Abdominal wall endometriosis is a rare clinical condition associated with abdominal pain and psychologic disorders. It's pathophysiology remains unclear. Clinical history and imaging findings are necessary for the diagnosis. Its management is challenging, and requires close collaboration between gynaecologists and visceral surgeons specially in complex procedures. The aims of our study are to present risk factors, clinical presentation, imaging findings and management features. It was a retrospective descriptive study including fifteen patients presenting abdominal wall endometriosis. Data about age, medical history, imaging findings, surgical procedures and outcome are reported.Fifteen women were included in our study. The most common symptom was cyclic abdominal pain. Twelve of them had history of caesarean section, and three had history of myomectomy. All patients underwent ultrasound and MRI. We performed surgical excision to all cases. One patient needed large excision with abdominoplasty procedure.Abdominal wall endometriosis is a rare clinical condition with unclear pathophysiology. It occurs frequently after gynaecologic or obstetric surgery. Most reported complaint was catamenial abdominal pain with abdominal wall mass. Ultrasonography, computed tomography and MRI are useful for diagnosis, specially to eliminate differential diagnoses. Abdominal wall endometriosis management is based on surgery. Excision goals are to remove the mass and to confirm histological diagnosis of parietal endometriosis.Parietal endometriosis is a rare and challenging condition with unclear pathophysiology. It requires specific management. This pathology will be encountered more frequently considering the increasing rate of caesarean section. •Extra pelvic endometriosis locations are rare, especially in the abdominal wall.•It is a challenging condition with an important social and medical impacts caused by symptoms.•We present a multicentric Tunisian experience of abdominal wall endometriosis management. Abdominal wall endometriosis is a rare condition. It is characterized by the development of ectopic endometrial cells and stroma in abdominal wall layer. It occurs commonly after surgical procedures such as caesarean section or myomectomy This challenging and rare condition is associated with chronic pelvic pain and psychologic disorders.In our study, we reviewed all cases of abdominal wall endometriosis in the last three years and managed in two gynaecologic surgery centers: Department B of gynaecologic surgery of Tunis maternity center and Maternity department of Tunis military hospital. Our study aims to assess clinical, diagnostic and therapeutic features of abdominal wall endometriosis. The work has been reported in line with the SCARE criteria 2It's a retrospective and observational case series study of patients with abdominal wall endometriosis managed in department B of gynaecologic surgery and obstetrics of Tunis maternity center and maternity department of Tunis military hospital. This study was conducted from January 2018 to December 2020. Fifteen patients with abdominal wall endometriosis were reported. Medical records regarding age, medical history, clinical presentation, imaging findings, surgery and postoperative outcome were collected and analysed. All surgical procedures were performed by senior gynaecologist seniors.3Fifteen patients were enrolled in our study. The mean age was 32 years ranging from of 24 to 38 years. No patient had history of pelvic endometriosis. A history of pelvic surgery involving the uterus was reported in all cases; caesarean section in twelve cases and three patients underwent myomectomy by laparotomy with intraoperative uterine cavity opening.The main complaint was cyclic pelvic pain. It was reported in all cases and located at the level of abdominal scar. Two of them presented skin changes; in fact, they showed scar ecchymosis and hyperpigmentation in menstrual period. One patient had a history of skin bleeding throughout a scar fistula.Clinical examination founds a palpable abdominal scar mass in all cases, measuring from 1,5 to 6 cm. In one case, a bleeding skin fistula was found.14,8 months was the average time separating first symptom to surgery.Abdominal wall ultrasound and pelvic MRI were performed in all patients. Rectus abdominis muscle invasion was found in all cases .Fig. 1AbSurgical excision of parietal endometriosis location was performed , Fig. 3.In one case, recurrence was reported 6 months after excision. The patient underwent a large excision with abdominoplasty to ensure abdominal wall closure and to ovoid abdominal hernia.All procedures were uncomplicated. All patient's data are reported in 4Abdominal wall endometriosis is a rare clinical condition with unclear pathophysiology. The leading theory suggests that endometrial cells are implanted outside uterine cavity after gynaecologic manipulation as caesarean section or myomectomy. It occurs frequently after gynaecologic or obstetric surgery, reported incidence ranges from 0,03 to 3,5% Esquivel and al described a clinical triad to suspect abdominal wall endometriosis Ultrasonography, computed tomography and MRI are useful for diagnosis, specially to eliminate differential diagnosis: parietal abscess, lipoma, hematoma, hernia, granuloma and tumour Ultrasonography is the first step to evaluate painful abdominal wall mass In ultrasonography imaging, abdominal wall endometriosis appears as heterogeneous and hypoechoic mass with echogenic spots MRI is the cornerstone technique to evaluate soft tissue tumours. In case of abdominal wall endometriosis, MRI is an interesting option to describe and evaluate mass size, extension, deep infiltration and involvement of surrounding structures In MRI, abdominal wall endometriosis appears as an hyperintense heterogeneous mass on both T1 and T2-weighted sequences.The management of abdominal wall endometriosis is based on surgery with large excision. Margins must be clean at least 1 cm The surgery goals are to remove the mass and to confirm histologic diagnosis of parietal endometriosis.For small nodule, surgery is easy to perform. With clean margins of 1 cm, recurrence rate is less than 5% Surgery strategies should be planned after considering recurrence and parietal defect risks. For complex procedures, close coordination between gynecologic and visceral surgeons may guarantee better outcome.Medical treatment based on oral contraceptives, analogues of gonadotropin-releasing hormone, aromatase inhibitors and dienogest may be useful if combined to surgical excision to avoid recurrence Abdominal wall endometriosis affects negatively social, sexual and professional activities, by generated pain, depression and absenteeism Several methods in gynaecologic surgery are described to avoid abdominal wall endometriosis.It is recommended to handle gently the uterine tissue, ensure meticulously the control of bleeding, wash of the intraabdominal cavity before closure and avoid subcutaneous dead spaces Randomised large studies are needed to define efficient methods to avoid abdominal wall endometriosis.5Parietal endometriosis is a rare clinical condition with unclear pathophysiology. Although rare, gynaecologists must be familiar with this pathology nowadays considering the high rate of caesarean section and gynaecological procedures. The diagnosis of abdominal wall endometriosis is based on clinical data, patient history, ultrasound and MRI. Surgical excision remains the cornerstone of treatment, it may require coordination with visceral surgeon in case of large and complex excision.No source of funding to declare.Ethical approval of case series is not needed in our institution.Written informed consent was obtained form patients for publication of this case series and accompanying images. A copy of the written consent was available for review by the Editor-in-Chief of this journal on request.Mohamed Aymen Ferjaoui and Ramzi Arfaoui: surgery and study design.Slim Khedhri, Kais Abdessamia and Mohamed amine Hannechi: manuscript redaction.Kmar Hajjami, Raja Azri and Manel Benghalia: data collection.Monia Malek, Mohamed Radhouane Rachdi and Khaled Neji: Manuscript supervisors.Not mandatory.Mohamed Aymen Ferjaoui and Ramzi Arfaoui.Not commissioned, externally peer-reviewed.No conflicts of interest to report.


Background: Haematological malignancies, such as lymphoma and leukaemia, can have a variety of clinical manifestations. The most frequent cause of death from haematological malignancies is multiple organ failure due to neoplastic organ infiltration and/or septic shock. Histiocytic sarcoma (HS) is a rare malignant nodal or extranodal tumour with histiocytic immunophenotype that originates from a lymphohematopoietic precursor. The patients with HS usually have a poor prognosis due to its aggressive clinical behaviour. Rare cases of undiagnosed sudden HS death have been described in the literature. Methods: A forensic autopsy of a 46-year-old white male who died at home suddenly and unexpectedly without warning conditions or known diseases. Gross analysis, histology and toxicology were also performed. Results: The diagnosis of HS of the ileum with secondary nodal and cardiac metastatization was made. Conclusions: A prompt diagnosis of HS in life is paramount because it can make a difference in prognostic outcomes. Histiocytic sarcoma (HS), also known as histiocytic lymphoma, is an exceedingly rare malignant neoplasm that originates from a lymphohematopoietic precursor . It is mA 46-year-old white male died suddenly and unexpectedly at home without warning conditions or known diseases. A forensic autopsy was performed 72 h after death in accordance with the Recommendations on the Harmonization of Forensic Autopsy Rules of the Committee of Ministers of the Council of Europe (1999).Histology and immunohistochemistry. For each organ, sampling was carried out. After fixation in 10% neutral buffered formalin and paraffin embedding, extensive sampling of the ventricles and atria was performed. The tissues along the course of the coronary arteries with the anterior and posterior descending branches and of the septum including the conduction system were also sampled. Sections for haematoxylin–eosin staining (H&E) for morphological diagnosis and serial sections of 4 microns for immunohistochemistry (IH) were performed. Human monoclonal anti-CD68 and anti-lysozyme antibodies (1:100) with the DAB Substrate-Chromogen system and Alkaline Phosphatase/RED (DAKO) were used.Femoral blood was analysed for alcohol (ethanol) and volatiles by headspace gas chromatography coupled with a flame ionization detector (GC/HS-FID). All post-mortem specimens  were screened for the presence of the main different classes of drugs  by immunological or chromatographic methods as appropriate. A systematic toxicological analysis (STA) was performed by means of an LC–MS/MS system (API 3200 triple quadrupole ABI-SCIEX) in the multiple reaction monitoring (MRM) mode.No blood fluid in the abdominal or thoracic cavity, while the serous surfaces of the ileum appeared to be widely congested with blood collections in the perivisceral fat and enlarged lymph nodes. Macroscopically, the heart and other systemic organs appeared normal in diameter and features. The heart was 397 g in weight . After fToxicology analysis showed no evidence of drugs or alcohol.The degenerative changes of the ileum mucosa with oedema, fibrosis and diffuse trans-parietal lymphoid infiltrate with associated middle perivisceral elements and absence of continuous solutions were observed. The perivisceral lymph nodes appeared subverted from predominantly atypical epithelioid or sarcomatoid medium-sized cells that were CD68- and lysozyme-positive , as wellHaematological malignancies, such as lymphoma and leukaemia, can have a variety of clinical manifestations. Symptoms are often caused by myeloid or lymphoid cell dysfunction or by the proliferation of neoplastic blasts in the bone marrow .Among haematological malignancies, HS is classified also as part of the adult non-Hodgkin lymphomas and occurs in both sexes and in a wide age range  ; up to nHS is an extremely rare and aggressive disease with a rapidly progressing clinical course. In the majority of cases, the primary presentation is an extranodal mass involving other organs  ,9, and tThe vast majority of GI HS is located in the bowel, and few cases are found in the stomach. When the bowel is affected, the symptomatology can be made worse by perforation with bleeding and hypovolemic shock.However, patients may be also asymptomatic or may have no warning conditions or known diseases. Rarely the diagnosis is made after death which can occur for pulmonary emboli, cardiopulmonary arrest secondary to superior vena cava syndrome, multiple organ failure, sepsis, intracranial haemorrhage or anoxic brain injury and other causes. The most frequent cause of death is multiple organ failure due to neoplastic organ infiltration and/or septic shock ,5.Furthermore, the secondary metastatic spread of malignant cells may result in systemic effects such as blood hypercoagulability ,12. OnlyIn each sudden unexpected death, pathologists and physicians are requested to find evidence of a specific cause of death, and in particular to establish whether cardiac death is secondary to arrhythmic or nonarrhythmic mechanisms or otherwise to heart failure ,22,23,24


