The most common tender cutaneous neoplasm is a dermatofibroma. The characteristics of three women (35 to 42 years old) with painful dermatofibromas are described. One woman was receiving immunosuppressive therapy for the past decade following a liver transplant; the other women were healthy. The dermatofibroma was located on the buttock, shoulder, and arm, respectively; tumor-related pain had been present for several months to at least a year. The dermal nodules ranged in diameter from 5 millimeters to 12 millimeters and were either flesh-colored to slightly red or brown or tan; one tumor was surrounded by a hyperpigmented patch. Microscopic examination showed the following dermatofibroma variants: classic (fibrocollagenous) in two women and histiocytic in one woman. All the women experienced resolution, without recurrence, of pain following the punch biopsy that only removed the majority  of the dermatofibroma. Tender cutaneous neoplasms include not only dermatofibromas and other fibrous lesions, but also adipose, bone, calcium, cartilage, eccrine, infiltrative, lymphoproliferative, muscle, neural, and vascular tumors. Acronyms and acrostics are mnemonic devices that have been used by clinicians to aid in recalling the diagnoses associated with painful skin tumors. When there were only 11 or less number of pain-related cutaneous conditions, shorter acronyms associated with either a woman’s name, a country or an egg were used. A unique acrostic inspired by Charlotte’s Web, a children’s book by E. B. White, was created when the differential diagnosis of tender cutaneous neoplasms expanded to 25 tumors. The sequential discovery of additional tender skin lesions resulted in two subsequent minor revisions to the original, hog-related, mnemonic. Herein, a new acrostic -- that is not only able to incorporate the inspiration from Charlotte’s Web, but also includes cutaneous lymphoma and a final category of “everything else” in order to maintain the future integrity of mnemonic -- for the painful tumors of the skin is proposed: HOG FLED PEN AND GETS CALM LIFE BACK . Painful tumors of the skin include a variety of benign and malignant neoplasms. They include adipose, bone, calcium, cartilage, eccrine, fibrous, infiltrative, lymphoproliferative, muscle, neural, and vascular tumors. Mnemonic devices -- such as acronyms and acrostics -- have been created to aid clinicians in remembering the differential diagnosis of painful skin tumors -8.Dermatofibroma is a common benign cutaneous neoplasm. A solitary tumor may be the consequence of an insect bite at the site of the lesion whereas some of the individuals with multiple lesions may have an acquired condition that alters their immunity or be a recipient of treatment with an immunosuppressive agent or both. Most dermatofibromas present as asymptomatic dermal papules or nodules; however, albeit less frequently, the tumor may be painful -15.The clinical features of three women, each of whom developed a painful dermatofibroma, are described. In addition, the characteristics of tender dermatofibromas that have been reported in other individuals are summarized. Also, previous and current mnemonics for tender cutaneous lesions are reviewed and a new acrostic for painful tumors of the skin is proposed.Case oneA 42-year-old Philippine woman presented with a tender lesion on her left buttock of approximately one-year duration. It had begun as a small dark spot and had continued to slowly enlarge. She had no previous skin cancers or dermatologic conditions.A complete cutaneous examination was done. A protuberant, painful, brown 12 x 12-millimeter dermal nodule surrounded by a hyperpigmented 3 x 2-centimeter patch was observed on the lateral and inferior area of her left buttock Figures -1C. A thMicroscopic evaluation of the tissue specimen showed a benign spindle cell tumor, consisting of a predominance of collagen and fibroblasts in a whorled arrangement, extending from the deeper portion of the papillary dermis into the reticular dermis and extending to the lateral margin of the specimen; compressed collagen bundles, having a keloidal appearance, were noted at the periphery of the neoplasm. A grenz zone of the normal-appearing papillary dermis was noted between the underlying dermal tumor and the overlying epidermis. The epidermis showed acanthosis and orthokeratosis Figures -2D.The correlation of the clinical presentation and the pathology findings established the diagnosis of a painful dermatofibroma of the classic variant; this tumor has also been referred to as a fibrocollagenous dermatofibroma. Most of the benign dermal neoplasm had been removed during the biopsy; therefore, the residual tumor and surrounding hyperpigmentation were observed. The dermatofibroma-associated pain completely resolved after the biopsy site had healed and there has been no recurrence of pain at the site.Case twoA 37-year-old Hispanic woman presented with a painful lesion on her left shoulder of several months duration. It had begun as a small tan patch that had grown to its current size and become painful. She also had warts on her feet.Her medical history was significant for a liver transplant 10 years earlier and episodic migraine headaches. Her transplant-related medications included 1,000 milligrams daily of mycophenolate mofetil and two milligrams twice daily of tacrolimus. She would take 50 milligrams of sumatriptan when she began to develop a headache. Her plantar warts were being treated topically by applying 40% salicylic acid pads daily.A complete cutaneous examination was done. A slightly raised, painful, tan 5 x 5-millimeter dermal nodule was noted on the left shoulder proximal to her arm Figures , 3B; dimTwo benign-appearing brown patches were noted on the left shoulder  and left chest; clinically the lesions were consistent with junctional nevi. In addition, there was a healed scar on her left chest above the breast. Also, several verrucous papules were present on the great toe and plantar surface of her left foot; these were plantar verruca.Microscopic evaluation of the tissue specimen from the left shoulder showed a non-circumscribed, benign-appearing, fibrocellular tumor in the reticular dermis. The tumor consisted of intertwining bands of collagen interspersed with predominantly fibroblasts and occasional histiocytes. The epidermis was acanthotic and its basal layer was hyperpigmented.The correlation of the clinical presentation and the pathology findings established the diagnosis of a painful dermatofibroma of the classic variant. Almost all the dermal tumors had been removed during the biopsy and the site of the residual dermatofibroma was clinically observed. The pain previously associated with the dermatofibroma did not persist after the biopsy and did not recur.Case threeA 35-year-old Caucasian woman presented with a painful mass on her left upper extremity of at least 12 months duration. It had begun as a small bump and became tender as it grew. The patient was healthy and had no other skin conditions.A complete cutaneous examination was done. A prominent, tender, flesh-colored to slightly red 8 x 8-millimeter dermal nodule was observed on the left extensor mid-arm Figures , 4B. A tMicroscopic evaluation of the tissue specimen showed a dermal tumor, consisting of numerous histiocytes and some fibroblasts that extended to the lateral margin of the specimen; within the benign neoplasm, there was hemosiderin and phagocytosis of lipid by the histiocytes. There was acanthosis of the overlying epidermis.The correlation between the clinical presentation and the pathology findings established the diagnosis of a painful dermatofibroma of the histiocytic variant. The biopsy site healed; the site and residual tumor were clinically observed. The dermatofibroma-associated pain resolved without recurrence.Several neoplasms are included in the differential diagnosis of painful dermal tumors Table  1-6]. T-6. T1-6]Dermatofibroma, also referred to as a fibrous histiocytoma, maybe a painful dermal tumor. However, this benign neoplasm was not included in the manuscript that summarized tender cutaneous lesions by Thompson in 1985 Table  [1-8]. SSeveral clinical and pathologic variants of dermatofibroma have been described ,10. A deMicroscopic examination of a dermatofibroma shows a dermal tumor consisting of benign fibroblasts and/or histiocytes. Compression and thickening of the collagen at the periphery of the tumor may be present creating keloidal-appearing collagen bundles. The overlying epidermis may demonstrate hyperkeratosis , acanthosis (appearing as thickening of the epithelium), and basal layer hyperpigmentation . In addition, some of the tumors have a Grenz zone of normal-appearing papillary dermis between the epidermis above and the dermal neoplasm below ,10.Dermatofibroma researchers have observed that some of the tumors were symptomatic. In a retrospective study of 122 biopsy-confirmed specimens of dermatofibromas from 92 patients, nearly 60% of the lesions were asymptomatic; however, 20.5% (25 tumors) were tender, 11.5% (14 tumors) were painful, and 9.8% (12 tumors) were pruritic . AlthougAneurysmal dermatofibroma is a unique variant of this neoplasm with distinctive clinical presentation and pathologic features. Morphologically, they can be larger, cystic, and appear blue, black, or dark red; in addition, they can be associated with rapid growth and pain. Histologically, in addition to fibrous solid areas, up to half of the tumor consists of large, blood-filled, histiocyte-lined tissue spaces containing hemosiderin pigment, fibroblasts, and foam cells ,12.Cruz and Kyriakos are credited for the initial description of aneurysmal dermatofibroma. Their study included 17 cases from 14 women and three men. The lesion was described as painful in five (29%) of the patients . SubsequAtrophic dermatofibroma is another variant of dermatofibroma with characteristic morphologic and histologic features. Clinically, the tumor presents as a solitary patch with a central umbilication; it is most commonly found on either the shoulder, leg, or back of women over age 48 years. Pathologically, it has similar changes observed in non-atrophic dermatofibromas; however, in the central atrophic portion of the tumor, the dermis is often at least 50% thinned and the elastic fibers are either decreased or absent in this area .Atrophic dermatofibroma has been reported in at least 102 individuals; two of these men had tumors that were transiently painful and demonstrated not only atrophic changes but also aneurysmal histologic characteristics. The first patient was a 27-year-old man who presented with an asymptomatic brown atrophic patch of two years duration on his back that had been temporarily painful and swollen one year earlier; a biopsy of the tumor was diagnosed as an aneurysmal dermatofibroma with atrophic features and the residual neoplasm was completely excised. The second patient was a 40-year-old man who had an asymptomatic nodular lesion of ten years duration on his left upper trunk that had become painful during the previous 12 hours; examination showed a five-centimeter hemorrhagic plaque that evolved into a hematoma during the subsequent week and then progressed into an asymptomatic depressed lesion two months later. The lesion was completely excised and demonstrated an atrophic dermatofibroma in the atrophic zone accompanied by aneurysmatic changes in the non-atrophic area .Single case reports of painful tumors in individuals with other variants of dermatofibroma have also been described. A 25-year-old woman with a deep penetrating dermatofibroma of her right foot presented with a slowly growing painful mass of two months duration that made walking difficult; the tumor was completely excised and the dermatofibroma-associated pain gradually subsided . AnotherThree patients, each with a painful dermatofibroma, are described in this report; their characteristics are summarized in Table In addition to dermatofibroma, there are many other tumors of the skin that are painful. Mnemonic devices have been used as memory aids to facilitate remembering the differential diagnosis of painful skin tumors Table  1-6]. A-6. A1-6]Earlier acronyms for painful skin tumors were limited to seven, nine, or 11 conditions ,3. HowevWhen these investigators subsequently encountered an individual with a painful skin lesion caused by a foreign body (solder) and the cutaneous reaction to that foreign body, they modified their original acrostic by adding a “something else” category . Yet, wiSince the most recently proposed acrostic, a thorough review of the literature has revealed another painful tumor of the skin: lymphoma. Shelley and Wood observed that pain was a prominent symptom associated with occult malignant lymphomas in the skin of 50% (three of six) of their patients . SubsequThus, lymphoma of the skin should be added to the list of differential diagnoses for tender cutaneous neoplasms. However, in an attempt to maintain the integrity of the acrostic for painful skin tumors, it is also reasonable to add an additional category (which would be designated “everything else”) for new painful cutaneous lesions that are subsequently discovered. Hence, after modification and word rearrangement of the most recent mnemonic device, a new acrostic for painful skin tumors that still maintains its inspiration from Charlotte’s Web is proposed: HOG FLED PEN AND GETS CALM LIFE BACK Table  1-6].-6.1-6].There are several benign and malignant tumors that can present as tender cutaneous neoplasms. Dermatofibroma is a commonly occurring benign tumor that usually appears as an asymptomatic dermal papule or nodule. However, approximately 30% of dermatofibromas have presented as a painful or tender lesions. Indeed, dermatofibromas are the most common diagnosis associated with a painful tumor of the skin. The characteristics of three women with symptomatic dermatofibromas are reported; a punch biopsy, which removed some but not all, of the painful dermal nodule, not only established the diagnosis of the neoplasm but also resulted in resolution of the dermatofibroma-associated pain without recurrence. In addition to dermatofibromas and other fibrous lesions, tender cutaneous neoplasms include tumors of adipose, bone, calcium, cartilage, eccrine, lymphoproliferative, infiltrative, muscle, neural, and vascular origin. Mnemonic devices have been created to aid clinicians in remembering the differential diagnosis of painful skin tumors. Shorter acronyms  were adequate when the number of possible pain-related cutaneous conditions was 11 or less. When the differential diagnosis of tender cutaneous neoplasms expanded to 25 tumors, an acrostic inspired by E. B. White’s children’s book Charlotte’s Web was created; subsequently, two minor revisions to the mnemonic were published for each additional tender skin lesion that was identified. With the addition of cutaneous lymphoma and a final category of “everything else” to maintain the integrity of mnemonic, a new acrostic for the painful tumors of the skin -- that is still able to incorporate the inspiration from Charlotte’s Web -- has been proposed: HOG FLED PEN AND GETS CALM LIFE BACK.


