Buccal bifurcation cyst (BBC) is a rare inflammatory odontogenic cyst, which commonly affects children in the first decade of life. We report a case of a seven-year-old healthy boy with bilateral BBC, which involved unerupted incomplete permanent mandibular first molars. A review of the literature in English language revealed few similar cases. We reviewed 16 manuscripts of bilateral mandibular BBC, reporting a total of 20 cases since 1970. The clinical features of bilateral mandibular BBC summarized here could assist specialists with an accurate diagnosis and provide patients with optimal management. Children between the age of 4 and 14 years are most commonly affected. In addition, BBC occurs more in the mandible and most likely involves the permanent mandibular first molar. The prevalence of BBC is less than 1% of all odontogenic cysts2. Typically, BBC causes many undesirable oral manifestations such as buccal swelling at the affected area, delayed tooth eruption or partially tooth eruption associated with deep periodontal pockets. In some cases, pain and infection associated with pus could be present3. Buccal bifurcation cyst (BBC) is a rare inflammatory odontogenic cyst. The first case of BBC was reported in 1983 by Stoneman and Worth4. Histopathologically, the BBC cyst wall is lined by a non-keratinized stratified squamous epithelium with inflammatory lymphocytes infiltrate. Surgical excisional procedure is performed for complete removal of the cysts with the involved teeth to reduce the risk of recurrence of the epithelial cysts5.Radiographically, BBC is illustrated as a well-defined radiolucent lesion surrounded by sclerotic rim. The lesion either involves the roots of a partially erupted tooth or surrounds an unerupted tooth; usually the permanent mandibular first molarHere, we report a case of a seven-year-old healthy boy with a chief complaint of painful, slowly growing lower jaw swellings on both right and left sides consistent with bilateral BBC, which involved unerupted incomplete permanent mandibular first molars. A review of the literature revealed few similar cases. We report this case to add to an additional case of bilateral mandibular BBC to the literature.A seven-year-old healthy boy presented to the Oral and Maxillofacial Surgery Clinic with a chief complaint of painful, slowly growing lower jaw swellings on both right and left sides. The patient had no past medical, surgical, or dental history. His guardian reported no known drug and food allergies. The guardian also denied having family history of genetic related diseases or syndromes. The swellings caused esthetic disfiguring of the patient’s face, which led to social exclusion.On examination, extraoral, bilateral asymmetric swellings of the lower part of the face with no lymph node involvement were noted. Intraorally, painful bilateral, hard, bony mandibular swellings covered with normal color mucosa, extending from the mesial aspect of the mandibular second primary molars on both sides including the retromolar areas, was observed. On palpation, the affected site revealed expansion of the buccal cortical plates on both sides. Both mandibular permanent first molars were not clinically erupted.A diagnostic panoramic radiograph was taken which illustrated bilateral, well-defined radiolucencies surrounded by sclerotic margins and including the unerupted incomplete permanent mandibular first molars . The panoramic image showing right radiolucency measured around 1 cm in its greatest dimension, and involved the unerupted permanent mandibular first molar , and not involving the inferior mandibular cortical bone and the adjacent areas. On the other hand, the left radiolucency measured around 3cm in its greatest dimension, and involved the unerupted permanent mandibular first molar , the inferior mandibular cortical bone and the adjacent areas. Moreover, the left radiolucent cyst distally displaced the permanent mandibular second molar tooth bud  . ProvisiThe guardian was informed about the patient’s condition, possible management and complications. Then, a consent form was signed before starting the complete surgical removal of the lesions under general anesthesia. An incision was made, then a buccal mucoperiosteal flap was raised in the mandible at the mandibular permanent first molar area on both right and left sides to expose the bone. The buccal plate was expanded and thinned; especially in the left side. An access to the cyst area was made by performing an ostectomy on both sides. The whole cyst lining was removed with the attached mandibular first molars. Then, the cavity was enucleated and curetted thoroughly , to reduThe specimens were sent to the histopathology laboratory in two containers. The histopathologic analysis of the multiple serial sections of the two specimens revealed multiple pieces of cyst wall lined by small strip of non-keratinized stratified squamous epithelium with mixed inflammatory reaction and surrounding edematous granulation tissue formed of numerous proliferating capillary type vascular spaces. Also, the specimens under the microscope showed bland looking spindle-shaped fibroblasts and degenerated bony spicules, which was consistent with cystic lesions . Based oSix months later, the clinical and radiographic examination of both surgery sites showed signs of gradual healing and resolution and increased bone density in the areas. One year later, no clinical evidence of lesion recurrence, and radiographic imaging illustrated that the affected areas were completely filled up with bone.6. It is also postulated that enamel projections at the area covered by reduced enamel epithelium between the cementoenamel junction and the furcation of the tooth could lead to cyst formation7. It is worth mentioning that the etiopathogenesis of BBC is the same of paradental cyst, according to World Health Organization Classification of Head and Neck Tumours (2017).Bilateral BBC is an uncommon inflammatory odontogenic cyst. It usually arises from the buccal side of the mandibular permanent first molar in children. The pathophysiology of BBC is still undetermined. It is claimed that the inflammation is caused by the deep periodontal pocket caused by tilted tooth. Other authors claim that the reason behind the cyst formation could be because of a defect in the tooth eruption, which may lead to inflammation, proliferation of the epithelial cells and cyst formation9. Dentigerous cyst is classified as a developmental cyst that may also occur in the mandibular first molar areas and sometimes interfere with the process of normal tooth eruption in children. On the other hand, paradental cyst is considered as an inflammatory cyst, which commonly occurs in the lateral surface of a vital tooth usually the first and second mandibular molars of primary teeth.Clinically, bilateral BBC has few challenging similarities with dentigerous cyst, and paradental cyst. For example, all previously mentioned cysts are commonly associated with a tooth. However, paradental cysts and dentigerous cysts usually occur in adults in the mandibular third molar regionRadiographic interpretation is essential to assist recognize and differentiate between different cystic lesions. Normally cystic lesions have a well-defined, unilocular radiolucency with a sclerotic margin; however, cysts sometimes present with different features. For instance, paradental cysts present on the lateral crown of an incompletely erupted tooth, while dentigerous cysts usually surround the crown of an unerupted tooth. Every rule has an exception, it is challenging to differentiate between the circumferential dentigerous cyst and the BBC radiographically as both illustrated as radiolucent cysts involving a completely unerupted tooth. Histopathologically, the cyst wall under the microscope shows chronic inflammatory lymphocytes and two to four cell layers thick of proliferating lining of nonkeratinized stratified squamous epithelium.The diagnosis of BBC should be established based on the clinical, radiographical and microscopic characteristics. The clinical examination, radiographic interpretation and histopathologic analysis of both lesions displaying cystic origin with bilateral buccal expansion involving both mandibular first molars in a seven-year-old child associated with bilateral BBC was presented here. This reported case had similar clinical, radiographical and histopathological features of bilateral mandibular BBC. It is worth mentioning that the case presented here is very rare because it describes bilateral mandibular BBC. To the best of our knowledge, the bilateral characteristic of BBC is very rare; there are only few cases have been reported in the English-language literature. In this article, we reviewed 16 manuscripts of bilateral mandibular BBC, reporting a total of 20 cases in the period between 1970 to 2019. Furthermore, these cases occurred in different ages starting from 4 years to 13 years with predilection to male patients .18. On the other hand, other cases underwent surgical enucleation of the cysts either with the involved tooth or maintaining the involved tooth. In the current case, we chose to perform a surgical procedure under general anesthesia to enucleate both bilateral mandibular BBCs with the involved teeth to reduce the risk of recurrence and to fulfill the patient’s parent’s desire.Through the past years different approaches have been performed to manage the BBC. In some cases, the way of management was only by following up the lesion with no interventions; some lesions showed different degrees of regression and others needed interventionBBC typically affects children in the first decade of life. BBC occurs in the buccal area of the mandibular first molar. Only a few bilateral mandibular BBC cases were reported in the literature. Although bilateral mandibular BBC is uncommon, the diagnosis would be less challenging if it is established by the correlation of the clinical examination, radiographic interpretation and histopathological analysis. Moreover, the clinical features of bilateral mandibular BBC summarized in this review could assist specialists to an accurate diagnosis and provide patients with optimal management.The parents mentioned that the surgery has an excellent impact on their son’s life. Also, the outcomes met their expectations as the facial swellings disappeared.Written informed consent was obtained from the patient’s father for publication of this case report and accompanying images.All data underlying the results are available as part of the article and no additional source data are required. My previous comments were not answered by the authors. Therefore, I suggest for the manuscript "Approved with major reservations".Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?PartlyIs the case presented with sufficient detail to be useful for other practitioners?NoIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?NoIs the background of the case’s history and progression described in sufficient detail?PartlyReviewer Expertise:Oral PathologyI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. The table of cases from past literature is very informative.Some points need to be explained.Please add the prevalence rate of such unusual cases to the introduction with citation.To evaluate the lesion extent, was CT or CBCT performed prior to surgery?  If so, please include a photograph of representing cut.Please also include an extraoral clinical photograph.Add details on the prescribed drugs and instructions for postoperative care.Please address previous citations regarding surgical management of this unusual cyst with complete tooth removal in the discussion.Thank you for this interesting, well written case study.Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?PartlyIs the case presented with sufficient detail to be useful for other practitioners?YesIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?PartlyIs the background of the case’s history and progression described in sufficient detail?YesReviewer Expertise:Oral & Maxillofacial SurgeryI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. Please add the prevalence rate of such unusual cases to the introduction with citation              (Done)To evaluate the lesion extent, was CT or CBCT performed prior to surgery?  If so, please include a photograph of representing cut.              No CT or CBCT were performed.  Please also include an extraoral clinical photograph.             No extraoral photograph was taken Add details on the prescribed drugs and instructions for postoperative care. (Done) This is a case of bilateral buccal bifurcation cysts that is unusual in that (a) it caused an extensive esthetic disfiguration leading to “social exclusion” and (b) necessitated extraction of two unerupted permanent molar teeth. However, as there are no clinical photographs, intra-oral or extra-oral, the first feature cannot be documented. Even more, the authors do not contemplate on the reasons that dictated extractions: does the risk of recurrence (how common is it?) balance the loss of two permanent first molars? A more thorough discussion is necessary. More specific comments:IntroductionThe Introduction contains information (i.e. “Children between the age of 4 and 14 years are most commonly affected.”) not supported be relevant references.Clinical PresentationThe swelling are described as “painful” by the patient’s guardian. It would be interesting to know whether those swellings were, also, painful on palpation and/or showed pus excretion, or whether the authors noticed any orifice on the mucosa through which they could probe the cyst.The teeth are described as “ unerupted incomplete permanent mandibular first molars” . Do they mean partially/incompletely erupted, as they were clinically not evident but radiographically not surrounded by bone?The histopathologic description should be reorganized: “multiple pieces of cyst wall” are profoundly consistent with “cystic lesions”, while it is the presence “of non-keratinized stratified squamous epithelium”, not “bland looking spindle-shaped fibroblasts and degenerated bony spicules” that make the findings “consistent with cystic lesions”. Fig. 3 is not of proper quality.DiscussionThe Discussion contains information not supported be relevant references.The main aim of Discussion is for the authors to discuss their findings and offer (if possible) new insights. For instance, the authors correctly state that the inflammation caused by a deep periodontal pocket, or a defect in tooth eruption are possible reasons for the development of . Which of those factors possibly caused the lesions in their cases? How do they explain the bilaterality of the lesion?nd paragraph they write:“Clinically, bilateral BBC has few challenging similarities with dentigerous cyst, and paradental cyst.” Only bilateral BCC has those similarities, not the usual unilateral variant?  “all previously mentioned cysts are commonly associated with a tooth”. This is common for most cysts, inflammatory or developmental!“paradental cysts and dentigerous cysts usually occur in adults in the mandibular third molar region” and later “paradental cyst… which commonly occurs in the lateral surface of a vital tooth usually the first and second mandibular molars of primary teeth.” Paradental cyst usually develops distally to the third permanent molar.The authors should be very careful in what they state. For instance, in the 2“Radiographic interpretation is essential to assist recognize and differentiate between different cystic lesions”. Please correct syntaxis.“on the lateral crown”. Wrong expression.“Every rule has an exception, it is challenging to differentiate between the circumferential dentigerous cyst and the BBC radiographically as both illustrated as radiolucent cysts involving a completely unerupted tooth”. The authors correctly include this variety of dentigerous cyst in the d.d. of the present case (why don’t they state it in the case presentation), but they should give more information not only for the shake of completeness of their work, but also for “tutorial” reasons. The “Every rule has an exception” does not to fit in that case, while the whole phrase needs reconsideration.“Histopathologically, the cyst wall under the microscope shows chronic inflammatory lymphocytes and two to four cell layers thick of proliferating lining of nonkeratinized stratified squamous epithelium”. Repetition of well known information. It would more interesting to know if histopathologic examination can differentiate the cysts included in the d.d.“The diagnosis of BBC should be established based on the clinical, radiographical and microscopic characteristics. The clinical examination, radiographic interpretation and histopathologic analysis of both lesions displaying cystic origin with bilateral buccal expansion involving both mandibular first molars in a seven-year-old child associated with bilateral BBC was presented here. This reported case had similar clinical, radiographical and histopathological features of bilateral mandibular BBC. It is worth mentioning that the case presented here is very rare because it describes bilateral mandibular BBC. To the best of our knowledge, the bilateral characteristic of BBC is very rare; there are only few cases have been reported in the English-language literature.” This paragraph says the same thing many times. Please reconsider.Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?PartlyIs the case presented with sufficient detail to be useful for other practitioners?NoIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?NoIs the background of the case’s history and progression described in sufficient detail?PartlyReviewer Expertise:Oral PathologyI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. There are no suggestions. The manuscript is ok.Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?YesIs the case presented with sufficient detail to be useful for other practitioners?YesIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?YesIs the background of the case’s history and progression described in sufficient detail?YesReviewer Expertise:Oral pathologyI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. This is an interesting and well-documented case report of bilateral buccal bifurcation cysts (BBC). It is important to include in the manuscript that the etiopathogenesis of BBC is the same of paradental cyst, according to World Health Organization Classification of Head and Neck Tumours (2017). In addition, the diagnosis should be established based in the clinical/radiographic and microscopic characteristics.Are enough details provided of any physical examination and diagnostic tests, treatment given and outcomes?YesIs the case presented with sufficient detail to be useful for other practitioners?YesIs sufficient discussion included of the importance of the findings and their relevance to future understanding of disease processes, diagnosis or treatment?YesIs the background of the case’s history and progression described in sufficient detail?YesReviewer Expertise:Oral pathologyI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above.


Importance: Spitzoid lesions are a group of melanocytic tumors characterized by spindle-like or epithelioid cells with variable malignant potential. While some spitzoid lesions are classified as evidently benign or malignant by clinic and histology, others present with unclear clinical and histological characteristics and are categorized as lesions of intermediate biologic potential. These lesions represent a challenge for pathologists and clinicians alike. No consensus on ancillary diagnostics and clinical management exists. Prediction of their clinical course is difficult. The implementation of ancillary diagnostics is currently subject of extensive discussions.Observations: We report three cases of spitzoid lesions in three young female patients  from a single reference center with different clinical and histological manifestations. In each case, uncertain clinical and histological presentation led to the stepwise application of additional diagnostics using immunohistochemistry and a custom next generation sequencing panel optimized for melanocytic lesions (MelArray). Combining ancillary diagnostics helped determine clinical management in all cases by characterizing the biology of these lesions.Conclusions and Relevance: We illustrate how clinical, histological and molecular features contribute to an optimized management plan in these critical situations and present a possible algorithm for the assessment of spitzoid neoplasms. Spitzoid lesions are a diverse group of rapid-growing melanocytic tumors characterized by spindle-like or epithelioid cells. Certain spitzoid lesions show evidently benign or malignant clinic and histology, while others present with unclear characteristics and are therefore categorized as lesions of intermediate biologic potential . These lThe implementation of ancillary diagnostics such as immunohistochemistry (IHC), comparative genomic hybridization (CGH) and next generation sequencing (NGS) is currently subject of extensive discussions.The usefulness of IHC in these cases is widely accepted, although there is no consensus on which markers to apply , 2, 4. GMelArray is a customized NGS panel optimized for melanocytic lesions that focuses on 190 genes previously reported in melanoma .We use three cases to present the interplay between clinical presentation, pathology and molecular biology and illustrate how this currently influences clinical management.A 3-year-old female presented with a quickly developing lesion on the right calf. The parents had first noticed a flat, reddish macula at 29 months of age. Within 4 months, it had grown in size and elevation and developed a verrucous aspect. Due to unclear clinical features, the lesion was totally excised in April 2019. Histology, IHC and MelArray were performed see .Considering atypical clinical presentation and histology with inconspicuous molecular characteristics, clinical follow-ups were recommended after total excision. The patient has been followed up for 9 months without relapse.A 15-year-old female presented with a 13 × 18 mm dome-shaped, pinkish papule on the lower right back. Right inguinal lymphadenopathy was detected concomitantly. The patient reported to have first noticed the skin lesion in October 2017 and an inguinal swelling in February 2019. A biopsy of the skin was taken in March 2019. Histology, IHC and MelArray were performed see .Based on this diagnostic constellation, a re-excision with 1 cm safety margin was carried out shortly after.Fine needle aspiration of the enlarged right inguinal lymph node with a diameter of 1 cm and thereafter a modified lymphadenectomy was performed in April 2019.Histology and IHC of the lymphadenectomy sample showed locoregional spreading.A PET/CT performed before the operation showed no further manifestations of disease.The family was informed about the uncertain prognostic outcome. We recommended a close follow-up regimen with clinical assessments every 6 weeks and imaging every 3 months, alternating ultrasound and PET/CT scans and no adjuvant therapy. After 9 months, no relapses have occurred.A female was referred to our clinic in April 2017 at the age of 14. The girl initially presented with a 15 mm, asymmetrically pigmented lesion on her back. The lesion had been present for years but had increased in size and thickness. Upon biopsy, a distinctly malignant histology and IHC features were seen; MelArray was performed to assess mutational status  were performed. The SLNB showed a 2.5 mm metastasis. Adjusted lymphadenectomy was carried out in a peripheral center and was tumor-free. The patient was treated with adjuvant Ipilimumab without complications from August to November 2017. Follow-ups took place every 3 months and showed no recurrence until February 2019, when multiple lymph node, lung and brain metastases were detected during routine diagnostics. The biggest brain metastasis was excised and irradiated stereotactically. Systemic immunotherapy with Ipilimumab and Nivolumab was initiated in March 2019. The patient had to discontinue immunotherapy due to progressive disease and myositis of the ocular muscles after her second and third infusions.The patient is now undergoing targeted therapy with Dabrafenib and Trametinib since May 2019. She is followed up clinically every month, with imaging every 3 months.The last PET/CT performed in December 2019 showed complete response of all extracranial lesions and cMRI showed stable disease.Variability of histologic classification , 7, 8 anIn our first patient, unremarkable IHC and molecular patterns lead us to believe that the biology of the lesion was most likely benign and a loose regimen with regular clinical follow-ups was chosen.The lesion in our second patient showed regular nevus architecture with large cells in conventional histology. Focal Ki-67 activity raised concern. Additionally, NGS showed a variant in the RASA2 gene, a tumor-suppressor for which inactivating mutations are described in 5% of melanomas . To our The frequency of non-synonymous coding mutations suggested an intermediate genetic instability. The constellation of clinical presentation of primary lesion, histology, IHC and NGS, lead us to postulate an intermediate biologic instability of the lesion. Although the tumor had already developed lymph node spreading, we expect a low risk of developing further metastases.In cases like hers, where prognosis is unpredictable, counseling of patients is crucial. A close follow-up regimen was chosen in agreement with the family, with close clinical follow-ups and frequent imaging. Adjuvant immunotherapy was not recommended.BRAF (p.V600E) mutation as well as a variety of other mutations  and a high amount of copy number variants (CNVs) in the genes analyzed by MelArray. All technologies pointed towards high potential for malignant course of disease, supporting the clinicians’ decision to proceed according to melanoma guidelines including adjuvant immunotherapy. Our primary goal was to define the dignity of these lesions and find a suitable therapeutic strategy, rather than classify them by nomenclature. Nevertheless, we would like to note that, since Spitz nevi only rarely present BRAF (p.V600E) mutations [In our third patient, IHC corroborated with histology, showing distinct signs of malignancy. NGS showed utations , 11, wheutations , we deciThe combination of specific IHC-staining and NGS gave valuable insight on lesion biology in all cases. As NGS accessibility increases, it is conceivable that it will play a role in routine assessment of biologic potential of spitzoid lesions in the near future. Although the mutational landscape of Spitz tumors differs from cutaneous melanoma , 4, 12, However, information on mutational burden and CNV analysis allows us to estimate the genomic alteration burden that might reflect malignant potential, as multiple mutations and CNVs tend to be more common in lesions with higher malignancy , 14, 15.We are confident that a standardized classification system, which routinely includes IHC and NGS, will give more clarity on spitzoid lesions in the near future , 16.For centers rarely confronted with spitzoid lesions and technologies such as NGS are not readily available, we suggest cases be referred to reference centers for further assessment .With these cases we portray three different clinical, histological and molecular manifestations of spitzoid lesions. All the information gained impacted the clinical management plan. Here we present a possible algorithm for the classification of biologic potential of spitzoid lesions .Larger, multi-center validation studies and registries with long-term follow-up are needed to evaluate feasibility and clinical relevance of routine implementation.


