Gallstone ileus and obstructed inguinal hernias are respectively, rare and common causes of small bowel obstruction. There are no published cases of these pathologies occurring simultaneously. Here, we describe a unique case of an elderly male patient presenting with a small bowel obstruction caused by these combined pathologies. Following an acute presentation with obstructive symptoms, a CT scan demonstrated small bowel obstruction due to a large gallstone lodged in the neck of an inguinoscrotal hernia with associated pneumobilia. The case may have been managed conservatively if it was not for the presence of the gallstone. Previous imaging had incidentally demonstrated gallstones in the gallbladder and a large uncomplicated right inguinoscrotal hernia. It is presumed that a cholecystoduodenal fistula formed and a gallstone then migrated downstream to lodge within the neck of the inguinoscrotal hernia. This case underscores the concept that even in the presence of an “obvious” cause of small bowel obstruction, such as an irreducible, large inguinoscrotal hernia, we must always maintain a healthy clinical skepticism and an open mind to other unexpected aetiologies, which may account for the clinical presentation that might impact subsequent management. Following cholecystitis, adhesions may form between the gallbladder and the duodenum and, with associated pressure from a gallstone, a cholecystoduodenal fistula can form. A gallstone may then proceed through the intestinal tract until it reaches an area of narrowed lumen, usually the distal ileum or a fixed obstruction point, such as an inguinoscrotal hernia in this case. Delayed diagnosis, due to atypical or misleading symptoms such as constipation may lead to further complications, especially when associated with synchronous pathologies.4 We discuss a rare case with no prior description in the medical literature, where the abdominal CT scan was essential in establishing the diagnosis. It highlights that past medical history and pre-existing imaging can provide important clues to aid an accurate diagnosis and execute appropriate management.Inguinal hernias are common with a lifetime risk of 27–43% in males and 3–6% in femalesA 93-year-old gentleman was brought in by ambulance to our emergency department with a 24-h history of abdominal pain and distension associated with one episode of vomiting. The patient was known to have a longstanding large right-sided inguinoscrotal hernia. Additionally, he had co-morbidities of asthma, chronic renal failure and a long-term urinary catheter for benign prostatic hyperplasia. On examination, the abdomen was soft but distended and a large irreducible right-sided inguinoscrotal hernia was noted.2 on a background of chronic kidney disease. Therefore, a non-contrast CT scan of the abdomen and pelvis was performed to further investigate his obstructive symptoms. The CT scan showed dilated small bowel loops to a transition point within the right inguinoscrotal hernia where a 42 mm calcified stone was lodged at the neck  and the wound was closed.A discussion with the patient and his family regarding further management was undertaken, emphasising concerns about his age and associated comorbidities, as well as disclosing the high mortality risk of 23.5%,  risk adjustment model). A right groin exploration was performed, and the obstructing gallstone was identified within the small bowel. An enterolithotomy   and less commonly hernias and strictures.6 Gallstone ileus is thought to be the underlying aetiology of 1–4% of hospital presentations with intestinal obstruction and for up to 25% of all non-strangulated cases of small bowel obstruction in patients over 65 years of age.7 Inguinal hernias account for up to three-quarters of abdominal wall hernias and may present with obstruction.8 In the case we present, the patient had features of small bowel obstruction secondary to a gallstone lodged within the neck of an inguinoscrotal hernia which makes it interesting and unique within the literature.Small bowel obstruction is one of the most common surgical emergencies seen in elderly populations, accounting for 51% of all emergency laparotomies in the UK.9 Ayantunde et al10 reported known gallstone disease in only 27% of cases and other case series show that 50% of the patients denied any biliary symptoms.7 Although a plain abdominal radiograph is routinely used in the initial assessment of small bowel obstruction, it only has a sensitivity of 40–70% in the diagnosis of gallstone ileus.11 To confidently make the diagnosis of gallstone ileus on plain film, a triad of dilated loops of small bowel, pneumobilia and presence of a gallstone is required.11 These features are also required on the CT scan, but it offers overall sensitivity, specificity, and accuracy of 93%, 100%, and 99%, respectively.12 In this case, the clinical presentation was of straightforward small bowel obstruction supported by the initial plain abdominal radiograph. However, the aetiology for the obstruction was not clearly demonstrated during the initial evaluation, as the hernia was not initially recognised as the site of obstruction, and the patient belongs to the minority who have a known history of asymptomatic gallstones. Although CT is routinely the main tool used to determine the cause of small bowel obstruction, it was of particular use in this unique case. The CT scan, besides providing the diagnosis, guided surgical management by localising the gallstone to the scrotum, allowing a groin incision rather than a midline abdominal incision laparotomy in a high-risk patient.Following cholecystitis, adhesions may form between the gallbladder and the adjacent bowel, most frequently the duodenum. Pressure effect from gallstones cause them to erode through and migrate into the bowel forming a cholecystoenteric fistula. The gallstone may then impact in any part of the intestinal tract, most commonly in the ileum , jejunum (16.1%–26.9%), duodenum (3.5%–14.6%) and colon (3.0%–4.1%).13 surgery via a midline laparotomy is indicated for most cases of gallstone ileus. There has been debate for some time for the best management for gallstone ileus. Enterolithotomy alone is preferred and sufficient in elderly patients without concurrent cholecystectomy, as this adds considerable risk to the procedure’s mortality.3,14Since there is a low rate of spontaneous stone passage of approximately 1.3%,15,16 Our patient underwent open inguinal hernia repair with removal of the gallstone via an enterotomy, that was subsequently closed. The hernia was reduced and following an orchidectomy, the posterior wall of the inguinal canal was reinforced with a biological mesh. The orchidectomy (for which consent had been obtained from the patient) was performed to enable closure of the large defected deep ring, to reduce the risk of a recurrence and is a well-documented approach for repairing large inguinal hernias.17 The patient recovered well with no significant post-operative complications.Recent studies have reported safe use of biologic mesh reinforcements in contaminated ventral hernia repair.An exhaustive search of literature and peer reviewed libraries was done, including Cochrane and PubMed Libraries, and did not identify a similar presentation. Even though gallstone ileus within an inguinal hernia is rare, it does exist and this described case emphasises the requirement for pre-operative CT to aid surgical planning. It carries a significant risk of morbidity and mortality; therefore, early diagnosis and treatment is essential for an improved final outcome. The patient’s previous history and prior radiological investigations play a vital role in identifying such conditions. Current radiological findings and consideration of the patient’s co-morbidities guide management. This rare case upholds the importance of the role of CT imaging in the diagnosis and management of a patient with bowel obstruction. Furthermore, early and considered surgical management, especially in an elderly patient with comorbidities, can significantly reduce the rates of morbidity and mortality associated with this pathology.CT imaging is essential in the diagnosis and management of small bowel obstruction, even in the presence of an “obvious” clinical cause. Keep an open mind to an unusual cause or combined pathology.The review of past imaging may be helpful in clinching the diagnosis.Early cross-sectional imaging facilitates early initiation of treatment.CT scanning may improve patient outcome by tailoring surgical approach and technique.Gallstone ileus occurs following migration of a gallstone via a cholecystoenteric fistula to lodge at a point of ileal luminal narrowing.Gallstone ileus is a rare cause of small bowel obstruction. The most common causes are adhesions, hernias and strictures.Gallstone ileus and inguinal hernias may be found as separate causes of small bowel obstruction but the finding of a gallstone ileus within an inguinal hernia is a very rare presentation and indeed could only have been identified pre-operatively by CT.


Report on clinical cases of spontaneous reattachment of rhegmatogenous retinal detachment (RRD). From 2014 to 2020 we diagnosed four patients with spontaneous reattachment of RRD. We conducted a review of the relevant medical records, focusing on the initial symptoms at presentation, the initial diagnoses, with a further observation of the patients next 3 years. The patients were re-examined 3 years later after cases of spontaneous reattachment of RRD. Three years after a case of spontaneous reattachment of RRD during reexamination in four patients (four eyes), redetachment was not detected. The mentioned clinical cases indicate the possibility of finding new approaches to RRD management. Rhegmatogenous retinal detachment (RRD) is a progressive state requiring surgical intervention aimed to block retinal tears (RT) and remove vitreoretinal traction. It is known that surgery is the sole management of RRD and must be performed immediately. Scleral buckling and vitrectomy are targeted to block RT and reduce vitreoretinal traction with retinal reattachment as a potential outcome. As far as literature review shows, undiagnosed RRD or delay in surgery may result with spontaneous reattachment of rhegmatogenous retinal detachment (SRRRD) .A 45-year-old female noted a sudden loss of vision on the right eye (OD) a month ago. The best-corrected visual acuity (BCVA) was incorrect light projection OD and 20/200 in the left eye (OS); low visual acuity OS caused by myopic maculopathy. Dilated fundus examination (DFE) OD revealed posterior vitreous detachment (PVD) and RRD in three quadrants with retinal folds and RT at 12:00 and 1:00 o’clock at the periphery. Vitreoretinal surgery was offered to the patient. One month later, SRRRD was diagnosed  and retiA 69-year-old female noticed flashes and a ‘curtain’ in front of both eyes (OU). BCVA was correct light projection OD and 20/40 OS. DFE OU revealed RRD: OD revealed PVD and RRD from 10:00 to 5:00 o’clock; OS revealed PVD and RRD in two quadrants with RT from 10:30 to 3:00 o’clock. One month later, at the time of admission for vitreoretinal surgery for RRD OD, SRRRD OS was diagnosed ; RLC wasA 56-year-old male noted a loss of vision and flashes OD. BCVA was 20/400 OD and 40/200 OS; low visual acuity OS caused by myopic maculopathy. DFE OD revealed attached posterior vitreous with RRD in three quadrants involving the macula with atrophic retinal holes at 1:00 and 11:00 o’clock, the retina had fibrotic changes with pigment deposits on its surface. Vitreoretinal surgery was offered to the patient, but one or a month later SRRRD was diagnosed  and RLC A 59-year-old male noted a decrease in vision OS. The patient had diabetic retinopathy, a proliferative stage with maculopathy. BCVA was 20/4000 OS and 20/50 OD; low visual acuity OD caused by diabetic macular edema. DFE OS revealed PVD with RRD in two upper quadrants involving the macula with RT at 12:30, 6:00 and 9:00 o’clock. Vitreoretinal surgery was offered to the patient. A month later, SRRRD was diagnosed  and RLC Individual patient data are summarized in Algvere et al.  and LeanDe Jong et al.  concludeSome studies have suggested that the basis for the development of SRRRD presumably involves the occurrence of a complete PVD that in most cases relieves vitreoretinal traction , 6. InflIn our study patients were not limited in head and eye movement or neither in a bed position, they had various types of RT, one patient had proliferative diabetic retinopathy, one patient had no PVD; the clinical variability of the SRRRD may contradicts studies above.There were several limitations in our study. First, the limited number of the cases and retrospective nature of this study. Second, all patients were not comprehensively examined by imaging tools with focus imaging of RT and detachment before SRRRD to determine the pathogenesis of SRRRD. We recognize and do not undermine the importance of vitreoretinal surgery in RRD; we strongly recommended surgery to all our patients.To summarize, SRRRD is a rare event in ophthalmologist’s practice, accumulating case reports may provide significant information in understanding the current phenomenon. In certain cases, persistent reattachment of the retina after RRD without any intervention is possible. The mentioned clinical cases indicate the possibility of finding new approaches to RRD management.