A urethral prolapse is a rare condition occurring most likely in prepubertal girls and post-menopausal women. The cause of this condition is not well known but an under-laying low level of estrogen is thought to have a role.This is an 8-year-old girl diagnosed with urethral prolapse, who was successfully managed by excision of the prolapsed urethral mucosa circumferentially. A three-month following up did not notice any particular challenge.The most common presenting sign is genital bleeding and the vaginal doughnut sign. Treatment of urethral prolapse should begin with medical therapy in most patients before resorting to surgical management. However, in case of bigger size, severe genital hemorrhage, and prolapsed mucosa with an appearance suggestive of vascular compromise, surgical management is the first-line option. We estimate a severe prolapse mimicking a uterine prolapse must be included in surgical management as a first-line option.Surgical excision may be the first-line option in certain urethral prolapses given its association with quick recovery versus the effectiveness of the long-time required for estrogen use, as well as the low likelihood of successful resolution linked to estrogen use. •Urethral prolapse is a rare condition.•A low estrogen level is thought to have a role.•Estrogen cream application is the treatment of choice.•Surgical management may be a first-line option in severe conditions such as one mimicking a uterine prolapse. There was no history of chronic cough or constipation.On examination, we found a bleeding and tender round doughnut-shaped mass (2 × 2 cm) protruding from the vagina, resembling a uterine cervix .Fig. 1PhA pelvic ultrasound scan was done and revealed a normal uterus at its usual location.A diagnosis of urethral prolapse was made, and the patient was taken to the theatre after her caretaker (her mother) was counseled about the diagnosis, the option management, and eventual complications. We got her informed consent. A preoperative assessment was made. Under general anesthesia, a urinary catheter was inserted into the bladder .Fig. 2PhPatient in lithotomy position, a circumferential excision of the prolapsed urethral mucosa was made followed by an interrupted hemostatic suturing using vicryl 4/0. There was no complication, and the blood loss was estimated to be less than 50 ml. The procedure lasted 12 min. The catheter was left in situ for 3 days before being discharged. After then she started applying Premarin cream, a conjugated estrogen cream, twice per day for 2 weeks. She had tender breast bulking and vulvar hyperpigmentation  secondarThree months later she was reviewed and was found asymptomatic.3The etiology of urethral prolapse is not yet well known and many theories are trying to explain its occurrence A congenital abnormalities theory has been made such abnormal urethral anatomy resulting in a weak attachment between the inner and outer smooth muscle layers of the urethra, a neuromuscular abnormality, and an elastic tissue deficiency We did not notice such conditions in our patient. As most cases occur in post-menopausal women and prepubertal girls, it is thought an under-laying low level of estrogen has a role The clinical diagnosis is not obvious, a high rate of misdiagnosis is observed Unlike in postmenopausal women, prepubertal girls are asymptomatic and the urethral prolapse is accidentally discovered, as a round, soft, and plain red-colored mass, donut-shaped from the vagina measuring 0.5 to several centimeters in diameter protruding from the distal urethra Genital hemorrhage is usually not profuse and urinary symptoms such as dysuria, urinary retention can be observed If there persist some doubts to make the diagnosis, a urinary catheter can be inserted into the bladder through the round mass to confirm that the meatus lay at the prolapsed mucosa.Treatment of urethral prolapse should begin with medical therapy in most patients We also thought applying conjugated estrogen cream could fasten wound healing and prevent recurrence in this young girl supposed with low estrogen levels: estrogens act on the cutaneous wound healing response by modulating the inflammatory response, cytokine expression, and matrix deposition Our young girl had a 2 cm urethral prolapse mimicking a uterus prolapse. We estimate a surgical excision was the better management option given the size of the prolapsed mucosa and the effectiveness of this option.4Surgical excision may be a first-line option given the effectiveness of the long-time required for estrogen, as well as the low likelihood of successful resolution, especially in case of thrombosed or strangulated prolapse, severe prolapse mimicking a uterus prolapse, and a high likelihood of recurrence and loss to follow up.Written informed consent was obtained from the caretaker of the patient for publication and accompanying images.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Not applicableNot applicable.John Kasereka Muteke.Not commissioned, externally peer-reviewed.JKM managed the patient, AA and JKM participated in the follow-up; JKM and FKS wrote the first draft. All co-authors read and approved the final manuscript.The authors declare that there is no conflict of interest in this article.


Teaching Point: Lymphomatous involvement of the skeletal muscle is rare; however, because lymphoma can be treated with chemotherapy, it is crucial to consider it in the differential diagnosis of a soft tissue mass in the appropriate clinical and imaging setting. A 37-year-old man presented to the emergency department with a one-month history of non-traumatic left knee pain and swelling. His previous medical history was irrelevant. Physical examination revealed a tumefaction on the left medial thigh. Laboratory tests were unremarkable.The patient underwent a knee radiograph that showed a lytic bone lesion in the femur (asterisk in Magnetic resonance imaging (MRI) revealed a large soft tissue mass infiltrating the thigh’s muscles . Contrast enhancement can be either homogeneous or heterogeneous [Imaging findings can be variable. Multiple muscles may be involved, and it can manifest as a diffuse or focal muscular enlargement, an infiltrative lesion, or a focal mass. In cases of tumor multifocality, involvement may be continuous or discontinuous. It may not be circumscribed by the muscle compartment and fascial planes .Distinguishing lymphoma from other neoplastic and non-neoplastic disorders can be challenging. Although relative cortical preservation in the context of a sizable extraosseous tumor is not specific, it is a useful finding for considering lymphoma in the differential diagnosis .