Adult penile lymphangioma (APL) is a rare multifactorial vascular malformation. Usually, it is asymptomatic and located on the coronal sulcus or shaft of the penis. It is benign in nature with rare chances of recurrence. APLs can be approached via various modalities from simple “watch and wait” to electrofulguration, laser ablation, and surgical excision. We report a rare variant of lymphangioma of the penis inculcating the shaft being excised surgically with favorable outcome along with brief review of literature. Lymphangioma is a benign condition which may occur due to abnormal development of the lymphatic system. Lymphangiomas are usually found in the head and neck region with rare exception of genital involvement. There are only few reported cases of penile lymphangioma in the literature. The congenital variant become more apparent over time as reported by Handa et al. , whereasA-24-year-old unmarried male patient presented with a mass over dorsum of the penis for the past 10 years. It was painless and gradually increasing in size. As per the history, there were no predisposing factors. On examination, the mass was approximately of size 3 cm × 2 cm lying on the dorsal aspect at the midshaft of the penis. It was nontender, mobile, and firm in consistency with absent inguinal lymph nodes. The ultrasonography of the penis showed cystic mass involving midshaft of the penis.A circumferential subcoronal incision was given to deglove the penis. The plane was developed below the external Colles fascia remaining above the Buck's fascia to avoid inadvertent injury to neurovascular bundles. On proximal dissection, the cystic mass was found severely adhered to midshaft. The two large feeding vessels were found at the ventral aspect of the mass which were dissected and ligated so as to remove the mass intact . The posLymphangiomas or “lymphatic malformations” were first described by Redenbacher et al. in 1828 . These lSo far, less than 50 cases have been reported since Ferris and Holmes first described penile lymphangioma in 1944 . The lymPenile lymphangiomas are commonly located on the coronal sulcus or shaft of penis.The presentation is asymptomatic in majority of patients but those symptomatic can have sexual dysfunction. Given the benign nature of penile lymphangioma and rare likelihood of recurrence, these can be managed conservatively in those asymptomatic. However, acquired large lymphangiomas causing disfigurement of the penis or for the cosmetic purpose surgical excision should be adopted.Various treatment modalities can be offered to treat lymphangiomas from simple “wait and watch” to electrofulguration, laser ablation, and surgical excision with favorable prognosis in each. However, during surgical excision, staged excision may be required depending on size . In our APL is a benign tumor of lymphatic system which can be excised surgically with favorable outcome to prevent long-term sequelae of being permanently disfigured.


We present the first case of pancreatic acinar cell carcinoma (PACC) with multiple lesions. A 55‐year‐old man with a pancretic tail mass on abdominal computed tomography (CT) was admitted to our hospital. Endoscopic ultrasound (EUS) showed a hypoechoic mass, and EUS‐guided fine‐needle aspiration (EUS‐FNA) revealed the mass to be PACC. The patient underwent distal pancreatectomy, and two masses were identified in the pancreatic tail and body. Histologically, both masses had tumor cells similar to acinar cells and were positive for BCL‐10. The patient was thus diagnosed with synchronous PACC. Ten months after the surgery, abdominal CT revealed a mass in the remnant pancreas. EUS showed a hypoechoic mass, and EUS‐FNA determined it to be PACC. The patient underwent total remnant pancreatectomy. The histological imaging results were similar to those of the first resection. Finally, the patient was diagnosed with synchronous and metachronous PACC. The possibility of multiple occurrences in the pancreas should be considered with PACC. We present the case of a 55‐year‐old man who had pancreatic acinar cell carcinoma (PACC) and underwent distal pancreatectomy. Two masses were identified in the pancreatic tail and body and were diagnosed as synchronous PACC. Ten months after the surgery, a mass was found in the remnant pancreas, and total remnant pancreatectomy was performed, resulting in the diagnosis of synchronous and metachronous PACC. Acinar cell carcinoma (ACC) of the pancreas is relatively rare, accounting for approximately 1% of all pancreatic neoplasms.Here, we report a rare case of multiple ACC that occurred synchronously and metachronously in the pancreas.A 55‐year‐old man with a pancreatic mass on contrast‐enhanced computed tomography (CT) performed due to acute alcoholic pancreatitis was admitted to our hospital. His serum cancer antigen 19‐9 level was mildly elevated at 54 U/mL . Abdominal CT revealed a 40‐mm heterogenous mass with a clear boundary in the tail of the pancreas Fig. . PancreaTen months after the surgery, abdominal CT demonstrated a 20‐mm hypovascular mass in the remnant pancreas, and EUS‐FNA revealed the presence of PACC. The patient underwent total remnant pancreatectomy. The histological features of the tumor in the remnant pancreas resembled the PACC histology of the first resected mass. No tumor cells were found in the resection margin. Finally, the patient was diagnosed with synchronous and metachronous PACC.ACC of the pancreas is relatively rare, accounting for about 1% of all pancreatic neoplasms.