The lacrimal caruncle is composed of numerous structures including different glands as well as hair follicles. Accordingly, the spectrum of benign and malignant lesions is broad, and the clinical diagnosis is often challenging. Here we systematically analyzed excised caruncular tumors over the past 22 years with special emphasis on the clinico-pathological correlation to provide a guidance for clinicians.Retrospective evaluation with clinico-pathologic correlation of surgically removed caruncular tumors between 1998 and 2020 at a tertiary referral center.n = 35), cystic lesions (n = 14), oncocytoma (n = 9), papilloma (n = 8), sebaceous gland hyperplasia (n = 8), and reactive lymphoid hyperplasia (n = 4) were observed most frequently. Besides, we are the first reporting herniated orbital fat accompanied by a pyogenic granuloma. 2.4% (n = 2) were malignant tumors .Eighty-two caruncular tumors were identified in the respective period. The patients were between 11 and 85 years of age . Nevi (Caruncular tumors show a broad spectrum of mostly benign tumors. They can occur in patients of any age. However, 8/9 oncocytomas and both malignant lesions were detected in patients older than 60 years. Although the clinical diagnosis was confirmed in only 68.3% by the histopathological analysis, the two malignant lesions were identified as such already clinically. Caruncular lesions with a history of growth or other signs of malignancy should be excised followed by detailed histopathological examination to allow a final diagnosis and exclude rare malignant tumors with lethal potential. The online version contains supplementary material available at 10.1007/s00417-021-05464-x. The lacrimal caruncle (herein referred to as caruncle) is located in the medial angle of the eyelids. It is covered partly by non-keratinized squamous epithelium with numerous goblet cells and partly by keratinized stratified squamous epithelium. The caruncle is composed of loose connective tissue rich in fibroblasts and may also contain adipose tissue. It contains numerous skin appendages such as hair follicles, sebaceous and sweat glands as well as occasionally accessory lacrimal glands , and striated muscle fibers. Melanocytes are present in the basal layer of the surface epithelium. The composition of the caruncle with its various structures is responsible for the variety of benign and malignant tumors arising in this specific location.Caruncular tumors are rare compared to conjunctival lesions; however, most of them 95%) are benign –5. The i% are benIn this study, we report 82 lesions surgically excised during the last 22 years at the University Eye Hospital Bonn, Germany. This clinico-pathological correlation aims to provide guidance for clinicians with regard to differential diagnoses and treatment of patients with caruncular lesions.All primary caruncle tumors were surgically excised between 1998 and 2020 at the University Eye Hospital Bonn and histopathologically diagnosed at our Ophthalmopathologic Laboratory. The suspected clinical and final histopathological diagnoses were correlated. For this study, the clinical data including the clinical diagnosis of the patients was retrospectively evaluated. The histological evaluation was performed by light microscopy using routine hematoxylin–eosin staining (HE) and periodic acid-Schiff reaction (PAS) as well as immunohistochemical stains where necessary . Caruncular tissue which was excised during pterygium surgery was not included in the study nor was conjunctival intraepithelial neoplasia of the tarsal/bulbar conjunctiva involving the caruncle.The number and histological diagnosis of surgically removed conjunctival lesions which were excised during the same period were recorded allowing for a comparison with regard to frequency of conjunctival and caruncle tumors.The research was conducted in adherence to tenets of the Declaration of Helsinki. Ethics Board Approval of the University of Bonn was granted (328/16).Between 1998 and 2020, 82 excised caruncular tumors were identified. The patients were between 11 and 85 years of age  and more frequently female  and papillomas , followed by oncocytomas (n = 9), cystoid lesions (n = 9), and sebaceous gland hyperplasia (n = 7). Only 2.4% patients of the cohort (n = 2) exhibited a malignancy , Table n = 56) of the lesions were correctly diagnosed clinically respectively the final histological diagnosis was already included in the tentative clinical diagnosis.All lesions were unilateral and solitary. The most frequent tumors were benign melanocytic lesions  underwent complete surgical excision followed by two cycles of mitomycin C 0.04%. After 2 years, the patient developed a recurrence which was again treated with surgical excision and another cycle of mitomycin C 0.04%. The patient is now free of recurrence for 4 years .n = 1183) at our institution, caruncular tumors (n = 82) are much rarer and account for only 6.4% of the surgically removed lesions of the anterior segment.Compared to the conjunctival tumors excised during the same period .Clinical findings: brownish or whitish-reddish (amelanotic lesions); papillomatous .JA occurred more frequently in pigmented lesions compared to amelanotic melanocytic lesions . PatientClinical findings: yellowish (keratin cyst), variable (for other types).Histology: cyst lined by keratinized squamous epithelium (keratin cyst)/non-keratinized epithelium with apocrine/eccrine secretion (sweat gland cyst)/non-keratinized squamous epithelium with goblet cells /no epithelium (resorptive cyst); lumen filled with keratin lamellae (keratin cyst) or eosinophilic material.Clinical findings: reddish or whitish, with a smooth surface and a nodular or cystoid appearance.Histology: cystic cavities lined by a proliferating epithelium; cells with eosinophilic and granular cytoplasm , accompanying inflammatory component possible.The histologic appearance of oncocytoma resembles an apocrine epithelium — however, (accessory) lacrimal glands exhibit an eccrine secretion.Clinical findings: papillomatous epithelial lesion, reddish or whitish.Histology: proliferating keratinized or non-keratinized acanthotic squamous epithelium with goblet cells covering fibrovascular cords.Clinical findings: yellowish with subepithelial gland-like structures.Histology: composed of densely packed regular appearing sebaceous glands.Clinical findings: reddish/salmon-colored, with a smooth surface.Histology: dense lymphocytic infiltrates with formation of well-defined reactive lymphoid follicles with germinal centers.Normal caruncular tissue; dermoid (thickened collagen lamellae containing skin appendages); herniated orbital fat in the caruncle accompanied by pyogenic granuloma.Clinical findings: reddish, with adjacent papillomatous surface epithelium.Histology: conjunctival intraepithelial neoplasia  at the base of a typical pyogenic granuloma.Clinical findings: reddish, smooth surface, with infiltrative growth characteristics.Histology: subepithelial mass with a lobular growth pattern, cells with prominent nucleoli and a partly foamy cytoplasm, (bizarre) mitotic figures.The caruncle displays a different spectrum of tumors as the conjunctiva and lesions of the caruncle are rarer . As in oMalignant neoplasias at the caruncle comprise basal cell carcinoma, squamous cell carcinoma, basosquamous carcinoma, malignant melanoma, lymphoma, and sebaceous gland carcinoma , 17–20. Although clinicians may aim to make the correct diagnosis clinically , thBased on our own experience and data from the literature, four features should alert the clinician to suspect a carcinomatous malignant process at the caruncle: (1) fast growth, 2) indistinctive borders, (3) smudgy or ulcerated surface, and (4) older age > 60 years . These criteria do not apply for lymphoid lesions and Kaposi sarcoma  which ha indistinEvery cohort of caruncular lesions shows a specific spectrum: Our study comprises a relatively high number of oncocytoma comparable to Kaeser . ShieldsNevi consisted of benign melanocytic lesions  without any evidence of malignant transformation or malignancy. Overall, the mean age was (median: 37.8 years) lower than for the other lesions — except RLH. A slight female preponderance of melanocytic lesions was — in accordance with our study — observed in other studies [ studies . Eighty- studies , 26.cystic lesions are a heterogeneous group, and therefore the clinical assignment to the final histological diagnosis was difficult since cysts can originate from different structures . Only 64.3% of the cysts were clinically identified as cystic lesions.The The most frequent cysts diagnosed in our clinic were keratin cysts in which we observed histological differences from epidermoid cysts of the skin. Histologically they were lined by keratinized squamous epithelium, and the lumen contained keratin lamellae. However, a close proximity to sebaceous glands — often opening out into the epithelium of the cyst — were observed in these keratin cysts. Hairs were not present in the lumen of the cyst as one would expect in a dermoid cyst  and therOncocytoma is a specific but rare tumor of the caruncle which probably arises from caruncular accessory lacrimal glands named glands of Popoff [f Popoff , 31. It f Popoff , 32.n = 5), in only three of these cases it was the main primary clinical diagnosis. Due to their varying clinical appearance  they can be mistaken for sebaceous gland hyperplasia, cysts, reactive lymphoid hyperplasia/lymphoma, inflammatory processes, or other kinds of neoplasia, and thus the oncocytomas in this and other series [In our study, patients with oncocytoma were generally older  which is in accordance with other studies , 33, 34.r series , 6 were n = 5), while larger, cystic oncocytomas were more likely to appear whitish (n = 4). However, the transition between these two ends of the spectrum is fluent, and it may be more likely the material in the lumen of the lesion determining the clinical appearance since in particular in larger oncocytomas, surface epithelium (keratinized squamous epithelium or most often non-keratinized epithelium with goblet cells) may become incorporated into the oncocytoma. Different architectural patterns of oncocytoma have been reported before including cystic-micropapillary, confluent-glandular, solid-organized, solid-disorganized [The histologic type of oncocytoma may also affect the clinical appearance as small, solid oncocytomas were more likely to exhibit the classical reddish color  at the caruncle which are most frequently surgically excised (7.1–32%) [sed 7.1–3% [2, 3, 7.1–32%) , 14, 15.Hyperplasia of sebaceous glands is a typical lesion of the caruncle. In this cohort, only male patients were affected with an age range from 37 to 83 years. The typical clinical picture is yellowish with a subepithelial gland-like appearance. Thus, in most cases, the correct diagnosis was achieved clinically (83%). The one lesion which had a reddish appearance was clinically diagnosed as pyogenic granuloma. Histopathologic correlation showed a