Cesarean scar defect (CSD) presents as a cystic defect that connects the uterine cavity at the site of the previous cesarean section (CS). Endometriosis refers to the discovery of endometrial glands and stroma outside the uterine cavity. Cases of endometriosis cysts at CSD have not been reported.In this article, we will present a patient with an endometriosis cyst at CSD with symptoms of a prolonged menstrual cycle, periods without cyclic abdominal pain, and a history of cesarean delivery. The gynecologic ultrasound showed a CSD and a mixed mass in the right front of the uterus. After about 1 month, the tumor grew from a diameter of 4.75 cm to 8.06 × 6.23 × 3.66 cm. The patient eventually had an operation, which revealed a mass protruding from the incision in the anterior uterine wall, which was attached to the anterior uterine wall by a thin tip with a smooth surface. Intraoperative rapid cytopathology suggested that endometrial glands were seen within the smooth muscle tissue, similar to endometriosis. Subsequently, the patient underwent resection of the endometriotic cyst. Final paraffin pathology showed smooth muscle with visible endometrial glands and old hemorrhage, and a one-year follow-up showed no recurrence of endometriosis cysts at CSD.Endometriosis cysts at CSD are very rare. The clinical symptoms may be less obvious, and the diagnosis relies mainly on the patient’s previous surgical history and imaging. A finding of a pelvic mass in the location of the CSD, with or without symptoms of menstrual changes and intermittent abdominal pain, should be considered an endometriotic cyst at CSD. Surgical treatment is a good choice for this disease. Further studies are needed regarding the etiological mechanism of this case and why the mass enlarged rapidly in one mouth. Morris first proposed cesarean scar defect (CSD) in 1995, also referred to as cesarean scar diverticulum, isthmocele, uterine scar defect, uterine diverticulum, uterine niche, and uterine scar defect or sacculation , 3. NotaThis article will present a patient with an endometriosis cyst at CSD, with symptoms of prolonged menstrual cycle and periods, without periodic abdominal pain, and a history of CS. To our knowledge, no such cases have been reported.A 40-year-old woman complained of menstrual irregularity lasting for 2 years, and the patient reported menstrual periods lasting 3 to 15 days and cycles of 15-60 days. The patient’s menstrual volume varies; at most, it can be 4 times the average. However, the patient has never had periodic abdominal pain. The patient does have a history of two previous CS, and the last CS occurred 12 years ago. No other history beyond the cesarean was noted, and there is no knowledge of any familial genetic disease.The gynecological ultrasound of the patient showed that the incision at the anterior wall of the uterus had a triangular defect with an accessible base of 0.76 cm and a height of 0.55 cm. A dark area with an inner diameter of about 4.75 cm could be seen in the pelvis, and a grid-like high echo could be seen in the dark area. Notably, Color Doppler ultrasound showed no significant abnormal blood flow signal Fig. A, B. AlpOne month later, the patient returned, and gynecological ultrasound revealed a mixed mass measuring approximately 8.06 × 6.23 × 3.66 cm in the right anterior part of the uterus, with hypoechoic, anechoic, and hyperechoic areas, closely associated with the uterine body incision. Furthermore, Color Doppler ultrasound suggested a slight enrichment of blood flow within the mass Fig. C, D. TheFinally, the patient underwent laparoscopic surgery. This procedure found a cystic mass at the incision of the anterior uterine wall with a thin tip attached to the anterior uterine wall Fig. E and senAny myometrium defect in the lower part of the uterus after uterine surgery may be considered CSD . Most ofThere is no definite conclusion about the occurrence of CSD, which is mainly related to poor wound healing , 5. SomeThe possible reason for the formation of an endometriosis cyst at CSD described in this paper is that during CS, the endometrium leaves the uterine cavity and reaches the plasma surface of the uterus. Under the action of hormones and growth factors, the ectopic endometrium affects the healing of the cesarean incision. The two interact with each other, thus forming the endometriosis cyst at CSD. In this article’s case of the endometriosis cyst at CSD, the diameter of the mass increases from 4 cm to 8 cm in 1 month. In addition to the mass enlargement of the ectopic endometrium under the periodic action of hormones, there may also be the influence of CSD. CSD has scar uterine incision myometrium discontinuity. Menstruation may pass through the defective uterine base, directly increasing the tumor’s size and increasing the tumor rapidly in a short time. Or the environment of CSD is more conducive to the growth of peculiar smell endometrium, and the activity of ectopic endometrium is high, leading to the above situation. Alternatively, the interaction of two factors may lead to the rapid growth of a tumor.In summary of the patient diagnosis and treatment, the diagnosis of endometriosis at CSD is mainly based on the patient’s previous history of one or more CSs, clinical manifestations, and imaging examinations to confirm the diagnosis.Most CSD patients are asymptomatic, but 28.9-82% will show abnormal and/or post-menstrual bleeding (AUB) . Lumps aThe auxiliary examination for endometriosis cysts at CSD mainly involves imaging examinations. Ultrasound, CT, and MRI can assist in diagnosing, but the final diagnosis requires histological diagnosis , 12. On There is no current gold-standard treatment for CSD, and common treatment modalities include hormonal medication, surgical repair, or even hysterectomy for definitive treatment . For CSDCurrent treatment of scar endometriosis includes pharmacological management and direct surgical excision therapy . NotablyEndometriotic cysts at CSDs are rare, and no similar cases have been reported. Additionally, the clinical symptoms of endometriotic cysts at CSDs may be less pronounced. The diagnosis relies heavily on the patient’s previous surgical history and imaging. Ultrasound and MRI are good imaging tests to aid in the diagnosis. Patients with a history of gynecologic or obstetric surgery, the presence or absence of menstrual changes and/or periodic abdominal pain, and the finding of a pelvic mass should be considered for endometriotic cysts at CSD. Surgical treatment seems to be a promising treatment option for the patient. It is also important to note that the mass may grow rapidly in the short term and show abnormal blood flow signals on gynecologic ultrasound. However, this does not imply malignancy. It is particularly regrettable that this article only describes the disease but does not provide an in-depth study of the causes of its formation and some specific symptoms. Therefore, studies are needed regarding the causes of endometriotic cysts at CSD, the rapid increase in the mass size within a month, and whether there is an interaction between CSD and endometriotic cysts.


Genital outflow tract obstruction due to cervical agenesis is an uncommon Mullerian duct anomaly, increasingly being treated with conservative surgery by creation of an outflow tract by drilling or coring into the cervical remnant or by uterovaginal anastomosis. A 19-year-old woman with cervical dysgenesis in the present case underwent a successful uterovaginal anastomosis to relieve the obstructive menstrual symptoms and preserve the future reproductive function. The neouterovaginal canal was created over a mold of Foley's catheter by anastomosis anterior surface of the uterine corpus to the vaginal vault, bypassing the dysgenetic cervix and using the fibrous band of cervix as support. Normal cyclical menses were restored. Steps of the procedure are detailed in this case report. Mullerian duct anomalies affect 1 to 4% of the general population, of which congenital cervical anomaly represents a rare variant.We present a case with cervical dysgenesis in a 19-year-old woman who was treated with surgical construction of a genital outflow tract by a novel uterovaginal anastomosis technique using the fibrous cervical band as a scaffold for neocervix.A 19-year-old sexually active woman with primary amenorrhea presented with cyclical pelvic pain for 3 years. On examination, secondary sexual characters were well developed; breasts and pubic hair were Tanner Stages 3 and 4, respectively. No mass was palpable on abdominal examination. External genitalia was normal female type with a proximally blind but otherwise patent vagina of 8 cm. A normal-sized uterus could be palpated on bimanual examination through the blind vagina; however, the cervix was not discernible. Outflow tract obstruction at the level of the cervix was therefore suspected clinically. Imaging showed a normal-sized uterus (7.7 × 5.1 × 3.3 cm) with a hypointense endometrial collection (1.8 × 2.6 × 2.6 cm) suggestive of hematometra and left-sided hematosalpinx. An 18-mm long cervix was identifiable on magnetic resonance imaging (MRI), but the endocervical canal was not visualized . VaginaSurgery was planned via abdominoperineal approach. Intraoperatively, the uterus was small and the cervix was palpable as a small fibrous raphe, confirming outflow obstruction at the level of the cervix. Left fallopian tube was dilated and tortuous with hemorrhagic content within. The right tube did not show evidence of any retrograde collection. Both ovaries were normal . It wasThe vesicouterine space was dissected to expose the lower end of the uterus, the rudimentary cervix, and the vault of the blind vagina. Level of vaginal incision to create vaginal opening of the uterovaginal canal was guided by an assistant from the vaginal end. A 2-cm transverse incision was then made at the uppermost portion of the vagina by the surgeon under guidance . AnteriConstruction of the neouterovaginal canal was started by first suturing the posterior flap of the vagina with the inferior margin of the uterine stoma using Polydioxanone monofilament suture (PDS II), which formed the posterior wall of the future uterovaginal canal with the fibrous raphe juxtaposed to it posteriorly . HorizoThe postoperative period was uneventful. Foley's catheter was kept in situ for 4 weeks. She was given cyclical oral contraceptive pills for 3 months. A review hysteroscopy was done 3 months after the initial procedure to evaluate the outflow tract. The anastomotic channel was found patent and cylindrical, simulating a normal cervical canal. Healthy endometrial tissue was noted in the cavity in the proliferative phase. The patient reported to have been relieved of cyclical abdominal pain and having had cyclical menstrual periods for the last 6 months till the time this case is reported.Understanding and delineating the exact anatomy of the Mullerian anomaly is the critical step before reconstruction or surgical correction of the genital tract is considered. An outflow obstruction at the level of cervix was evident in the present case on clinical examination. This was confirmed on MRI, which remains the most reliable investigation for diagnosis and surgical correlation of the defect morphology in Mullerian anomalies.The ESHRE/ESGE classification for the present case was U0C4V0, which symbolizes a normal uterus (U0), an aplastic cervix (C4), and a normal vagina (V0).Management of cervicovaginal agenesis has evolved over the decades and extirpative procedures such as hysterectomy as a treatment option have fallen out of favor. In presence of sufficient cervical stroma, creation of a neocervical canal by drilling or coring with the insertion of a stent with or without grafting to expedite epithelization has been attempted successfully to relieve the obstructive menstrual symptoms. Chakravarty et alCanal reconstruction by digging through the native cervical remanent, however, carries overall higher rates of complications including restenosis, need for repeated surgeries, ascending infections, and even fatal peritonitis with septic shock.Spatulated anastomosis, instead of end-to-end attachment, was performed in the present case as the size and thickness of the uterine and vaginal stomas were different. The vaginal opening was fabricated in opposite obliquity to that of the hysterotomy incision and was further spatulated by giving a longitudinal incision in the middle of flap perpendicular to its transverse edge . SpatulThe main goals of surgery in our patient were to create an outflow tract and establish normal menstruation, which were satisfactorily achieved. The restoration of the tract patency renders a future possibility of using assisted reproductive technologies to achieve pregnancy. Sequelae of retrograde menstruation and endometriosis were not severe in our patient, highlighting importance of early diagnosis and timely treatment of the obstruction.Early surgical correction with uterovaginal anastomosis should, therefore, represent the primary therapeutic option in patients with cervical agenesis or dysgenesis for most optimal outcomes.


