells with an abundant clear to lightly eosinophilic cytoplasm; round, medium-sized nuclei with occasional moderately sized nucleoli; and a zellballen-type architecture with small tumor nests separated by thin fibrous septa containing capillaries. PEComas are generally grossly circumscribed, but some are histologically infiltrative into the surrounding soft tissue. Histologically, PEComas are composed of clear to lightly eosinophilic cells that are arranged into nests, fascicles, and occasionally sheets, often with a radial arrangement around blood vessels. Epithelioid and/or spindle cells  with a clear to pale eosinophilic cytoplasm are arranged as sheets, nests, and short fascicles, sometimes in a perivascular distribution. There is typically a prominent capillary network. Rare features have included extensive stromal hyalinization and ganglion-like cells. Atypical histological features associated with malignant behavior are noted as follows. An admixture of epithelioid and spindled cells is common. Some cases are predominantly spindled and identical to the so-called clear cell myomelanocytic tumors, and some are predominantly epithelioid and identical to CCST or monotypic epithelioid AML.PEComas have morphologically, immunohistochemically, ultrastructurally, and genetically distinctive features such as an epithelioid appearance with a clear to granular cytoplasm, a round to oval, centrally located nucleus, and an inconspicuous nucleolus. Most tumors have few, if any, mitotic figures (MFs), but some, especially those of higher nuclear grade, may have prominent mitotic activity [greater than 5 MF/50 high-power fields (HPFs)], including atypical MF. Coagulative necrosis and angiolymphatic invasion are uncommonly identified.Most tumors are composed of relatively uniform nuclei of low nuclear grade, but some have higher grade nuclei and prominent nuclear pleomorphism that is identifiable at low magnification. Multinucleated giant cells are common, and, occasionally, giant cells are encountered with a central eosinophilic zone surrounded by a peripheral clear zone reminiscent of the spider cells seen in adult rhabdomyoma. reported 26 cases of PEComas of soft tissue and gynecologic origin and proposed criteria for the classification of these tumors as “benign,” “of uncertain malignant potential,” and “malignant.” They observed a significant association of tumor size greater than 5 cm, infiltrative growth pattern, high nuclear grade, necrosis, and mitotic activity greater than 1/50 HPF with subsequent aggressive clinical behavior of PEComas. Malignant PEComa seems to be a very aggressive neoplasm leading to multiple metastases and death.,16,19 Malignant members of the PEComa family  have also been reported, but firm criteria for malignancy have not yet been established. In 2013, Doyle and Hornick claimed that these factors should be predominant predictors of malignant behavior, such as marked nuclear atypia, diffuse pleomorphism, >2 mitoses per 10 HPF, and metastases. Malignant PEComas typically demonstrate a high proliferative index with Ki-67 immunostaining. This tumor is composed of nests and sheets of usually epithelioid but occasionally spindled cells with a clear to granular cytoplasm and a focal association with blood vessel walls. The histological features predictive of poor outcome in gastrointestinal PEComa are uncertain, but the presence of coagulative tumor necrosis and size greater than 5 cm seem to be associated with early recurrence.In 2005, Folpe et al Im et al asserted that large tumor size, high nuclear grade, infiltrative growth pattern, and high proliferative activity seemed to be predictors of aggressive behavior. In addition, Lee et al reported that tumor size, location, and infiltrative growth patterns were considerable prognostic factors. Vascular invasion is uncommon. It is already apparent that a gray zone exists where prognosis cannot be defined with certainty. PEComa with definite evidence of aggressive behavior may be typically suggested by the presence of marked atypia, high mitotic activity, or coagulative necrosis.In addition to the criteria by Folpe et al, which is the most frequently positive melanocytic marker. PEComas typically show immunohistochemical evidence of both smooth muscle and melanocytic differentiation. They show immunoreactivity for both melanocytic (HMB-45 and/or melan-A) and smooth muscle (actin and/or desmin) markers. Defined by the coexpression of melanocytic and muscle markers, PEC tumors do not have predictable histopathological behavior. The therapy consists of radical resection. HMB-45 was the most sensitive melanocytic marker (96% of cases), followed by Melan-A (72%), microphthalmia transcription factor (MiTF) (50%), and desmin (36%). Although uncommon, some cells may also stain positive for pancytokeratins. Nevertheless, more cases should be analyzed to better understand the origin and histogenesis of PEComas.Knowledge of the patient's clinical history is crucial, but immunohistochemistry is also invaluable. In view of the potential wide differential diagnosis of these tumors, immunohistochemistry is usually required to confirm the diagnosis of PEComa. To date, the precursor lesion or cell of origin has not been identified. However, these tumors characteristically tend to stain positive for HMB-45, Immunohistochemical demonstration of melanocytic differentiation is the most reliable way to distinguish PEComa from GIST.Strong and diffuse expression of CD117 of the PEComas highlights an important differential diagnostic problem between PEComa and GIST because PEComa is a biphasic mesenchymal tumor with GIST-compatible morphology. Because less than 50% of tumor cells are CD117-positive in some cases of GIST, the use of melanocytic markers is mandatory because GISTs are negative for melanocytic markers.3.4PEComa is a rare neoplasm, and no standardized treatment has been established. Currently, surgery is the mainstay of treatment for primary PEComa at presentation, as well as for local recurrences and metastases, with the aim of obtaining clear resection margins. It is possible that PEComas of the colon occurred previously but could not be recognized as such because of the lack of current diagnostic capabilities. Primary excision is usually curative, as most tumors are benign. Surgical resection of the tumor with the adjacent tissue in the gastrointestinal tract is the mainstay of treatment of the primary tumor and of local recurrence.,11,13,27 Tumor size ranges widely, but most are between 4 and 6 cm at the time of excision. In our presented case, metastatic lesions were successfully managed by resection alone. Cheng et al reported a case with a recurrent PEComa of the sigmoid colon with pancreatic