massive inflammatory reaction with numerous vessels around and in parts superior to the sebaceous glands giving the lesion its reddish clinical appearance. The number of sebaceous gland hyperplasia varies between different studies from 1.8 up to 8% (as in our study) [r study) , 7. HoweReactive lymphoid hyperplasia (RLH) is a rare diagnosis in general. However, it has been shown that RLH is often located in the medial conjunctiva including plica and caruncle [caruncle . Althougcaruncle . Of the caruncle , 6, 7. Pcaruncle –3. Regarcaruncle .The clinical diagnosis of RLH at the caruncle is difficult. It may be considered in younger patients and in tumors with a salmon-colored appearance. Once a biopsy is performed showing a lymphoid lesion, it is mandatory to rule out a lymphoma by additional immunohistochemistrical stains and/or clonal analysis since age is not a reliable discriminating factor between RLH and lymphoma.To the best of our knowledge, we are the first to report herniated orbital fat accompanied by a pyogenic granuloma. Kaeser reported two cases of fatty infiltration of unknown origin in two woman of 51 and 82 years . Since tRegarding dermoids of the caruncle, one case report of a caruncular dermoid was reported before involving the upper eyelid and palpebral conjunctiva . Østergamalignant epithelial tumors, we have reported a case series of pyogenic granuloma with a conjunctival intraepithelial neoplasia on its base with one of these lesions occurring at the caruncle [With regard to caruncle . The othAlthough rare, premalignant and malignant epithelial lesions have been reported in nearly every larger cohort on caruncle tumors , 5–9, 30In summary, caruncular tumors have a different spectrum and are less common than lesions of the bulbar and tarsal conjunctiva. They can occur in patients of any age and appear to affect women more often. However, other studies and our cohort only report caruncular lesions that were surgically removed  which may explain a higher number of female patients who are more likely to go to a doctor and to have surgery for aesthetic reasons.Although most caruncular lesions are benign, the clinical diagnosis is often challenging justifying a histopathological examination of each excised lesion to achieve a histological diagnosis and exclude malignant tumors with lethal potential which have in the case of caruncular melanoma a higher risk for metastasis .Supplemental Fig. 1Diagnosis dependent age distribution of patients with caruncular lesions. (PDF 211 kb)High resolution image (TIF 4219 kb)Supplemental Fig. 3Sebaceous gland hyperplasia. Clinical picture of a yellowish lesion of the caruncle (a). The corresponding histologic figure shows caruncular tissue mainly composed of regularly appearing sebaceous glands . Higher magnification illustrates the holocrinic sebaceous glands  (JPG 659 kb)High resolution image (TIF 2989 kb)Supplemental TablePresence of junctional activity in benign melanocytic lesions. (PDF 90 kb)


In the pituitary sella, the coexistence of pituitary adenoma and primary pituitary lymphoma is exceedingly rare. Thus far, only six cases have been reported. Here, we present the seventh case of coexisting pituitary adenoma and primary pituitary lymphoma, which was difficult to differentiate from other sellar tumors. To our knowledge, this is the first case of the prolactin subtype of the pituitary adenoma in literature. We have also systematically reviewed the literature and summarized the characteristics of coexisting pituitary adenoma and lymphoma. We believe this report provides a new clinical reference for the diagnosis and treatment of collision tumors of pituitary adenoma and lymphoma. Collision tumor in the sellar region is a rare condition; it refers to two coexisting tumors of different shapes attached to each other. Typically, a collision tumor in the sellar region is characterized by the coexistence of pituitary adenoma and another tumor. Reportedly, the pituitary adenoma can be associated with meningioma , Rathke'In May 2020, a 41-year-old Asian male was hospitalized with a history of intermittent stabbing headache, without progressive deterioration of visual acuity or visual field. The patient underwent endoscopic endonasal transsphenoidal tumor resection. A tough tumor was encountered in the sphenoid sinus and sellar region. The tumor was abnormally hypervascular, and there was bone inside the tumor. The volume of intraoperative bleeding was about 200 mL. By the end of the surgery, all the tumors were removed in pieces. Postoperatively, the patient recovered well. The symptoms of headache were significantly relieved. Pathological results showed sparsely granulated lactotroph adenoma combined with primary pituitary diffuse large B-cell lymphoma . The immA postoperative head MRI showed that there was no residual tumor in the sellar region. A PET-CT scan did not show increased uptake in the pituitary gland and other parts. The results of bone marrow puncture showed that the degree of proliferation was V/VII, G:E = 2.3:1, and nuclear heterogeneous cells were occasionally seen. Then, he received four cycles of chemotherapy. The R-MI protocol (rituximab+methotrexate+ifosfamide) was used, accompanied by supportive treatment.The patient underwent follow-up examinations at postoperative 6 months and 1 year. The patient was generally in good condition, and the tumor did not recur. A series of brain MRIs showed no evidence of tumor regrowth . The patCollision tumors of pituitary adenoma and primary pituitary lymphoma in the sellar region are extremely rare. We systematically reviewed the literature to identify the characteristics of coexisting pituitary adenoma and lymphoma. To our knowledge, thus far, only seven cases have been reported, including this case. We have summarized the characteristics of these seven cases –10 and pThe pathophysiological mechanism of tumorigenesis remains unclear, as there are only a very small number of cases currently reported. The local immune changes in the pituitary gland may be brought about by tumorigenesis. It is reported that primary pituitary lymphoma can develop in immunosuppressed individuals . At the The coexistence of pituitary adenoma and primary pituitary lymphoma has no special clinical manifestations. The most common clinical manifestation is headache; it was noted in 5 of 7 cases . WhetherSurgical management is the most important treatment option for collision tumors of pituitary adenoma and primary pituitary lymphoma. The patients in the six previous cases underwent surgical treatment. In Au et al.'s  case repThe recurrence rate after total resection is low, but there is still recurrence. In the case report by Kuhn et al. published in 1999 , the tumThe coexistence of pituitary adenoma and primary pituitary lymphoma in the sellar region is extremely rare. The disease diagnosis, tumor control, and overall survival are more challenging to achieve for collision tumors than for pituitary adenomas alone. The gross total resection of both tumors is very important for satisfactory tumor control. The follow-up assessment of patients should be appropriately prolonged.Because the patient's preoperative diagnosis was a conventional pituitary adenoma, video recording was not performed during the operation. Therefore, there are no intraoperative images. Furthermore, the report lacks neuro-ophthalmological examination, because the patient claimed to have normal visual acuity and field. Lastly, only 1-year postoperative follow-up was performed on the patient. We plan to continue to monitor the situation of this patient.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.ML conceived the study and edited the final manuscript. SR and QL collected the clinical data and performed a literature review. YZ confirmed the pathological analysis. BL reviewed the clinical notes and produced the draft manuscript. YX and LZ conceived the research and helped with the writing of the manuscript. All authors read and approved the final manuscript.This work was supported by grants from the Taishan Scholar Project of Shandong Province of China (tsqn202103200) and the National Natural Science Foundation of China (81701159).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Lipoma is the commonest benign mesenchymal tumor composed of matured adipocytes. A literature search revealed few reported cases of giant scrotal lipoma. This article aims to report a giant scrotal lipoma weighing 1100 g and illustrate our challenges with the diagnostic process in a low-resource setting. A 28-year-old male presented with a huge right scrotal mass. Examination revealed the mass had no cough impulse. It was firm, non-tender and lobulated, with definite edges. Scrotal sonography was suspicious of lipoma. Intraoperatively, there was an encapsulated scrotal wall mass and an incidental inguinoscrotal hernia, content being the omentum. The scrotal mass was excised, hernia sac was ligated, and excised and the posterior wall was repaired. Histology confirmed the scrotal mass as a lipoma. Primary scrotal lipomas are rare but should be considered in the differential diagnosis of unusual scrotal masses. Ultrasonography is a useful diagnostic tool in resource-limited settings. Lipoma is the commonest benign mesenchymal tumor composed of matured fat cells , 2. It mThis article aims to report a 1100-g giant scrotal lipoma in a young male and review the literature. We also illustrate our challenges with the diagnostic process in a resource-limited setting.A 28-year-old male presented with a gradually increasing right inguinoscrotal swelling over 3 years. There were no other associated symptoms. On clinical examination, he was afebrile, not pale and well hydrated. Systemic examination was normal. The abdomen was flat with no visible and palpable cough impulse. There was a huge right hemiscrotal mass which measured 25 × 12 × 8 cm, irreducible, firm, non-tender and lobulated with definite edges as shown in Common scrotal masses like hydrocele, varicocele and inguinoscrotal hernia are often managed successfully by general practitioners in resource-limited settings. Clinical history and examination are often enough to make diagnose of common scrotal masses. Atypical scrotal masses are difficult to diagnose and are often referred to the urologist for expert management. A giant primary scrotal lipoma is a rare clinical occurrence that poses preoperative diagnostic challenges to the clinician , 5. But The use of radiological imaging provides invaluable information that may aid in preoperative diagnosis and surgical planning , 13. USGLiposarcoma of the spermatic cord is a differential diagnosis of clinical importance in this case. It accounts for 3–7% of all extra-testicular tumors . It growPrimary scrotal lipomas are rare but should be considered in the differential diagnosis of unusual scrotal masses. USG is a useful diagnostic tool for scrotal pathologies in resource-limited settings.Written informed consent was taken from the patient for publication of this report.None declared.