Narcolepsy, a neurologic disorder that leads to excessive daytime sleepiness, may represent a rare consequence of neoplastic lesions involving the sellar/parasellar and hypothalamic regions, the anatomical areas responsible for wakefulness. Optic pathway gliomas represent the most common neoplasm of these regions and present an excellent overall survival, while long-term neurologic impairments, such as visual loss, endocrinopathies, or sleep disorders, are the principal causes of morbidity. In this case report, we describe a non-NF1 patient suffering from a very extensive optical pathway glioma, who several years after the diagnosis in a radiological condition of stable disease, presented with severe narcolepsy, a rare complication, that led to the death of the patient. Narcolepsy is a neurologic disorder characterized by excessive daytime sleepiness and sudden sleep attacks, associated with cataplexy and rapid eye movement (REM) sleep-related phenomena. It is a rare pediatric condition that is predominantly idiopathic, and in its secondary form can sometimes be associated with central nervous system (CNS) tumors. This occurrence is very rare, although the exact prevalence of excessive daytime sleepiness in pediatric neuro-oncology patients is difficult to assess; several case series of secondary narcolepsy have been published in adults, whereas fewer notions are available in children ,5,6,7,8.Tumors in the hypothalamic/pituitary region have been linked to the development of narcolepsy in childhood, and craniopharyngiomas, pituitary adenomas, and low-grade gliomas (LGG) are among the most common ,11,12.Optic pathway gliomas (OPGs) represent the LGG that can develop anywhere in the optic pathway, including one or both optic nerves, chiasm, or hypothalamus, but can extend to and infiltrate the adjacent brain structures ,14,15,16We describe the case of a 3-year-old patient affected by OPG and never treated with radiotherapy, who presented with a sudden state of drowsiness that evolved into narcolepsy. The rapid clinical deterioration occurred several years after the diagnosis and in a phase of radiological stability of the tumorA 3-year-old boy came to our attention for visual impairment, convergent strabismus of the left eye, and evidence of hypotrophy of the optic disc in the fundus. He had a history of seizures with onset at 18 months treated with sodium valproate.At physical examination, the patient was in adequate general condition and neurocognitive development, severe bilateral hypovision  highlighting a retinal nerve fiber layer thickness (RNFL) severe reduction, visual loss quantifiable with Lea figures in 2/10 in left eye and 4/10 in right eye) horizontal nystagmus, divergent strabismus of the left eye and poor pupillary reaction to photostimulation.café au lait spot was detected, but neurofibromatosis type 1 (NF1) was previously excluded by genetic analysis.A single A magnetic resonance imaging (MRI) of the brain and spinal cord documented the presence of an infiltrative, space-occupying lesion involving the optic-chiasmatic region .The patient underwent a biopsy  that allowed the diagnosis of ganglioglioma grade 1 in accordance with the latest edition of the World Health Organization (WHO) classification of central nervous system .Chemotherapy treatment was started according to the International Society of Paediatric Oncology-Low Grade Glioma (SIOP LGG) protocol .Almost one year after starting therapy, the patient began to show a clinical picture of cachexia, and a brain MRI showed disease progression . A new oThe possibility of hydrocephalus contributing to clinical deterioration was considered but MRI did not show any signs of raised intracranial pressure. Other systemic causes of somnolence such as electrolyte imbalance or anemia were excluded.The possibility of a surgical procedure was discussed at the tumor board and considered futile since the tumor had an infiltrative growth and imaging did not show any signs of intracranial hypertension.The excessive daytime sleepiness (EDS) associated with sleep paralysis and hypnopompic hallucinations (upon awakening) did raise the suspicion of secondary narcolepsy in differential diagnosis with autoimmune encephalitis.Autoimmune antibodies  were negative. A multi-sleep latency test (MSLT) performed after a video-polysomnography recording more than 6 h of nighttime sleep demonstrated the presence of excessive daytime sleepiness and a very short sleep onset latency (<2 min) with 2 SOREM (sleep onset REM), thus resembling a narcolepsy pattern. Treatment for narcolepsy was started with modafinil, which was only partially effective in decreasing daily somnolence and caused side effects such as tachycardia and psychomotor agitation.Pitolisant as an add-on was then tried, but it was associated with severe anxiety symptoms without benefit on the hypersomnolence; therefore, it was withdrawn.In the following months, the child developed a progressive and significant neurological decline with the appearance of continuous dyskinesias, psychosis, mutism, encopresis, enuresis, and secondary autism spectrum disorders. The neurological impairment led to the death of the child within a few months.Narcolepsy is defined as a debilitating condition of excessive daytime sleepiness and sudden attacks of sleep and can be accompanied by cataplexy, sleep paralysis, hallucinations, and disrupted nocturnal sleep ,25,26,27However, indirect evidence of an autoimmune pathogenesis of narcolepsy is provided by the observation that narcolepsy occasionally occurs in association with paraneoplastic syndromes and other autoimmune diseases, such as multiple sclerosis, coeliac disease and systemic lupus erythematosus . In our A genetic predisposition has also been described involving a specific HLA allele (HLA-DQB1*0602) found in 85–95% of patients with narcolepsy .Narcolepsy may rarely result from secondary causes such as inherited disorders, encephalitis, head trauma, stroke, vascular malformation, or CNS tumors . PatientMechanisms by which cancer disrupts circadian rhythm are still unclear. One mechanism hypothesized to achieve this is via DNA methylation of specific clock genes, impacting both the peripheral circadian system and the central circadian clock, potentially leading to sleep problems. The regulation of wakefulness, sleep, and circadian rhythm is preserved through neuropeptide-producing neuronal networks involving the thalamus, hypothalamus, basal forebrain, and brain stem. When the tumor is located in these areas due to its position and/or its expansion as well as to surgery or cranial irradiation, the sleep-wake (SW) regulatory systems can be affected, disturbing the signaling pathways ,32.Following the appearance of characteristic symptoms, which include excessive daytime sleepiness, sudden attacks of sleep or cataplexy, the suspicion of primary or secondary narcolepsy is based on the performance of nocturnal polysomnography (PSG) and the multiple sleep latency tests (MSLT), a test that measures the tendency and rapidity of falling asleep under standardized conditions ,11,33,34B receptors [The treatment of narcolepsy is the same regardless of its etiopathogenesis. It is based on the use of drug therapy targeting dopaminergic , dopaminergic, and noradrenergic  or histaminergic (pitolisant) pathways . Anothereceptors . These teceptors ,9,11. Aceceptors ,38. AssoPediatric series of narcolepsy in pediatric patients with brain tumors are few and the percentage of incidence of this neurological complication is different among the available series. This evidence is due to the difficulty in defining the exact diagnosis of this sleep disorder.In In another study, Mandrell et al. analyzed a population of 110 pediatric patients with the diagnosis of a craniopharyngioma; the patients completed a baseline sleep clinic evaluation performing a PSG and MSLT. Among patients who completed PSG and MSLT, a diagnosis of excessive daytime sleepiness was found in 80% of them.Particularly, hypersomnia was diagnosed in 45% and narcolepsy in 35%, with a higher percentage in overweight or obese patient groups .Craniopharyngiomas are slow-growing tumors of the sellar and parasellar region and may also involve the hypothalamus; they are the most common intracranial tumors of nonglial origin in the pediatric population, representing 6–9% of pediatric brain tumors ,39,40,41One of the hypothesized mechanisms leading to daytime sleepiness in craniopharyngioma patients is the decreased melatonin production, documented in patients with craniopharyngioma, and caused by the involvement of the hypothalamic suprachiasmatic nucleus (SCN), a key component of the sleep-wake regulatory system and regulation of circadian rhythm. Indeed, it directs the diurnal secretion of melatonin from the pineal gland in response to the environmental light/dark cycle, and the melatonin itself is involved in sleep promotion and regulation of circadian rhythms ,43. MoreWeil et al. described the case of an 11-year-old girl diagnosed with a large sellar and suprasellar germinoma, in which narcolepsy led to the diagnosis and thus subsequently disappeared during treatments . In a fuTachibana et al. presented a case of an 11-year-old girl who developed narcolepsy following surgical removal of a craniopharyngioma with almost total removal of the hypothalamus that was seen on MRI, confirming the aforementioned hypothesis. Beyond the craniopharyngioma, LGGs are the most common CNS tumors in childhood found in the hypothalamic/chiasmatic region; LGGs account for approximately one-third of all SNC tumors ,49,50,51OPGs account for approximately 3–5% of childhood CNS tumors and constitute a subtype of LGG that generally cannot undergo complete surgical resection. They can be bilateral, potentially arising anywhere along the optic pathway, although about 40–75% involve the chiasmatic-hypothalamic region ,16,18,53The treatment of OPG may be based on simply careful observation; however, patients presenting with severe clinical symptoms or mass effects with significant progressive growth require more aggressive treatment based on the use of chemotherapy, radiotherapy, and/or surgery ,18,53,56OPGs can lead to variable clinical sequelae such as visual loss, endocrinopathies, and hypothalamic dysfunction, developmental/neuropsychological disorders, hydrocephalus, and focal neurological deficits because of their location ,53,57.Our patient suffering from OPG presented with rapidly progressing narcolepsy after several years of clinical and radiological stable disease. The narcolepsy diagnosis was confirmed by testing and had no improvement even though different lines of therapy were performed. Despite the relatively benign nature of the lesion, due to its dimension and hypothalamus involvement, it led to the death of our patient within five years from diagnosis. It is of interest to note that the patient did not have the typical suprasellar obesity.Narcolepsy is a rare condition in pediatrics. It is mainly idiopathic, but we must not forget that it can result from conditions that damage the hypothalamic area such as CNS tumors. In a broad spectrum of further clinical perspectives, it may be important to include somnolence disturbances and narcolepsy as signs of tumor progression and using these clinical evidences at the same plan of ophthalmological/endocrinological impairments to evaluate an oncological treatment choice or change.The treatment for narcolepsy is generally well tolerated with the resolution of symptoms; however, there are cases such as the case we have presented, resistant to treatment and with progressive and fatal evolution.