metastasis that was treated with surgical resection only. Surgery seems to be the only approach for aggressive cases, as chemo- and radiotherapy have not shown significant positive results.For most tumors, surgical resection is the first or only effective treatment. The most effective treatment for gastrointestinal PEComa is surgical resection.,19,34,35 Rigby et al reported an 11-year-old girl with metastatic renal PEComa treated with dacarbazine-based chemotherapy and imatinib mesylate. The tumor did not respond to an initial treatment of chemotherapy.The role of surgical resection and chemo- and radiotherapy is currently not well defined. However, locally advanced or metastatic disease portends a poor prognosis, and strategies incorporating chemotherapy, radiation, and immunotherapy have been reported. Park et al first reported a 7-year-old boy treated with surgical resection and adjuvant IFN-α2b immunotherapy for pediatric PEComa of the ascending colon.There are obvious difficulties in performing a therapeutic trial mainly due to the rarity of the disease. Some patients have been treated with adjuvant therapy (including Gleevec), but the efficacy of such therapy is not known. The effect of adjuvant or palliative treatment is uncertain and unpredictable. The outcome of our present case adds to the main evidence base of curative surgery for the treatment of malignant PEComa. Whether the tumor is benign or malignant cannot be predicted, so that the main treatment for the disease may be surgical resection, especially if malignant cases are encountered, as is done with GISTs.Furthermore, potential benefits of adjuvant chemotherapy have not been investigated.; however, malignant PEComas have been increasingly reported over the past 2 decades.,21,27,34 Because relatively few malignant PEComas have been reported, firm criteria for malignancy have not been established. Infiltrative growth or edges, marked hypercellularity, and marked nuclear pleomorphism/atypia may be secondary features suggesting aggressive behavior or malignancy.,33,34,35 Most reported malignant PEComas contained necrotic areas, and many revealed high mitotic index.,16,19,37 The risk of recurrence and metastasis risk is low if the Ki-67 marker index is less than 1% of PEC tumors. We noted that Ki-67 labeling of 5% of neoplastic cells was observed in PEComas in the colon and rectum that behaved aggressively in Table Clinically, most PEComas follow a benign course Therefore, metastatic spread of PEComas may, in some cases, be a late complication that presents after many years. This finding highlights both the need for criteria that more accurately predict the behavior of PEComas and the need for long-term follow-up of patients with PEComas, as widespread metastases may present as a late complication.In addition, in another report, PEComa was not recognized at initial presentation, and the diagnosis of PEComa was not made until the patient returned with a metastasis. At the 49th postoperative month, we found that liver metastases and metastatic tumor growth progression were slow after first operation. The treatment strategy for malignant PEComa is still ambiguous and controversial, especially in advanced or metastatic disease. It is difficult to diagnose PEComa by extensive imaging studies or tumor markers.PEComa can appear in many parts of the body, but there have only been a few reports of PEComa of the colon and rectum. Diagnosis is still unclear without a clear definition of malignant PEComa of the colon. Malignant PEComa is an aggressive disease leading to multiple metastases and death, as expected with a high-grade sarcoma.Postoperative patients need to be closely monitored because the treatment protocol for PEComa of the colon and rectum has not reached a worldwide consensus. It appears that long-term follow-up is necessary for all patients with PEComas of the colon because the prognosis of the disease is not entirely known currently.4To the best of our knowledge, this is the first case of intestinal PEComa of the rectum with liver metastasis without adjuvant chemotherapy or radiotherapy after 2 surgical resections and follow-up for 120 months. At 28 months after segmental hepatectomy, the patient was doing well. There is no gold standard guidance for the diagnosis and follow-up of PEComas. We believe that histopathological studies produce a valid definition for PEComa prognosis and the establishment of a clinical registry for PEComa of the colon seems to be necessary in the future.Conceptualization: Kung-Hung Lin, Li-Ren Liou.Data curation: Li-Ren Liou.Investigation: Ming-Shan Su.Methodology: Ming-Shan Su.Project administration: Meng-Lin Huang.Supervision: Nai-Jen Chang, Min-Jen Tsao.Validation: Min-Jen Tsao.Writing – original draft: Kung-Hung Lin.


Teaching point: The typical “cracked dry mud” pattern on ultrasound may help avoiding biopsy for proliferative myositis which is a self-resolving benign process. Muscular ultrasound (US) was required for a 53-year-old woman presenting with a rapidly growing firm and painless mass developing near the humeral insertion of the left pectoralis major. Longitudinal US Figure  and 1c sPM is a rare self-limiting and rapidly growing worrisome pseudosarcoma process of skeletal muscles affecting middle-aged patients . NeverthPM diffusely involves the muscular connective stromal network  with respect to the muscular fibres. It is constituted by a poorly demarcated network of myofibroblasts – accompanied by large basophilic giant cells resembling ganglion cells – infiltrating and dissociating the muscle fibers . The celComputed tomography lacks of any specificity but MRI may show more diagnostic features. T2-weighted MR images, STIR sequences and post-contrast T1-weighted images typically demonstrate a moderate to marked hyperintense and enhancing soft-tissue infiltrating mass. The geometrical network of hypointense bundles of muscle fibres surrounded by hyperintense inflammatory tissue may be seen on views perpendicular to the muscular fascicles.US has a high spatial resolution and unlimited capabilities of multiaxial imaging facilitating optimal exploration of superficial muscles along their longitudinal or transverse axis. US is usually able to recognize the typical network of peri-, inter- and intra-fascicular inflammatory infiltration of PM with respect of the muscle fibres. The very typical “dry cracked mud” US pattern found on transverse views is unequivocally suggestive of PM .As illustrated in our case, the robustness of US diagnosis of PM could allow a watch-and-wait option as an alternative method of follow-up, avoiding unnecessary biopsy and/or potentially mutilating surgery. Fine-needle biopsy would be reserved only for doubtful cases.