Bilateral thalamic primary gliomas are an exceedingly rare entity. Symptomology heralding a workup and diagnosis of bithalamic gliomas is diverse and varies between the pediatric and adult populations. Herein, we present a case of a 63-year-old female patient who presented with progressive gait imbalance and fatigue, prompting an outpatient brain MRI, remarkable for marked expansion of the bilateral thalami secondary to non-enhancing, T2-weighted-fluid-attenuated inversion recovery (T2-FLAIR) bright bithalamic lesions. The patient underwent a right frontal frameless stereotactic biopsy of the right thalamic lesion, with immuno-histology indicating a high-grade anaplastic astrocytoma with molecular features of glioblastoma (GBM). The patient’s functional status declined precipitously in the month following her diagnostic biopsy, precluding any therapy, and the patient ultimately pursued home hospice care without further treatment. This case details the clinical management of a very rare tumor, supplementing the available literature on the progression and treatment of this rare disease. Bilateral thalamic primary gliomas are an exceedingly rare entity, with fewer than 75 cases reported in the literature to date . BilaterA 66-year-old right-handed Caucasian woman with a medical history of obesity, hypertension, and unprovoked deep vein thromboses (DVTs), anticoagulated on warfarin presented with progressive gait imbalance and fatigue, prompting an outpatient brain MRI. The symptoms started approximately 12 months prior and progressively worsened, as she went from ambulating freely to necessitating a cane and eventually a walker by the time of hospital presentation with multiple falls reported in the months preceding evaluation. The patient noted corresponding progressive fatigue over the past year as well, and her family noted memory impairment over the past six months. The patient denied any associated fevers, unintentional weight loss, or change in appetite. Physical examination revealed a markedly slowed, wide-based gait with heavy reliance on upper extremities via an assistive walker and hesitation with each step, given her professed imbalance. On presentation, the patient was therapeutically anticoagulated on warfarin, with international normalized ratio (INR) of 2.0. Bilateral lower extremity ultrasound re-demonstrated chronic bilateral deep venous thromboses without evidence of acute thrombosis. Labs were otherwise unremarkable. A contrast-enhanced MRI of the brain was obtained, remarkable for asymmetric expansion of the thalamus secondary to non-enhancing T2-weighted-fluid-attenuated inversion recovery (T2-FLAIR) hyperintense lesions. The lesions demonstrated a mass effect on the adjacent lateral ventricles and basal ganglia without significant midline shift. Greater FLAIR hyperintensity was demonstrated on the right than the left with unilateral right-sided extension of FLAIR hyperintensity into the surrounding white matter and midbrain Figures , 2.The patient underwent a right frontal frameless stereotactic biopsy of the right thalamic lesion. Microscopic examination of the biopsy specimen of the right thalamic lesion showed an infiltrating glioma with moderate cellularity. Rare mitotic figures were visualized, but no microvascular "endothelial" proliferation or necrosis was present. The histologic features were consistent with anaplastic astrocytoma, World Health Organization (WHO) grade III. However, immunohistochemistry and next-generation sequencing (NGS) confirmed isocitrate dehydrogenase 1 (IDH-1) wild-type status and the presence of a telomerase reverse transcriptase (TERT) promoter mutation. Therefore, the molecular findings were most consistent with a final diagnosis of diffuse astrocytic glioma, IDH wild-type, with molecular features of glioblastoma (GBM), WHO grade IV . The speThe diagnosis of bithalamic glioma generally portends a poor prognosis. Treatment options are limited, with surgical resection precluded by the vitality of the structures involved. In cases with associated obstructive hydrocephalus, cerebrospinal fluid (CSF) diversion may offer symptomatic relief of elevated intracranial pressure but does not otherwise alter the course of the disease. The role of radiation and chemotherapy, in conjunction or individually, remains a subject of debate . FactorsCharacteristic radiographic findings associated with primary bithalamic gliomas include bilateral thalamic enlargement with homogeneous low signal intensity and no contrast enhancement on T1-weighted MRI. Both thalami typically demonstrate high-signal intensity on T2-weighted images, with T2 hyperintensity sometimes extending to surrounding white matter and midbrain structures ,7. The pThis patient had an immuno-histologically high-grade anaplastic astrocytoma with molecular features of GBM. Proposed treatment consisted of radiotherapy and temozolomide, or radiation alone. However, the patient’s functional status declined precipitously in the month following her diagnostic biopsy, precluding either therapy, and the patient ultimately pursued home hospice care without further treatment.Bilateral thalamic primary gliomas are a very rare entity with a generally poor prognosis. Given the rarity of this disease, there is presently a dearth of literature pertaining to its diagnosis and management. Herein, we present the clinical manifestations of a bilateral thalamic glioma in a 66-year-old woman from symptomology to tissue diagnosis and rapid clinical decline thereafter. This case report supplements the available literature on this rare entity. Specifically, this case illustrates the utility of obtaining a tissue biopsy in order to make a definitive diagnosis, as MRI alone may be insufficient.


This study describes an autopsy case of pancreatic/peripancreatic myeloid sarcoma in a 70-year-old man, initially presenting with obstructive jaundice. Pathologically, diffuse infiltration of round cells containing atypical nuclei with marked cleavage was observed mainly in the pancreas head, peripancreatic lymph nodes, spleen, bilateral lung, and bone marrow. Immunohistochemically, the tumor cells were negative for CD20, CD79a, CD3, CD5, c-kit, CD34, and TdT and positive for myeloperoxidase, CD33, CD68, and CD163. Flow cytometry of the peripheral blood showed underexpression of CD11c and aberrant expression of CD56 in the monocyte subset. The peripheral blood smear showed an increase in monocytes and atypia in neutrophils and monocytes, as well as enlarged platelets and polychromatic erythroblasts. Hence, it was suggested that the myeloid sarcoma was derived from the acute transformation of chronic myelomonocytic leukemia. Myeloid sarcoma is an extramedullary-mass-forming hematologic malignancy that is difficult to diagnose, especially when the initial presentation is a pancreatic mass. However, early diagnosis is important for appropriate therapy. Although bone marrow examination could not be performed because of the patients' severe condition, the pathological specimen obtained with autopsy helped subtype the patient's leukemia. The immunohistochemical features of this case merit attention. Myeloid sarcoma (MS) is a rare disease characterized by a tumor mass consisting of immature myeloid cells at an extramedullary site . MS may It was highly suspected that the patient had chronic myelomonocytic leukemia (CMML) in the background of MS because of the peripheral blood findings. CMML is a rare clonal hematopoietic malignancy with features of myeloproliferative neoplasm (MPN) and myelodysplastic syndrome (MDS) , 5.Herein, we present an autopsy case of MS of the pancreas head region possibly derived from acute transformation of CMML in a patient whose initial presentation was obstructive jaundice due to pancreatic mass. He had never previously been diagnosed with hematologic malignancy. He died in a week after his administration to our hospital with disseminated intravascular coagulation (DIC) due to a pulmonary tumor thrombotic microangiopathy- (PTTM-) like condition.A 70-year-old man who was previously healthy visited our hospital because of yellowing of the skin and conjunctiva that started 7 days before his visit to our hospital. He had not undergone a health checkup in the last 15 years and was previously a heavy drinker but not a smoker.3, platelet (PLT) count, 119 × 103/mm3; aspartate aminotransferase, 135 U/L; alanine aminotransferase, 211 U/L; lactate dehydrogenase, 762 U/L; total bilirubin, 9.01 mg/dL; direct bilirubin, 6.79 mg/dL; and C-reactive protein (CRP), 1.76 mg/dL. Abdominal computed tomography (CT) with enhancement showed a 44 mm, gradually enhanced mass with multiple enlarged lymph nodes, including peripancreatic and mesenteric nodes  count, 12,000/mmic nodes . Magnetiic nodes . CT did For bilirubin reduction, endoscopic retrograde cholangiopancreatography was initially performed, but due to compression by the tumor, the ampulla of Vater was not detected; hence, a hepatogastrostomy was performed. Tumor biopsy was performed from the periampullary site; however, no tumor cells were observed on pathological evaluation.3 and CRP level of 2.1 mg/dL, and he developed respiratory failure. An increase in monocytes (>1 × 109/L), atypical neutrophils, enlarged PLTs, polychromatic erythroblasts, and atypical monocytes were seen in the peripheral blood  M1 (AML without maturation) and M2 (AML with maturation), whereas recent reports have shown that the M4 (acute myelomonocytic leukemia) and M5 (acute monoblastic and monocytic leukemia) subtypes are also frequent , 6. The We considered the patient had CMML in the background of MS, based on the following three findings: [1] monocytosis and multilineage dysplasia seen in the peripheral blood, [2] aberrant expression of CD56 and underexpression of CD11c in the monocyte subset seen in the flow cytometry of the peripheral blood, and [3] the microscopic and immunohistochemical features of the tumor cells observed in autopsy. The aberrant expression of CD56/underexpression of CD11c in the monocyte subset has been reported to be fairly/highly specific for CMML, respectively , 8. The CMML was previously considered a subtype of myelodysplastic syndrome in the initial FAB classification , but in The present case showed obstructive jaundice due to the pancreas mass as its initial symptom. Several swollen lymph nodes were seen near the mass at pancreatic head in this case; hence, it is possible that the pancreatic parenchyma may be infiltrated from the tumor in the peripancreatic lymph nodes. MS of the pancreas is rare. According to Norsworthy et al., only five cases of MS of the pancreatic head without a previous hematologic disorder have been reported in the English literature , 14–17. No chemotherapy was administered in the present case because the biopsy specimen did not contain tumor cells and the patient's condition was rapidly deteriorating due to the PTTM-like condition. Autopsy specimens from the ampulla of Vater showed massive tumor cell infiltration to the ampullary site with occasional edematous areas without tumor cells, suggesting that the biopsy specimens might have been obtained from nontumorous sites.PTTM is a state in which tumor cells embolize to the pulmonary vasculature resulting in the activation of the coagulation cascade, pulmonary hypertension, and DIC. PTTM is most commonly associated with gastric adenocarcinoma but has been described with numerous other carcinomas. To our knowledge, PTTM associated with a hematologic malignancy has not been reported except for one case of myelodysplastic syndrome . AlthougRegarding the subtyping of leukemia, analysis of cell surface antigens by flow cytometry and molecular data of bone marrow specimens are usually utilized. However, in the present case, since we could not perform a biopsy of the bone marrow due to the patient's severe respiratory condition, we attempted subtyping by microscopic/immunohistochemical examination of autopsy material and the peripheral blood smear. The present case showed some confusing results in immunohistochemistry; the tumor cells were completely negative for CD34, an early hematopoietic antigen, which is known to be positive for the majority, but not all of AML . For exaIn conclusion, we present a case of MS of the pancreatic head accompanied by a PTTM-like condition. Further attention may be warranted for our findings that [1] the cause of obstructive jaundice/pancreas mass may be hematologic malignancy and (2) immunohistochemistry for pathological specimens obtained during autopsy and smear/flow cytometry data of peripheral blood may help subtype leukemia.