Chondromyxoid fibroma is a rare benign tumor of cartilaginous origin with myxoid and fibrous components. It accounts for approximately 1% of bone tumors. Metaphysis of long bones is the most common location of this tumor. However, there a few case reports of this tumor arising from epiphysis of short tubular bones of the hand and feet.An 11-year-old girl presented to our OPD with complaints of pain and a gradually progressive swelling of the right great toe. On examination, the swelling was diffuse with no signs of inflammation. X-ray examination revealed a well-defined, longitudinally oval lytic lesion in the right distal phalanx of great toe, involving the growth plate and, eroding the medial cortex. Computed tomography (CT) scan did not show any evidence of calcification, septations or involvement of soft tissue. Open biopsy and curettage was done and the specimen was sent for histopathological examination. Histopathological examination (HPE) showed a lobular pattern consisting of myxomatous stroma and immature cartilaginous cells in lacunae. The lobules were separated by fibrous septae. It was reported to be Chondromyxoid fibroma. The patient presented six months later with persisting pain and X-ray showed recurrence of the tumor. Hence, complete excision of the tumor was done and the defect was filled using synthetic bone graft. At six months follow up, the patient did not complain of pain and X-rays showed signs of bone formation with incorporation of the graft.Chondromyxoid fibroma is a low grade tumor, which may demonstrate nuclear atypia histologically and mimic chondrosarcoma. Differentiating these two is of paramount importance to avoid over-diagnosis and aggressive treatment. Recurrence is common with marginal excision and especially in younger patients like in our case. Complete resection is the mainstay of management. Long-term follow up of patients is necessary to watch for malignant transformation, a rare complication. Chondromyxoid fibroma is an extremely rare neoplasm of bone. There are no specific radiologic features, and histopathology provides a definitive diagnosis. It should be considered in differential diagnosis of lytic lesion, and differentiated from other tumors, especially from chondrosarcoma to treat the patient appropriately. Chondromyxoid fibroma is the rarest benign tumor of cartilaginous origin characterized by incomplete cartilage differentiation. It accounts for less than 1% of all bone tumors . It is fAn 11-year-old girl presented to our OPD with complaints of pain and a gradually progressive swelling of the right great toe for six months. The pain was dull aching of mild intensity, without any diurnal variation, and associated with difficulty while walking. On examination, there was diffuse swelling of the great toe as shown in Figure X-ray examination revealed a longitudinally oval lytic lesion in the right distal phalanx of great toe, involving almost the entire shaft, abutting the growth plate and eroding the medial cortex, “hemispheric bite like lesion as shown in Figure With these findings, the patient underwent open biopsy and curettage of the lesion. Medial approach to the distal phalanx was used. Intraoperatively, there was no involvement of the soft tissue. The lesion and the walls of the cavity were excised until healthy cortical bone was seen Figure .Grossly, the curetted specimen consisted of multiple irregular tissue fragments, which were grey-white and pale brown in color as shown in Figure HPE showed a lobular pattern consisting of myxomatous stroma and immature cartilaginous cells in lacunae. Spindle shaped cells with hyperchromatic nuclei were dispersed in the myxoid matrix. The lobules were separated by hypercellular fibrous septae Figures -8. GiantThe patient presented six months later with complaints of pain in the great toe. X-ray right foot showed a lytic lesion in the distal phalanx of the great toe with well defined sclerotic and scalloped margins, coarse bony trabeculations, and without cortical expansion Figure .Since the tumor recurred, complete excision of the tumor with bone grafting was planned.The same medial approach was used again, and a cortical window was made to visualize the lesion. The cortex was found to be interrupted, and there was no involvement of the soft tissue. The lesion and the walls of the cavity were excised until healthy cortical bone was seen. The defect was filled using synthetic bone graft (G bone) as shown in Figures At six months follow up, the patient did not complain of pain, and X-rays showed signs of bone formation with incorporation of the graft Figure . Scar haChondromyxoid fibroma is a rare benign tumor of cartilaginous origin. It was first described by Jaffe and Lichtenstein in 1948 . The repRadiologically, it is seen as an eccentric lesion in metadiaphysis of long bones and as a central lesion involving the entire width of the bone in short tubular bones. The lesion is usually surrounded by well-defined scalloped margins with intralesional trebaculations. Cortical thinning and expansion are seen. However, periosteal reaction is rare ,7. The lDefinitive diagnosis can be made only on histopathology. The typical histopathology involves a lobular arrangement of stellate cells in a myxoid and chondroid background. The lobules are hypocellular with hypercellular fibrous septae. Osteoclast like cells can be seen in the peripheries .Differential diagnosesChondrosarcoma: Chondromyxoid fibroma is a low-grade tumor, which may mimic chondrosarcoma. Radiologically extensive and irregular cortical destruction with soft tissue involvement is seen in chondrosarcoma. Histologically, both of them share the lobular pattern, myxoid stroma, and peripheral cellularity with nuclear atypia. However, chondrosacrcomas are more monotonous, have larger lobules, show abundant myxoid ground substance and less fibrous component. Mature hyaline cartilage is more likely to be seen on chondrosarcoma . ChondroSimple bone cyst: Commonly seen in children and adolescents, and cannot be differentiated radiologically. Grossly, the cyst is lined by a thin fibrous membrane and filled with fluid. HPE of bone cyst shows fibrous tissue containing giant cells and hemosiderin-laden macrophages, which is not seen in chondromyxoid fibroma .Enchondroma: Commonly seen in fingers and toes. HPE shows paucicellular cartilage with mottled calcification. Myxomatous and chondroid stroma surrounded by fibrous septa, which is typical of chondromyxoid fibroma, is absent .Aneurysmal bone cyst: Both of them share similar radiological features such as cortical expansion with bubbly appearance. Histology helps in differentiating them .Low-grade infections can mimic chondromyxoid fibroma in radiology. However, periosteal reaction is commonly seen in infections, and cortical breach is seen in chondromyxoid fibroma. Both of them can be distinguished histologically .The treatment options include curettage alone or with bone grafting or cement, en bloc excision or amputation .Curettage alone, without packing the defect results in recurrence in about 20%-80% of the tumors . This isAmputation may be considered in malignant cases and multiple recurrences . AlthougDistal phalanx of the great toe is an uncommon location for chondromyxoid fibroma and only a few case reports can be found. Bahk et al. reported a case of right great toe swelling which was diagnosed initially as osteosarcoma . InterphChondromyxoid fibroma is an extremely rare neoplasm of the bone. There are no specific radiologic features, and histopathology provides a definitive diagnosis. It should be considered in the differential diagnosis of a solitary bone lytic lesion that has well-defined scalloped margins, and differentiated from other tumors, especially from chondrosarcoma to avoid aggressive treatment. To the best of our knowledge, our case of chondromyxoid fibroma of distal phalanx of the toe is the first in the literature to be reported that occurred before the closure of physis, as it is commonly seen among adults in short tubular bones.


Neuroendocrine tumors (NETs) are rare neoplasms, with an estimated annual incidence of 6.9/100 000. They arise from cells of the diffuse endocrine system, which are mainly dispersed throughout the gastrointestinal (GI), pancreatic, and respiratory tracts. The incidence of GI‐NETs has recently begun to show a steady increase. According to the Surveillance, Epidemiology, and End Results database, 53% of patients with NETs present with localized disease, 20% with locoregional disease, and 27% with distant metastases at the time of diagnosis. Surgery is the mainstay for the treatment of locoregional GI‐NETs. Endoscopic resection is an option for well‐differentiated early GI‐NETs, which are thought to very rarely metastasize to lymph nodes. A lesion that is technically difficult to resect via endoscopy is an indication for local resection . GI‐NETs with possible lymph node metastasis is an indication for enterectomy with lymph node dissection. For NETs with metastatic lesions, cytoreduction surgery can control hormonal hypersecretion and alleviate symptoms; therefore, cytoreduction surgery is recommended. The indications for surgery vary and are based on the organ where the NET arose; therefore, an understanding of the patient's clinical state and individualized treatment that is based on the characteristics of the patient's GI‐NET is needed. This review summarizes surgical treatments of GI‐NETs in each organ. The indications for surgery vary and are based on the organ where the neuroendocrine tumors (NET) arose; therefore, an understanding of the patient's clinical state and individualized treatment that is based on the characteristics of the patient's gastrointestinal NET are needed. They arise from cells of the diffuse endocrine system,Neuroendocrine cells are derived from either epithelial or neuroectodermal cells. GI‐NETs mainly arise from epithelial cells.Most NETs grow slowly over the years, and patients with NETs show signs and symptoms related to the mass of the tumor; however, about 30% of patients can have signs and symptoms associated with the hypersecretion of hormones from the tumor. Patients with nonresectable functional NETs who show signs and symptoms of hormonal hypersecretion can require systemic treatment for both control of the tumor and their signs and symptoms.1.2A study based on the Surveillance, Epidemiology, and End Results (SEER) database showed that the age‐adjusted annual incidences of NETs arising from the appendix, cecum, duodenum, colon, stomach, jejunum/ileum, and rectum were 0.15, 0.16, 0.19, 0.20, 0.30, 0.67, and 0.86, respectively.1.3Whereas tumor grade, as measured by the mitotic rate and/or the Ki‐67 index, refers to the proliferative activity of neoplastic cells, differentiation refers to the extent to which tumor cells resemble their normal counterparts. Tumor grade is defined numerically, as follows: low‐grade (grade 1 [G1]) tumors have a mitotic rate from 0 to 1 per 10 high‐power fields (HPFs) or a Ki‐67 index from 0% to 2%; intermediate‐grade (G2) tumors have a mitotic rate from 2 to 20 per 10 HPFs or a Ki‐67 index from 3% to 20%; and high‐grade (G3) tumors have a mitotic rate >20 per 10 HPFs or a Ki‐67 index >20%.1.4111In) pentetreotide somatostatin receptor scintigraphy  1,4,7,10‐tetraazacyclododecane‐1,4,7,10‐tetraacetic acid (DOTA)–octreotate (DOTATATE) PET/CT scanning is useful for baseline whole‐body staging, the detection of small lymph node or bone metastases, and identification of the primary site.The diagnosis of NETs is based on histopathology, imaging, and clinical presentation. Computed tomography (CT) and magnetic resonance imaging (MRI) play a key role in assessing the location and extent of GI‐NETs. In a series of 64 patients with metastatic GI‐NETs, MRI was found to be more sensitive than CT for the detection of small liver metastases.2Surgery is the mainstay treatment of locoregional GI‐NET, which may be curative in the case of R0 resection. Patients with operable disease have better prognosis than those with inoperable disease, even in advanced stages.2.1Gastrointestinal NETs arise from subepithelial, histamine‐secreting, enterochromaffin‐like cells. Type A gastritis is strongly associated with GI‐NETS. The recommendations for treatment suggest that the selected surgical procedure should be based on the Rindi classification Table .24Type I GI‐NETs occur in patients with chronic atrophic gastritis. They account for approximately 80% of gastric carcinoids.Type II gastric carcinoids are caused by hypergastrinemia in the setting of an underlying gastrinoma, primarily in patients with multiple endocrine neoplasia 1 (MEN1). Consequently, patients who have type II gastric NETs usually present with symptoms of Zollinger‐Ellison syndrome, such as diarrhea, heartburn, and peptic ulceration. Tumors tend to be small, multifocal, and relatively unaggressive. Elevated serum gastrin levels and low gastric pH are frequent in patients with type II gastric carcinoids. Management is conservative, and tumors might regress with successful treatment of the underlying gastrinoma.The frequency of lymph node metastasis in GI‐NETs with tumor diameters of 1‐2 cm was reported to be 21%.2.2Duodenal NETs often present as individual small, pale sessile lesions located mostly in the first or second part of the duodenum.2.3Most small bowel NETs originate in the distal ileum.2.4Neuroendocrine tumors of the appendix have been found in one of 300 appendectomy specimens, and most are found incidentally after surgery for appendicitis. The malignant potential of appendiceal NETs appears to be strictly related to tumor size. A study of a large series of patients did not find metastatic disease in 127 patients who had tumors smaller than 2 cm.2.5Colonic NETs most frequently arise in the ascending colon. Colectomy with regional lymphadenectomy has been recommended for these tumors.Rectal NETS with a tumor diameter greater than 2 cm show a very high frequency of lymph node metastasis (58%‐76%), and therefore these tumors are indications for rectal resection plus lymph node dissection.2.6In recent years, the therapeutic armamentarium for metastatic GI‐NETs has expanded considerably. New systemic treatments for tumor and syndrome control have been shown to delay progression as well as diminish symptoms related to hormone secretion. Everolimus is an inhibitor of the mammalian target of rapamycin (mTOR). The RADIANT‐4 trial showed that everolimus significantly improved PFS over placebo in advanced well‐differentiated, nonfunctional lung or GI‐NETs.Moreover, since cytoreduction surgery for NETs with metastatic lesions can control hormonal hypersecretion and alleviate symptoms, cytoreduction surgery is recommended.P = .001).For patients with liver metastases, the 5‐year survival rate after hepatectomy has been reported to range from 61% to 81%, and rates of improvement of associated signs and symptoms, including hormonal symptoms, has been reported to range from 90% to 100%.The resection of primary tumor has been recently reported to possibly lead to the prolonged survival of patients with GI‐NETs associated with metastases.3In general, surgery is the first‐line treatment for patients with localized GI‐NETs, and it should also be considered for palliation in patients with metastatic or bulky disease. The indications for surgery vary, based on the organ where the NET arose; therefore, an understanding of the patient's clinical state and individualized treatment that is based on the characteristics of the patient's GI‐NET are needed.Conflicts of Interest: The authors declare no conflicts of interest.Author Contribution: Study conception and design – K. Eto, N. Yoshida, S. Iwagami, and H. Baba; acquisition of the data – K. Eto, N. Yoshida, S. Iwagami; writing of the manuscript – K. Eto, N. Yoshida, S. Iwagami, and H. Baba. All authors approved the final manuscript.