Melanoacanthoma is a rare variant of seborrheic keratosis, which is notable for dark pigmentation and fast radial growth, making it difficult to distinguish from melanoma. Histologically, it is characterized by proliferation of keratinocytes and dendritic melanocytes. The authors report a scalp lesion, fast growing, suspected by dermoscopy and confocal microscopy examination, with dendritic cells distributed throughout the lesion. Based on these findings, it was not possible to classify this lesion as clearly benign, so it was excised. Histopathologic evaluation and immunostain were consistent with melanoacanthoma. Cutaneous melanoacanthoma (CM) is a rare variant of seborrheic keratosis (SK) that occurs mainly in fair-skinned adults, preferentially in the head, neck, and chest.A 76-year-old male patient presented a rapidly growing 9-mm asymmetric, dark-brown patch on the scalp. Dermoscopic examination showed comedo-like openings, fat fingers, and irregularly distributed dots .Figure 1The first diagnostic hypothesis was SK, but the irregularly distributed dots lead the authors to perform RCM to exclude melanoma. It showed a typical honeycomb pattern at the spinous-granular layer, epidermal projections, keratin filled invaginations, and widespread dendritic pagetoid cells. At the dermo-epidermal junction (DEJ), densely packed polymorphous dermal edged papillae were observed, in addition to an infiltration of dendritic cells between dermal papillae. At the papillary dermis level, melanophages were observed . Based oOn histopathological evaluation, hyperplastic epidermis, hyperkeratosis, acanthosis, and dendritic melanocytes were seen throughout the lesion. Melan-A and CD1a immunostain were positive and confirmed an increased density of melanocytes and Langerhans cells . These fCM is an uncommon neoplasm histologically characterized by proliferation of epidermal keratinocytes and large dendritic melanocytes. The melanocytes are scattered throughout the tumor.Four case reports have previously described dermoscopic appearance of CM, and features of both benign as well as malignant lesions have been reported. Rossiello et al. described a starburst pattern,The assessment of the reported lesion with polarized light (PL) dermatoscopy demonstrated comedo-like openings, fat fingers, and irregularly distributed dots. PL offers a better view of structures located deeper into the skin, whereas non-polarized light allows a better visualization of more superficial structures. With PL, milia-like cysts are less visible; the melanin appears sharper and darker, and comedo-like openings can be interpreted as dots. Thus, the use of PL instruments, increasingly popular among dermatologists, may hinder the diagnosis of CM.In equivocal clinical and dermoscopic SK cases, Ahlgrimm-Siess et al. showed that at least three RCM criteria for SK diagnosis can be found in almost of all lesions; including epidermal projections, keratin-filled invaginations, and corneal pseudocysts at the suprabasal levels; densely packed polymorphous dermal papillae at the DEJ level. Furthermore, there is absence of any features suggestive of malignancy.in situ.On RCM examination, the presence of pagetoid spread of dendritic melanocytes within the epidermis, as well the presence of these cells at DEJ, is an important clue for melanoma, in particular the lentigo malign subtype.RCM features of CM have been described in only one case by Shahriari et al. The authors observed numerous, tangled dendritic cells, at the basal and supra-basal layers. This pattern was interpreted as suspicious for melanoma and the lesion was excised, confirming CM.In the present case, a regular honeycomb and polymorphous papillae were identified, as well as an infiltration of dendritic cells at the basal and supra-basal layers and at the DEJ level. Concerned with these diffuse dendritic cells, even in the presence of other RCM criteria of benignity, the authors performed a biopsy. In hindsight, dendritic cells scattered throughout the tumor in the presence of RCM criteria for SK are consistent with the histopathological description of CM.In conclusion, ruling out melanoma was difficult after RCM imaging in the present case, as it was for Shahriari et al. The present authors believe that the appearance of uniform dendritic melanocytes throughout the lesion, in the context of a thickened epidermis with RCM features of SK, may be a possible clue of CM. Reports of more cases analyzed with RCM and the definition of criteria for diagnosis of this benign tumor is necessary in order to prevent CM from being a pitfall of RCM examination.None declared.Ana Carolina Souza Porto: Approval of the final version of the manuscript; conception and planning of the study; elaboration and writing of the manuscript; obtaining, analyzing and interpreting the data; effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic conduct of the cases studied; critical review of the literature; critical review of the manuscript.Tatiana Pinto Blumetti: Effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic conduct of the cases studied; critical review of the literature; critical review of the manuscript.Mariana Petaccia de Macedo: Effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic conduct of the cases studied; critical review of the literature; critical review of the manuscript.Juliana Casagrande Tavoloni Braga: Effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic conduct of the cases studied; critical review of the literature; critical review of the manuscript.None declared.


Juvenile polyposis is an autosomal dominant inherited disease characterized by the development of numerous hamartomatous and nonneoplastic polyps of the gastrointestinal tract. Juvenile polyposis has also recently been reported as a predisposition for gastrointestinal cancer.SMAD4 and BMPR1A. However, based on the few characteristic physical findings and histopathological features, the final diagnosis was juvenile polyposis restricted to the stomach.A 63-year-old man underwent esophagogastroduodenoscopy because of anemia and hypoalbuminemia during a follow-up for gastric polyposis, which showed multiple reddish polyps and two elevated lesions in the stomach. The elevated lesions were diagnosed as well-differentiated adenocarcinomas by biopsy. He had no specific physical findings or family history. Computed tomography showed gastric wall thickening without lymphadenopathy or distant metastasis. Colonoscopy showed an adenoma in the transverse colon. He underwent laparoscopy-assisted total gastrectomy with Roux-en-Y esophagojejunostomy. The resected specimen revealed numerous variously sized non-pedunculated polyps throughout the stomach, diagnosed histopathologically as hamartomatous polyps. The two elevated lesions were diagnosed as a well-differentiated adenocarcinoma restricted to the mucosa and a well-to-poorly differentiated adenocarcinoma invading the submucosa with prominent lymphatic permeation, respectively. Genetic analysis failed to identify any germline mutations in the genes usually associated with juvenile polyposis, including This patient represented a rare case of non-familial juvenile polyposis of the stomach with gastric cancers. Juvenile polyposis has malignant potential, and patients should therefore be carefully followed up. Surgical treatment, particularly total gastrectomy, is recommended as a standard treatment in patients with juvenile polyposis of the stomach with gastric cancer. SMAD4 and BMPR1A have recently been identified as causative genes for juvenile polyposis [Juvenile polyposis is a gastrointestinal polyposis characterized by the development of numerous hamartomatous and nonneoplastic polyps , with a olyposis . Furtherolyposis –8. We reAPC, KRAS, TP53, and ERBB2 genes in carcinoma, but failed to detect any germline mutations, including in SMAD4, BMPR1A, or PTEN, in hamartomatous polyps and carcinoma. However, based on the few characteristic physical findings and the histopathological features of the polyps, the final diagnosis was juvenile polyposis restricted to the stomach with gastric cancers. The patient was discharged on postoperative day 8 and has been monitored carefully with no adjuvant chemotherapy, by his request. There is no evidence of recurrence 16 months after surgery.A 63-year-old man was evaluated for anemia (hemoglobin 11.8 g/dl) and hypoalbuminemia  in another hospital. He had been diagnosed with gastric polyposis 5 years ago. He underwent esophagogastroduodenoscopy, which showed multiple reddish polyps accompanied by bleeding and erosion throughout the stomach Fig.  and two Juvenile polyposis is a rare disease characterized by the development of numerous hamartomatous and nonneoplastic polyps throughout the gastrointestinal tract . DiagnosSMAD4 [BMPR1A [SMAD4 and BMPR1A. The current patient had no family history of juvenile polyposis or germline mutations in the SMAD4 and BMPR1A genes but was diagnosed with juvenile polyposis of the stomach based on physical findings and histopathological features. Case reports of non-familial juvenile polyposis published in PubMed from 1985 to 2017, including the present case (n = 12), are summarized in Table Hamartomatous polyposis syndrome of the gastrointestinal tract with autosomal dominant inheritance includes not only juvenile polyposis, but also Peutz–Jeghers syndrome and PTEN hamartoma tumor syndrome . In addi [BMPR1A  as genes [BMPR1A , which m [BMPR1A . The pre [BMPR1A , 18, whiSMAD4 germline mutation [SMAD4 or BMPR1A, but did have somatic mutations in APC, KRAS, TP53, and ERBB2 genes. In addition, the histopathological findings showed a well-differentiated adenocarcinoma, or intestinal type according to the Lauren classification [TP53 mutation, and RTK-RAS activation [Although juvenile polyposis is a nonneoplastic lesion, several cases have suggested that it represents a predisposition to gastrointestinal cancer –8. The rmutation . The curfication , directltivation .Accordingly, patients with juvenile polyposis should undergo regular follow-ups, including gastrointestinal endoscopy, with surgical treatment in the event of gastric cancer. However, recurrences of gastric cancer and juvenile polyps in the remnant stomach have frequently been reported in patients who underwent partial gastrectomy , 32, andWe report a rare case of a patient with non-familial juvenile polyposis of the stomach with gastric cancers. This case highlights the malignant potential of juvenile polyposis and the need for careful follow-up of such patients. Total gastrectomy is recommended as a standard treatment in patients with juvenile polyposis of the stomach with gastric cancer.