Of the benign tumors of the middle ear, the paraganglioma is the most common, and the facial nerve schwannoma comes in second placePotentially, middle ear schwannomas may stem not only from the facial nerve, but also from its side branches: the chorda tympani; the stapedial nerve; the tympanic branch of the glossopharyngeal nerve and the auricular branch of the vagus nerveMale, 55 year-old patient complaining of left ear tumor with six months of evolution which in the last 15 days started to hurt. He also reported hypoacusis on the same side for 7 years. Upon physical exam we found a polypoid tumor on the left ear which came out through the external ear canal, of soft consistency and painless (photo 1). There was no facial nerve involvement. Audiological tests showed moderate mixed hypoacusis with a good cochlear reserve in the middle and lower frequencies. A contrasted CT scan showed a radiopaque image which occupied the entire external ear canal (EEC), the tympanic and mastoid cavities. The EEC was enlarged with irregular bone outlining (Photos II and III). We biopsied the lesion and the pathology exam reported Schwannoma (Photo IV).During surgery we noticed a sessile tumor occupying the entire lumen of the EEC. The lesion seemed to have originated in the middle ear, shifting the EEC skin on the postero-superior region. During tumor shifting, we noticed a purulent oozing coming from the middle ear. We then performed a mastoidectomy, aticotomy and we brought the canal wall down as much as possible and, later on, after we had apparently removed the entire tumor, we did a type III tympanoplasty. The tumor involved the hypotympanum, the mesotympanum, aticoantral and peritubal regions. Among other findings, we found an erosion of the long arm of the incus, the stapes superstructure was preserved and we did not notice tumor adhering to the promontory, and the tympanic portion of the facial canal was intact. The patient evolved favorably with functional integrity of the facial nerve with preserved audiometric thresholds. He is currently in the fourth year of follow up, with a favorable outcome.We suppose the schwannoma came from the chorda tympani because of its anatomical location, facial nerve integrity on the tympanic-mastoid portion and the lack of adherence to the promontory.Since 1966 until today, there are five reports in the world literature of cases of schwannoma associated with the chorda tympani nerve. The main complaint seen in the papers studiesIn most of the cases, upon otoscopy, the lesion aspect corresponded to a bulging in the postero-superior region of the tympanic membrane or the EEC posterior wall, differently from our patient, whom had a polypoid tumor covered by skin and filling up the entire EEC, bulging out through the canal. There is a report of a cystic tumor which totally occluded the canal and which corresponded to an EEC schwannomaCT scan and MRI are tests which enable to define tumor extension and the very nature of the lesion. It would be ideal to count on both studies and, depending on the clinical case, even refrain from biopsing.In most of the cases published, the surgery was conservative because of the small size of the tumor. In our case, thanks to the large tumor extension, we did a canal wall down mastoidectomy and type III tympanoplasty.The middle ear schwannoma, stemming from the collateral branches of the facial nerve is uncommon, but should be considered in the differential diagnosis of middle ear tumors. Biopsy is a risky procedure, but it was the main diagnostic method in most of the cases published. CT scan and MRI are useful to assess tumor extension and recognize the nature of the lesion. Ultimate treatment is surgical removal, as conservative as possible.


Her past medical history included an ovarian borderline serous tumour diagnosed in 2018, which was treated with abdominal hysterectomy and omentectomy, and subsequently, following recurrence of a serous carcinoma, with two cycles of carboplatin.9/L (150–400 × 109/L). A peripheral blood film showed circulating blasts. A bone marrow aspirate revealed trilineage dysplasia and an increased population of medium‐large blasts with nuclear folding and prominent nucleoli (10%) , white blood count (WBC) of 1.7 × 109/L (4–11 × 109/L) and platelets 62 × 10Aberrant expression of cytokeratins in acute myeloid leukaemia is a recognised but extremely rare phenomenon, which can potentially pose a diagnostic pitfall. Performing a wider panel of immunohistochemistry can help to identify these cases and diagnose them accurately. Our case highlights the importance of interpreting unusual immunohistochemical findings in the context of other investigations including morphology, flow cytometry and the clinical presentation.Yasin Dhonye and Wael Al‐Qsous wrote the original manuscript. Haifaa Saadi reviewed the bone marrow aspirate and provided slides for images. Hadil Abu Arqoub and Wael Al‐Qsous reviewed the bone marrow trephine and took images. All authors reviewed and approved the final manuscript.The authors declare they have no conflicts of interest.The authors received no specific funding for this work.No research on human was performed on this case. A written informed consent was obtained from the patient.


Choriocarcinoma is a malignant tumour of trophoblastic origin. Most are gestational choriocarcinomas, which usually occur in women with an epithelial origin of the placental chorionic villi and are associated with pregnancy. It mainly originates in the gonads such as the ovaries and testes. However, it rarely occurs in the stomach and is known as primary choriocarcinoma (PGC).A 69-year-old man complained of abdominal distention for 3 years, which worsened 1 week later. Gastroscopy showed chronic atrophic gastritis C1 (C1: indicates atrophic gastritis involving the sinus region); the pathology report of the gastroscopic specimen showed high-grade epithelial tumours in the mucosal glands. We diagnosed an occupying lesion in the stomach and performed a laparoscopically assisted distal gastrectomy and Billroth type 1 anastomosis. Postoperative pathology showed “gastric choriocarcinoma with cancerous tissue invading the entire gastric wall”. The patient was discharged on the 11th postoperative day as there were no postoperative complications. The patient was followed up until June 2022 with a good recovery and no recurrence.We encountered a case of Primary Gastric Choriocarcinoma, where the cancerous tissue invades the full thickness of the gastric wall. Choriocarcinoma is a malignant tumour of trophoblastic origin . The vasA 69-year-old male presented to our hospital complaining of abdominal distension and discomfort for 3 years, worsening for 1 week. His medical history included chronic appendicitis and he had undergone an appendectomy 2 years ago. Recently, there was no significant change in weight. One week ago, the patient felt a marked increase in abdominal distension and pain, mainly in the upper and middle abdomen, and presented to our hospital. The results of the examination were as follows: normal vital signs, fecal occult blood test (++), ALB: 33.05 g/L, Hb: 96 g/L and HCG-β: 66.12 mIU/ml. Liver and kidney function, electrolytes and blood glucose were normal. Abdominal Doppler ultrasound showed a solid mass in the upper abdomen, which was suspected to be gastric cancer . GastrosPrimary gastric choriocarcinoma was first described by Davidsohn in 1905  and acco