Hemoptysis is defined as bleeding from the lower respiratory tract. It can be life-threatening and requires urgent investigation and intervention. Common causes of hemoptysis include bronchiectasis, tuberculosis, aspergilomas and malignancy. We present an unusual case of hemoptysis in a young woman with a history of surgery for scoliosis 18 years earlier.A 30-years-old woman was admitted to our institution for recurring hemoptysis since one year. She had a history of scoliosis and had undergone antero-lateral Th7 through Th12 spinal fusion surgery 18 years earlier. The hemoptysis was slight and resolved spontaneously or after empirical antibiotic therapy, and was attributed to bronchitis. Computed tomography revealed spinal rod penetration into the lung resulting in injury, while the caudal edge of the rod migrated into the liver and the joining screws had entered the mediastinum. Hemoptysis was due to penetration of the rod into the lung. The patient underwent extensive surgery, which was successful.The case highlights the need for thorough evaluation of patients with hemoptysis. Every incident of hemoptysis, even if minor, should be promptly investigated, because it can be life-threatening. •This case demonstrates a rare cause of hemoptysis due to penetration of the rod inserted during surgery for scoliosis into the lung 18 years after surgery; this condition was accompanied by migration the caudal edge of the rod into the liver and displacement of the joining screws into the mediastinum threatening to damage the aorta.•It highlights the need promptly and thoroughly evaluate patients with even slight hemoptysis because it can be life-threatening.•It indicates the need for periodic follow-up of patients after scoliosis surgery to prevent or minimize its complications. It can be life-threatening and requires urgent investigation and intervention. The diagnostic procedures should identify both, the source, and the underlying cause. Common causes include bronchiectasis, tuberculosis, and malignancy; however many other conditions such as cardiac, rheumatologic or vascular diseases can also cause hemoptysis . We pres2A 30-year-old woman was admitted to our institution in 2019 for hemoptysis. She had a history of scoliosis and had undergone surgical antero-lateral Th7 through Th12 spinal fusion 18 years earlier. She was a smoker, but denied any history of drug abuse or the inhalation of intoxicants. In the past, she had 2 pregnancies - in 2010 and 2018 - terminated by caesarean section. She had three episodes of hemoptysis during the year. The patient was expectorating sputum mixed with a small amount of fresh blood the size of a few pin heads, without chest pain or dyspnea. Hemoptysis resolved spontaneously or after empirical antibiotic therapy, and the patient did not require emergency treatment or admission. Few days before hospitalization at our institute, the hemoptysis was more pronounced – she expectorated about 5–10 mL of blood daily, and complained of chest tightness. At admission, the patient's general condition was good. Physical examination revealed advanced scoliosis of the thoracic and lumbar spine to the right, blood pressure 110/60 mmHg, pulse 82 bpm, respiratory rate 14/min, saturation 97%, and body temperature 36.5 C. Results of laboratory tests showed no abnormalities, C-reactive protein (CRP) was normal, there was no anemia, and the clotting parameters  were normal. Chest computed tomography (CT) showed penetration of the spinal rod into the right upper lobe of the lung; around the edge of the rod pulmonary consolidations and nodules corresponding to lung injury were visible . The cau3Scoliosis is defined as a lateral curvature of the spine of ≥10ͦ in the coronal plane. Surgery is recommended when the curvature reaches 45ͦ to 50ͦ or if curves progresses at an accelerated rate. The goals is to correct the deformity, prevent progression of the curve, restore trunk symmetry, and minimize pain and morbidity . After sAlthough modern instrumentation and new surgical techniques result in good surgical outcomes, the complication rates have remained somewhat constans ranging between 5% and 23% . DelayedDelayed migration of the scoliosis rod is an exceedingly rare complication. However, in most cases, passage of the rod into the rectum or the lower extremity ,10, as wThe complexity of spinal surgery is reflected in the diversity of complications that maight occur months or years later ,16. SpinTreatment of hemoptysis is challenging for physicians, because in about 20% of the cases and in up to 42% of the smokers, an etiological diagnosis cannot be established even after bronchoscopy and chest CT . Our pat4In conclusion, this case highlights several important issues: 1) it demonstrates an unusual cause of hemoptysis due to penetration of the rod inserted during surgery for scoliosis into the lung 18 years after surgery; this condition was accompanied by migration the caudal edge of the rod into the liver and displacement of the joining screws into the mediastinum threatening to damage the aorta, 2) it highlights the need to promptly and thoroughly evaluate patients with even slight hemoptysis because it can be life-threatening, 3) it indicates the need for periodic follow-up of patients after scoliosis surgery to prevent or minimize its complications, and, finally 4) this unusual case describes successful treatment despite the rare and potentially morbid complication.Not commissioned, externally peer reviewed.Ethical approval has been exempted by our institution for reporting this case.No founding was sought or secured in relation to this case report.Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of written consent is available for review by the Editor-in-Chief of this journal on request.All authors have contributed to conception and design of the study, drafting the article, revising it critically for important intellectual content. All authors have approved the final article.Not applicable.Justyna Fijolek.The authors declare that they have no conflict of interest regarding the publication of this case report.


We report the case of a 72-year-old female never-smoker with stage IV endothelial growth factor receptor (EGFR) mutated lung adenocarcinoma. This patient was started on first line tyrosine kinase inhibitor (TKI) and seemingly developed new bone metastases under this treatment. As there was a remarkable discrepancy between the partial response seen in the primary tumor and non-osseous metastatic locations, the possibility of a bone flare phenomenon was considered. In this case report, we demonstrate that new bony lesions are not always synonymous with disease progression. The development of bone metastases is common in non-small cell lung cancer (NSCLC) as about 20–40 percent of patients will be diagnosed with metastatic bone lesions at some point during the course of their disease . In the 2A 72-year-old female never-smoker was diagnosed with stage IV adenocarcinoma of the lung. The primary lesion was located in the right upper lobe and measured 2.9 cm in its greatest diameter. Staging was performed using computed tomography (CT) of chest and abdomen as well as a 18-F-Fluorodeoxyglucose Positron Emission Tomography-CT (PET-CT). These examinations showed affected hilar, mediastinal and supraclavicular lymph nodes and distant metastases in liver and bone marrow (TNM 7: cT1bN3M1b). There was no evidence on CT of focal lytic or blastic bone lesions. Because next-generation sequencing (NGS) revealed an activating EGFR mutation in exon 21, she was started on daily erlotinib 150 mg, an oral targeted EGFR TKI. This therapy was well tolerated. On her first follow-up three months into treatment, CT showed shrinking of the primary tumor, mediastinal lymph nodes and liver metastases  TKI. As these targeted therapies are becoming more common practice, the possibility of osteoblastic flaring should be considered to avoid a misinterpretation of radiologic findings leading to a premature cessation of a successful treatment. Timely follow-up imaging and a critical analysis of both clinical and radiological evolution are therefore vital for making the right therapeutic decisions.