Congenital nasopharyngeal masses (CNMs) are rare. Presenting symptoms vary, and the differential diagnoses cover a wide spectrum of possibilities. As it is uncommon, most examples discussed in literature are described as case reports or series. Guidelines on CNM patient management do not exist. In this study, we present two (2) cases of neonates with CNMs that were encountered at our tertiary center. Additionally, to best elaborate a comprehensive, case-based approach to CNM management, we offer an up-to-date, diagnosis-to-treatment review of current literature.Case series and systematic literature review.Twenty-eight (28) studies are included since January 2000 to October 2021, with a total of 41 cases. Most common diagnosis was teratoma (78%). Female-to-male ratio was 2.5:1. Twenty percent of cases presented prenatally with polyhydramnios or elevated alpha-fetoprotein. Postnatally, the presenting symptoms most frequently encountered were respiratory distress (78%), oral mass (52%), and feeding difficulties (29%). Seventy-five percent of affected newborns showed symptoms within the first 24 hours of life. Forty percent of cases had comorbidities, especially in the head and neck region.Congenital nasopharyngeal masses can be detected antenatally, or symptomatically immediately after birth. Airway protection is a cornerstone in the management. Selecting the right imaging modality and convening a multidisciplinary team meeting are important toward the planning of next steps/therapeutic approach. Typically, a transnasal or transoral surgical approach will be deemed sufficient to address the problem, with a good overall prognosis. The nasopharynx—the embryonic intersection of the neural axis and alimentary and respiratory tracts—is subject to a variety of congenital anomalies. Congenital head and neck irregularities are rare, with a 5.5% prevalence among all congenital anomalies.2As opposed to anterior nasal masses, which are usually seen on external examination of the nose, posterior nasal cavity and nasopharyngeal masses are often difficult to visualize. Moreover, CNM diagnosis can be delayed since signs and symptoms are seldom specific and may mimic other upper respiratory tract problems. However, while feeding difficulties may be the only presenting symptom in patients with smaller lesions, larger CNMs usually present with acute respiratory distress and may require emergent airway protection. Therefore, as newborns are obligate nasal breathers, it is essential to quickly diagnose and treat situations with nasopharyngeal obstruction.Due to the rarity of cases, most CNMs described in the literature are case reports or series. This study presents two cases treated in our tertiary center, along with a systematic review of the current literature. Our purpose is to offer an up-to-date, case-based approach to CNM management, from diagnosis to treatment.A 3400-g female neonate was born to a primigravida, healthy mother, by a vaginal delivery, at 40+1 weeks’ gestation. Delivery was uneventful, with 9/10 Apgar scores and normal physical exam. Prenatal evaluation revealed polyhydramnios, which was attributed to gestational diabetes. During the first hours of life, dyspnea, desaturation events during feeding, and increased salivation were noticed. A nasogastric tube passed with difficulty through both nostrils; therefore an anatomical blockage was suspected. Nasal endoscopy was performed and revealed a nasopharyngeal mass. Protective intubation was performed, and the neonate was then transferred to our neonatal intensive care unit (NICU). Magnetic resonance imaging (MRI) revealed a heterogeneous lesion bulging from the nasopharynx into the oropharynx and the base of tongue, which resembled radiographically a teratoma . A biopsTwo days old, a male neonate presented with feeding and breathing difficulties. Physical examination was normal except for mild dyspnea and micro-retrognathia. Desaturation and dyspnea were relieved when the neonate was placed prone after insertion of a nasal airway. History revealed an uneventful pregnancy and spontaneous vaginal delivery at 38+6 weeks’ gestation, with a birth weight of 3785 g. Prenatal evaluation was normal. Laboratory studies and echocardiography were normal, and the neonate was transferred to our NICU for further evaluation for upper airway obstruction. Upon arrival, a nasal endoscopy exam was performed, revealing a cystic mass blocking the nasopharynx and the soft palate. A neck ultrasound (US) demonstrated a midline cystic mass posterior to the tongue. Brain US was unremarkable. Head and neck MRI demonstrated an elliptical, cystic, well-defined mass with fluid content and a stipe with partition extending from the posterior aspect of the nose to the oropharynx .As well-defined planes were observed between the mass and its adjutant structures, no biopsy was taken preoperatively. Transnasal endoscopic surgery was carried out to fully resect macroscopically a cystic nasopharyngeal mass. Extending from the roof of the nasopharynx to the soft palate, the mass, which presented with some liquid and liquefactive properties during surgery, was entirely blocking passage to the choana. Due to epistaxis, postoperative care required the administration of one blood unit and a local tamponade with tranexamic acid. The neonate was extubated on postoperative day 2. Mild stridor was treated with budesonide inhalation. On postoperative day 7, the neonate was discharged home with no feeding or respiratory symptoms.Later, on histopathological exam, the tumor was found to be positive for glial fibrillary acidic protein, suggesting the presence of brain, and the diagnosis of heterotopic brain tissue, which is similar to nasal glioma, was confirmed. Twelve months after surgery, the child was examined in our follow-up clinic for noisy breathing and snoring. Nasal endoscopy and MRI showed nasopharyngeal midline tissue, which was suspicious for enlarged adenoid tissue. Endoscopic excision of the tissue was performed, during which time no other suspicious nasopharyngeal mass was observed. Pathology report confirmed that the tissue consisted of epithelium and fibrotic stroma, which was consistent with adenoid hypertrophy.The goal of our systematic review was to find and discuss congenital nasopharyngeal masses (CNMs), as this topic is not well described in literature, and no management guidelines exist.According to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement, we searched PubMed, Embase, MEDLINE, and Google Scholar from January 2000 to October 2021 using the following search terms: “congenital nasopharyngeal mass” (or tumor or lesion), or “teratoma,” “heterotopic neuroglial tissue,” “brain tissue,” or “cephalocele.” From this, we identified 153 citations and fully reviewed reports . After eThe most common diagnoses for CNMs were teratomas  followed by hairy polyp . Twenty percent of cases presented prenatally with either polyhydramnios or elevated alpha-fetoprotein levels. Female-to-male ratio was 2.5:1. Biopsies were performed in 25% of cases.The most common presenting symptoms were respiratory distress (78%), oral mass (52%), and feeding difficulties (29%). There was a combination of symptoms in 33%.Seventy-five percent of patients showed symptoms within the first 24 hours of life, 10% within the first month, and the rest within the first year.Imaging was used in 72% of cases (30 patients)—of these, 55% had a computed tomography (CT) scan, and 45% had MRI. In 20% of cases, both modalities were used.Forty-one percent of cases (17 patients) had co-morbidities, especially in the head and neck region: of them 60% had cleft palate, 30% were found to have systemic diseases , and about 10% of patients had anatomical abnormalities (e.g. micrognathia). Fifty percent of cases occurred in developing countries.The nasopharynx represents the most superior portion of the pharynx, bounded superiorly by the skull base and inferiorly by the soft palate, and is lined by stratified squamous epithelium and by respiratory epithelium at the roof and nasal choanae. The nasopharynx derives from the neural crests of the ectodermal leaflet.In the literature CNMs are rare, and most research is based on case reports and series. The purpose of this study was to review relevant cases in a select body of literature and to develop a diagnostic approach to CNMs, regardless of the histopathological type of the mass.We presented two cases of newborns who were admitted to our hospital with CNMs leading to airway obstruction. Both cases were managed similarly, although the final histopathological reports revealed different tumor types. In our literature review, most CNM cases were teratomas, but other tumors were described as well. Based on the cases we presented and our systematic literature review, we suggest the following approach to CNM management .Prenatally, CNMs may present with polyhydramnios due to impaired swallowing.22ex-utero intrapartum treatment (EXIT) procedure to secure a newborn’s airway before division of the maternal–fetal circulation in cases of large lesions.Any lesion, especially in the head and neck area, requires referral to a genetic consultation. Parents should be referred to a tertiary hospital with an adequate team of anesthesiologists, neonatologists, and neonatal intensive care unit that can be prepared to carry out a potentially complicated airway management. Prenatal diagnosis also enables planning for an The most common presenting symptoms for CNMs in these infants were respiratory distress, oral mass, and feeding difficulties. Airway protection is, therefore, a cornerstone in the management of CNMs. In a newborn who is suspected of upper airway obstruction, a blocking mass should be suspected. Otolaryngology consultation is advised, and preventive intubation should be considered. In the case of a difficult intubation, placement of a tracheostomy tube is advisable.Although no such cases were described in the literature, it should be mentioned that CNMs can block the eustachian tube and potentially cause serous otitis media.Prior to delivery of a child with CNM, it is highly advisable to convene a multidisciplinary team (MDT) consisting of neonatologists, otolaryngologists (head and neck surgeons), radiologists, and anesthesiologists. Following the birth, the group should meet again and discuss the next steps.Selecting the appropriate imaging modality is mandatory. The purpose of imaging is to better acquaint physicians with the components of a given lesion and its relation to adjacent structures, especially the dura mater and the brain. Although an MRI with contrast agent is the recommended modality—as it easily distinguishes different types of tissues and offers reduced radiation exposure in comparison to a CT—its limited availability, long duration, and the need for patient sedation are disadvantages. A CT with contrast agent is an alternative, but its execution may require sedation, as well, and is associated with increased lifetime risk for cancer mortality (as follow-up imaging tests may be necessary). Based on our literature review, we conclude that it is not advisable to perform any CNM-related surgery without imaging, as it will increase the chances for incomplete resection, major bleeding, or adverse outcomes. When there is a concern regarding synchronic malformations, neonatologists may also require additional imaging tests.A presurgical biopsy can inform physicians about the characteristics of a given lesion and its level of aggressiveness, as well as guide their decision-making regarding the extent of resection necessary, the need for