The patient in this case report had male and female genitalia simultaneously. We created a flowchart of the differential diagnosis for clinicians.A 41-year-old male was admitted to the hospital complaining of lower quadrant abdominal pain for 1 day. Physical examination revealed that his penis size was normal, but a urethral orifice was located in the perineum area between the scrotum and anus. One small testicle was in the left scrotum, but no testicle was present on the right. The patient’s abdomen was bulging, and he had lower abdominal pain. According to the emergency CT scan, a lesion (74*65 mm) was found in the right pelvis between the bladder and rectum. The lesion showed an unclear boundary and hematocele appearance. The lesion was removed by emergency surgery, and the pathology report indicated a mixed germ cell tumor with a seminoma and yolk sac tumors.This article is a case report of germ cell tumors in 46XY PGD patients. The literature review summarizes the clinical diagnosis, and a flowchart is provided for physicians in future practice. The importance of this report is that it will help acquaint physicians with this rare disease and make the right initial clinical decision quickly through the use of this flowchart. However, the variants of special subtypes of 46XY DSD are myriad, and all the diagnoses could not be covered in one flowchart. Sex development is a highly precise process and is regulated by many factors. Genetic factors, hormone levels and changes in the external environment may cause disorders of sex development (DSDs) . DSDs caThe case in this article is rare. The patient was diagnosed with 46XY PGD with the rupture of a seminoma mixed with yolk sac tumors and had male and female genitalia simultaneously. After informing the patient and acquiring his consent, we deidentified the personal information and placed the unique radiology images and the patient’s photos in this article. We also reviewed the literature and summarized a diagnostic flowchart for clinicians.A 41-year-old male patient was admitted to the emergency room on the afternoon of September 30th, 2020 due to the complaint of “lower abdominal pain for 1 day, aggravating for 2 h”. He had mild nausea and vomiting without diarrhea, bloody stool/urine or fever. He reported no familial tumor history or hereditary disease, except ambiguity external genitalia with a normal size penis and a female-like urethral orifice located in the perineum between the anus and scrotum ; platelet (PLT) count, 214 × 109/L (125–350 × 109/L); white blood cell (WBC) count, 13.68 × 109/L (3.5–9.5 × 109/L); alpha fetoprotein (AFP), > 1210 ng/mL (< 7 ng/mL); human chorionic gonadotropin (HCG), 898 mIU/mL (< 5 mIU/mL); and lactate dehydrogenase (LDH), 2583 U/L (313–618 U/L).Laboratory test results were as follows: hemoglobin (Hb), 80 g/L(130–175 g/L); red blood cell (RBC) count, 4.25 × 102 intramuscular injection for 3 continuous days, showed that the 1st and 4th day testosterone levels after the test were 1.53 and 2.05 nmol/L, respectively.The results of hormone analysis were as follows: follicle-stimulating hormone (FSH), 20.8 mIU/mL (1–13 mIU/mL); luteinizing hormone (LH), 9.79 mIU/mL (1–8.4 mIU/mL); early morning total testosterone (T), 0.54 nmol/l (4.27–28.24 nmol/L); free testosterone (FT), 1.75 pg/mL (15–50 pg/mL); and serum dihydrotestosterone (DHT); 40 pg/mL (112–955 pg/mL). Estradiol (E2) and prolactin (PRL) levels were 0.8 nmol/L  and 15.32 (2.1–17.7 µg/L), respectively. The short-term hCG stimulating test, in which the patient was given 3000 IU/mA karyotype of 46,XY was found by testing peripheral blood lymphocytes. We proposed a testicular biopsy to the patient to obtain a histological analysis, but the proposal was refused by the patient.The patient was diagnosed with a disorder of sex development, acute lower abdominal pain and suspected pelvic tumor rupture. He underwent emergency surgery immediately after admission to the ward. We explored his abdominal area and found that the ruptured tumor was located on the right side of the pelvis, with a uterus-like structure between the bladder and the rectum. No ovary was found in the abdomen. Eight hundred milliliters of free uncoagulated blood was collected in the abdomen. The 8 by 10 cm uterus-like structure with the ruptured tumor Fig. a, b was The pathological results showed that the tumor was a seminoma (mainly) mixed with nests of yolk sac tumors  have disorders of testicular development.Congenital adrenal hyperplasia (CAH) is caused by mutations in the 17β-HSD3 or SRD5A2 genes and is involved in androgen synthesis disorders. The 17β-HSD3 gene mutation can cause deficiency in the conversion of androstenedione to testosterone. SRD5A2 gene mutation can cause deficiency in 5α-reductase that can make the conversion of testosterone to its more potent form of dihydrotestosterone (DHT).Androgen receptor gene mutations can lead to androgen insensitivity syndrome (AIS), which also includes partial (PAIS) or complete (CAIS) forms.The inconsistency among genetic (XY or XX), gonadal (ovaries or testis), external genitalia (penis or vulva), and internal genitalia (Mullerian or Wolffian ducts) causes DSD . 46XY DSThe first-line testing in newborns for diagnosing 46XY DSD should include karyotyping, imaging (ultrasound/CT/MRI), and serum hormone analysis. Molecular testing for gene mutations is limited by accessibility and cost . The difThe clinical presentation of 46XY CGD includes unambiguous female genitalia, bilateral streak gonads, and amenorrhea . PGD patIn clinical circumstances, ultrasound, CT or MRI are the most commonly used tools to determine the internal genitalia status of DSD patients. For CGD patients, there is usually no uterus or just a rudimentary uterus in the pelvis. Undescended streak gonads, Wolffian remnants, or fallopian tubes could also be found . The imaThe most important diagnostic tool for 46XY, DSD, is hormone testing. For CGD patients, gonadotropin hormones (LH and FSH) are elevated in the absence of gonadal steroid production . Short-tIn this report, the patient had ambiguous external genitalia, gynecomastia, right-sided cryptorchidism and sparse body hair. Wolffian derivatives  were revealed by pelvic ultrasound. A solid tumor was also detected on the posterior part of the bladder. The image of the tumor after resection shown in Fig. 46XY PGD may be hard to diagnose because the clinical presentation, laboratory test, and imaging findings are usually not consistent with the theory. There are some special subtypes of 46XY DSD that always confuse clinicians. For example, ovotesticular DSD (OT-DSD) patients are very similar to PGD patients in terms of clinical presentations, image findings, and biochemical profiles. The only difference is a rare condition with both ovarian and testicular tissue simultaneously present in an individual after histopathological examination . There iIn this report, we present the case of a 46,XY PGD patient with a ruptured germ cell tumor and review the literature to summarize the differential diagnostic flowchart for 46,XY DSD patients. This flowchart is easy to use and will help clinicians make the initial diagnosis. However, our diagnostic flowchart does not include all the rare subtypes of 46XY DSD.


Urinary bladder lymphangioma is a rare and benign lesion that is often causes symptoms related to irritation and urinary tract obstruction. Because a lymphangioma may resemble a true neoplasm of the urinary bladder clinically, the lesion must be removed for accurate histologic diagnosis and to rule out malignancy.We present a case of a 40-year-old female who was evaluated for painless gross hematuria. Clinical and diagnostic work up revealed a sharply defined mass involving the wall and bulging into the cavity on the dome of the bladder. Partial cystectomy was performed and histologic findings were compatible with cavernous lymphangioma. The symptom of hematuria relieved after the procedure and the patient was in good status without evidence of recurrence by cystoscopy at follow-up 6 months later.Lymphangioma of the urinary bladder is treated with surgical excision and seems to have no recurrence once completely resected, but long-time follow-up may be needed. Lymphangioma is rare benign vascular lesion composed of a localized collection of dilated lymphatic channels, which has been classified into three histologic groups depending on the size of lymphatic spaces as capillary, cavernous, and cystic . LymphanA 40-year-old woman presented with painless gross terminal hematuria for 6 months. Urinalysis showed red blood cells notable for 65 per high power field and a negative urine culture. Computerized tomography (CT) scan showed a sharply defined, heterogeneously enhancing mass involving the dome of the urinary bladder wall measuring 4.0 cm in the maximum diameter Fig. a. CystosThe cut surface of the resection specimen demonstrated a predominantly solid tumor of 3.8 cm in the maximum diameter and gray red in color with numerous tiny dilated cysts containing chylous to yellow cheese-like fluid Fig. . The covRegarded as an abnormality of morphogenesis rather than as a neoplasm, lymphangioma is now also referred to as lymphatic malformation . It affeMesenchymal tumors of the urinary bladder are rare, and lymphangiomas among them are even rarer. Of the previously reported 5 patients with lymphangioma involving the urinary bladder Table , 3 were Since its rarity, lymphangioma in the urinary bladder is extremely difficult to properly diagnose preoperatively, and histological examination is essential for the correct diagnosis. In most cases, the histologic diagnosis of lymphangioma is straightful, although some with secondary hemorrhage can resemble a cavernous hemangioma, and those with a significant amount of smooth muscle within the wall of the lymphatic vessels resemble a venous malformation. Histologic features that favor the diagnosis of lymphangioma over a hemangioma are lymphoid aggregates in the stroma and more irregular lumens with widely spaced nuclei . IHC forLymphangioma is typically benign, but because of its propensity for involvement of deeper tissue planes, recurrences have been documented in as many as 20% of patients after removal of superficial lesions . IncludiIn conclusion, lymphangioma arising in the urinary bladder is extremely rare, and only five cases have been identifed in studies reported in English. It is difficult to diagnose bladder lymphangioma according to its clinical features. The gold standard method used for its diagnosis is histopathology.Lymphangioma of the urinary bladder is treated with surgical excision and seems to have no recurrence once completely resected, but long-time follow-up may be needed.