To analyze a case of neglected cervicovesical fistula with intrauterine adhesions caused by cesarean section.A 36-year-old female patient with a history of two previous cesarean sections complained of the absence of menstruation for the last 18 months. The diagnosis of the cervicovesical fistula was made through hysteroscopy and cystoscopy. The reconstruction of the uterus and bladder was achieved by a laparoscopic repair technique.The patient resumed normal menstruation postoperatively without complaining of any complications. Uterine continuity and cavity had been restored to normal at the second look of hysteroscopy.Cervicovesical fistula with intrauterine adhesions is very rare in our clinical work. Hysteroscopy might play an essential role in diagnosing cervicovesical fistula and IUA. In our literature review, a surgical approach was the mainstay and definitive management of the cervicovesical fistula following a cesarean section. Vesicouterine fistula is an abnormal communication between the bladder and the uterus. It represents a rare urogenital complication, accounting for approximately 1%–4% of genitourinary fistula , and latA 36-year-old Chinese female patient, with a history of two previous cesarean sections, was referred to our outpatient clinic. Her chief complaint was the absence of menstruation for the last 18 months, accompanied by periodic lower abdominal distension. She said that the second cesarean section was taken emergently in a rural hospital and it was a tough procedure. She denied any allergies and had no special drug history or family history of other diseases. The physical examination at the presentation was unremarkable. A gynecological three-dimensional ultrasound examination revealed intrauterine adhesions and hydrosalpinx in the left fallopian tube.We carried out hysteroscopy examinations before the surgery. A huge defect was discovered at the 12 o'clock position in the cervical canal when the scope was moved forward . Then thThe adhesion was released carefully, and the scar was removed; finally, the fistulous tract was ended by the posterior wall of the bladder and the anterior cervix of the uterus  after thNo surgical complications were observed postoperatively. After the reconstructive procedure, bladder catheterization was established for one month. Furthermore, the uterine balloon was indwelled for two months. The patient could micturate normally after the catheterization was removed. Moreover, she returned to normal menstrual cycles and volume after the surgery without complaining of abnormal vaginal discharge, urinary incontinence, or menouria. The second hysteroscopy examination was carried out two months later. The uterus restored its continuity, with no new adhesion in the uterine cavity. In addition, no obstruction or adhesion was found in the cervical canal .Cervicovesical fistula represents a rare urogenital complication. The diagnosis of cervicovesical fistula is generally based on symptoms and clinical examinations. The symptoms of cervicovesical fistula are variable. These symptoms include amenorrhea and periodic hematuria coinciding with the time of the menstrual cycle or menouria, vaginal leakage of urine, and recurrent urinary tract infections , 4, 5. MFor this reported case, the patient had neither the typical Youssef's syndrome nor the symptom of continuous urinary leakage. The patient chiefly complained of secondary amenorrhea after the second cesarean section. Possibly suggesting that the uterine incision was made low down in the lower uterine segment close to the cervix where the bladder could have become adherent to the scar. Due to inadequate separation of the lower uterine segment, the bladder was mistaken as the cervical side of the lower uterine segment, which was sutured with the uterine body side during that emergent procedure. The patient did not pay more attention to menstruation during lactation. With the hyperplasia of scar, the posterior uterine wall adhered to bladder resulting in outflow obstruction and amenorrhea. When the period recovered postpartum, menstrual reflux and inflammation led to atresia of the fimbria, hematosalpinx, and periodic abdominal pain.Various imaging procedures have been found useful to diagnose cervicovesical fistula. Cystoscopy, cystography, hysteroscopy, and hysterosalpingography (HSG) play a crucial role. Additional imaging examinations include contrast-enhanced CT, magnetic resonance imaging (MRI), and transvaginal ultrasound . HoweverAmenorrhea is a common symptom of intrauterine adhesions, especially in severe Asherman syndrome. It was difficult to distinguish IUA from cervicovesical fistula after the second cesarean section. As a preliminary diagnosis of intrauterine adhesions, the possibility of the cervicovesical fistula was overlooked in this patient, for which the relevant imaging tests were deficient. While the application of hysteroscopy by examining the no-dilated cervical canal also played a vital role in diagnosis.Treatments for cervicovesical fistula include conservative treatments and surgery. The conservative therapeutic options include bladder catheterization, hormonal therapy, and cystoscopic fulguration, all with favourable results. Nevertheless, only 5% of the cases respond to conservative therapy –13. SurgThe surgical approach has been established as the mainstay and definitive management of the cervicovesical fistula following the cesarean section in our literature review. For this patient, the uterus had been constructed, the hematosalpinx had been cured, and the bladder had been repaired by laparoscopy. Meanwhile, intrauterine adhesions had been released by hysteroscopy. The normal shape of the uterus, uterine cavity, and bladder had been restored.Cervicovesical fistula with intrauterine adhesions is very rare in our clinical work. Therefore, it can be neglected easily. It is necessary to restore the correct anatomical structure during multiple cesarean sections, and meanwhile, early identification and diagnosis are necessary. Hysteroscopy might play an essential role in diagnosing cervicovesical fistula and IUA. Surgery is the vital approach to repairing the cervicovesical fistula. The uterus should be reconstructed as far as possible instead of hysterectomy to preserve women's reproductive function.


Mesenteric cysts are rare lesions of the abdominal cavity or retroperitoneum. The exact etiopathogenesis is still undefined. Clinical manifestation can vary from asymptomatic patients to symptoms of an acute abdomen, making diagnosis very challenging.We present a case of a 47-year-old male with new ongoing polyuria and nocturia as well as episodes of slight abdominal pain. An abdominal ultrasound showed ascites and the computer tomography (CT) scan raised suspicion of an internal hernia. We performed a diagnostic laparoscopy and open resection of a cystic lesion of the small bowel mesentery. The histological examination revealed a lymphatic mesenteric cyst.Mesenteric cysts represent rare intra-abdominal tumors that physicians should consider as a differential diagnosis in patients with abdominal pain and an intra-abdominal mass.Surgery should be advised to prevent the development of complications and to confirm the histopathological diagnosis. •Idiopathic mesenteric cyst with an unusual clinical presentation•Mesenteric cysts are rare but should still be considered as differential diagnosis.•Surgery is only an option to clarify diagnosis. Nowadays most authors define the term mesenteric cyst as a heterogeneous group of cystic formation located anywhere in the mesentery, omentum or retroperitoneum Benevieni, an Italian anatomist, reported a mesenteric cyst for the first time. He described this lesion in 1507 in an autopsy on an 8-year-old boy. Rakitansky published the first accurate description of a chylous cyst in 1842. Tillaux carried out the first successful surgical resection in 1880 We present a case of a 47-year-old male patient with a lymphatic mesenterial cyst of the proximal jejunum. This case report has been reported in line with the SCARE criteria 2A 47-year-old male patient initially presented new urological symptoms such as polyuria and nocturia. Furthermore, he reported episodes of slight abdominal pain and a pre-existing gastroesophageal reflux. The urological examination revealed a benign prostatic enlargement without signs of urethral obstruction. An abdominal ultrasound showed ascites, other intra-abdominal pathologies were not described. A contrast-enhanced computed tomography (CT) scan of the abdomen was performed for further investigation A + B. ItThe patient was referred to our institution for surgical consultation. The physical examination remained unremarkable with no sign of an abdominal mass, abdominal tenderness, or peritonitis. A recent colonoscopy a year earlier had shown no significant findings. The personal history to that point remained unremarkable as well with no previous abdominal surgery or medication. The lab results showed no abnormalities.Due to the unclear findings, the potential internal hernia, the possible differential diagnosis of a pseudomyxoma as the origin of the unclear calcification and the ascites, surgery was planned. A diagnostic laparoscopy was performed by an attending and a surgical resident. It showed no sign of ascites or an internal hernia. Yet, we found a cystic lesion with perifocal milky fluid accumulation, located in the mesentery of the proximal jejunum. We performed a mini-laparotomy and a complete excision of the lesion. Due to the proximity of the lesion to the small bowel, we performed an “en bloc” intestinal loop resection of about six centimeters and an end-to-end anastomosis.The histopathological examination confirmed a lymphatic mesenteric cyst with a diameter of 11 cm , Fig. 3.No postoperative complications occurred. The patient remained at fasting for the first three postoperative days and there was no complication during the following oral food intake. The patient was discharged on full diet and without any complication on the ninth postoperative day. On the 6-week follow-up the patient was doing well and mentioned no discomfort or abdominal pain.Retrospectively, the patient remembered a hospitalization over several days due to unspecific abdominal pain, nausea and vomiting at the age of 10. The pain was located in the epigastric area and was also associated with abdominal cramps and incapacity of oral food intake for several days. The diagnostic assessment carried out during that hospitalization remained inconclusive but no abdominal imaging had been available at that time. The patient also reported, he suffered from several similar episodes of the same unspecific abdominal pain during his teenage years until they resolved at the beginning of adulthood.3The clinical manifestation of mesenteric cysts is mostly non-specific and can present with various symptoms. As in our case, up to 40 % of mesenteric cysts The symptoms can vary from non-specific, chronic abdominal pain to symptoms of an acute abdomen mimicking rupture of an abdominal aortic aneurysm In patients with non-specific, usually chronic diffuse abdominal pain, symptoms are thought to be caused from traction and stretching on the root of the mesentery Acute symptoms are usually related to complications and may include hemorrhage, shock, rupture, volvulus or bowel infarction, resulting in surgical emergency situations Mesenteric cysts can also present clinical or radiological findings similar to malignant lesions such as appendiceal cancer, most of all pseudomyxoma peritonei (PMP). CT imaging of our patient, showing focal calcification and fluid collection between small bowel loops in the right lower abdominal quadrant, also aroused the suspicion of PMP and led to consider a diagnostic laparoscopy.The etiopathogenesis of mesenteric cysts is still unknown. Hypotheses include traumatic, infectious, or embryonic origins As reported by several authors, most mesenteric cysts are located in the small bowel mesentery (about 60 % of cases), followed by the large bowel mesentery (24 %), and the retroperitoneum As mentioned before, several definitions and classifications have been proposed. The most accepted classification nowadays divides mesenteric cysts according to their histopathological features. This classification includes the 6 following groups: cysts of lymphatic origin, mesothelial origin, enteric origin, urogenital origin, mature cystic teratomas and pseudocysts. Surgery is the treatment of choice for this kind of lesion, although good results have been reported with percutaneous drainage and sclerosis of the cystic wall If feasible, laparoscopy should be preferred to open surgery to reduce postoperative pain, need for analgesic drugs and postoperative length of hospital stay.4Mesenteric cysts are a rare entity. Nevertheless, they should be considered as a differential diagnosis as the clinical features may be unspecific. Complete surgical resection is the best option to confirm the diagnosis and prevent recurrences.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer-reviewed.N/A.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit-sectors.MvSuT.N/A.All authors contributed to the article. The first draft of the manuscript was written by MG and MvSuT. KJN and DCS critically reviewed previous versions of the manuscript. AH obtained and interpreted the imaging and provided The authors have no conflicts of interest to declare.