Nodular hidradenoma is a rare benign adnexal tumor. It is most frequently encountered in the head and neck region, trunk, and extremities. This tumor exhibits a high recurrence rate, and an association with malignancy.Many names have been used to describe this pathology.We report the case of a nodular hidradenoma in a 30-year-old moroccan woman who presented with a 2-year history of a swelling in her right preauricular region. Histological examination revealed the typical appearance of a nodular hidradenoma. The tumor was excised and one year after the initial presentation, there was no sign of recurrence.We emphasize the importance of wide surgical excision with appropriate margins to prevent local recurrence. A close follow up of the patients is recommended. •Nodular hidradenoma is a rare benign adnexal tumor. Itis most frequently encountered in the head and neck region, trunk, and extremities.•This tumor exhibit a high recurrence rate, and an association with malignancy.•We emphasize the importance of wide surgical excision with appropriate margins to prevent local recurrence. A close follow up of the patients is recommended. Considerable controversy surrounds its histogenesis. It has been proposed that clear cell variations are of apocrine differentiation whereas only a minority of tumors are of true eccrine derivation . It occuA 30-year-old female patient, with no significant past medical or family history, presented at the department of ENT with a swelling in the right preauricular region, increasing gradually in volume over the last 2 years.There is history of serous discharge from the swelling.Physical examination revealed a reddish multilobulated pedunculated fleshy mass (4.0 × 3.0 cm) which bleedson touchin the right preauricular region, with an thickening of the overlying skin. The mass was freely mobile, non-compressible and non-pulsatile . RegionaThe patient underwent an incisional biopsy of the massunder local anesthesia of the mass to exclude any malignant disease.Histopathologic examination of the specimen revealed epithelial tumor proliferation with solid and cystic componentsmade of clear cells surrounded by myoepithelial cells. trabecular structures, were also seen in some areas.Rare cytonuclear atypia and mitosis patternswere noted. The histopathological pattern was consistent with a nodular hidradenoma.Therapeutic intervention: Surgical intervention was performed by our chief resident who has 5 years of operative experience. Local anesthetic of 4ml 2% lidocaine with 1 : 200,000 epinephrine was administered at the base of the tumor. Elliptical excision with wide margins was performed sharply using scalpel.After hemostasis with bipolar electrocautery, subcutaneous tissue and skin were sutured separately without tension using polyglactin and polypropylene interrupted sutures. The posThe patient was reevaluated in the outpatient clinic of our department 1 month after discharge. There was no sign of facial nerve deficit.Routine follow up 3, 6 and 12 months later showed no signs of recurrence.This case has been reported in line with the SCARE criteria .2Nodular hidradenoma is a rare benign sweat gland tumor. It is known under variable names . Table .The firsNodular hidradenomas are usually seen in patients in their 40s–80s, with a pick incidence in the 6th decade .ExceptioClinically, It appears usually as a small, solitary, nodular, superficial dermal lesion with intact overlying skin. Some tumors may exhibit ulceration on the surface or serous fluid leakage . Our casThe differential diagnosis includes metastatic disease  and primary skin tumors with follicular differentiation, sebaceous differentiation, or sweat gland differentiation .Histologically, Nodular hidradenomas may have variable patterns reflectedby the various terms used to describe this entity .It preseMalignant transformation has been sporadically reported in the literature . HoweverSeveral cases are also described with deceptively benign histological appearances, but aggressive behavior, which make difficult thedistinction between hidradenocarcinoma and hidradenoma .Recurrence of nodular hidradenoma is common, up to 10% , most prHouse et al.  have sug3Nodular hidradenoma is a rare skin adnexal tumorwith potential of aggressive behavior. We stress the importance of wide surgical excision with appropriate margins to prevent local recurrence. A close follow up of the patients is recommended.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.The authors declared that this study has received no financial support.Walid Bijou: Corresponding author writing the paperRabii Laababsi: writing the paperYoussef Oukessou: study conceptReda Abada: study conceptSami Rouadi: study conceptMohamed Roubal: correction of the paperMohamed Mahtar: correction of the paperNot commissioned, externally peer-reviewed.Authors of this article have no conflict or competing interests. All of the authors approved the final version of the manuscript.


Lingual osseous choristoma is a rare benign tumor consisting of normal matured bone tissue. It was first reported in 1913, and less than 100 cases of lingual osseous choristomas, mainly in their twenties and thirties, have been reported in the English literature until now. Here, we report an additional case of lingual osseous choristoma, in an elderly patient, that was incidentally removed by coughing and cured without additional interventions. An 89-year-old male patient was referred to our department for an evaluation of chronic cough. When we examined his oral cavity and pharynx, he expectorated a 10 -mm mass which was histologically diagnosed as an osseous choristoma. We confirmed the well-defined, rounded, high-density mass with a tiny pedicle on the base of the tongue in previous cervical spine CT images. No signs of recurrence were found during the 15-month follow-up examination. Our case serves as a reminder of this rare entity in the diagnosis of tongue masses of the elderly. The term choristoma was coined to define a tumor-like lesion composed of normal tissue in an abnormal region . SubsequAn 89-year-old male was referred to our department for an evaluation of prolonged cough from a physician after screening tests. The patient's past medical history revealed presence of hypertension, subdural hematoma, and lumber canal stenosis. The patient had no history of intraoral trauma.When we examined the patient's oral cavity and pharynx, the patient furiously coughed and expectorated a small mass. The mass was approximately 10 mm in diameter and was covered with normal mucosa . We onlyHistological examination revealed that the mass consisted of circumscribed bone, and it was surrounded by benign-appearing stratified squamous epithelium . The bonLess than 100 cases of osseous choristomas of the oral and maxillofacial region have been reported in the English language literature, and the most common site is the tongue, especially the dorsal posterior third of the tongue near the foramen caecum and circumvallate papillae . Gorini Osseous choristoma appears as a sessile or pedunculated mass usually covered by normal mucosa. The size of the lesion may vary from 3 to 50 mm at their largest diameter, and most osseous choristomas of the tongue are frequently asymptomatic . The mosThe clinical differential diagnoses include tumor-like lesions, benign tumors, and malignant tumors , 14. We The treatment of lingual osseous choristoma is surgical excision. Although recurrence of osseous choristomas of the masseter muscle has been reported , there hIn conclusion, we have reported the oldest case of lingual osseous choristoma known thus far. CT scans are useful for the radiological diagnosis of lingual osseous choristomas. The “watch and wait” approach could be an option for asymptomatic lesions because lingual osseous choristomas with tiny pedicles spontaneously resolve.


The torsion of normal adnexa is rare during pregnancy, especially in the third trimester. Nonspecific symptoms and signs as well as the limitations of ultrasound (US) make the diagnosis difficult, resulting in the loss of adnexa and fetal compromise. The magnetic resonance imaging (MRI) features of the torsion of normal adnexa are not classically described during pregnancy and only reported in a few cases. We find some different MRI features of the torsion of normal adnexa in late pregnancy and its diagnosis and treatment values are discussed in our report.A 27-year-old woman at 31 + 5 weeks’ gestation presented to the emergency department with a three-day history of the left lower abdominal pain. US discovered a mass of 87 × 61 mm in the left abdomen, but did not show whether the mass originated from the left ovary or the uterus. MRI showed the left ovary was increased in size to 82 × 42 × 85 mm with peripheral follicles. On fat-suppressed T2-weighted images, the signal intensity of the lesion was significantly decreased compared with the right ovary. The adjacent fallopian tube was found to be thickened. The radiologists diagnosed ovary infarction secondary to adnexal torsion. With the provisional diagnosis of adnexal torsion, the patient was taken to surgery. The left adnexal torsion was found during surgery. There was extensive hemorrhage and necrosis, so a left salpingo-oophorectomy was performed. The histopathology confirmed an extensively hemorrhagic fallopian tube and ovary with partial necrosis.We believe MRI is helpful where US is indeterminate in diagnosis of the torsion of normal adnexa in advanced pregnancy. We found that aside from hyperintensity on fat-saturated T1-weighted images, the low signal intensity on T2-weighted images can also reflect adnexal hemorrhage in conjunction with the torsion of normal adnexa. Adnexal torsion refers to the total or partial twist of the ovary around its vascular axis and can result in vascular compression and necrosis. A total of 13.7% of all adnexal torsion episodes are found in pregnant women . The nonMagnetic resonance imaging (MRI) is a safe and effective method for making diagnosis in advanced gestation . MRI canA 27-year-old woman at 31 + 5 weeks’ gestation  presented to the emergency department with a three-day history of the left lower abdominal pain. The pain was constant and getting progressively more severe. She did not complain of nausea or vomiting. There were no history of urinary symptoms, fevers, vaginal bleeding or uterine contraction. Ovarian hyperstimulation therapy was not performed. The patient’s ovaries were noted to be normal on ultrasound prior to the start of pregnancy.On examination, the patient’s vital signs were stable. Abdominal palpation revealed a tender abdomen on the lower left side without signs of peritoneal irritation. The uterus was enlarged corresponding to 31 + 5 weeks, and the cervix was closed with a little discharge. An obstetric US demonstrated the fetal parameters that corresponded to gestation with normal amniotic fluid and fetal activity. A heterogeneously hypoechoic mass of 87 × 61 mm was discovered in the left abdomen adjacent to the left uterine border with unclear boundaries, which contains several non-echoic millimetric cysts. Doppler revealed a lack of perfusion within the mass  around its pedicle and the left tube. The left ovary was 80 × 60 × 50 mm with a purple hue. The right adnexa and uterus were normal. As there was extensive hemorrhage and necrosis, a left salpingo-oophorectomy was performed. The histopathology confirmed an extensively hemorrhagic fallopian tube and ovary with partial necrosis Fig.  and descAdnexal torsion is frequently associated with ovarian hyperstimulation therapy or ovarian masses. The torsion of normal adnexa is rare during pregnancy, especially in the third trimester. Early diagnosis and treatment are essential to save the adnexa and decrease maternal and fetal morbidity . AdnexalMRI is a useful diagnostic modality when visualizing ovaries by routine US is difficult in the case of adnexal torsion suspected during the second and third trimesters of pregnancy . The speUS is a safe, accurate, fast, noninvasive, and inexpensive primary method for detecting adnexal torsion. The sonographic findings of adnexal torsion included the unilateral ovarian enlargement (> 4 cm), uniform peripheral cystic structures, coexistent mass within the affected ovary, free pelvic fluid, lack of arterial or venous flow, and twisted vascular pedicle . The preCompared with US, MRI has different potential advantages, such as large FOV, multiplanar imaging, excellent soft tissue contrast, and ability to differentiate blood from other fluids. A study conducted by Béranger-Gibert et al.  reportedIn conclusion, the diagnosis of adnexal torsion in advanced gestation is difficult to make due to nonspecific symptoms and signs and the limitations of US. MRI is helpful where US is indeterminate in diagnosis of the torsion of normal adnexa in advanced pregnancy. MRI is sensitive in detecting adnexal hemorrhage when the torsion of the normal adnexa occurred during pregnancy, and on imaging is displayed as hyperintensity on fat-saturated T1-weighted images or low signal intensity on T2-weighted images.