Testicular torsion is a surgical emergency. Early diagnosis and surgical treatment are vital in order to preserve the affected gonad. Current surgical teaching emphasizes sudden, severe, persistent, unilateral scrotal pain as a cardinal symptom of testicular torsion. We present the case of unilateral testicular torsion in a 14-year-old patient who presented with the absence of severe pain. Despite a delayed presentation to the emergency department, the gonad could be salvaged successfully. Literature on the topic of testicular torsion presenting with minimal pain is limited. Nevertheless, pediatric surgeons might be faced with cases similar to the one we describe. Underestimating this phenomenon might lead to a delay of treatment. In such cases, ultrasound can be a beneficial addition in the diagnosis and accelerate definitive operative treatment. The presented case clearly demonstrates that, although we do not include testicular torsion without severe pain in our surgical teaching algorithms, we might encounter it in our clinical practice. Testicular torsion is a surgical emergency. Early diagnosis and surgical treatment are vital in order to preserve the affected gonad. The majority of cases occur during childhood and puberty, and commonly associated symptoms include sudden, severe pain and vegetative symptoms ,2. The iSudden, severe, persistent, unilateral scrotal pain;Vegetative symptoms such as nausea and emesis;Visible displacement of the testicle, either in rotation or height;Swelling and/or tenderness of the scrotal sac;Absence of cremasteric reflex.The majority of textbooks and scientific articles define testicular torsion as being characterized by one or more of the following symptoms or clinical signs:These symptoms usually lead to a timely presentation of the primary care physician or to the emergency room, although the majority of patients do not present immediately ,2. PossiTesticular torsion;Torsion of appendix testis;Hydrocele;Varicocele;Scrotal hernia;Epididymitis/orchitis;Tumor;Trauma.Differential diagnoses of scrotal pain and swelling in children include:Detorsion of the testicle should be achieved within 6–8 h in order to avoid the necrosis of the testicle . One recTesticular torsion is a surgical diagnosis, if it manifests with the above-described symptoms. Current surgical teaching emphasizes sudden, severe, persistent, unilateral scrotal pain as a cardinal symptom of testicular torsion. We present a case of an oligosymptomatic patient with testicular torsion, which reminds us to consider testicular torsion in the absence of this cardinal symptom.A 14-year-old patient presented to our interdisciplinary pediatric emergency room with scrotal discomfort of 3–5 by the numeric rating system (NRS) and the swelling of the right testicle. These symptoms were first noticed two days prior to presentation but had never been perceived as particularly severe by the patient. No initial point of onset could be identified. Recent trauma or strenuous activities were plausibly denied by the patient. Fever, nausea, emesis or other vegetative symptoms were not described. Sexual activity was negated. The patient did not appear to be shy or embarrassed about the symptoms or the physical examination.On inspection, the right scrotum was enlarged and showed slight redness but no significantly increased local temperature. Clinical examination revealed an enlarged and slightly elevated right testicle compared to the left testicle. The testicle was neither tender nor painful on palpation. Prehn sign was negative. The cremasteric reflexes were regular on both sides. The value of commonly known symptoms as it is emphasized in surgical teaching is set against the actual symptoms of the patient in As severe scrotal pain was absent, laboratory tests and ultrasound were performed and revealed a slightly elevated C-reactive protein. Point-of-care ultrasound in the emergency room showed a right testicle with an increased volume of 13 mL versus 7.6 mL contralaterally. There were no clear signs of perfusion on the right side .Thus, immediate exploratory surgery via a scrotal approach was initiated. Intraoperatively we found a 540° torsion of the right testicle with hypoperfusion and significant static swelling of the testicular vessels, as well as a bell-clapper deformity. After detorsion, the testicle had recovered sufficiently after 20 min in warm towels for the decision to be made to perform orchidopexy . This waThe patient could be discharged the following day and the follow-up examination 8 weeks after the operation showed completely healed wounds and an excellent testicular parenchymal structure and perfusion, as shown in the ultrasound performed by a pediatric radiologist . InteresDuring the hospital stay and upon follow-up, the history was taken regarding pain perception and neurological deficits. The patient had not previously noticed unexplained wounds or hematomas, or difficulties in temperature sensing. He had not experienced lack of balance or motion steadiness. According to him and his family, he had been eating a balanced diet with appropriate portions for his age. The neurological examination showed no signs of neuropathy, including inconspicuous reaction to painful stimuli, normal reflexes, muscle profile, vibration sensitivity and no deformity of the feet.The case was discussed with the pediatric neurologist and neither history nor clinical examination warranted any further diagnostic procedures such as EMNG at this time. However, we advised the family and pediatrician to be attentive to new symptoms and in such a case advised for consecutive early reevaluation.Written consent was given by the patient and his guardian to use clinical information as presented in accordance with the institutional consent form as approved by the Ethikkommission Nordwest- und Zentralschweiz (EKNZ).Testicular torsion is a surgical and clinical diagnosis. In the literature as well as in medical and surgical textbooks, it is described as associated with sudden, intense, unrelenting pain, often resulting in vegetative symptoms, the combination of which should prompt the patient to seek medical support. Our case presentation clearly shows that this is not always the case. The patient was plausibly and objectively not in great distress. His discomfort levels were, even taking interpersonal differences in pain perception into consideration, mild to moderate. His main complaint was the scrotal swelling more than the pain. While clinical presentations asked for further investigations to check the differential diagnoses, the ultrasound findings did not. This case raises several questions for the pediatric surgeon to consider. Is this just an exceptional case? Is the patient’s pain perception compromised? Why do we not teach the possibility of a nearly painless testicular torsion? What is the value of ultrasound for diagnosing testicular torsion?n = 3), moderate (n = 2) or even absent pain (n = 2) [Notably, an article by Lyon in 1961 reported that “the classic symptoms … may often not be the first sign of torsion” . The aut (n = 2) . Among tThe primary diagnosis of testicular torsion can be made clinically in the presence of severe classical symptoms as described above, ideally more than one. Studies have shown that a classical symptom such as an absent cremasteric reflex has a high sensitivity of up to 99%, while a normal cremasteric reflex does not rule out torsion ,3. A stuSeveral imaging methods are available for assessing the differential diagnoses of testicular torsion in pediatric patients. Mainly, ultrasound might be beneficial in the acute situation. Ultrasound can be used as a tool to complement the clinical impression and to distinguish differential diagnoses . In a stAlthough ultrasound is easily accessible and not harmful due to the lack of ionizing radiation, its quality is highly dependent on the examiners’ experience as well as the sophistication of technical equipment ,19. MultIn the case of a questionable diagnosis and an inconclusive ultrasound, immediate exploratory surgery should be performed. Surgery is therefore still considered the gold standard for diagnosing testicular torsion . Pain reIn the presented case, we have no clinical reason to suspect an underlying pathology of pain perception. Subjective pain experience in healthy children may vary according to multiple factors such as sex, age, and coping mechanisms . StudiesTaken together, the literature on the topic of pain-reduced testicular torsion is limited. Nevertheless, pediatric surgeons might be faced by cases similar to the one we describe. Clinically, there is no explanation for the absence of severe pain in this particular patient. Underestimating the possibility of painless testicular torsion might lead to a delay of treatment. In such cases, ultrasound can be an important beneficial addition in the diagnosis and accelerate definitive operative treatment. The presented case clearly demonstrates that, although we do not include testicular torsion without severe pain in our surgical teaching algorithms, we might encounter it in our clinical practice.