additional surgeries, follow-up period intervals, and the need for adjuvant therapies. Due to the fear of bleeding or cerebrospinal fluid leak, it is not recommended to biopsy a vascularized lesion or one with a direct connection to the dura . As only 25% of cases in the analyzed literature were biopsied before surgery, the question may be raised whether a presurgical biopsy is necessary at all. Knowing the characteristics and aggressiveness of the mass can dictate the extent and the importance of macroscopically negative margin. Regardless, sometimes the urgent need for surgery can overcome the usefulness of carrying out a biopsy.Fiberoptic intubation is the preferred technique for establishing airway access in patients with suspected masses that bulge to the oropharynx; nasal intubation is not advised as the nasopharyngeal mass may be injured, resulting in bleeding. Surgeons must be prepared with an option of surgical airway (tracheostomy) in case of failed intubation, especially in patients with large lesions. Tracheostomy techniques in newborns require experienced teams and is beyond the scope of our article.Depending on the extent of a given mass, several surgical approaches are accepted. Most cases in the studied literature were handled via the transnasal approach or combined with the transoral approach. However, recurrent or aggressive tumors may require a more definite approach or even open surgery. Once again, airway management requires a multidisciplinary approach .Parents of newborns must be included in the process of decision-making and choosing treatment options.If preoperative imaging suggests an isolated mass with no relation to brain or dura mater, there is a low risk for meningitis or other major infectious sequelae. Based on systematic literature review, postoperative prophylactic antibiotic was not indicated, unless symptoms of infection  were encountered. However, it should be noted that many authors did not discuss whether they opted to administer preoperative antibiotics, and this point should be treated with caution.Most cases either placed low emphasis on or did not document the need for follow-ups. Nevertheless, we believe regular follow-up visits are important, especially during the first few years after resection. In cases where lesions have not been fully resected, attentive follow-ups are absolutely indicated.Due to the rarity of cases, most CNMs are described in literature as case reports, and prospective studies are almost impossible to manage in these patients. In most cases, the prognosis is good. It is important to refer and manage these cases in tertiary centers and to involve MDTs in decision-making processes.This is the first study to conduct a systematic literature review on this topic. In it, we have recommended an approach for managing newborns with CNMs based on our experience and updated literature review .


The appendix duplication is a sporadic malformation in which the horseshoe form is the uncommon described variant. To our knowledge, we report the first Moroccan case of a horseshoe appendix in a girl admitted to managing of pain at the right iliac fossa.. The objectives of this work is threefold: i) to report this sporadic case of horeshoe appendix, ii) to emphasize the importance of suspicion of appendicular duplication in appendicular syndrome and iii) to recommend the exploration of the ileoceacal region to avoid surgical complications and medicolegal problems.Through this article, we present a very rare case of appendicular duplication. It has not been objectified in radiological exploration and discovered by chance during the operation. Resection then closure of the appendicular bases allowed our patient to heal.Our case report shows that we have to take into consideration this sporadic presentation of appendicular syndrome and this even in the absence of radiological signs. Our work brings enriched the literature by a new case of horseshoe appendicitis highlighting the importance of surgical treatment •We report a case report of an appendix duplication witch is a very rare congenital malformation. To date, only fifteen cases were published.•After careful examination of the literature, we found that this is the first case of this rare entity in Morocco.•Appendicular duplication is a rare malformation that often escapes preoperative radiological imaging.•We believe that this manuscript can add new perspectives to the surgery practice of this very rare presentation. It is a2A 26-year-old married woman without children from eastern Morocco and under insulin treatment for type I diabetes for 6 years was admitted to the emergency department for ketoacidosis and presented with acute onset of abdominal pain and vomiting. The clinical examination found a patient with fever at 39 °C with a positive urine test strip and tenderness in the right iliac fossa (Alvarado score> 6). We performed an infectious assessment that showed hyperleukocytosis , an increase of C-reactive protein (CRP) at 66 mg/L with slight metabolic acidosis on arterial gasometry. Ultrasound examination was in favor of acute appendicitis. After discussion with the patient, the consent for surgical exploration was obtained and showed a horseshoe appendix . An appe3Duplication of the vermiform appendix is an extremely rare malformation with an estimatedincidence of 0.004% . It was rare anatomical variations such as acute appendicitis with absence of appendix  andappendicular ectopia . Cave clanatomical location . Since tand was named “Cave-Wallbridge”. In 2010, Calotă et al. added the horseshoe shape to theold classification . Recentldescribed. We reported a new case of a horseshoe appendix that is a very raremorphological entity in addition to the fifteen reported cases described in the literature.Several hypotheses have explored the genesis of the a horseshoe appendix such as fusion of the two appendicular points, appendiculo-cecal fistula and division of the appendicular mesoappendix explained by the presence of a single vessel in the mesoappendix . The int4Appendicular duplication is a sporadic malformation that often escapes preoperative radiological imaging. Surgeons must rigorously examine the ileocecal region in order to avoid missing any appendicular duplication in case of symptomatic recurrence, and we insist on the radical surgical treatment which alone allows the cure.The procedure of surgery was explained to the patient with all advantages and possible complications. He agreed on the procedure and informed consent was taken from her.The author(s) received no financial support for the research, authorship and/or publication of this article.not applicable.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Jabi Rachid: Writing, review and editing of the manuscript.Siham Elmir: Contributed for diagnose and treatment of the patient.Mohamed Bouziane: Review, Supervision and surgeons of the patient.1Name of the registry:2Unique identifying number or registration ID:3Hyperlink to your specific registration (must be publicly accessible and will be checked):Jabi Rachid.Not commissioned, externally peer-reviewed.The authors declared no potential conflicts of interests with respect to research, authorship and/or publication of the article.


Naevus sebaceus (NS), also referred to as NS of Jadassohn, is a rare non-melanocytic congenital cutaneous hamartoma with mainly sebaceous differentiation. NS has pluripotent potential with the possible evolution of benign and/or malignant neoplastic transformation. Literature of clinical audit and retrospective analyses conclude that there is no need for prophylactic excision except for cases in which malignant transformation is suspected. Although malignant transformation is rare, there are psychosocial issues with which to contend. We present a case of a 5 year-old girl with a cerebriform mass to her right parietal scalp, which was present at birth. Sebaceous glands occur over the entire body surface, with the exception of the volar aspect of the hands in addition to the dorsal and plantar aspects of the feet. The scalp possesses the largest number and most concentrated collection of these glands . NumerouNaevus sebaceus (NS) was first described by Jadassohn in 1895; hence, it is known as NS of Jaddassohn . NS is aThere is a transition in tissue morphology; hormonal influences from the mother may briefly increase the prominence in an infant, whereas pubertal hormones enhance the verrucoid appearance in an adolescent . SecondaThe patient’s mother was gravida 3 and parity 3 who received antenatal care with the recommended antenatal ultrasounds which did not detect any congenital anomalies. There was no known family history of any mass lesions or other dermatological pathologies. She was born at term, with a mass to the right parietal region of the scalp, which was documented on her hospital chart. An magnetic resonance imaging brain study, done at 6 weeks of life, exhibited a ~3-cm diameter exophytic right parietal scalp mass that appeared to be superficial and separated from the cranium by a layer of scalp fat. There were no underlying osseous or brain abnormalities. The impression was that of a right parietal scalp mass with a benign appearance. Differential diagnoses included an epidermoid tumour or another benign tumour.The patient presented to the plastic surgery outpatient clinic at 5 years of age. The well-circumscribed pink cerebriform-like tumour was camouflaged by her hair styling . The meaOne month after her first presentation to the clinic, an excisional biopsy of the mass was performed under general anaesthesia . The scaThe pathology report consisted of a 5 × 4 × 1.2 cm portion of cerebriform skin and 2.4 × 1.2 × 0.5 cm portion of grey brown tissue. The microscopic findings demonstrated skin, with the most prominent feature being the increase in the number of sebaceous glands. Many sebaceous glands communicate directly with the epidermis. The epidermis was hyperplastic in places. The overall features were those of NS. A separately submitted specimen was reported as a portion of loose fibrous tissue.The medical importance of a solitary NS relates to the possible evolution of benign or malignant neoplastic changes. NS has pluripotent potential . A seconP < 0.05) [A 2014 retrospective analysis of 707 cases of NS revealed that trichoblastoma followed by syringocystadenoma papilliferum were the most frequent benign tumours arising from NS . The mal < 0.05) . An 18-y < 0.05) .A clinical audit published in 2007 concluded that prophylactic excision of NS is not warranted, especially in young children. Excision was recommended only when benign or malignant neoplasms are clinically suspected or for cosmetic reasons .Although the more recent literature supports that malignant transformation may be less than previously documented, full excision was completed in this patient. In addition to the possible development of a secondary tumour, psychosocial problems, such as a high level of social anxiety, social avoidance, social discrimination and interaction, and interference with personal life must be taken into consideration. The dissatisfaction with facial appearance may lead to behavioural difficulties as well . Three yThe rhombic flap was selected for scalp closure as it was easily designed, quick, provided excellent contour, texture, thickness and colour match and took advantage of the skin laxity adjacent to the defect , 13. TheIn conclusion, we would recommend that NS should be excised as early as possible to prevent the occurrence of any secondary neoplasms and to prevent psychosocial issues.None declared.None.