Osteochondromas are a very common and usually asymptomatic entity which may originate anywhere in the appendicular and axial skeleton. However, the ribs are a rare site of origin and here they may prove symptomatic for mechanical reasons. In this case report, we describe an unusual case of a symptomatic osteochondroma of the rib secondary to its location and unique shape, ultimately requiring surgical intervention. They are estimated to occur in 3% of the general population and account for 20–50% of all benign bone tumors and 10–15% of all bone tumors.1Mostly they develop as a solitary bone proliferation, however up to 15% of osteochondromas occur in the spectrum of Hereditary Multiple Exostoses syndrome.4Osteochondromas can originate anywhere in the appendicular or axial skeleton, however the majority develops in the lower limb with a prevalence of 50% around the knee.1–5Although osteochondromas are benign in nature, they may cause symptoms for mechanical reasons and rarely may undergo malignant transformation.In this case report, we present a rare case of a solitary osteochondroma of the rib causing significant pulmonary symptoms due its unique shape and location.A healthy 15-year-old boy presented to the Emergency Department with a 2-week history of a persistent productive cough. The sputum occasionally contained fresh blood in small quantities. He had been on a short haul flight a few days prior to symptom onset.There was no history of fever. Family history was positive for ankylosing spondylitis only.He had recently seen the general practitioner, who had prescribed Codeine with limited effect.−1 with normal clotting and infection parameters.At presentation he was hemodynamically stable. Physical examination revealed a left-sided pleuritic rub only. Blood investigations demonstrated a Hb of 8.4 mmol lA chest radiograph was performed and showed a small left-sided apical pneumothorax with a basal pleural adhesion and consolidation in the basal left upper lobe .Subsequently, a CT angiography of the thorax was performed to rule out potential pulmonary embolisms and for further investigation of the radiograph findings.No signs of pulmonary embolism were seen. Instead, there was a left-sided pneumothorax, associated with a consolidation in the lingula and basal pleural adhesion. The underlying reason for these findings was a 4 cm long spear-shaped osseous proliferation originating from the visceral side of the left ventrolateral sixth rib . There wThe patient underwent an uniportal video assisted thoracic surgery the following day confirming the CT findings of an internally directed sharp osseous proliferation originating from the sixth rib perforating the visceral pleura . AdjacenA post-operative chest radiograph showed complete resorption of the pneumothorax and the patient was discharged after 2 days in good clinical condition.Histological examination confirmed the osseous proliferation consistent with a osteochondromatous proliferation with no signs of dysplasia or concern of malignancy. Pleural specimens showed reactive and old hemorrhagic changes.This case is a rare example of a solitary osteochondroma leading to serious complications because of its shape and location.2,4.Osteochondromas are considered to develop as a chondromatous epiphyseal herniation through the periosteal cuff which implant intracortically in the metaphysis. They tend to grow away from the joint through tensile forces of tendons and ligaments. Radiologically, they show medullary and cortical continuity with the parent bone and a small hyaline cartilage cap, generally smaller than 2 cm1,4 These symptomatic osteochondromas usually present in younger patients, 75–80% are discovered before the 20th year of age.4Most osteochondromas are incidental findings, however they may cause symptoms ranging from deformity, fractures or mechanical problems with compromise of neurovascular bundle, impingement on ligaments or tendons or secondary bursae.5 and are mostly benign (55%).3 Fibrous dysplasia is the most common cause of a benign rib growth (22–30%),3,5 followed by osteochondromas (3–8%).2,3Primary chest wall tumors are very rare and account for 5–10% of bone tumors, 95% of which occur in the ribs6It is estimated that 55% of rib osteochondromas occur in patients with Hereditary Multiple Exostoses.Osteochondromas of the ribs generally originate near the costochondral junction but may also occur at the costovertebral junction. Although our patient presented with an osteochondroma slightly more lateral to the costochondral junction, it was histopathologically confirmed to be consistent with a benign osteochondromatous proliferation.7–10 and hemothorax7,11–13 or simply a palpable lump.14When symptomatic, they can cause pulmonary symptoms such as pneumothorax,15 pericardial thickening,16 hiccups,17 diaphragmic erosion and rupture7,18 and spinal cord compression.19Cases have also been rarely reported with extrapulmonary symptoms ranging from acute coronary syndrome due to extrinsic compression,8,9,12 and labor.15 The absence of a provoking event is consistent with a large population-based study which showed 76% of spontaneous pneumothorax in the general population occur in rest or sleep.20No significant traumatic event prior to symptoms has been described in these case reports and neither was the case in our patient. However, some patients had a history of previous athletic activity21Our patient reported cough and hemoptysis shortly after an intercontinental flight. Pulmonary embolism was therefore excluded. Pneumothorax and air travel do have an known association but this is considered to be the result of rupture of a superficial lung cyst due to volume expansion with drop in barometric pressure at higher altitude.Although there was no known history of underlying lung disease, we cannot rule out volume expansion of the lung as a predisposing factor for a traumatic pneumothorax in our patient.We presented a case of a solitary osteochondroma of the ventrolateral sixth rib causing a pneumothorax, pulmonary hemorrhage and hemoptysis due to its unique shape and location.Osteochondromas are very common, however the ribs are a rare site of origin.These osteochondromas tend to be asymptomatic, but may cause symptoms for mechanic or cosmetic reasons.In the case of a patient with a symptomatic osteochondroma of the rib, symptoms may range from hemo- or pneumothorax to cardiac symptoms, diaphragmic rupture and spinal nerve injuries due to extrinsic compression.Osteochondromas are a very common entity, but rarely occur at the ribs.Osteochondroma of the rib may cause non-specific, but serious complications for mechanical reasons depending on its location.Surgical intervention should be considered in the case of a symptomatic osteochondroma of the rib.


Congenital peritoneal encapsulation is a rare congenital malformation in which all or part of the small bowel is covered by a thin accessory peritoneal membrane. Despite being usually asymptomatic and an incidental finding during surgery or autopsy, there is a small number of reports in the literature whose diagnosis was established in the context of intestinal obstruction. The authors review the topic and describe a case report undergoing surgery for intestinal obstruction. Intraoperatively, there was a partial peritoneal encapsulation of the small bowel with signs of intestinal malrotation. Peritoneal membrane excision, terminal ileum release and complementary appendicectomy were performed. There was a favorable clinical evolution in the postoperative period. Although rare, it is important to remember this entity in the differential diagnosis of patients with intestinal obstruction, in the absence of other etiologic factors. Congenital peritoneal encapsulation (CPE) is a rare, congenital entity in which the small bowel is surrounded by an accessory peritoneal membrane. Its diagnosis is usually accidental, during surgery or autopsy . AbdominA 41-year-old male patient, without past medical or surgical history, denying communicable disease exposure or previous trauma history, presented to the emergency department with colicky epigastric abdominal pain associated with bilious vomiting, but normal bowel transit the day before. The patient was at the hospital the previous day with similar complaints. The physical examination was normal and he was discharged after analgesia, being asymptomatic.On physical examination, he was eupneic, hemodynamically stable and apyretic. His abdomen was soft, not distended, painless, with normal bowel sounds. Rectal examination showed an empty ampulla. Blood analysis revealed mild leukocytosis and little increase in C-reactive protein. Plain radiograph of the abdomen revealed dilated small bowel loops with air/fluid levels. Abdominal ultrasound revealed swollen intestinal loops on the right flank and interloop fluid. For further clarification, abdominal and pelvic computed tomography (CT) scan showed small bowel distension, air/fluid levels and free fluid ; signs oIn the presence of an intestinal obstruction of an unclear cause, an exploratory laparotomy was proposed. A peritoneal encapsulation  of the sCPE is a rare congenital malformation in which the small bowel is partially or totally involved by an accessory peritoneal membrane derived from the peritoneum of the yolk sac , 4. It dAlthough preoperative diagnosis is difficult, imaging findings may suggest the diagnosis. CT may reveal aggregation of small bowel loops involved by the peritoneal membrane and signs of intestinal obstruction , 4. A heSurgical treatment involves excision of the peritoneal membrane and lysis of adhesions from its fixation points, since the bowel loops within the capsule lie freely without adherence to each other , 2, 5, 7SEP was first described by Owtschinnkow in 1907 , 7, initet al [ACS was first described by Foo et al  in 1978.et al , 9, 10. et al , 4—probaet al .The actual incidence of CPE is difficult to determine, given the difficulty in differential diagnosis with ACS and SEP, entities in which the small bowel is also involved by an accessory peritoneal membrane. Contrasting from SEP, whose postoperative mortality can reach 60–80% , 4, CPE CPE is a rare clinical entity to bare in mind in the differential diagnosis of intestinal obstruction in which other causes have been excluded. A high index of suspicion allows for timely surgical intervention, avoiding ischemia and consequent intestinal resection, which are associated with greater morbidity and mortality.