This paper presents the first report of osteomyelitis in heterotopicossification in a patient with macrodystrophia lipomatosa. Careful review ofmagnetic resonance imaging allowed correct diagnosis and design of a limitedsurgical excision. Osteomyelitis should be considered in the differentialdiagnosis of pain and discharge when heterotopic ossification is present. It is characterized by abnormal overgrowthof mesenchymal structures in a limb, often within a specific sclerotomaldistribution . Although there is a possible association with aPIK3CA gene mutation , the aetiology remains unclear . It is usually unilateral and diagnosed at birth. Clinically, theaffected region grows disproportionately larger in length and girth comparedto the rest of the extremity and usually stops at skeletal maturity . Pathologically, it is characterized by hyperproliferation of adiposetissue, interlaced with a fibrous network . ML has beenclassified into two subtypes depending on the pattern of growth : in the progressive type, the affected region grows at a faster ratecompared to the rest of the limb, whereas in the static type, which is more common, there is the same rate of growth for the affected and non-affected areas . Patients usually have major cosmeticconcerns and can find the large, heavy limb functionally challenging.Function may also be impaired due to early joint degenerative changes orneurovascular compromise .2A 47-year-old man with gigantism due to static ML of his left leg was referred with a 2-year history of two spontaneously discharging sinuseson the lateral aspect of his left leg . He had a historyof soft tissue debulking 30 years prior to presentation which was complicated by postoperative haematoma formation needing surgical drainage.After this surgery, his leg healed well. There were no concerns aboutinfection at that time and he had no sinus drainage. He returned to hisprevious mobility and had no problems with the leg for over 20 years.He recalled sustaining blunt trauma to the front of his left leg a number ofyears prior to presentation. This was a minor injury but was complicated bya cellulitic reaction in the skin. This persisted for a few weeks and thensettled completely. Again, he had no skin breakdown or sinus formationrelated to this injury.He was reviewed several times over the following years by his local surgeons, with mobility issues due to the large bulk of his leg. He then presentedwith pain and new spontaneous sinus formation, without any new injury orother infection. It was assumed that he must have haematogenousosteomyelitis of the tibia or fibula. Treatment options were discussed. Itwas decided that resection of infected bone from the tibia or fibula wouldbe possible but that closure of the wound in this very large leg would be problematic. Amputation was also considered. He was reviewed by a plasticsurgeon who concluded that amputation would not be possible as the gigantismextended to above the hip joint and would require a very majorreconstruction to close the residual stump . Prosthetic fittingwould be challenging. Similarly, local excision would require a free tissuetransfer which would be extremely difficult in this huge lower limb. It wastherefore considered that this was not operable due to his massive limbenlargement.At presentation to us, he was systemically well. The soft tissues on thelateral aspect of his left leg were indurated with two obvious drainingsinuses . A hard, non-tender lump was palpable deep to his scars and sinuses. There was no tenderness along the tibia and fibula. Plainradiography showed subcutaneous calcification consistent with heterotopicossification or possible sequestration and infection . There wasno periosteal reaction, lysis or sequestration in the fibula or tibia. Amagnetic resonance imaging (MRI) scan demonstrated fluid around theheterotopic bone, with oedema in the surrounding soft tissues . This fluid extended to the skin at one of the sinuses . Thetibia and fibula were normal. There was no sign of any tumour or otherpathology.Enterobacter cloacae, Pseudomonas aeruginosa and Staphylococcus aureus. All threeorganisms were cultured from at least three specimens. The Staph. aureus was fullysensitive and the Gram negatives were only resistant to nitrofurantoin, Ceftazidime and Aztreonam. He was treated with intravenous amikacin and ciprofloxacin according to these sensitivity results, followed by 6 weeks of oral ciprofloxacin. He recovered quickly from surgery with good wound healing and return to weight-bearing. At 1-year follow-up, the wound over the lateral leg was well healed and there were no signs of local recurrenceof osteomyelitis . The patient has been reviewed at 30 monthsafter surgery and has remained infection-free, with no recurrence of thesinus and no local signs of infection.Intraoperative findings confirmed the presence of subcutaneous heterotopicossification with osteomyelitis, with no deeper extension of the infection.The ossification had formed superficial to the deep fascia in the lowerdermis. At the start of the operation, the sinus tract was excised anddiscarded. The heterotopic bone was exposed and five samples were taken fromthis bone and the soft tissue attached to it, with separate instruments.These were cultured for anaerobic and aerobic organisms. The infected anddead bone was excised completely and the tissues were closed primarily. Intraoperative bone cultures showed 3Localized limb hypertrophy may be caused by a number of conditions, including ML, neurofibromatosis type 1, Klippel–Trenaunay–Weber syndrome, haemangiomatosis and Proteus syndrome . However, with theexception of ML, these conditions usually have a positive family history andare associated with cutaneous and systemic signs . Althoughmostly asymptomatic, patients with ML typically present with two primaryconcerns: cosmetic and mechanical. Cosmesis may be the presenting complaintat all ages but mechanical problems tend to develop in adolescence withsecondary degenerative joint changes and resultant reduction in function. Patients may also present due to repetitivetrauma to the affected region . This is likely to be due to thesheer size of the affected region, making it more prone to injury.Heterotopic ossification forms within soft tissues which normally do notossify . It can either be genetic or acquired. The latter ismore common, often secondary to soft tissue trauma, including burns,orthopaedic procedures, musculoskeletal trauma and brain and spinal cordinjury . Osteomyelitis has not been described as acomplication of heterotopic ossification (HO) and it is more common to have HO forming in response to infection, particularly around infected joint prostheses .It is difficult to determine when the heterotopic ossification developed inthis limb. It may have developed in the scar after the debulking surgery,particularly as there was a history of haematoma formation. Ossification oflimb haematomas was first described by Van Arsdale in 1893 , but there are no reports of osteomyelitis developing in such bone.However, our patient was completely unaware of any HO after this surgery andhis infection did not present until many years later. We suspect that his HOwas secondary to the blunt trauma sustained to his left leg several yearsprior to presentation. This trauma produced a small haematoma along thefascia which may have ossified, forming an area of HO. Our patient reports a “cellulitic episode” following his traumatic event. This may have been a true episode of skin cellulitis, but hissymptoms of erythema, swelling, pain and increased warmth may alternativelyhave been due to the initial inflammatory phase of HO formation which canpresent with these symptoms . The two diagnoses are in factoften confused . A review of 235 patients with HO after spinalcord injury reported that 6.4 % exhibited features which mimicked activeinfection . As the HO matures, with calcification developingat its periphery, findings on MRI become less specific, making diagnosismore difficult. However, once fully formed, the characteristic features ofhyperintense cancellous marrow on T1- and T2-weighted images with a rim ofhypointense cortical bone make the diagnosis clearer .Mature, uninfected HO should not exhibit peri-osseus or medullary oedema, which are more characteristic of osteomyelitis . In this case,the presence of long-standing sinuses diagnosed the osteomyelitis, which wasconfirmed and localized by the imaging. Newer imaging modalities such assingle-photon emission computed tomography in combination with computed tomography (SPECT-CT) are increasingly being used to aid in the diagnosis ofHO. SPECT-CT has also been used successfully to distinguish betweenosteomyelitis and HO .4Osteomyelitis has not previously been reported in gigantism orpost-traumatic HO in the limbs. Clinical review of this patient suggested atypical lower limb chronic osteomyelitis, arising in the tibia or fibula.The presence of severe gigantism prevented simple surgical resection ofthis, so he was denied curative treatment. Careful review of the MRI scansrevealed no involvement of his tibia or fibula but demonstrated an area ofunexpected HO in the soft tissues on the lateral aspect of his leg,communicating with the sinus tracts. This was much more amenable to surgicalresection with easy soft tissue closure.