Hemolymphangioma is a rare, noninvasive benign tumor of mesenchymal origin resulting from malformation of vascular and lymphatic vessels. The incidence of hemolymphangioma in the spinal canal is low.This report describes 2 patients with a lesion located in the thoracic spinal canal or spinal epidural space, who were misdiagnosed with suspected meningioma or suspected schwannoma, respectively, based on magnetic resonance imaging (MRI).Hemolymphangioma.The application of a surgery was designed to treat the 2 patients.2 patients stated that symptoms were improved after the operation.This report should raise awareness among clinicians that careful image analysis and consideration of patient history and pathology is required for accurate differential diagnosis of hemolymphangioma in the spinal canal and spinal epidural space. Previously, reports have described hemolymphangioma in the abdomen, mediastinum, extremities, oral region, and on the tongue and orbit. To the author's knowledge, there are no reports of hemolymphangiomas in the spinal canalin international papers. This article presents 2 cases of hemolymphangioma in the spine that were misdiagnosed based on findings from magnetic resonance imaging (MRI)Hemolymphangioma is a rare, noninvasive benign tumor of mesenchymal origin resulting from malformation of vascular and lymphatic vessels. Hemolymphangioma can be congenital or acquired, but are most frequently congenita,2Approval was obtained from the Ethics Committee of The First Hospital of Jilin University, and all subjects gave their informed consent to participate.Case 1: A 58-year-old male presented to our hospital with thoracic back pain, with weakness and hypoesthesia in both lower extremities. The patient was administered rehydration therapy for 1 day, and his thoracic back pain was slightly alleviated, however, weakness with hypoesthesia in the lower extremities, gradually worsened. Physical examination, including the Glasgow Coma Scale, revealed that the patient was conscious (spontaneous eye response: +4) verbally fluent (oriented: +5) but had no motor response (+1). The patient had normal upper limb strength (grade 5/5), but grade 0/5 (no contraction) lower limb strength, although muscle tension was normal. The patient was experiencing numbness from T6 to T12 and had pain in the mid-thoracic spine (T4 to T6). Residual neurological examination revealed no obvious abnormalities. The MRI scan demonstrated a 2.5 cm × 1.5 cm lesion at T3/T4 that appeared hyperintense on T1-weighted images  verbally fluent  but had no motor response (+1). The patient had normal upper limb strength (grade 5/5) and grade 4/5 lower limb strength. Sensitivity to pain, temperature, and coarse touch of both lower limbs were decreased, and there was a positive bilateral Babinski sign. The MRI scan revealed a 6.1 cm × 0.9 cm lesion in the spinal epidural space at T10 to T12 that infringed upon the adjacent intervertebral foramen and appeared hypointense on T1-weighted images (Fig. 3 On immunohistochemistry analysis, hemolymphangiomas are CD31 and D2-40 positive. Hemolymphangiomas may be congenital or acquired. Congenital hemolymphangiomas result from an obstruction of the venolymphatic communication between the systemic circulation and the dysembryoplastic vascular tissue. Acquired hemolymphangiomas occur due to inadequate lymph drainage and damage to the lymphatics resulting from surgery or trauma. Evidence suggests that imaging characteristics of hemolymphangiomas vary according to the location in the body, proportion of blood and lymphatic vasculature, and imaging modality. Ultrasound, computed tomography, and MRI are useful for the diagnosis of hemolymphangiomas, with findings dependent on the amount of water-based substance and number of blood vessels in the lesion. The patients in this report underwent contrast-enhanced MRI. In Case 1, MRI findings indicated a lesion resembling a meningioma, a commonintra-spinal canal tumor, and a more likely diagnosis than spinal hemolymphangioma. However, careful analysis of imaging results should distinguish between meningioma and hemolymphangioma, as meningiomas arise from the arachnoid layer of the meninges, most have a dural tail attaching them to the dura mater, and some contain calcified deposits. MRI imaging of Case 1 revealed hyperintense signals in T1- and T2-weighted images of the lesion. Combined with pathological results, these findings were indicative of subacute thrombosis, which is considered a characteristic feature of hemolymphangioma; this suggests that MRI is an effective tool for the diagnosis of this lesion. Hemolymphangioma was considered responsible for the patient's back pain and weakness in the lower extremities as it is a slow growing lesion that is associated with gradual onset of clinical symptoms. The acute onset of serious dysfunction of the lower limbs was attributed to thrombosis.Hemolymphangiomas present as cystic or cavernous lesions that consist of dilated veins and lymphatics interspersed with normal stromal tissue and vasculature; dilated vessels may contain thrombosis. As hemolymphangiomas show “crawling” progressive growth, rather than the expansion growth characteristic of schwannomas, careful analysis of imaging results should distinguish between the 2.The patient in Case 2 had lower limb symptoms for 4 years. This is consistent with chronic progressive spinal cord compression and the presence of a slow growing mass. MRI imaging of Case 2 revealed a lesion in the spinal epidural space that was infringing upon the adjacent intervertebral foramen. This resulted in a diagnosis of an atypical schwannoma, the most common extramedullary spinal tumor. Schwannomas generally appear as rounded lesions on imaging; however, they often protrude out of neural exit foramen and forma dumbbell shape. Schwannomas are hypointense on T1-weighted images and hyperintense on T2-weighted images with necrotic and cystic areas.Both patients in this study were treated with surgical resection, which is considered the most effective management for hemolymphangioma. Recurrence rates are reported at 10% to 27% following complete removal of a hemolymphangioma.In conclusion, the incidence of hemolymphangioma in the spine is low, and clinical diagnosis remains challenging. This report describes 2 patients with hemolymphangioma in the thoracic spinal canal or spinal epidural space who were misdiagnosed with a suspected meningioma or suspected schwannoma, respectively. Our findings suggest that MRI combined with patient history and pathology is required for an accurate diagnosis of hemolymphangiomas in the spinal canal and spinal epidural space. This report should raise awareness among clinicians of the importance of MRI, patient history, and pathology in the differential diagnosis of hemolymphangioma.