Thyroid-stimulating hormone (TSH)-secreting pituitary adenomas (TSHomas) are rare and usually present with hyperthyroidism. Calcification in pituitary tumors is an infrequent finding. Herein, we report an extremely rare case of TSHoma with diffuse calcification.via endoscopic transnasal-sphenoidal surgery. Microscopically, nests of cells were inconspicuous among the diffuse psammoma bodies. Expression of TSH was patchy, and only several TSH-positive cells were observed. Postoperatively, the serum levels of TSH, FT3, and FT4 decreased to their normal range. Follow-up MR images showed no evidence of residual tumor or regrowth after the resection.A 43-year-old man was admitted to our department with a complaint of palpitations. An endocrinological examination revealed elevated serum levels of TSH, free triiodothyronine (FT3), and free thyroxin, whereas the physical examination revealed no obvious abnormality. Computerized tomography (CT) showed a sellar mass with diffuse calcification. Contrast-enhanced T1-weighted images revealed a less-enhancing tumor without obvious suprasellar or parasellar expansion. The tumor was completely removed via endoscopic transnasal-transsphenoidal surgery (eTSS), and thyroid function was normalized after the operation.Herein, we report a rare case of TSHoma with diffuse calcification that presented with hyperthyroidism. A correct and early diagnosis was made according to the European Thyroid Association guidelines. This tumor was completely removed  Among all functional pituitary adenomas, thyroid-stimulating hormone (TSH)-secreting pituitary adenomas (TSHomas) are rare and account for only 0.5%–3% of all pituitary tumors in surgical series , 2. TSHoA 43-year-old man visited the outpatient department complaining of palpitations. His height was 174 cm, and his body weight was 66 kg. He had continuously perspired profusely. His blood pressure was 160/95 mmHg. No abnormal findings were noted on physical examination, including the thyroid gland. Electrocardiogram showed atrial fibrillation, and the heart rate was 97 beats/min. Endocrinological examination revealed hypersecretion of TSH, free triiodothyronine (FT3), and free thyroxine (FT4); as a result, central hyperthyroidism was in consideration Table 1.To prevent a thyrotoxic crisis during the perioperative period, thiamazole was administered at a dosage of 30 mg/day, and the hyperthyroidism was significantly relieved. After careful preoperative evaluation, endoscopic transnasal-sphenoidal surgery (eTSS) was performed in December 2020. During the operation, a solid yellowish mass full of calcifications was identified in the sellar area  for the publication of any potentially identifiable images or data included in this article.HY and CY wrote and revised the manuscript together. JM participated in the surgery and handled the pictures. WJ gave some important advice for the revision of the manuscript. All authors contributed to the article and approved the submitted version.


A 58-year-old female patient with a history of left temporal glioblastoma multiforme (GBM) World Health Organization (WHO) grade IV that had been diagnosed and treated by total tumor resection 12 months earlier Figs. . At the Open biopsy and partial tumor resection were performed the following day with the patient under general anesthesia in a park bench position using intraoperative neurophysiological monitoring. The partial tumor resection was performed via a retrosigmoid approach. Macroscopically the tumor showed a grayish color, had a soft consistency and was adherent to the cranial nerves. Due to the adherence of the tumor in relation to the cranial nerves, a complete resection could not be performed.The postoperative course on our intensive care unit showed missing swallowing and gag reflexes. An extubation could therefore not be performed, a tracheotomy was refused by the otherwise neurologically intact patient. In the following days an acute decrease of consciousness occurred. A CT of the head demonstrated an extensive tumor progression with compression of the brainstem. In fulfilment of the patient’s will, the treatment was discontinued and the patient passed away the following day.arrows). T2 weighted images  show regressive changes on T1 weighted images after administration of gadolinium (Gd). The lesion displays distinct contrast enhancement . Early postoperative (< 24 h) MRI obtained after tumor surgery confirms complete tumor resection  located in the prepontine cistern. The lesion compresses and dislocates the brainstem and the myelon . On T1-weighted MRI after administration of Gd the lesion shows some degree of homogeneous enhancement of contrast medium .Initial preoperative MRI shows a space-occupying lesion of the left-sided temporal lobe Fig. , arrows.ges Fig. a, arrow ion Fig.  showing ium Fig. f, arrow.Primary central nervous system (CNS) lymphomas (PCNSL) account for a small proportion of all primary brain tumors and usually involve the brain parenchyma. A rare but distinct subtype of primary CNS lymphoma is primary dural lymphoma (PDL), which as a special feature histopathologically very often corresponds to low-grade B‑cell marginal zone lymphoma with a better prognosis, whereas most PCNSL are histopathologically diffuse large B‑cell lymphomas .Primary dural lymphomas share similar imaging characteristics with other dural masses and can present as an extra-axial lobulated mass, which is hyperdense to brain parenchyma on non-enhanced CT imaging and especially in this modality cannot be distinguished with certainty from meningiomas. Bony involvement was described in about 3/4 of the cases in a case study .In addition to the typical imaging features of extra-axial masses with broad-based dural attachment with a “dural tail” in contrast-enhanced sequences and cerebrospinal fluid rim (CSF cleft) in T2-weighted sequences, MRI shows strong and mostly homogeneous contrast enhancement as well as diffusion restriction with DWI hyperintensity and corresponding apparent diffusion coefficient (ADC) signal depression as signs of hypercellularity. In contrast to high-grade glioma, MR perfusion shows decreased relative cerebral blood volume (rCBV) values.Skull base meningiomas are predominantly benign WHO grade 1 tumors when located at the skull base and only in rare cases WHO grade 2 or 3 tumors and then more often located along the convexity. On CT intralesional calcifications as well as hyperostotic changes of the adjacent bone can be seen. MRI shows the typical signs of an extra-axial, well-circumscribed tumor localization with the dural tail sign in contrast-enhanced sequences and the CSF cleft sign in T2/FLAIR (fluid attenuated inversion recovery) sequences. On MRI, calcification is best pictured with signal loss in SWI sequence. Contrast enhancement is usually homogeneous and strong, although meningiomas may also appear atypical with necrotic areas [Dural metastases are among the most common causes of dural masses. The leading underlying primary tumors are breast, prostate, kidney, head and neck or lung cancer .The cause may be direct extension of bony metastases, but also hematogenous, lymphathic, or via venous vertebral plexus retrograde metastases. Especially in the case of solitary occurrence of dural metastases, differentiation from other extra-axial masses such as meningioma or hemangiopericytoma is very often not reliably possible during initial imaging, as these entities may jointly show homogeneous contrast enhancement, calcifications and hyperostoses. Concomitant infiltration of the brain parenchyma or bony erosions in about 1/3 of cases each or infiltration of the cerebral sinuses is also described . In addiGlioblastoma multiforme (GBM) accounts for more than half of adult cases of malignant brain-derived tumors. MRI usually shows a typical appearance of an intra-axial mass with peripherally inhomogeneous, but strong contrast enhancement and cerebral blood volume increase on perfusion imaging, which usually surrounds a central zone of necrosis. In addition, peritumoral T2/FLAIR hyperintense edema is usually seen, with histologic evidence of tumor cell infiltration in addition to vasogenic changes. Most GBM patients experience tumor recurrence near the resection margins and despite infiltrative growth, metastases are generally rare and when they do occur are usually leptomeningeal extracranial via CSF seeding. Few cases of extraparenchymal dural metastases have been described worldwide in a subtype of glioblastoma with a primitive neuroectodermal component (PNET), although these may also occur after chemoradiotherapy .Ecchordosis physaliphora (EP) is a rare benign, in most cases retroclivally and prepontine located hamartomatous mass derived from notochord remnants, that is usually asymptomatic due to its size below 2 cm and should be differentiated from intradural clival chordoma, which also originates from ectopic notochord but is neoplastic .In rare cases EP demonstrates space-occupying effects such as encasement of the basilar artery. CT shows a circumscribed bony substance defect, MRI usually shows a stalk-like connection to the clivus with predominantly T2-hyperintense, CSF isointense signal, and contrast enhancement is mostly absent. In contrast to this, intradural clivus chordoma demonstrates more frequently contrast enhancement after gadolinium injection.A rare differential diagnosis of dural space-occupying lesions is solitary dural plasmacytoma, which is a benign, single plasma cell tumor lesion without clinical and imaging-based evidence of systemic myeloma manifestation at the time of diagnosis but may evolve into malignant multiple myeloma later in life . First cNearly one year prior to the surgical removal of the clival tumor, we had assessed a tissue biopsy from the same patient from the temporal lobe. We identified a glial tumor with a diffuse infiltration into neighboring CNS tissue Fig. a. The tuWe now obtained a small tissue biopsy for intraoperative neuropathological assessment. A cryostat section was stained with hematoxylin and eosin , the immunohistochemical reaction for GFAP now remained negative Fig. c. The tulls Fig. d.The immunohistochemical reactions for the pan-cytokeratin markers MNF116 and AE1/3 as well as epithelial membrane antigen (EMA), transcription termination factor 1 (TTF1) and melan‑A remained negative. Vascular structures were labelled in the immunohistochemical reaction for alpha-smooth muscle actin (SMA). Desmin, signal transducer and activator of transcription 6 (STAT6) and S100 were negative. Nuclear expression of integrase interactor 1 (INI-1) was preserved.Only a few cells were labelled in the immunohistochemical reactions for leukocyte common antigen LCA, CD45), B‑lymphocyte antigen CD20 and B‑cell antigen receptor complex-associated protein alpha chain CD79a). The tumor cells showed partial positivity in the reaction for vimentin (Fig. , B‑lympha. The tuMalignant gliomas with a primitive neuronal component have first been described in 2009 . Using aThe present case is highly interesting for several reasons. Firstly, glioblastomas with a primitive neuronal component are extremely rare. When more than 75,000 brain tumors were analyzed, only 63 harbored the same characteristic methylation profile that was detected in both of our specimens . Secondl