Desmoid fibromatosis (DF) is a rare and locally infiltrative monoclonal fibroblastic proliferation arising from connective tissues, with lack of metastatic potential. About 10% of all DF cases are intra‐abdominally sited. Complications in this site, due to the locally infiltrative nature of the disease, may be severe and potentially life threatening. However, data on incidence, management, and outcome of these complications are limited.Data of patients with sporadic or FAP‐related intra‐abdominal DF treated at Istituto Nazionale dei Tumori (INT) in Milano from 2005 to 2020 who developed a serious complication during the course of their disease were retrospectively collected and analyzed with a descriptive statistics.Out of 72 intra‐abdominal DF, 8 cases were identified : 3 with bowel obstruction, 5 with bowel perforation. In 4 cases the serious complication was the first evidence of disease; in the other 4 cases it occurred at a time interval from diagnosis in the range of 4–44 months (during an active surveillance program in one case and during chemotherapy in the other 3 cases). A surgical treatment was feasible and successful in 5 cases. In 3 surgically unmanageable patients, all progressing and experiencing acute complications while on chemotherapy, a non‐surgical approach with intensive supportive treatment and with a prompt change of chemotherapy regimen was implemented, being successful in two, the other patient dying due to a concomitant progressive lymphoma thereafter.In this series of intra‐abdominal DF, the incidence of serious complications was 11%. Most patients were successfully treated with surgery. When surgery was deemed to be unfeasible, a conservative management with intensive supportive care and a careful choice of chemotherapy was adopted, ensuring a favorable outcome in most. In the sporadic setting, the disease is most commonly located to girdles, extremities and abdominal wall. In these sites, the locally infiltrative nature of the disease may result in functional impairment and pain, with a significant impact on quality of life, but low risk of death. Intra‐abdominal‐site is rare in sporadic setting, accounting for less than 5%–10% of cases, but is very common in FAP‐related cases, accounting for 80% of cases.On this basis, we retrospectively selected patients with sporadic or FAP‐related intra‐abdominal DF treated at the Fondazione IRCCS Istituto Nazionale dei Tumori (INT), Milan, Italy, over 15 years, and reviewed healthcare records of patients who experienced a serious complication.2The medical records of all consecutive patients with sporadic or FAP‐related intra‐abdominal DF treated from 2005 to 2020 at INT who experienced a serious complication during the course of their disease were retrospectively reviewed. The following data were collected: time of occurrence of the serious complication from diagnosis of DF; treatment on‐going at the onset of the serious complication; type of serious complication, management, and outcome. Details on DF treatment strategy adopted before and after the serious complication were also recorded. Data collected included also gender, age, DF size, and multifocality. Follow‐up was updated as of July 2020. Descriptive statistics were used. This study was approved by the institutional Ethics Committee.3Seventy‐two patients with intra‐abdominal DF were treated at INT over the study period: 51 with mesenteric DF and 21 with retroperitoneal or pelvic DF. In 61 cases the disease was sporadic and in 11 cases it arose in the context of FAP. Out of these 72 patients, 8 patients (11%), who experienced a serious complication, were identified for this study. All 8 cases had mesenteric DF, so that the incidence of serious complication was 16% when only mesenteric DF was considered. Two cases had FAP‐related DF  and 6 had sporadic disease . Three patients experienced a bowel obstruction and 5 patients had a bowel perforation. Three patients were females and 5 were males. The median age at the onset of the event was 35 years (range 20–56 years). The median maximum diameter of the lesion at the time of the event was 18 cm (range 6–35 cm). Three patients had multifocal intra‐abdominal disease.In 4 patients, the diagnosis of DF followed the serious complication and the radiological evidence of an intra‐abdominal lesion. In the other 4 cases, the serious complication occurred after 4, 5, 6, and 44 months from diagnosis, respectively. In particular, in one case the serious complication occurred during an active surveillance program and in the other 3 cases it occurred during chemotherapy .In 5 cases, the management of the serious complication was based on a surgical treatment . Two FAP patients were treated with a palliative surgery after the onset of a small‐bowel obstruction and the CT evidence of an intra‐abdominal lesion. After the histological confirmation of DF diagnosis, both patients received a low‐dose chemotherapy with methotrexate and vinorelbine (MTX‐VA), for 40 and 43 cycles, respectively, achieving a dimensional response. Ileostomy was removed after 1 year from the withdrawal of chemotherapy in both cases. Another patient was initially treated with a palliative surgery after the CT evidence of an intra‐abdominal lesion causing a small‐bowel perforation. A complete surgery with ileostomy removal was performed 6 months later. A further patient, initially managed elsewhere with active surveillance because asymptomatic, was treated with a macroscopic intralesional surgery after 5 months from the diagnosis, following the evidence of a progression of the disease, causing a small‐bowel perforation. Subsequently, because of the presence of a macroscopic residual disease, the patient received a low‐dose chemotherapy, achieving a dimensional shrinkage. One additional patient was treated with a complete surgery after the onset of fever, abdominal pain and the radiological evidence of an intra‐abdominal mass causing large‐bowel perforation. After 30 months from the event, in this last case, a CT scan showed a local relapse and a treatment with pazopanib was started. Treatment was on‐going at the moment we were writing this report. After a median follow‐up of 41 months (range 25–59 months) all patients are alive, 4 of them free from progression, one with an event of local relapse.A conservative management of the serious complication was adopted in 3 cases, because a surgical treatment was deemed to be unfeasible. These 3 cases are detailed hereafter. Details about chemotherapy doses administered are reported in Table 44.1After a diagnosis of mesenteric DF (10 cm), this patient , who was asymptomatic, was seen at INT in April 2012. An active surveillance program was started and a progressive dimensional shrinkage (up to 7 cm) was observed until August 2015, when, following a dimensional increase of the lesion (up to 11 cm) with the appearance of a new satellite lesion (4.5 cm), a liposomal doxorubicin‐based chemotherapy was started. After rapid progression of disease , conditioning bowel sub‐obstruction with fever, the patient was admitted to INT on September 18, 2015. Antibiotic and analgesic therapy were implemented and one cycle of doxorubicin plus dacarbazine was administered after 2 weeks. A few weeks later, following an episode of severe acute anemia and abdominal pain, radiological signs of duodenal and small bowel perforation were detected Figure . A surgi4.2This patient , who was asymptomatic, came to our attention in October 2019, after the diagnosis of mesenteric DF (9.5 cm). Low‐dose chemotherapy with MTX‐VA was recommended. Because of a subsequent diagnosis of follicular lymphoma, 3 months later a treatment with R‐CHOP  was started. After rapid worsening of clinical condition, with acute abdominal pain, on January 30, 2020 the patient was admitted to INT. CT scan showed a tumor response as far as lymphoma was concerned, but a dimensional increase (19 cm) of the DF lesion was observed, with signs of bowel perforation. A surgical treatment was excluded, and a conservative management was undertaken. A doxorubicin‐based treatment was continued during hospitalization, with weekly administration of doxorubicin for three cycles. Symptomatic improvement was obtained and the patient was discharged after 2 months of hospitalization. Since the following CT showed a shrinkage of the DF lesion (16 cm) but an increase of mediastinal and abdominal lymph nodes, R‐CHOP was resumed, with evidence of further progression after two cycles. A rescue chemotherapy with rituximab, oxaliplatin, and cytarabine (R‐DHAOx) was started, but after one cycle further lymphoma progression and dimensional increase of DF lesion (18 cm) were observed. Best supportive care was selected and 8 months after the onset of the severe complication this patient died.4.3In October 2018, this patient , who was asymptomatic, came for an outpatient visit at INT, after being diagnosed with intra‐abdominal DF (20 cm) a few weeks earlier. An active treatment with low‐dose MTX‐VA was proposed. On March 29, 2019, the patient was again evaluated at INT, after 12 weekly cycles of chemotherapy. General condition appeared worsened, with symptoms of bowel sub‐obstruction and malnutrition, and the patient was admitted. A CT scan showed a dimensional increase of the lesion (up to 35 × 22 cm) Figure  with bil5Out of 72 patients with intra‐abdominal DF treated at INT over the last 15 years, the incidence of a serious complication was 11%, in terms of bowel obstructions or perforations, which in 4 cases were the first evidence of disease. A surgical treatment was feasible and successful in 5 cases, while a non‐surgical approach was used in 3, being successful in two, the other patient dying due to a concomitant progressive lymphoma thereafter.In this retrospective case series analysis of exclusively intra‐abdominal DF patients treated at a reference Center over 15 years, the number of serious complications was low, but not negligible, especially in comparison to extra‐abdominal DF and abdominal wall DF. Clearly, intra‐abdominal DF is a rare condition, so that one can speculate even on such a limited number of cases. Indeed, the incidence was 11% of all patients, but 16% when only mesenteric DF is considered. One should be aware that DF is a non‐metastasizing disease, with an overall very good prognosis in terms of life expectancy. Even in this series, none of our patients died to the complication .The importance of complications in intra‐abdominal DF is due to the increasingly widespread conservative approach to DF in general. Up to some years ago, surgery was viewed as standard treatment in DF. Today, front‐line surgery is generally not of choice, given the high risk of local recurrences even after adequate resections and the high rate of functional sequelae at several sites.Our approach was non‐surgical in three patients, because any surgery was deemed to be unfeasible. All three patients experienced acute complications during chemotherapy, as a consequence of progressive disease. Intensive supportive treatment was administered, along with antibiotic therapy, and a careful change of chemotherapy regimen was established soon after the stabilization of the acute condition. The outcome was favorable in two patients, the other dying of a concomitant non‐Hodgkin's lymphoma after 8 months. A good tumor response was achieved with an anthracyline‐based chemotherapy in one of them (after progression to low‐dose chemotherapy) and vice versa with low‐dose chemotherapy in the other (after progression to anthracyclines). Both regimens are known to be active in DF.Non‐surgical policies in DF basically mean active surveillance or medical therapy. Active surveillance is based on the reported number of long‐term disease stabilizations, and even spontaneous regressions, without any treatment.In conclusion, a policy of active surveillance is tenable also in abdominal DF, as in DF in general, but the clinician needs to be aware that serious complications are relatively more common. When surgery is not resorted to even at the time of any such complications, intensive supportive care and an accurate choice of chemotherapy may well be resorted to.The following represents disclosure information provided by authors of this manuscript. All relationships are considered compensated unless otherwise noted. Relationships are self‐held unless noted.F.B., M.F., A.O., C.C., M.V., G.C., C.M.: No competing interests to declare.S.P., R.B., E.P.: Institutional research funding Amgen Dompé, Bayer, GlaxoSmithKline, Novartis, Pfizer, PharmaMar, Eisai, Eli Lilly, Advenchen Lab, AROG Pharmac, Blueprint Medicines, Deciphera, Epizyme, Karyopharm Pharmac, Daiichi Sankyo.A.G.: Honoraria from Novartis, Pfizer, Eli Lilly, PharmaMar; consulting or advisory role for Novartis, Bayer, Eli Lilly, Pfizer, Nanobiotix, PharmaMar, SpringWorks; research funding from PharmaMar (Inst), PharmaMar, Nanobiotix.P.G.C.: Consulting or advisory Role for Bayer, Deciphera; speakers' bureau for Pfizer; institutional research funding from Amgen Dompé, Bayer, GlaxoSmithKline, Novartis, Pfizer, PharmaMar, Eisai, Eli Lilly, Advenchen Lab, AROG Pharmac, Blueprint Medicines, Deciphera, Epizyme, Karyopharm Pharmac, Daiichi Sankyo.Conceptualization, E.P. and F.B.; Methodology, E.P.; Investigation, E.P., A.O., and F.B.; Formal Analysis, E.P. and F.B.; Resources, M.F., G.G., and C.M.; Writing ‐ Original Draft, E.P., F. B.; Writing ‐ Review & Editing, F.B., E.P., S.P., R.B., C.C., M.V., A.G., and P.G.B.; Supervision, P.G.C. The corresponding author attests that all listed authors meet authorship criteria and that no others meeting criteria have been omitted.All authors had full access to the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. This study was approved by the Ethical Committee of IRCCS Istituto Nazionale dei Tumori (reference number INT 191/20). All participants provided written informed consent. This study was performed in accordance with the Declaration of Helsinki.


Hemorrhagic lesions in CNS lymphoma are extremely rare. We report the case of a 75-year-old patient admitted to the emergency room following a classic hemorrhagic stroke. The CT scan showed a hyperdense tumor-like process with perilesional edema, the diagnosis reinforced by (MRI). The patient underwent macroscopically total surgical excision and the anatomo pathological examination concluded a diffuse non-Hodgkin's B large cell lymphoma. The follow up was marked by a clear clinical improvement. Primary cerebral lymphomas can be polymorphic, so this diagnosis should always be kept in mind during stroke manifestations. This case illustrates the diagnostic difficulty of this rare and poor prognosis condition. •Primary central nervous system (CNS) lymphoma is relatively rare, representing only 1%–2% of all primary CNS malignancies. Hemorrhagic lesions in CNS lymphoma are extremely rare.•This paper presents a case of A 75-year-old man admitted to the emergency room following a classic hemorrhagic stroke The CT scan showed a hyperdense tumor-like process with perilesional lesional edema, the diagnosis reinforced by (MRI). The patient underwent macroscopically total surgical excision and the anatomopathological examination concluded a diffuse non-Hodgkin's B large cell lymphoma. The follow up was marked by a clear clinical improvement.•The clinical expression of primary cerebral lymphomas can be polymorphic, so this diagnosis should always be kept in mind during stroke manifestations. This case illustrates the diagnostic difficulty of this rare and poor prognosis condition. Hemorrhagic lesions in CNS lymphoma are extremely rare  or in a glass sample .Rubenstein et al. introduced an interesting immuno-histochemical result consisting of higher VEGF immunor-eactivity in the hemorrhagic case compared with 4 other primary CNS lymphomas that did not show hemorrhagic evidence .On VEGF and hemorrhage in brain tumors, Arita et al. investigated the relationship between intratumoral hemorrhage and overexpression of VEGF in pituitaryadenomas, and they reported the positive relationship between VEGF expression and intratumoral hemorrhage in pituitary adenomas. Although the pathologies are different, their results on CD34 were similar to ours, and they also concluded that the amount of vascularity had no effect on hemorrhage . We receliterature review, it is possible that hemorrhage of primary CNS lymphoma is related to VEGF activity .Surgery has only a diagnostic role in this highly infiltrative, chemo and radiosensitive pathology. The therapeutic problems of PCL are multiple. In addition to the tumor-specific biological characteristics which seem to confer a poor prognosis to PCL in large B-cell NHL, the bioavailability of chemotherapeutics in the CNS is hampered by the BBB characterized by tight intercellular junctions, high Pgp expression and numerous cellular efflux mechanisms . In addiAlthough vascular manifestations are exceptional in PCL, this diagnosis must be suspected in front of recurrent neurological episodes of undetermined mechanism .4The clinical expression of primary cerebral lymphomas can be polymorphic, so this diagnosis should always be kept in mind during stroke manifestations. This case illustrates the diagnostic difficulty of this rare and poor prognosis condition.Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient.Ethical approval has been exempted by our institution.None.Oufaa JAMAL: writing the paper.Marouane MAKHCHOUNE: writing the paper.Yassine TAHRIR: Corresponding author and writing the paper.Khadija EL GUETTABI: Correcting the paper.Abdelhakim LAKHDAR: Correcting the paper.None.MAKHCHOUNE MAROUANE.Provenance and peer review Not commissioned, externally peer-reviewed.The authors declare having no conflicts of interest for this article.