Ectopic pancreas is basically a benign disease and is not always necessary to be removed. However, all types of neoplasms occurring in the normal pancreas such as ductal adenocarcinomas and intraductal papillary mucinous neoplasms (IPMNs) may develop even within ectopic pancreas. We recently encountered an extremely rare case of ectopic pancreas in the gastric antrum associated with IPMN possessing a GNAS mutation.GNAS at exon 8 (Arg201Cys).A 71-year-old Japanese woman complained of epigastric pain. Computed tomography and upper gastrointestinal endoscopy showed an intramural cystic mass in the antrum of the stomach. Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) biopsy did not give a definitive diagnosis, and the patient underwent resection of the lesion. Histology of the resected specimen showed that the gastric intramural lesion was ectopic pancreas. Moreover, the lesion contained dilated duct components with tubulo-villous epithelial proliferation consistent with pancreatic IPMN. Since the covering epithelial cells had highly atypical nuclei, the lesion was diagnosed as IPMN with high grade dysplasia. Immunohistochemistry showed that the IPMN component showed to be MUC2-, MUC5AC-, and CDX2-positive but MUC1- and MUC6-negative. Mutational analyses using genomic DNA revealed that the IPMN component had a mutation of GNAS mutation. Although there were 17 cases of ectopic pancreas with IPMN including 6 cases of gastric ones reported in the English literature, this is the first case of ectopic pancreas with IPMN which was proved to have GNAS mutation. Intimate preoperative examinations including imaging analyses and EUS-FNA biopsy/cytology are recommended to decide whether the lesion has to be resected or not even if they are not effective for getting the right diagnosis.We finally diagnosed this case as gastric ectopic pancreas accompanied by intestinal type IPMN with high grade dysplasia possessing  Ectopic pancreas, also known as pancreatic heterotopia or aberrant pancreas, is defined as pancreatic tissue that presents in organs other than the pancreas. Its predilection site is the stomach, duodenum, and Meckel’s diverticulum of the ileum, and it often affects submucosal, proper muscular and/or subserosal layers of them , 2. EctoIn the pancreas, many different types of neoplasms can occur. Most of them originate from pancreatic exocrine tissue especially duct component. Ductal adenocarcinomas are the most common tumor type of ductal origin with solid characterization, and intraductal papillary mucinous neoplasms (IPMNs) are a less common tumor type of ductal origin with cystic change. Although IPMNs generally proliferate within the pancreatic duct, they could have intraductal malignant lesion with rather high incidence and even might invade outside the ductal structure as the result of tumor progression. Thus, they can be divided into three categories: intraductal papillary mucinous adenoma (IPMA), IPMN with high-grade dysplasia , and invasive IPMC.On the other hand, IPMNs can be classified into four subtypes by histology and immunohistochemistry (IHC) for mucin core proteins (MUCs)/CDX2: gastric, intestinal, pancreatobiliary, and oncocytic types , 4. GastK-ras, GNAS, p16, TP53, CTNNB1, etc [K-ras mutation could be found in both pancreatic duct adenocarcinomas and IPMNs, but GNAS mutation at exon 8 (Arg201Cys or Arg201His) is specifically detected in IPMNs [Pancreatic neoplasms such as pancreatic duct adenocarcinomas, IPMNs and solid pseudopapillary neoplasms might have gene mutations of GNAS mutation. We report here the case with a review of the literature.Ectopic pancreas is basically a benign disease and does not always require resection. However, all types of neoplasms occurring in the normal pancreas such as ductal adenocarcinomas and IPMNs may develop even within ectopic pancreas –21. TherA 71-year-old Japanese woman complaining of epigastric pain came to Hyogo College of Medicine Hospital. Computed tomography showed an intramural cystic tumor having a maximum diameter of 70 mm in the antrum of the stomach  was observed in the gastric antrum Fig. a. Cut suGNAS mutation at codon 201 in exon 8 (Arg201Cys) . Thus, the lesion was finally diagnosed as gastric ectopic pancreas accompanied by intestinal type IPMN with high grade dysplasia (non-invasive adenocarcinoma) possessing GNAS mutation (Arg201Cys). The lesion was considered to be completely resected.Moreover, the lesion contained dilated duct components with tubulo-villous epithelial proliferation Fig. c at the ys) Fig.  but not Ectopic pancreas is generally a benign disease, but there is a possibility that neoplasms occurring in the normal pancreas such as ductal adenocarcinomas and IPMNs may develop even within the ectopic pancreas –22. We ePathological changes other than neoplasms such as cyst formation and inflammation/abscess which can occur in the normal pancreas could also happen in ectopic pancreas. The lesions might cause symptoms such as abdominal pain, epigastric discomfort, nausea, vomiting, and bleeding , althougPancreatic IPMN exhibits papillary epithelial proliferation and mucus production, and is classified into four subtypes  according to mucus traits and histological features , 4. InteGNAS mutation at exon 8 (Arg201Cys or Arg201His) among pancreatic neoplasms [GNAS mutation but none of pancreatic duct adenocarcinomas have such a mutation [K-ras mutation, while approximately half of pancreatic IPMNs have the mutation [GNAS and K-ras mutations, only GNAS mutation, only K-ras mutation, or neither of them. Each subtype  of pancreatic IPMNs appears to have GNAS and/or K-ras mutations at similar frequency [GNAS mutation but not K-ras mutation. This is the first reported case of ectopic pancreas with IPMN component which is proved to have GNAS mutation. Since all of intestinal type IPMNs have GNAS mutations and since invasive cancer associated with IPMNs appears to be much more frequent in intestinal type IPMN, detection of GNAS mutation in the specimen from intramural gastric lesion by preoperative EUS-FNA biopsy/cytology could become an indicator suggesting that the tumor should be excised.Pancreatic IPMNs specifically have eoplasms , 6. Apprmutation . On the mutation . Thus, arequency . In the Imaging tests could suspect ectopic pancreas of the stomach, but preoperative accurate diagnosis of the lesion by them is usually difficult. In the case of gastric ectopic pancreas with IPMN, the situation may be similar. Moreover, preoperative EUS-FNA biopsy/cytology and incisional biopsy cannot always obtain appropriate material for pathological diagnosis like in our case. Thus, definitive diagnosis of gastric ectopic pancreas without IPMN or with IPMN is often made by the histological examination of the resected tissue. Many gastric GISTs have solid appearance by imaging, but some GISTs might show cystic change especially in those with platelet-derived growth factor receptor alpha gene mutation. Indeed, preoperative clinical diagnosis of our case was GIST with cystic change, and the final diagnosis of ectopic pancreas with IPMN was made by the resected specimen. Although both ectopic pancreas with IPMN and GIST with cystic change have to be resected because of possibility with malignant characteristics, systematic lymph node dissection might not be required in the case of GISTs because they usually could not metastasize to lymph node. In any case, we have to consider that gastric intramural lesion with cystic change might be ectopic pancreas with IPMN component.GNAS mutation. This is the first case of ectopic pancreas with IPMN which was proved to have GNAS mutation. Intimate preoperative examinations including imaging analyses and EUS-FNA biopsy/cytology are recommended to decide whether the lesion should be resected or not even if they are not effective for getting the accurate diagnosis.We experienced an extremely rare case of gastric ectopic pancreas accompanied by intestinal type IPMN with high grade dysplasia possessing


Tumor lysis syndrome is a potentially lethal condition caused by rapid cell death, releasing a high level of toxic cytokines. It is common in patients with hematological malignancy but rare in solid tumors.A 64-year-old patient presented to our unit with a 17.3-cm hepatocellular carcinoma and marginal liver reserve. The first-stage operation of associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) was performed.The patient was found to be anuric with grossly deranged electrolytes after the first-stage operation. Tumor lysis syndrome was diagnosed.The patient was transferred to the intensive care unit for aggressive fluid administration and continuous venovenous hemofiltration for the management of tumor lysis syndrome.The patient recovered and then underwent the second-stage operation of ALPPS with extended right hepatectomy 8 days after the initial operation without any long-term sequelae.ALPPS is a relatively new technique in liver surgery, entailing an increased risk of tumor lysis syndrome due to an in situ tumor after the first-stage operation. Clinicians should have a high index of suspicion regarding this potentially lethal complication with prompt management. The majority of TLS in solid tumors is associated with chemotherapy, while there is only a handful of TLS cases related to surgery. In this case report, tumor lysis syndrome occurred in a patient who had undergone initial surgery for a huge hepatocellular carcinoma (HCC).TLS occurs when tumor cells release their contents into the bloodstream. While TLS is most frequently encountered after systemic treatment of aggressive hematologic malignancy, it is rare in solid tumors with only several case reports in the literature.2Ethical approval was not necessary for the report as this was a retrospective review of anonymous clinical data. The anonymized patient has given permission to be included in the manuscript and has provided informed consent to the publication of the case. The alpha-fetoprotein level was 20 ng/mL. An indocyanine green retention test yielded a result of 20.0%, and the volume of the left lateral liver segment was 24% of the estimated standard liver volume. Liver enzyme levels were mildly elevated with a bilirubin level of 26 μmol/L, while the baseline renal function was normal. Given the patient's limited liver reserve, associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) was scheduled.A 64-year-old hepatitis-B virus carrier was referred to us for treatment of a massive HCC. Contrast triphasic computed tomographic scan showed a 17.3-cm tumor in the right liver lobe with arterial enhancement and portovenous washout (Barcelona Clinic Liver Cancer stage B).The first stage of ALPPS took 3 hours and 18 minutes, with a blood loss of 500 mL. The moderately cirrhotic liver was occupied by a 17-cm tumor over the right lobe, compressing the right hepatic vein and the middle hepatic vein. Over 70% of the left medial segment was occupied by the tumor. Temporary clamping to mark the left liver lobe and an intraoperative indocyanine green retention test were performed. The overall time of