Abdominal apoplexy due to short gastric artery rupture following vomiting is an exceedingly rare condition. It results from non-traumatic and non-iatrogenic causes. This entity has variable clinical presentation and patients usually present with non-specific abdominal pain. Imaging plays a vital role in early diagnosis, as immediate exploratory laparotomy is the treatment of choice for successful outcome and helps to reduce mortality rate. We report the case of a 27-year-old male patient who presented to the emergency department with acute-onset abdominal pain after multiple episodes of vomiting following binge alcohol drinking. Contrast-enhanced CT revealed intraperitoneal haemorrhage secondary to vessel rupture, probably from a short gastric artery. Intraoperatively, the short gastric artery was identified as the bleeding source and ligated. The patient had an uneventful postoperative course. He reported a pain score of 10 at presentation . Laboratory investigations showed a haemoglobin level of 13.7 g dl−1 (13–17), leukocyte count of 11.2 × 109 l−1 (4–11) and haematocrit of 42.6% (42–52). Prothrombin time, activated partial thromboplastin time and international normalized ratio were within normal limits. Liver function tests and serum amylase level were within normal limits.A 27-year-old male presented to the emergency department with severe epigastric and left hypochondriac pain of 1-day duration. He also complained of pain radiating to the left shoulder, worse with inspiration and lying down. He gave a history of drinking about 1.5 l of alcohol over a period of 2 h. He subsequently had repeated episodes of vomiting, following which he developed severe abdominal pain. The duration, from onset of pain to presentation, was about 8 h. There was no significant past clinical or surgical history. There was no history of abdominal trauma. On physical examination, he was tender, predominantly in the epigastric and left hypochondriac region with no guarding or rebound tenderness. No abdominal distension was noted. The remainder of the physical examination was unremarkable. On presentation, his vital signs were stable; heart rate was 69 minBased on the clinical presentation of several episodes of vomiting followed by acute onset severe abdominal pain after binge alcohol intake, the clinical differentials considered included acute alcoholic pancreatitis and acute gastritis. Other possible differentials considered were Boerhaave’s syndrome and Mallory–Weiss tear, given the multiple episodes of vomiting.Chest and abdominal radiographs were normal. In view of the continuous abdominal pain, contrast-enhanced CT of the abdomen and pelvis was performed. The portal venous phase images demonstrated an approximately 13 × 12 × 10 cm large hyperdense collection [50–60 Hounsfield units (HU)] in the left hypochondriac region adjacent to the greater curvature of the stomach. There was a focus of active contrast extravasation (230 HU) within this collection . Delayed−1 (13–17) and 38% (42–52), suggesting volume loss. In view of the imaging findings of active intraperitoneal bleed and falling haemoglobin levels, the patient was immediately taken for exploratory laparotomy. The intraoperative findings echoed the preoperative imaging findings, with the presence of haemoperitoneum. Around 1.2 l of blood clot was evacuated from the peritoneal cavity, largely from around the greater curvature of the stomach (−1 (13–17); hence no blood transfusion was required. The patient was well when discharged 3 days later.The patient was haemodynamically stable during and after the scan. Repeat haemoglobin and haematocrit levels done 8 h after admission were 11.5 g dl stomach . The sou stomach , which w1 The term abdominal apoplexy was coined by Green and Powers2 in 1931.1 Carmeci et al3 found only 110 cases of abdominal apoplexy between 1909 and 1998.1 This condition usually occurs in late middle age between 55 and 64 years and affects males more commonly than females in a ratio of 3 : 2.1Barber first reported idiopathic spontaneous intraperitoneal haemorrhage in 1909, in a pregnant female with no history of trauma.4 Other causes include malignancy , gynaecological disorders , blunt trauma and idiopathic or excessive anticoagulation.5 The common vessels to rupture are the left gastric artery, superior mesenteric artery and the splenic artery. Abdominal apoplexy secondary to rupture of short gastric vessels is exceedingly rare.1Most cases of abdominal apoplexy occur secondary to vascular pathology such as arteriosclerosis with or without hypertension; aneurysm of splanchnic artery, mainly splenic and hepatic artery; and vascular erosion caused by an adjacent neoplastic or inflammatory process.1 Hayes et al6 postulated that excess vomiting and retching may pull the gastrosplenic ligament, secondary to partial gastric volvulus, creating a shearing force that tears the short gastric artery. In our case, the likely antecedent factor could be vomiting.It has been postulated that vomiting can predispose to short gastric vessel rupture. Olaoluwakitan et al found only 13 reported cases of short gastric vessel rupture in the literature, of which 8 cases occurred following vomiting.1 Our patient presented in the initial phase.Clinically, patients with haemoperitoneum may present in three phases. The initial phase, which is characterized by mild-to-severe abdominal pain due to generalized peritoneal irritation. The pain severity depends on the volume and the rate of bleeding. This is followed by a latent period, which is asymptomatic. In the terminal phase, symptoms usually become more severe and the patient may go into hypovolemic shock.7 In a haemodynamically stable patient, as in our case, contrast-enhanced CT scan of the abdomen and pelvis is the most useful imaging investigation to diagnose abdominal apoplexy preoperatively.1 CT scan findings of haemoperitoneum include a sentinel clot, hyperdense mesenteric fluid and active arterial extravasation. These signs help radiologists in identifying the source of intraperitoneal haemorrhage and help direct management.8 The imaging findings may also help in identifying patients going into hypovolemic shock at an early stage, which may need prompt fluid resuscitation and restoration of circulating volume. The earliest CT imaging sign of decreased vascular volume is flattening of the inferior vena cava. If there is further volume loss leading to hypoperfusion complex, the characteristic imaging findings are increased enhancement of the bowel wall, kidneys, adrenal glands, vasculature and decreased enhancement of the spleen and pancreas. This may help the clinician expedite the initiation of life-saving intervention before the patient’s instability manifests clinically.9In haemodynamically unstable patients, the diagnosis is generally made on exploratory laparotomy. In such patients, focused assessment by sonography in trauma examination may be a useful preliminary tool to detect intra-abdominal haemorrhage.1 In haemodynamically stable patients and those who are poor surgical candidates, if the site of active bleeding has been identified on a CT scan, percutaneous transarterial embolization therapy11 using coils may be considered as a non-invasive, alternative option to surgery. It can also serve as an adjunct to surgery to overcome life-threatening bleeding prior to definitive surgical treatment. However, immediate exploratory laparotomy is required for haemodynamically unstable patients.11 Early diagnosis and intervention determines patient prognosis, as non-surgical mortality may reach 100%. Hence, it is imperative to diagnose this entity at an early stage followed by prompt surgical intervention. This may help reduce mortality rates to less than 8.6% if a bleeding point was located and ligated, as opposed to 56% in cases where a bleeding point could not be identified.12Surgery is a definitive treatment for abdominal apoplexy, in addition to restoration of circulating volume. Another treatment option described is transarterial embolization. The choice of treatment is decided based on the age and condition of the patient, as well as the bleeding site.Short gastric artery bleed is a rare cause of abdominal apoplexy, and knowledge of this entity helps in prompt diagnosis and early surgical intervention, escalating the prognosis rate.Abdominal apoplexy secondary to short gastric rupture is known to be associated with frequent bouts of vomiting. Hence, in a patient presenting with abdominal pain following frequent episodes of vomiting, with or without signs of peritonism, the differential of abdominal apoplexy should be kept in mind and investigated promptly.If clinically suspected, early imaging with CT may help achieve a correct preoperative diagnosis of abdominal apoplexy, localize the bleeding site and help direct management.Written informed consent for the case to be published  was obtained from the patient for publication of this case report.