Dear Editor,Seizures induced by micturition are rarely reported in literature He was born from non-consanguineous parents by normal delivery after a normal pregnancy. No risk factors for epilepsy had been reported. Psychomotor developmental milestones were reached and were normal. No genitourinary or sexual dysfunctions had been reported.General and neurological examinations were unremarkable. In particular, the external genitals appeared morphologically normal.In addition, an outpatient urological evaluation, including ultrasonography of the lower urinary tract, performed a few days before the admission revealed no abnormalities.High-field 3 T MRI brain scan was normal. The boy underwent long term video-EEG monitoring, that showed rare interictal spiking over the left fronto-temporal head region and vertex, exclusively during NREM (N1-N2) sleep. Moreover, video-EEG clearly demonstrated that micturition quickly precipitated stereotyped ictal events: nine reflex seizures were induced by micturition and one reflex focal to bilateral tonic-clonic seizure, as well as eleven focal spontaneous seizure were recorded.Ictal clinical semiology of focal seizures consisted of an aura characterized by sudden genital pain followed by manipulation lasting few seconds, without impaired awareness. In the recorded focal to bilateral tonic-clonic seizure behavioural arrest, right head deviation and arm/truncal asymmetric tonic posturing (right > left) was also observed.Ictal EEG was characterized by the onset of rhythmical delta activity evolving to sharp waves located over the midline invovling the central-parietal and left fronto-temporal channels, with the recruiting rhythm evolving to a bilateral tonic-clonic seizure .Fig. 1ViBased on history and electro-clinical data, we diagnosed a focal epilepsy of unknown etiology with micturition-induced reflex seizures. The boy was started on carbamazepine, that was promptly discontinued after a few days due to an allergic skin rash. He was then switched to lacosamide, after which he remained seizure free for 3 months with a normal follow-up standard EEG.Reflex seizures triggered by micturition are very rare, and few cases have been previously reported We report a case with both focal micturition-induced and spontaneous seizures, as previously described in other reflex epilepsy reports Micturition is a complex physiological process that involves different hierarchically integrated anatomical central and peripheral structures, including cerebral cortex – in its mesial frontal, anterior cingulate and insular aspects -, brainstem and spinal cord To our knowledge, only ten cases with micturition reflex epilepsy have been reported so far.On the one hand, most of previous cases had similarities to the case we are reporting: unknown etiology, a midline-central epileptogenic zone, tonic seizures, and normal urinary tract function. On the other hand, our patient experienced not only reflex but also spontaneous seizures and did not show co-existing neurodevelopmental delay as often reported In conclusion, we reported this case to further describe the anatomic-electroclinical correlation of the epileptogenic networks’ hypothesis of this extremely uncommon epilepsy syndrome that is often of unknown etiology. We also aimed to emphasize the crucial role of video-EEG monitoring in the correct differential diagnosis of rarely encountered paroxysmal events, that must be differentiated from more‐common conditions including micturition syncope, urinary incontinence due to sphincter relaxation during generalized seizures, and the ictal urge to urinate in patients wtih nondominant temporal lobe epilepsy This work did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.


The lingual cyst with respiratory epithelium (LCRE) is a very rare congenital cyst of the tongue, floor of the mouth, pharynx, or hypopharynx with 21 cases reported in the literature [Differential diagnosis is very important for patients presenting with lingual cysts, as this may impact treatment and follow-up. The LCRE should be included in the different diagnosis of a dermoid cyst , teratoiIn order to differentiate the LCRE from other types of developmental cysts, Manor et al.  recommenrd week of embryonic development, the foregut divides into a ventral part, containing components of the endoderm that lead to the development of the laryngo-tracheo-bronchopulmonary tree, and a dorsal part that becomes the proximal gastrointestinal tract. During this time of differentiation, embryonal rests may be misplaced and entrapped in the pharyngeal arches (which contains the developing tongue), due to their proximity with the primitive foregut. These entrapped rests, which are pluripotential, can differentiate into respiratory epithelium and form a lingual cyst [The pathogenesis of LCRE is unknown, but it most likely represents a congenital abnormality that arises from a misplacement of undifferentiated cells of the ventral portion of the foregut in week 4 of embryonic development ,9. In thual cyst .per se consistent with the diagnosis of LCRE [We have recently treated a case of a 44-year-old male with a palpable, soft, tender mass occupying the entire width of the tongue, causing a mild restriction of tongue movement and elevation of the anterior floor of the mouth. Magnetic resonance imaging (MRI) showed a heterogeneously hyperintense cystic mass measuring 6 × 6 × 4 cm in size, located in the sublingual space . Histolo of LCRE .To date, 21 cases of LCRE have been reported in the literature  and one In conclusion, various well-established types of developmental cysts have been described in the tongue. The LCRE represents a distinct entity histologically characterized by the presence of respiratory tract epithelium, pseudostratified ciliated columnar and cuboidal, with the absence of any other structures within the cyst wall. These characteristics should always be considered as, due to its rarity, the LCRE is often overlooked with consequences on the treatment and prognosis of affected patients.


We report the case of a bile duct traumatic neuroma in a 76‐year‐old man who presented with obstructive jaundice one year after cholecystectomy. Despite the radiological examinations, the preoperative diagnosis was difficult. The patient underwent a biliary resection with choledoco‐duodenal anastomosis We report the case of a bile duct traumatic neuroma in a 76‐year‐old man who presented with obstructive jaundice one year after cholecystectomy. Despite the radiological examinations, the preoperative diagnosis was difficult. The patient underwent a biliary resection with choledoco‐duodenal anastomosis It consists of a disorganized proliferation of a cutted nerve end. This lesion can develop following any surgery, especially amputation. Cholecystectomy has been rarely implicated in its pathogenesisWe present a case of a bile duct traumatic neuroma occurring after cholecystectomy. The clinical presentation was a biliary stenosis whose benign or malignant nature could not be confirmed preoperatively.2A 76‐year‐old man, who underwent a laparoscopic cholecystectomy one year ago, presented for jaundice without abdominal pain or fever. Physical examination revealed a jaundice. Vital signs were stable. The abdomen was soft with no evidence of organomegaly. Laboratory tests showed bilirubin: 20 mg/l , alkaline phosphatase 524 U/L (1.9× ULN), and gamma‐GT 1001 U/L (18× ULN).Abdominal ultrasound showed a dilation of intra‐ and extrahepatic bile ducts. The common bile duct measured 12mm, and the main bile duct was not dilated. The transition zone was the site of a small hypoechoic nodule measuring 5 mm.A magnetic resonance cholangiopancreatography (MRCP) revealed Figure  a dilatiThe patient underwent a surgical resection of the main bile duct with a choledoco‐duodenal anastomosis.The postoperative course was favorable. He was discharged from hospital on the fifth postoperative day.Pathology revealed traumatic neuroma of the bile duct with positive S100 stains and without any evidence of malignancy Figure . At subs3Traumatic neuromas are benign tumors that can develop after nervous system trauma.The period between cholecystectomy and the onset of the neuromas varies from several months to 45 years.They may also present with a jaundice due to obstruction or with an intermittent right upper quadrant pain due to sympathetic nerves stimulation.Imaging shows dilatation of the biliary ducts. MRCP can reveal the bile duct stenosis.Endoscopic ultrasonography fine needle aspiration (EUS‐FNA) can be effective in the diagnosis of biliary stenosis (accuracy 68%–91%).Thus, despite all the modern imaging techniques, the certain preoperative diagnosis of traumatic neuroma is difficult.Neuroma formation affects nerves that are encased in Schwann cells. It has been suggested that it is caused by an increased levels of fibroblast growth factor and its receptor.Considering that cholangiocarcinoma is one of the differential diagnoses, an aggressive resection is recommended: extrahepatic bile duct resection with negative margins and periportal lymphadenectomy.Some authors recommend using intraoperative pathologic examination to exclude a malignant tumor and thus avoiding excessive surgery.4Traumatic neuroma should be considered in the differential diagnoses of patients with jaundice who have had a previous cholecystectomy. Surgical resection would be the recommended approach for diagnosis and cure given that cholangiocarcinoma is one of the differential diagnoses. Traumatic neuroma could also explain right upper quadrant pain in cholecystectomized patients.None declared.Dr Salwa Nechi was involved in the definition of intellectual content and prepared the manuscript. Dr Abdelwaheb Nakhli designed the concept, was involved in the definition of intellectual content, in the literature search and preparation of the manuscript. Dr Wiem Ben Hamida gathered the information and assisted in the preparation of the manuscript. Dr Amina Bani extracted imaging data and assisted in the preparation of the manuscript. Dr Amal Khsiba assisted in the preparation of the manuscript. Dr Asma Ben Mohamed contributed to the critical review of the manuscript. Dr Emna Chelbi contributed to the critical review of the manuscript. Dr Lamine Hamzaoui was involved in the definition of intellectual content and contributed to the critical review of the manuscript. Dr Hassan Touinsi revised the manuscript and acted as guarantor for the research.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.