clamping of the right hepatic artery was 15 minutes. The right portal vein was controlled with Hemolocks and divided. The liver was transected with Cavitron Ultrasonic Surgical Aspirator and Thunderbeat, with 2 20-minute cycles of Pringle maneuver (PM). The patient was transferred to the intensive care unit and was given generous intravenous isotonic fluid, frusemide, calcium chloride, and febuxostat. Continuous venovenous hemofiltration was started on postoperative day 1 and resulted in improved serum electrolytes. Urine output improved and was above 2.0 mL/kg/h since postoperatively day 2. Reassessment computed tomography showed patchy hypodense areas within the tumor and over the right liver parenchyma, suggestive of tumor and liver necrosis. The left lateral segment showed significant hypertrophy, amounting to 54% of the estimated standard liver volume. Hence, second-stage ALPPS with extended right hepatectomy was performed 8 days after the first operation. Histopathological examination reviewed a moderately differentiated HCC, measuring 17.0 × 15.0 × 11.5 cm, with lymphovascular permeation and portal venous invasion. The specimen had a clear resection margin, and multiple areas of necrosis were noted.The patient developed anuria after the operation. Potassium rose to 5.4 mmol/L. Uric acid was elevated to 609 μmol/L while creatinine increased to 462 μmol/L. Phosphate went up to 2.52 mmol/L, and adjusted calcium dropped to 1.98 mmol/L. This fulfilled the Cairo and Bishop diagnosis of clinical TLS, which comprises at least 2 metabolic abnormalities, including hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia, together with an increased creatinine level and anuria.The patient's renal function subsequently improved, with a creatinine level similar to baseline. With further rehabilitation, the patient felt restored and returned home a few weeks after the second operation.3 Other risk factors include chemosensitive tumors, a large area of tumor necrosis, and pretreatment renal dysfunction.Patients with significant tumor burdens and/or rapidly dividing tumors are at greatest risk for TLS.–8 or target therapy for metastatic HCC.,10 Mehrzad et al reported a case of spontaneous TLS in metastatic HCC, and Lehner et al reported a case of TLS after radiofrequency ablation for HCC.In the literature, there are only a handful of case reports of TLS in HCC, and the majority of them are related to transarterial chemoembolization for inoperable intrahepatic HCC We postulate that the principles behind ALPPS lead to a higher risk of TLS. In any liver surgery, when major bleeding is encountered, PM is applied to limit the blood inflow in order to control the bleeding temporarily. Even though it has been shown that PM causes significant oxidative stress in human hepatocytes, in most cases, this will not cause any significant damage to the liver parenchyma, with the readily restored inflow after the release of the vascular clamp. However, since the portal vein to the diseased lobe is ligated in ALPPS, the liver may not recover from the stress caused by PM. This condition is further worsened by the presence of the in situ liver tumor after the first-stage operation. While HCC is more dependent on the hepatic artery supply, it can be damaged and undergo necrosis after PM. The intracellular content from dead tumor cells and the damaged portion of the liver is readily released into the systemic circulation before the second-stage operation, posing a constant threat of TLS. Moreover, most patients having ALPPS have a large tumor load, and thus compression and prolonged manipulation of the tumor during surgery are inevitable, further increasing the risk of TLS. Lastly, a heavy tumor burden with high cell turnover is commonly observed in these aggressive liver tumors, which makes up another essential predisposition for TLS.TLS in liver resection has never been reported, but the first case of TLS in ALPPS was encountered in less than a decade since the popularization of this treatment procedure. Due to its rare occurrence, difficulty in diagnosis, and subsequent treatment delay, the reported mortality of TLS in solid tumors is up to 35%, significantly higher than that in hematological malignancy at 1.9%.TLS requires a high index of suspicion and prompt treatment due to its potential mortality, manifesting as renal failure, arrhythmia, and recurrent seizure. Compared with TLS in hematological malignancy, where the metabolic derangement occurs soon after the initial systemic treatment, TLS in solid tumors can have an insidious onset with a delay onset up to a few weeks. To prevent uric acid crystals formation in renal tubules, uric-acid lowering agents such as allopurinol and febuxostat are also given to avoid renal failure. Compared with allopurinol, febuxostat has a higher potency due to its action to oxidized and reduced forms of xanthine oxidase. It also requires no renal adjustment and has a lower risk of hypersensitivity. In the case of renal failure, dialytic modality is needed. Continuous modality is preferred to intermittent hemodialysis to reduce the risk of “rebound” accumulation of toxic substances. In our patient, besides aggressive fluid resuscitation, febuxostat and hemofiltration were started once the diagnosis of TLS was established. He experienced no arrhythmia nor seizure and made a full recovery. This stresses the importance of early recognition and prompt treatment in patients with TLS, which is paramount to patients’ survival.The principle of treatment covers 3 critical areas: hydration, correction of metabolic abnormalities, and treatment of renal failure. Aggressive fluid administration at 3 L per square meter per day reduces serum concentrations of harmful electrolytes, increases renal blood flow, and prevents crystal precipitation in renal tubules.4This is the first case report of TLS in ALPPS. Vigilance for this potentially lethal complication and prompt management may help prevent mortality and improve the safety of this novel surgical technique.Conceptualization: Wong Hoi She.Data curation: Ho Hung Cheung, Wong Hoi She.Formal analysis: Ho Hung Cheung.Investigation: Ho Hung Cheung, Wong Hoi She, Desmond Yat Hin Yap, Simon Hing Yin Tsang.Methodology: Ho Hung Cheung, Wong Hoi She.Project administration: Ho Hung Cheung.Resources: Tan To Cheung.Software: Tan To Cheung.Supervision: Wong Hoi She, Tan To Cheung.Validation: Ho Hung Cheung, Desmond Yat Hin Yap, Simon Hing Yin Tsang.Visualization: Wong Hoi She, Simon Hing Yin Tsang.Writing – original draft: Ho Hung Cheung.Writing – review & editing: Ho Hung Cheung, Wong Hoi She, Desmond Yat Hin Yap.


In contrast to pituitary adenomas, pituitary carcinomas represent a rare malignant neoplasm with a remarkable high mortality. Pituitary carcinomas can arise from any pituitary tumor cell line and are determined to be carcinomas when there is distant metastasis or central nervous system dissemination. In this case vignette, we describe a rare case of malignant prolactinoma with intraspinal metastasis, and we also provide a review of relevant literature and treatment. Anterior pituitary adenomas/tumors (pituitary tumors) are not uncommon , 2. In aPituitary carcinomas comprise 0.2% of all pituitary tumors , 18. Of Pituitary carcinomas are difficult to treat, with patients having an average of 2-3 surgeries in addition to radiation or chemotherapy treatment . These cThe patient is a 71-year-old right-handed and physically active female with an extensive history of pituitary macroprolactinoma first diagnosed in 2006. Her treatment course has been complicated with recurrence and has required two resections. Initial treatment for her macroprolactinoma included a debulking procedure in September 2006, followed by partial transnasal transsphenoidal surgery in November 2012, and gamma knife external beam radiation treatment localized to the cavernous sinus in January 2014. She underwent radiographic and hormonal surveillance with MRI in August 2014 and July 2019. She concurrently underwent hormonal treatment with cabergoline. Pituitary MRI on August 2014 showed resolution of the tumor in the cavernous sinus. Pituitary MRI on November 2019 showed no evidence of tumor. She had normal prolactin levels between November 2014 and August 2017, with a steady increase from November 2017–July 2020 from 21.74 ng/ml to 656.60 ng/ml despite increasing the dose of cabergoline to 2 mg oral daily and a repeat course of gamma knife external beam radiation to the cavernous sinus in 2019.She presented in August 2020 with constant sharp, burning 9/10 low back pain, and left lower extremity pain worsening for the past 2 months. She described her presenting pain as most intense in the left buttock, posterior thigh, and calf. Pain was aggravated by motion initially, with only slight relief by taking naproxen or ibuprofen. She denied weakness, numbness, bowel, or bladder difficulties. She was treated with physical therapy, with only slight benefit, and had otherwise not had any other treatment for her pain. On her neurologic exam, her sensation was intact to light touch and pinprick in all dermatomes except a slight decrease in pinprick sensation to both modalities at the lateral aspect of her left foot. Her reflexes were 2+ throughout except for absent left Achilles' jerk. No evidence of Hoffmann sign, Babinski sign, or clonus. Positive straight leg raise on the left side. She had an antalgic gait, but was able to walk with her heels, toes, and heel-to-toe. She was sent for neuroimaging of the lumbar spine  are the most commonly diagnosed hormone-producing pituitary adenomas, comprising about 40% of all functional pituitary tumors , 5. ThouA small subset of pituitary adenomas, 5%, show aggressive features, which are more difficult to manage, leading to increased mortality and morbidity , 6, 14. Clinicians must have a high index of suspicion when managing a patient that has multiple occurrences after initial pituitary surgery , 20. On The European Society of Endocrinology (ESE) recently published clinical practice guidelines for the management of these forms of pituitary lesions, including aggressive pituitary tumors and carcinomas. They outline that optimal conventional medical and surgical treatment should be utilized as the primary treatment, but useful adjuvant therapies in aggressive prolactinomas can include radiation therapy and radiation therapy combined with temozolomide (TMZ) , 26. UnfPituitary tumors are not uncommon. They represent a highly heterogeneous group varying histologically and in clinical relevance. They are routinely considered benign, but a surprising number can be locally invasive and up to 15% can be clinically aggressive. Pituitary tumors are classified as carcinomas when any anterior pituitary tumor cell line demonstrates distant metastasis or central nervous dissemination. Due to the rare incidence, the management of pituitary carcinoma is still very challenging and needs further investigation. Clinicians should suspect pituitary carcinoma with metastasis when there is a discrepancy between prolactin levels and MRI neuroimaging.