Tuberculosis is a communicable disease caused by mycobacterium tuberculosis that primarily affects the lungs. Primary tuberculosis of the nose in the pediatric age group is rare. The diagnosis of this common entity in the present case was challenging.We report the case of a 3-year old girl who presented with a painless swelling over the dorsum of the nose for 7 months. Imaging revealed a mass lesion eroding nasal bones, septum and frontal bone with intracranial extension. Endoscopic examination showed a friable mass in the superior aspect of the nasal septum, extending intracranially. Histopathology confirmed the diagnosis of nasal tuberculosis, and the patient improved on category I anti-tubercular therapy.Midline nasal swelling in children needs to be differentiated from congenital nasal swelling. A high index of suspicion is required for correct diagnosis of a patient with nasal tuberculosis. Anti-tubercular therapy is the mainstay of treatment. Tuberculosis in the head and neck region mainly affects the cervical lymph nodes, pharynx, larynx and ear . Nasal tA 3-year old girl presented with swelling over the root of the nose. Her parents had noticed swelling 7 months previously, which was gradually progressive and was the size of a small lemon at the time of presentation. The swelling was not associated with fever, nasal obstruction, bleeding or any other nasal complaints. The patient was prescribed antibiotics and anti-inflammatory drugs by the general practitioner, following which the swelling decreased slightly. The patient had recurrent episodes in which the swelling increased in size. The size of the swelling decreased on medication, but never completed resolved. There was no history of tubercular contact or any other family history of tuberculosis. On examination, the root of the nose was broadened by the ill-defined 2.5×2.0 cm swelling, but the overlying skin was normal .On palpation, the swelling was soft and non-tender with a normal temperature. Anterior rhinoscopy revealed no abnormality. Blood analysis showed raised erythrocyte sedimentation rate . The patient had a positive Monteux test, and a chest X- ray showed no abnormality. Contrast enhanced computed tomography (CT) of the nose revealed an enhancing soft tissue mass lesion in the superior aspect of both of the nasal cavities, measuring 2.0 ×1.8 × 2.8 cm (AP × TR × CC), eroding the intervening nasal septum, frontal and nasal bones at the glabella and reaching to the subcutaneous tissue . The base of the skull was eroded with a small intracranial extension. Magnetic resonance imaging (MRI) of the brain and paranasal sinus revealed enhancing soft tissue mass in the basifrontal extra-axial region with dural enhancement extending inferiorly into the superior parts of the nasal cavities with osseous destruction . The patient underwent endoscopic examination and biopsy under general anesthesia. On endoscopy, a smooth reddish mass was seen in the left nasal cavity. The mass was friable and found to be eroding the nasal septum, reaching the opposite nasal cavity . The mass was also found to be eroding the most superior aspect of the nasal septum with intracranial extension. As the mass was invasive with intracranial extension, a biopsy was taken and further definitive surgery was deferred for final histopathology diagnosis. Histopathology showed lesional tissue focally lined pseudostratified ciliated columnar epithelium with subepithelial epithelioid cell granuloma admixed with Langhans giant cells, suggestive of tuberculosis . The patient was started on category I anti- tubercular treatment. Isoniazid, rifampicin, pyrazinamide and ethambutol was prescribed for the first 2 months followed by isoniazid and rifampicin for the next 4 months. The swelling subsided on anti-tubercular therapy. At the time of writing, the patient has completed 6 months of anti-tubercular treatment and has been symptom free for 7 months following anti-tubercular therapy.Tuberculosis is a communicable disease caused by mycobacterium tuberculosis. The mode of spreading of tuberculosis is a droplet infection, and the lungs are the most common site of infection. Primary nasal tuberculosis is a rare entity .Nasal infection may result from direct inoculation, nose picking, by open tuberculosis or by haematogenous dissemination of a primary infection. Three forms of nasal tuberculosis have been recognized. Lupus vulgaris, or the nodular form, presents as papules and crusting over the nasal vestibule and the adjoining skin. The ulcerative form involves the cartilaginous nasal septum or the inferior turbinate and presents with nasal obstruction, crusting, discharge and epistaxis. The third form is sinus granuloma, which present as a mass in the paranasal sinuses with osteomyelytic changes .In the present case, a 3-year old girl presented with swelling at the root of the nose. Pediatric nasal tuberculosis has previously been reported in the literature at the age of 10–12 years, and the present case is the youngest case of nasal tuberculosis. Nasal tuberculosis has been reported in the literature presenting as nasopharyngeal mass, sino-nasal mass, pansinusitis epistaxis and epilepsy, facial abscess, proliferative lesion over the dorsum of the nose and as nasal bone osteomyelitis with cervical lymphadenitis -14. NasaMidline nasal swelling in the pediatric age group need to be differentiated from nasal dermoids, teratomas, gliomas, encephaloceles and hemangiomas . In the Nasal endoscopy showed a pale polypoidal, non-pulsatile mass arising from the roof of the nasal cavity and the superior-most part of the nasal septum. The mass did not increase on anesthetic Valsalva maneuver. The nasal mass was found to be friable and eroding the superior-most part of the bony nasal septum, reaching the other nasal cavity with erosion of the cribriform plate with intracranial extension. Unlike the classical description in which nasal tuberculosis involves the cartilaginous part of the nasal septum, our case had involvement of the bony part of the nasal septum. The diagnosis of nasal tuberculosis was confirmed on histopathology, which showed an epithelioid granuloma with Langhans giant cells . In addiThe present case report highlights the uncommon presentation of primary nasal tuberculosis as a midline nasal swelling in the pediatric age group, with the absence of nasal symptoms and the involvement of bone with intracranial extension.