Choroid plexus papillomas (CPPs) are rare, usually benign, neoplasms originating in the central nervous system. In this study, we present the first case of a giant airway-obstructing CPP in the pharynx of a newborn.A cystic mass located in the pharynx was noted in a fetus at the 29th week of gestation. Elective cesarean section was performed at the 38th week of gestation with successful intubation and ex utero intrapartum treatment. On computed tomography, there was a huge airway-obstructing cystic mass in the choana and pharynx. Elective surgery with total excision was performed, and histological examination confirmed the diagnosis of CPP.We report the first case of an extracerebral airway-obstructing CPP in the pharynx of a newborn. Radiologic examinations are not enough for the diagnosis of CPPs, and complete excision of the tumor with histological confirmation is indispensable for accurate diagnosis and treatment. Choroid plexus papillomas (CPPs) are rare tumors originating from the central nervous system. Almost all CPPs are histologically benign neoplasms. Its prevalence rates in adult and children are 0.5–1% and 3–4% of primary intracranial neoplasms, respectively . The papA 30-year-old woman undergoing regular follow-ups at an antenatal clinic and no treatment with any medicines except iron supplementation presented to our clinic. In the 29th week of gestation, a cystic mass approximately 1.8 × 2.0 cm in size located in the pharynx was noted on a routine ultrasound examination of the fetus. One month later, follow-up sonography revealed the lesion to have the same size. The amniotic fluid index (AFI) was 21.43 cm in the 30th week of gestation and increased up to 24.2 cm in the 34th week of gestation, and polyhydramnios was defined as AFI > 24 cm. There was a possibility of intubation failure. We planned an elective cesarean section with ex utero intrapartum treatment (EXIT) for prolonged stabilization of the fetal hemodynamic environment at the 38th week of gestation. A multidisciplinary team was assembled involving 2 neonatologists, 2 otorhinolaryngologists, 2 obstetricians, and 4 scrub nurses. A neonatologist performed successful intubation immediately after exposure of the fetal body, followed by umbilical cord clamping and delivery of the baby. The patient presented at birth with stable vital signs during endotracheal intubation.oxoplasma and rubella, whole blood polymerase chain reaction (PCR) of Herpes simplex virus-1 (HSV-1) and HSV-2, and real-time PCR of cytomegalovirus from urine, and all were negative.The female neonate was the first child born to the 33-year-old father and 30-year-old mother. The Apgar scores at 1 and 5 min were 7 and 8, respectively. She had a body weight of 2330 g (3rd percentile), height of 45 cm (25th percentile), and a head circumference of 32 cm (20th percentile); thus, she was diagnosed with asymmetric intrauterine growth restriction (IUGR). On the third day of life, the TORCH screening was done with serum immunoglobulin M (IgM) of tA 3D computed tomography (CT) of the chest airway performed shortly after birth showed airway obstruction owing to a cystic mass in the choana, nasopharynx, and oropharynx Fig. ; we coulAn elective surgery was performed at 16 days of age by otorhinolaryngologists. Under general anesthesia, removal of the pharyngeal mass through a transoral approach was performed in the Rose position. A whitish, protruding mass adhered to the prevertebral fascia and nasal septum, but was easily dissected to complete extirpation. The mass was 1.6 × 1.1 × 0.4 cm in size and 0.3 g in weight (Fig. CPPs were first reported in 1832 by Guerard in a 3-year-old child . In 2016To our knowledge, we report the first case of an extracerebral CPP in the pharynx presenting with airway obstruction that was resected completely without complications. Newborns are usually considered obligate nasal breathers despite being anatomically capable of breathing orally. For this reason, congenital nasal masses can cause respiratory distress in newborns. In infancy, the most common midline nasal masses are dermoids, encephaloceles, and gliomas . Nasal dThe pathological diagnosis of CPPs is similar to normal nonneoplastic choroid plexus tissue. Fibrovascular core lined by a single layer of columnar or cuboidal cell is typical finding . In caseIn the treatment of CPPs, complete surgical resection is recommended and essential for an accurate diagnosis. The survival rates associated with CPP after surgery are high. A meta-analysis revealed that the 1-, 5-, and 10-year survival rates associated with CPP are 90, 81, and 77%, respectively . No adjuObstetric ultrasonography is important for reducing the risk of unexpected poor outcomes or death of newborns. In this case, prenatal sonographic diagnosis of a slowly growing tumor, which can lead to respiratory distress owing to airway obstruction, allowed for the preparation for an EXIT process. A period of sustained utero-placental gas exchange was required to partly deliver the baby, expose the trachea, and reverse the airway occlusion. This procedure can be used to treat a variety of respiratory-distress conditions at delivery because of the prolonged stable fetal hemodynamic environment it provides .In summary, we herein report the first case of extracerebral CPP in the pharynx of a newborn with respiratory distress due to airway obstruction. Prenatal ultrasound is crucial for preventing poor outcomes in infants, and all pediatric patients with a laryngeal mass should undergo CT and MRI to rule-out a tumor with an intracranial connection; however, this is not enough to reach a definite diagnosis. Complete excision of the mass and histological confirmation are both indispensable for accurate diagnosis and treatment.


A meningeal hemangiopericytoma (MHPC) is an aggressive tumor characterized by a high rate of local recurrence and late distant metastasis. The objective of this study was to share our experience with the treatment of a MHPC and how to distinguish this tumor from a meningioma.A 62-year-old woman presented with symptoms of hypomnesia, hyperopia, and double vision for 1 month. Complete tumor excision was performed 6 years before. A biopsy sample was diagnosed as an atypical meningioma.MHPC with late delayed hepatic metastasis.Hepatic resection was performed initially, followed by secondary neurosurgery for complete excision of the bilateral frontal lesion 1 month later.Based on the tumor pathology and consensus of oncologic surgeons, radiation therapy was initiated. Adjuvant therapy was well-tolerated and the patient remained recurrence-free at 6 months after surgery.Here, we report a case of local brain tumor recurrence and multiple hepatic metastases from a MHPC. Craniotomy combined with radical metastasectomy may be useful in such cases. Detailed immunohistochemical staining is helpful to distinguish a MHPC from a meningioma. Long-term follow-up is recommended. The common anatomic locations of primary HPCs are the extremities, axilla, pelvis, retroperitoneum, head, and neck, and the sites of metastasis include the lung, bone, liver, pancreas, breast, and kidney.Here, we report a case of a recurrent meningeal HPC that had metastasized to the liver and was treated by craniotomy combined with metastasectomy 6 years after complete removal of the primary lesion.2A 62-year-old women presented with symptoms of hypomnesis, hypopsia and double vision for 1 month. She was previously diagnosed with bilateral frontal lobe meningioma and underwent tumour excision at our medical centre 6 years before. Since then, the patient remained healthy and complained of no neurological symptoms. The tumour pathology was interpreted as an atypical grade II meningioma according to the World Health Organization (WHO) criteria. No adjuvant chemo-radiotherapy was performed.On admission, her blood pressure, body temperature, and heart rate were within normal limits. A physical examination revealed no abnormalities. Laboratory studies revealed a decrease in white blood cell and platelet counts. In order to eliminate hematopathy, the patient underwent bone marrow puncture, which revealed that all blood cells were within normal ranges. An overview of the patient's laboratory studies is described in Table Cranial magnetic resonance imaging (MRI) revealed 2 bilateral frontal lobe tumours. The larger 1 was located in the frontal lobe, measuring 4.49 × 4.60 cm at the largest dimensions, the smaller 1 in the right frontal lobe, measuring 1.11 × 1.00 cm. In addition, MRI showed a narrow dural attachment in both sides  during arterial portography showed that the larger hepatic tumour located in the right lobe measured 3.76 × 2.51 cm at the largest dimensions  and anaplastic (grade III). However, irrespective of the histopathological classification, there is a significant consistency of data reporting that total tumour resection during the initial surgery is the most important factor for tumour control. Indeed, total surgical resection is regarded as the best treatment option to avoid recurrence or systemic metastases in patients with low-grade (grade II) HPCs.HPCs are aggressive tumours that have a high rate of local recurrences and late and distant metastases. Extracranial metastases occur in 23% of cases and appear at a mean of 8 years after initial therapy, with the liver and bone as the most common sites of metastases. Furthermore, Rutkowski et al demonstrated that aggressive surgical intervention provides the best survival outcome regardless of tumour location and patient age, and that if radiation is included, the total radiation dose should be limited to <50 Gy.However, some authors recommend surgical excision and postoperative radiotherapy at a dose of 50 Gy or more as an initial treatment, as adjuvant radiotherapy results in significantly better local control than surgery alone.The use of postoperative stereotactic radiosurgery for tumours that are deeply located or cannot be removed with microsurgical techniques is associated with extended survival and maintenance of neurological function. Stereotactic radiosurgery, including Cyber-Knife radiosurgery and Gamma surgery, are effective for the treatment of meningeal HPCs and associated with a low risk of adverse effects. Furthermore, anti-angiogenic agents have been proposed as potential promising therapeutic options for the treatment of HPCs.Chemotherapy dose not result in any improvement in tumour control. However, a retrospective trial of 15 patients that investigated the effect of different chemotherapies demonstrated an overall survival rate of 14 months. Furthermore, careful histological and immunohistochemical examinations, including the recently discovered signal transducers and activators of transcription 6, are essential to achieve a correct diagnosis.Microscopic examination is crucial for diagnosis of HPCs, which are composed of monomorphous spindle cells with moderate cellularity, arranged around staghorn blood vessels. HPCs and meningeal solitary fibrous tumours (SFTs) have significant similarities. Nevertheless, intracranial tumours are more cellular than HPCs or SFTs of soft tissues and contain fewer collagen bands. Additionally, meningeal HPCs demonstrate more mitoses, a higher Ki-67 index, and lower expression of CD34 and B-cell lymphoma 2 than HPCs and SFTs of soft tissues.4Liver metastasis of meningeal HPCs follows a typical protracted clinical course. Salient imaging characteristics and histological features correlated with clinical history are helpful to achieve a correct diagnosis. Craniotomy combined with radical metastasectomy may be useful in these patients. Here, we report the longest duration between initial onset and the development of local recurrence and hepatic metastases of HPCs, suggesting that providing long-term follow-up is necessary after surgery. Primary staging examinations, such as CT of the chest, abdomen and spine, seem to be indispensable and are highly recommended.The authors would like to acknowledge Anaerguli Maimaiti for her help in the preparation of this report.Aihemaiti Hasimu: Participated in the sequence alignment and drafted the manuscript.Bo Liu: Conceived the study and participated in the design, coordination and helped to draft the manuscript.Chen Liu: Participated in the surgical consultation.Dangmu – Jiafu Geng: Participated in the surgical consultation.Hui Wang: Provided immunohistochemical images and analysis.Qiang Fu: Participated in the study design.Qing – Jiu Zhou: Performed the surgery.Shao-Shan Li: Collected data and searched the Chinese and English literature.All authors read and approved the final version of the manuscript