Segmental arterial mediolysis (SAM) is a nonatherosclerotic, noninflammatory and nonimmune arteriopathy of unknown aetiology. We present the case of a 43-year-old male who presented to the emergency department with abdominal pain. A computed tomography of abdomen and pelvis showed a narrow, hypodense superior mesenteric artery after the origin, raising the possibility of thrombus or vasculitis. He was commenced on rivaroxaban and steroids. He subsequently presented with an acute abdomen in a collapsed state. Repeat imaging of his abdomen and pelvis revealed an ischaemic ileal segment and caecum. He required an emergency laparotomy with resection of the ischaemic segment and formation of a double-barrelled stoma. SAM is an important diagnosis for clinicians and radiologists to be aware of, given the risks of life-threatening haemorrhage and acute organ ischaemia. This is a commonly overlooked cause of abdominal pain, where an early diagnosis with lifestyle modifications may prevent disease progression and subsequent development of life-threatening complications. Segmental arterial mediolysis (SAM) was first described in the literature by Slavin in 1976 . SAM is A 43-year-old male presented to the emergency department with sudden onset epigastric pain, radiating to the umbilical region. The pain was associated with nausea and vomiting. At presentation, the patient was afebrile and hypertensive at 160/103 mmHg. On initial examination, the patient’s abdomen was soft with mild tenderness in the epigastric region. Bowel sounds were normal, and no masses or organomegaly were palpable. He had a 13-pack year history of smoking. An urgent CT abdomen and pelvis was ordered.The initial blood count and metabolic panel are shown in Our initial working diagnosis was vasculitis based on the findings from the CT angiography. Other differential diagnoses included mesenteric angina or a mesenteric thromboembolic event. Following discussion with the consultant rheumatologist and vascular teams, the patient was commenced on therapeutic low molecular weight heparin (LMWH) and intravenous (IV) steroids. A vasculitic screen was sent which was negative . The patHe represented within 1 week of discharge with severe abdominal pain, which was post-prandial in nature. During this visit, a repeat blood count, metabolic panel and lactic acid levels were all within the normal ranges. A repeat CT abdomen and pelvis was unchanged from his initial imaging. He was placed on bowel rest, commenced on total parental nutrition (TPN), IV unfractionated heparin and IV steroids and was accepted for transfer to the vascular and rheumatology specialities. At this time, our working diagnosis was mesenteric angina secondary to an SMA thrombosis/vasculitis.His imaging was discussed at the regional multi-disciplinary team meeting, where a diagnosis of SAM was made based on imaging findings and exclusion of other causes. The vascular team recommended medical management as his anatomy was not amenable to bypass surgery or endovascular repair. The patient was discharged on a tapering dose of steroids and LMWH with outpatient follow-up.in situ from the duodenum-jejunal flexure. The histology showed ischaemic necrosis suggestive of a low flow state with no evidence of thrombosis or vasculitis, confirming the diagnosis of SAM. His stoma was subsequently reversed 20 months post-emergency laparotomy, restoring intestinal continuity. Despite the anatomical concerns regarding the mesenteric blood flow, the patient encountered no post-operative complications.The following month the patient presented to a Model 4 hospital with an acute abdomen in a collapsed state secondary to septic shock. A repeat CT of his abdomen and pelvis, including CT angiogram, showed an ischaemic ileal segment and caecum. He was brought to theatre for a laparotomy, ileo-caecal resection and creation of a double-barrelled stoma (jejunum and ascending colon). Two-hundred centimetres of small bowel remained et al., reported that the most common abdominal artery associated with SAM was the SMA (53%), followed by the hepatic artery (45%), celiac artery (36%), renal artery (26%) and the splenic artery (25%) [We present the case of a 43-year-old male who presented to our ED with non-specific abdominal pain. This case highlights the difficulty in diagnosing SAM, and the importance of clinician awareness. The most common acute presentation is abdominal pain, as SAM frequently involves the splanchnic vessels but carotid and retroperitoneal arteries have also been reported . Skeik e the SMA 3%, follory (25%) .Histological diagnosis remains the gold standard to confirm the diagnosis of SAM. However, with the advancement in CT angiography and magnetic resonance angiography, more cases are being diagnosed based on a combination of imaging and clinical criteria . This haThe pathology of SAM consists of two phases: the initial insult, followed by the remodelling and restoration phase. The initial phase begins with mediolysis of the arterial media. Subsequently, a tear develops, which separates the outer media from the adventitia layer. This process can encompass a portion, or the entire circumference of an artery, and it has a segmental distribution which leaves normal arterial segments in between , 10. AftNo standardized guidelines exist at present for the management of patients with SAM due to the lack of randomized controlled trials. Lifestyle modification, control of hypertension and dyslipidaemia should be commenced to reduce the cardiovascular risk profile. Endovascular interventions are recommended as first-line treatment modality in patients with haemodynamic instability or evidence of end-organ ischaemia .The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.This research did not receive any specific grant from funding agencies in the public, commercial or not-for-profit sectors.All ethical standards were complied with while conducting this research.Informed consent was obtained for this paper. The manuscript is not under consideration elsewhere. The work is original. All authors have agreed to this submission, and all authors have seen, edited and approved the final version of this paper.


Accessory articulation of the transverse processes of the cervical vertebrae is an extremely rare congenital anomaly. We present two cases of accessory articulation of the transverse processes of the left C5/C6 and C6/C7 cervical vertebrae. The articulation at C5/C6 was found in a 34-year-old male following a mob assault, the C6/C7 accessory articulation occurred in a 28-year-old female involved in a high velocity motor-vehicle accident. Cervical spine fractures were suspected in both cases. Recognition of this variant anatomy and differentiation from a fracture is important especially in an acute trauma setting to prevent unnecessary immobilization and inappropriate specialist referral of these patients. Our search revealed only five previously reported cases in the literature mostly occurring at the C5/C6 level. This is only the second case ever described at the C6/C7 level. A 34-year-old male presented with multiple facial fractures following a mob-assault. Computerized Tomography (CT) scan was performed using a helical multi-detector 16-slice CT scanner (Phillips Brilliance Netherlands) to further assess the facial fractures and to exclude associated brain and cervical spine injuries. Reformatted images were obtained in the axial, coronal and sagittal planes. A left C5/C6 transverse process articulation was found incidentally.A 28-year-old female with no known medical co-morbidities presented to our emergency room following a high velocity motor-vehicle accident. She was an unrestrained driver with a Glasgow Coma Scale (GCS) of 14/15 who reported cervical spine tenderness. A CT scan was done in accordance with the Canadian CT head rules. A helical 16-slice multi-detector CT scanner (Phillips Brilliance Netherlands) was used with reformatted images obtained in the axial, coronal and sagittal planes. The patient was found to have mild traumatic brain injury with an uncommon left C6/C7 transverse process accessory articulation mimicking a fracture. No acute fractures or dislocations of the cervical spine were demonstrated.1 Our search revealed five previously reported cases in the literature that mainly occurred at the C5/C6 articulation.2–6 This is only the second case documented at the C6/C7 level.2Accessory articulation of the transverse processes of the cervical vertebrae is an extremely rare congenital anomaly that was first described in the 1960s.7 A separate ossification center may develop in the sixth fetal month from which the anterior costal transverse process arises. By 6 years of age, it fuses with the main ossification centres of the transverse process. The costal portion of the transverse process may enlarge and elongate the anterior tubercle, giving rise to an unusual variant, an accessory articulation of the transverse processes, which is a form of the more common supernumery cervical rib.2–4,6–9The anomaly can be explained through embryological developments in the cervical spine. Each cervical vertebra has three ossification centers: a centrum and two vertebral neural arches. The transverse processes are formed by the lateral extension of the centres of the neural arches .7 A sepa3 The elongation of the anterior tubercle with the accessory articulation was best demonstrated on CT with multi-planar reformations (Plain film radiographs were not indicated as part of the initial management but were performed retrospectively for academic reasons in case 2. The plain radiographs showed that there was a bony projection extending anterior to the vertebral bodies with a radiolucent line between the lateral masses of C6/C7  that corrmations  and volurmations . This anAccessory articulation of the cervical transverse processes is extremely rare, particularly at the C6/C7 level. In the acute trauma setting, both plain films and CT are of value, with CT being more superior at distinguishing this variant anatomy from fractures and osteo-degenerative changes. These normal variants should be kept in mind when reporting on CT for traumatic injuries to reduce unnecessary immobilization and inappropriate specialist referrals.Radiologists should be aware of the normal ossification of the cervical vertebrae. Each cervical vertebra has three ossification centres, the transverse processes are formed by the lateral extension of the centres of the neural arches.The costal portion of the transverse process may enlarge and elongate the anterior tubercle to articulate with the vertebrae below giving rise to an unusual variant which may simulate a fracture in an acute trauma setting.CT is superior to conventional radiographs with regards to anatomical delineation and is, therefore, the preferred choice at distinguishing variant anatomy from acute fractures.


Ecchordosis physaliphora is a rare congenital benign hamartomatous lesion originating from nodal cord remnants. This is histopathologically indistinguishable from chordoma, and hence imaging plays a key role in diagnosis. These lesions are hypointense on T1-weighted and hyperintense on T2-weighted images, and follow CSF signal. In contrast to chordoma, Ecchordosis Physaliphora does not demonstrate contrast enhancement. Here, we present a case of 32–year-old male with no prior medical history, who presented to an outside facility for chronic headache workup and incidentally detected indeterminate lytic defect in the bony clivus with a well demarcated smoothly corticated margin. Further assessment with MRI brain showed findings characteristic of Ecchordosis physaliphora, a benign congenital hamartomatous lesion originating from nodal cord remnants requiring no additional follow-up imaging or intervention. Ecchordosis physaliphora is a rare congenital benign hamartomatous lesion, found in approximately 2% of autopsies ,2. It orEcchordosis physaliphora is an imaging diagnosis, as it is histopathologically indistinguishable from chordoma . CT is nHere we present a case of Ecchordosis physaliphora with classic imaging characteristics along with differential considerations for midline middle cranial fossa masses, to include neoplastic and non-neoplastic lesions.The patient is a 32 –year-old male with no prior medical history, who presented to an outside facility for chronic headache workup. On presentation, he was afebrile and had stable vital signs. Initial laboratory workup is unremarkable. CT head without intravenous contrast was performed , and shoMRI brain with and without contrast was performed at our facility . This deGerman pathologist Hubert von Luschka first described the finding of pathologic ectopic notochordal tissue at the posterior clivus in 1856 .Neoplastic lesions in the central skull base include metastasis, myeloma and lymphoma originating from the clivus; invasive pituitary macroadenoma; meningioma, metastatic disease, diffuse leptomeningeal glioneuronal tumor and lymphoma originating from the pachymeninges; chordoma and Ecchordosis physaliphora originating from notochord remnants; nasopharyngeal carcinoma (direct invasion from nasopharynx) and chondrosarcoma . Additionally, nonneoplastic primary osteofibrous lesions in this region include fibrous dysplasia, ossifying fibroma, Paget's disease and Langerhans' cell histiocytosis .The central skull base also houses some anatomic variants, which also do not require any intervention or imaging follow-up. These include craniopharyngeal canal, canalis basilaris medianus, fossa navicularis magna, arrested sphenoid pneumatization, asymmetric pneumatization petrous apex, aberrant internal carotid artery, fibrous dysplasia and cephalocele .Ecchordosis physaliphora can be distinguished from its pathologic counterpart chordoma based on its imaging presentation, including the presence of CSF attenuation and the absence of contrast enhancement or osseous destruction. In contrast, chordoma typically presents as a lobulated extra-axial hyperdense soft tissue mass with osseous destruction, with or without internal calcification and mass effect on the brainstem (resulting in characteristic thumb sign). Additionally, chordoma demonstrates heterogenous increased T2 signal, and shows a variable enhancement pattern. ,7No patient identifiers are disclosed in the current report.