To discuss about the possible treatment modalities of supernumerary tooth in place of a impacted permanent toothNumerical anomaly with an excessive number of teeth are termed as supernumerary teeth.This paper describes a case with erupted supernumerary tooth in the place of missing left maxillary central incisor. Radiographically the left maxillary central incisor was impacted below the root of the supernumerary tooth with an additional impacted supernumerary tooth in relation to the right maxillary central incisor.A multidisciplinary approach is required in managing the supernumerary teeth. When orthodontic extrusion of the impacted permanent tooth is not possible the possibilities of retaining the supernumerary tooth should be considered.This paper describes about the treatment of supernumerary tooth erupted in the place of missing left maxillary central incisor not amenable for orthodontic extrusion.Thomas J, Harris A, Hedge S, Morgan G, Prabha EA, Rinu. Alternative Management of Impacted Permanent Central Incisor. Int J Clin Pediatr Dent, 2018;11(6):529-531. Supernumerary teeth are classified morphologically as rudimentary and supplementary teeth. They are usually associated with different syndromes. They may also appear as an isolated finding in patients with no pathology. They are prevalent in ranges from 0.5–3.8% in permanent teeth and 0.35–0.6% in primary dentition.2 The supernumerary teeth occurring between or just posterior to the central incisors are termed as “mesiodens”. They may or may not erupt, and if erupted, it causes malalignment of teeth; if impacted, it has to be evaluated radiologically.3Single supernumerary teeth are most commonly noted in anterior maxilla and are associated with the permanent dentition.A 12-year-old male patient reported to the department of pediatric and preventive dentistry, Rajas Dental College and Hospital with a chief complaint of unesthetic appearance. Intraoral examination revealed a cone-shaped supernumerary tooth present in the place of left maxillary central incisor . The lefSince the permanent left maxillary central incisor was above the root of the supernumerary tooth, it was decided to retain the supernumerary tooth and surgically remove the left maxillary central incisor and the additional supernumerary tooth. The permanent left maxillary central incisor and the additional supernumerary tooth were removed surgically, and sutures were placed  to 5. Ro1 Maxillary central incisors impaction is mentioned in the literature.4 The impacted central incisor usually erupts after the extraction of the supernumerary tooth, provided they have the eruptive force, and there is sufficient place in the arch.5The supernumerary tooth is formed from geminated tooth bud or due to the super production of the dental lamina. The conical or tubercular supernumerary tooth do not erupt usually. They may also cause impaction of the associated tooth or retard the eruption of the associated tooth.In this case, the patient's age was taken into consideration (12-year-old), and the radiograph revealed that root of permanent left maxillary central incisor was already completed, this would make spontaneous eruption difficult. Moreover, the tooth was placed in a horizontal position, above the root of the supernumerary tooth making orthodontic repositioning of the tooth difficult. Hence, it was decided to retain the supernumerary tooth since it was free from caries and periodontally sound.A multidisciplinary approach is required in managing the supernumerary teeth. When orthodontic extrusion of the impacted permanent tooth is not possible, the possibilities of retaining the supernumerary tooth should be considered.This paper describes the treatment of supernumerary tooth erupted in the place of missing left maxillary central incisor not amenable for orthodontic extrusion.


Giant parathyroid adenoma is a rare type of parathyroid adenoma defined as weighing > 3.5 g. They present as primary hyperparathyroidism but with more elevated laboratory findings and more severe clinical presentations due to the larger tissue mass. This is the first reported case of giant parathyroid adenoma from the Middle East.A 52-year-old Indian woman presented with a palpable right-sided neck mass and generalized fatigue. Investigations revealed hypercalcemia with elevated parathyroid hormone and an asymptomatic kidney stone. Ultrasound showed a complex nodule with solid and cystic components, and Sestamibi nuclear scan confirmed a giant parathyroid adenoma. Focused surgical neck exploration was done and a giant parathyroid adenoma weighing 7.7 gm was excised.Giant parathyroid adenoma is a rare cause of primary hyperparathyroidism and usually presents symptomatically with high calcium and parathyroid hormone levels. Giant parathyroid adenoma is diagnosed by imaging and laboratory studies. Management is typically surgical, aiming at complete resection. Patients usually recover with no long-term complications or recurrence. The normal parathyroid gland weighs approximately 50–70 mg. Parathyroid adenomas (PTAs) are usually small, measuring < 2 cm and weighing < 1 gm . Both PTTo the best of our knowledge, this case report describes the first case in the Middle East of a patient with non-ectopic GPTA presenting with visible neck swelling. This case report also reviews the published literature to report on the clinical characteristics and typical presentation of GPTA as well as diagnosis and treatment.A 52-year-old Indian woman was referred to our Surgical Endocrinology clinic at Hamad General Hospital in Doha, Qatar. She complained of a neck swelling and generalized fatigue. Laboratory results showed hypercalcemia and elevated PTH. Her past social, environmental, family, and employment history (housewife) were unremarkable. She did not smoke tobacco and never consumed alcohol. There was no past history of symptomatic kidney stones; however, a recent computed tomography (CT) scan of her abdomen and pelvis showed a 2 mm non-obstructing calculus in the lower pole calyx of her right kidney with no hydroureteronephrosis. Her past medical history indicated that she had dyslipidemia, controlled with medication; however, she was not on any other medication. On physical examination, a right-sided neck swelling was obvious on inspection; on palpation a mobile non-tender nodule could be felt, approximately 3 cm in size. The rest of the physical examination was unremarkable. A neurological examination was unremarkable. On admission, her pulse, blood pressure and temperature were normal.Serology laboratory tests showed corrected calcium of 3.12 mmol/L, an intact PTH of 503 ng/L, vitamin D of 19.97 nmol/L, and normal thyroid-stimulating hormone (TSH) level. Her renal functions were within normal limits, serum creatinine was 67 μmol/L, and 24-hour urine calcium was 4.30 mmol/L per 24 hours. Her complete blood count (CBC) and liver laboratory findings were within normal limits. Microbiology laboratory tests were not deemed necessary.Imaging investigations included an ultrasound of her neck that showed a complex nodule 4.1 × 2.3 cm) with solid and cystic components, and vascularity was observed in the mid to lower pole of her right thyroid gland  she had renal involvement, and, (2) serum calcium was 3.12 mmol/L  . She undA 52-year-old woman presented with a visible palpable right-sided neck mass and generalized fatigue. Ultrasound showed a complex nodule with solid and cystic components, and a Sestamibi nuclear scan confirmed a GPTA. Focused