An extremely rare extrapelvic position of endometriosis with a precise incidence of 0.07%–0.47%, leading usually to delayed and false diagnosis. Differential diagnosis should include that rare condition while ultrasonography remains a pivotal tool to unravel that enigma, especially in women with no specific symptoms and surgeries in the past. An extremely rare extrapelvic position of endometriosis with a precise incidence of 0.07%–0.47%, leading usually to delayed and false diagnosis. Differential diagnosis should include that rare condition while ultrasonography remains a pivotal tool to unravel that enigma, especially in women with no specific symptoms and surgeries in the past. Differential diagnosis should include that rare condition and ultrasonography remains a pivotal tool to unravel that enigma as well.A 34 years old woman was presented 4 years ago to our facility complaining for pain due to a palpable nodule in the abdominal wall of previous cesarean sections. No other symptom was reported. She had a personal history of two cesarean sections (2007 and 2012), and a laparoscopic surgery for ruptured ovarian cyst in 2013. Clinical examination revealed a palpable lesion in the left abdominal side and precisely into the abdominal wall. Vaginal examination and laboratory examinations were all normal. Transabdominal and translabial ultrasonography were performed (Figures The authors declare that they have no competing interest.SS made substantial contribution to acquisition of data. SS, ED, and IP made substantial contribution to conception, analyzing, and drafting the manuscript. SS contributed in analyzing data and revising the manuscript. IP contributed in acquisition of the data. ED: contributed in analyzing data. PD revised the manuscript. PD and AR agreed to be accountable for all aspects of the work. PD and AR gave final approval of the version to be published.Patient consent has been collected. The Ethics Committee of the Hospital has approved this Clinical Image.The authors have confirmed during submission that patient consent has been signed and collected in accordance with the journal's patient consent policy.


To report a unique presentation of hemangiopericytoma and discuss the clinical course, pathological features, and management of this tumor.An otherwise healthy 54-year-old Caucasian female presented with a painless conjunctival mass. The lesion gradually enlarged over a three-week period and was unresponsive to corticosteroid treatment. The mass was surgically removed, and histopathologic findings were consistent with hemangiopericytoma.Conjunctival hemangiopericytoma should be considered in patients with conjunctival lesions unresponsive to medical management. Surgical excision is diagnostic and therapeutic and is the strongest predictor of clinical course. Incompletely excised lesions are at a greater risk of local recurrence and subsequent metastasis. Given the neoplasm's malignant potential, patients should be followed in the outpatient setting. PrednisMicroscopic examination revealed proliferative spindle-shaped cells with vesiculated, fusiform nuclei and prominent nucleoli arranged in sheets with associated vascular channels, some of which demonstrating a staghorn pattern highlighted with immunohistochemical stains for CD 31 A. Reticu3Hemangiopericytoma is a rare, vascular tumor originating from the pericytes surrounding capillaries.4,Hemangiopericytomas are solitary neoplasms that demonstrate a haphazard arrangement of spindle-shaped cells outside the endothelial cell basement membrane.,,.Conjunctival hemangiopericytomas present similarly to hemangiopericytomas that originate elsewhere in the body.To our knowledge, there are no documented cases of conjunctival hemangiopericytomas demonstrating malignant potential.Total local excision is the recommended initial treatment modality. Surgical considerations include ensuring complete excision of any capsule, if present, and maintaining hemostasis. Given the propensity of these lesions to recur, patients should have frequent follow-up. Additional surgery is recommended for local recurrence. Postoperative radiation therapy has been used in conjunction with surgery in orbital tumors, but there is no consensus on its use for conjunctival hemangiopericytomas.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.No funding or grant support.All authors attest that they meet the current ICMJE criteria for Authorship.The following authors have no financial disclosures: WE, CC, JRT, HG, JW.


Concha bullosa (CB) is a pneumatized air cell within the nasal turbinates, often in the middle turbinate. CB is one of the most common anatomical variations found in the middle turbinate. It generally tends to be asymptomatic but can cause symptoms such as nasal obstruction, facial pain, and a decrease in the sense of smell. Moreover, CB can be involved in chronic rhinosinusitis, which can lead to its confusion with other lesions, as the radiographic picture might mimic other nasal pathologies. Here, we report a case of massive CB involved with allergic fungal sinusitis (AFS) and mimicking a bony tumor in order to highlight the diagnostic challenge of this clinical entity. Concha bullosa (CB) is the most common anatomical variation of the middle turbinate. It is defined as pneumatization of the middle turbinate, and its incidence varies from 14% to 53%. CB can be unilateral or bilateral. CB is commonly asymptomatic and is usually an incidental finding on computed tomography (CT). However, CB may cause symptoms such as nasal obstruction, facial pain, and a decrease in the sense of smell . TypicalA healthy 20-year-old female came to our clinic with a complaint of progressive, continuous, and long-standing history of bilateral nasal obstruction that was worse on the left side. She was having frequent thick mucoid nasal secretions, left-sided facial pain, and intermittent hyposmia, also it was associated with moderate to severe allergic rhinitis symptoms; she had no visual disturbances. The patient had already tried intranasal corticosteroids with no satisfactory improvement. A nasal endoscopic exam revealed a deviated nasal septum to the right side, a mildly hypertrophied right inferior turbinate, and a large mass occupying the left nasal cavity. This mass had displaced the nasal septum to the contralateral side and compressed the left inferior turbinate. Using the freer and fraiser suction, the mass was found to be hard but with normal overlying mucosa Figure .It originated from the skull base on the endoscope, with small nasal polyps noted beside it, but a further passage to the nasal cavity was not possible. The rest of the clinical exam, including her cranial nerves and orbital exam, was unremarkable. The patient came to our clinic for a second opinion, as she was concerned about the results of her medical imaging, which included CT scans and magnetic resonance imaging (MRI) of the paranasal sinuses. These reports commented on a bony nasal lesion measuring 1.7 × 3.7 × 3.0 cm and arising from the left skull base. The lesion was obstructing all ipsilateral paranasal sinuses with complete opacification and displacing the nasal septum to the contralateral side. Fortunately, there was no intracranial or orbital extension. No comments were made regarding heterogeneous opacification. These findings were thought to be an aneurysmal bone cyst, mucocele, mucopyocele, or other sinonasal tumors. However, upon careful study of the imaging, AFS was considered due to the heterogeneous nature of the content, the bony expansion of the lesion in the CT scans, the low signal intensity on T2-weighted images, and the heterogeneous high signals on T1-weighted images Figures .She was then assured and counseled for surgery. The patient had a routine and unremarkable preoperative assessment and then underwent functional endoscopic sinus surgery, polypectomy, and septoturbinoplasty (resecting whole middle turbinate) under general anesthesia. Intraoperatively, the mass was found to be a huge CB of the left middle turbinate that was filled with debris. Biopsies were taken from its mucosa and debris for histopathological staining and regular culture. It was resected, and all ipsilateral sinuses were opened and drained. The skull base and orbital wall were intact, and the surgery was uneventful Figure . The patient was given a tapered dose of oral steroids over 15 days and had an uneventful post-operative course. The histopathologic examination revealed amorphous debris with focal calcification and inflammatory cells with no evidence of malignancy. The material sent for fungal stain was reported positive. The intraoperative nasal swab result showed no pathogenic bacteria isolated after 48 hours of incubation at 37°C. In the subsequent postoperative visits, the patient remained asymptomatic, and the endoscopic exam showed a patent nasal airway, healthy mucosa, and no signs of recurrence.Bolger et al. classified CB according to its anatomical location: lamellar when it involves the vertical lamella and bulbous when it involves the inferior bulbous. If the pneumatization involves the whole concha, it is described as extensive . As noteFungal rhinosinusitis (FRS) is generally classified into invasive and non-invasive, according to the mucosal layer invasion. AFS is considered to be a non-invasive type of FRS. AFS is defined largely by the presence of basal polyposis with allergic mucin, that is, thick, tenacious, and eosinophilic secretions with characteristic histologic findings. The endoscopic finding associated with AFS is nasal polyposis, which generally affects multiple sinuses with a unilateral predominance. Furthermore, radiological features found in AFS on CT have been described as intrasinus serpiginous areas of increased attenuation causing heterogeneous images with bony expansion and thinning. Additionally, MRIs have demonstrated very hypointense regions surrounded by mucosal inflammation ,9. Bent and Kuhn established the standard criteria for the diagnosis of AFS. These criteria are based on clinical, radiological, immunological, and histological characteristics of the disease, including 1) type I hypersensitivity confirmed by history, skin tests, or serology; 2) nasal polyposis; 3) characteristic CT features; 4) eosinophilic mucus without fungal invasion; 5) positive fungal stain of sinus contents removed intraoperatively . The curIn this case, the main cause of confusion was the radiological readings, which were misinterpreted as other nasal lesions. Thus, the possibility of AFS involvement was not considered due to the lack of experience in this area. We would like to highlight the importance of clinicians carefully reading the images provided and combining them with clinical knowledge of differential diagnoses to avoid such confusion.This case report highlights the importance of including the diagnosis of AFS in CB. Radiological modalities can show bony expansions that may be confused with neoplasm of the nasal cavity. Endoscopic surgical treatment can confirm the diagnosis of AFS and is usually curative.