surgical neck exploration was done and a GPTA weighing 7.7 gm was excised. This case of non-ectopic GPTA is unusual in that it presented mainly with visible palpable right-sided neck mass, which was large to the extent that the initial ultrasound suggested that it could be a thyroid nodule.PTAs are well-reported tumors that cause PHPT. However, when their weight exceeds 3.5 gm, they are classified as GPTA . Ours weIn terms of presentation, the classic presentation of PTA is with PHPT accompanied by recurrent kidney stones, and psychiatric, bone, and gastrointestinal symptoms . HoweverIn terms of physical examination, the majority of GPTAs in the neck had a visible and palpable mass in the neck , 24, 25.et al. (2007) concluded that if preoperative PTH is > 232 ng/L, there is 95% likelihood of finding a PTA weighing > 250 mg [As for diagnostic laboratory studies, GPTA investigations start with serum calcium and PTH and proceed to imaging for localization Table . Hyperca> 250 mg . This is> 250 mg , its lim> 250 mg . Our pre> 250 mg .99mTc-sestamibi scintigraphy (MIBI) scan. The limitation of neck ultrasound in GPTA is that it may not show the extent of a lesion; when the GPTA is ectopic, a neck ultrasound will show no finding [In terms of imaging, localizing a GPTA is imperative to guide management. The most commonly used method is a combination of neck ultrasound and  finding . In medi finding –22. In n finding . Ultraso finding . As for  finding , 32.For the management, the 2014 US National Institutes of Health (NIH) guidelines for PTA management are either medical or surgical if it fulfills specific criteria . As withPostoperatively, larger sized PTAs may be associated with a higher incidence of postoperative hypocalcemia . Table 1GPTA is a rare subset of PTAs that weigh > 3.5 gm, it is benign, but can manifest with the symptoms of extreme hypercalcemia. From our literature review, we conclude that GPTA generally presents symptomatically, with high preoperative PTH and serum calcium directly proportional to the adenoma weight. The most accurate method for localizing a GPTA is a combination of neck ultrasound and MIBI scan. MIP with intraoperative PTH monitoring is the suggested management, although the need for the ioPTH monitoring is debatable in GPTA due to their large size and accuracy of preoperative imaging.


Chondromyxoid fibroma (CMF) is a rare, benign neoplasm of the chondroid, myxoid, and fibrous tissue. It characteristically affects the lower extremity long bones, although it may rarely arise within the craniofacial skeleton. We report the diagnosis and management of a 31-year-old male with a large, incidentally discovered CMF originating from the sphenoid sinus. A subsequent review of the literature reveals the need to differentiate from more aggressive neoplasms, such as chondrosarcoma and chondroma, which share radiographic features. A histopathologic examination is crucial for proper diagnosis and treatment. We discuss clinical sequelae, highlight the importance of a thorough pre-operative evaluation, and summarize previously suggested treatment paradigms. Originally described in 1948 by Jaffe and Lichtenstein, the chondromyxoid fibroma (CMF) is a neoplasm of cartilaginous origin -4, with Cases have been reported in all the paranasal sinuses. Given its benign nature, patients may not present until secondary symptoms manifest. These symptoms depend on tumor size and location but may include diplopia, facial pain, exophthalmos, neuralgia, dysarthria, epistaxis, nasal congestion, headache, bony swelling, or persistent chronic rhinosinusitis . Given tCase presentationA previously healthy 31-year-old male presented to the Otolaryngology - Head & Neck Surgery clinic, with right-sided nasal airway obstruction. Physical examination, including nasal endoscopy, revealed a large mass within the right nasal passage. He had no associated symptoms such as visual complaints, paresthesia, and facial pain. A biopsy was performed in the operating room, with the histopathologic evaluation revealing a lobular growth pattern with stellate or spindle-shaped cells in a chondroid background  and magnetic resonance imaging (MRI) imaging were completed for anatomic mapping and surgical planning. CT revealed a homogeneously enhancing mass with scattered coarse central calcifications. There was extensive bony remodeling and displacement without aggressive osseous erosion. Although centered within the right nasal cavity, the lesion was found to erode through the nasal septum into the left nasal cavity Figure . The masOne month later, the patient underwent endoscopic resection under general anesthesia. Intraoperatively, the tumor was noted to attach to the planum sphenoidale without dural involvement. The tumor was found to extend from the sphenoid with erosion through the posterior septum to involve the left posterior ethmoid cavity Figure .In addition to complete tumor resection, bilateral maxillary antrostomies and sphenoethmoidectomies were performed as well as a posterior septectomy. The left middle turbinate was also resected due to tumor involvement. There was no intraoperative cerebrospinal (CSF) fluid leak.The postoperative course was uneventful and a follow-up CT scan was obtained two weeks later without evidence of residual disease, as seen in Figure CMF is a slow-growing and rare neoplasm consisting of chondroid, myxoid, and fibrous tissue. CMF accounts for less than 0.5% of all bone tumors -4, with A review of the literature demonstrates inconsistent presenting symptoms. Table Given its rarity, an early differential would seldom, if ever, have CMF as the primary diagnosis. More common lesions, such as a chordoma or chondrosarcoma, should be considered for any lesion demonstrating calcification within a chondroid-like matrix . These nThese lesions are also difficult to differentiate radiologically. The identification of calcifications on CT should prompt the consideration of a cartilaginous or chondroid matrix-containing lesion . AlthougGiven this overlap of imaging findings across various lesions, a detailed histopathologic evaluation becomes crucial in making the proper diagnosis. In their series of 36 patients with CMF, Zilmer and Dorfman reported a 22% initial misdiagnosis rate and pointed out the potential of more aggressive treatment than is required, such as amputating the affected limb for a benign lesion -31. The Although the standard of treatment for CMF is surgical excision, the cosmetic and functional consequences of extensive resection of craniofacial lesions have led many authors to recommend curettage specifically for facial CMF with close follow-up. Baujat et al. asserted that long-term control is best achieved via wide surgical resection by demonstrating the high recurrence rate achieved after curettage . Select Another reason to favor surgery involves the risk of residual tissue undergoing malignant transformation. Although the transformation rate is low , cases oA review of the literature demonstrates that although an exceedingly rare tumor, representing <0.5% of all bone tumors, CMF has been reported to occur in the sinonasal cavity. Although CMF may appear similar to chondrosarcoma and chordoma on imaging, proper treatment depends on histopathologic differentiation. A biopsy will reveal stellate cells within an abundance of a myxoid or chondroid matrix. Although curettage may be necessary in the case of inaccessible tumors, complete surgical resection is preferred to minimize the risk of recurrence.