Hydatid disease is endemic in Mediterranean countries and most commonly occurs in the liver followed by the lung. A primary localization in the retroperitoneum is extremely rare.We report the case of a 29-year-old Tunisian patient presenting with progressive left flank pain and skin urticaria. On abdominal ultrasonography and computed tomography scan, a ruptured retroperitoneal hydatid cyst was diagnosed, which was confirmed by positive hydatid serology. The treatment consisted of resection of protruding dome. The evolution was favorable. No local recurrence was detected during postoperative follow-up.Primary retroperitoneal hydatid cyst is extremely rare and has uncommon presentation, but we should learn the keys to its diagnosis. In endemic regions, high suspicion for this disease is justified regardless of localization. Echinococcus granulosus [Hydatid cyst is a zoonosis caused mainly by the larval stage of the cestode worm anulosus . Hydatidanulosus . Therefoanulosus –5. BecauWe report a case of ruptured retroperitoneal hydatid cyst in a 29-year-old patient. Attention is drawn to the difficulty in diagnosis and management of such a rare presentation. This study is reported in line with the SCARE criteria .3) along with hypereosinophilia at 640/mm3. C-reactive protein (CRP) was elevated to 140 mg/L.A 29-year-old Tunisian patient, with no pathological history, presented with progressively worsening left flank pain and skin urticaria. He had no history of trauma, nausea, or vomiting. His medical history was not significant, in particular lacking history of prior surgery. The patient is a construction laborer living in Sidi Bouzid, a governorate in the center of the country. Examination showed tenderness in the left flank. Hematological tests showed slight anemia with leukocytosis (13430/mm3 with a detached membrane complicated with intraperitoneal rupture (Fig. Ultrasonography of the abdomen revealed a large cystic lesion in the left iliac fossa. Contrast-enhanced CT scan revealed a hydatid cyst of 12 × 7 × 21 cmExploratory laparotomy revealed a large cystic lesion with laminated membrane in the retroperitoneal space, extending from the lower pole of the left kidney down to pelvis, pushing the colon anteriorly and fissured in the peritoneum. We also identified four small daughter cysts along with peritoneal effusion of low abundance. The peritoneal cavity was washed out, then enucleation and partial cystectomy were carried out, observing the usual precautions Fig. . AfterwaEchinococcus granulosus. When infested, about 95% of the larvae are trapped in the liver or the lung, while only 5% of them escape into the systemic circulation to involve other organs [Human is an accidental host in the life cycle of r organs .A primary retroperitoneal hydatid cyst is extremely rare , 5. It iClinical features include flank pain, abdominal mass, and nonspecific symptoms such as nausea and vomiting. Symptoms are usually due to compression as cysts increase in size . When ruThe radiological appearance of the hydatid disease of musculoskeletal system mimics tumors and other inflammatory conditions. Therefore, preoperative diagnosis is sometimes difficult clinically and radiologically . AbdominSurgery is the cornerstone for treatment of hydatid cysts , 14. TotA retroperitoneal localization of the hydatid cyst is extremely rare. Rupture is a serious complication. The clinical symptoms can be confusing; therefore, preoperative diagnosis is sometimes difficult. In endemic regions, high suspicion for this disease is justified regardless of the localization affected, because delayed diagnosis increases the risk of impairment, recurrence, and sepsis.


Plasmablastic lymphoma (PBL) is a rare and highly aggressive type of lymphoma, which is commonly associated with human immunodeficiency virus (HIV) infection. Spontaneous regression of aggressive lymphomas is rare as they typically require administration of chemotherapy and radiotherapy for treatment. Here, we describe a case of a spontaneous regression of PBL after nasal biopsy and computed tomography (CT) guided biopsy of paravertebral mass in an immunocompetent patient. We postulate that the patient's immune system may be activated as a result of the stress and physical trauma brought on by nasal and paravertebral mass biopsy. Our case highlights the rare phenomenon of spontaneous regression of lymphoma which needs to be further studied on to establish its underlying pathophysiology. Plasmablastic lymphoma is a rare and aggressive B cell lymphoproliferative disorder. Delecluse et al. first described this rare disease in the oral cavity of patients with HIV in 1997 .However, the diagnosis of PBL remains a diagnostic challenge due to its overlapping features between lymphoma and myeloma. PBL is a high-grade B cell lymphoma that expresses plasma cell markers , but typically tests negative for B cell markers . The Ki-67 proliferation index of these tumours is usually high .–23.16–23Our case highlights the rare phenomenon of spontaneous regression of PBL in an elderly patient. The exact mechanism of this phenomenon remains unknown. However, we postulate that this could be triggered by activation of his immune system after nasal and paravertebral biopsy. Further research is needed to help better understand the pathophysiology behind this phenomenon as this could potentially have major therapeutic implications.


Only two cases of concomitantly occurring adenosquamous and neuroendocrine tumours (NET) have been reported in the literature. We report a case where both NET and adenosquamous are simultaneously occurring. A 42-year-old lady was hospitalized following painless jaundice and loss of weight. Computer tomography scan showed 3.0 x 2.9 cm hypo enhancing and hypovascular mass in the uncinate/head process. Another hypervascular mass was seen in the body of the pancreas. Pathological examination showed that the lesion in the uncinate process/neck of pancreas was an adenosquamous in the main pancreatic duct intraductal papillary mucinous neoplasm. The second tumour was a NET measuring 36 mm with no metastatic involvement. These findings suggested concurrently occurring but separated adenocarcinoma and NET tumours. This is the third case in the literature where both NET and adenosquamous are happening concomitantly, and the previous two other cases are also reviewed in the article. A patient presenting double-primary tumour in the pancreas is rare. Most case reports have described double-pancreatic tumours where a single tumour is of mixed histology such as adenoneuroendocrine or where a pancreatic ductal adenocarcinoma is either occurring simultaneously or derived from an intraductal papillary mucinous neoplasm (IPMN) or there is synchronous pancreatic neuroendocrine tumour (NET) and IPMN . ConcomiA 42-year old lady presented to the accident and emergency department with painless jaundice (both eyes and skin), loss of appetite, itching, pale stool, dark urine, lethargy, nausea and significant weight loss which started 3–4 months ago . On examination, her abdomen was soft, non-tender but distended and clinically jaundiced. Her past medical history consisted of Type 2 Diabetes Mellitus, dyspepsia, migraine and hypertension.Her initial blood tests showed normal full blood count and renal function tests. However, her liver function tests were deranged showing an obstructive picture. Bilirubin was 100 μmol/L, alanine transaminase was 452 μmol/L, alkaline phosphatase was 616 μmol/L and gamma-glutamyl transpeptidase was 1155 iu/L. Amylase was normal at 53 μmol/L with a slightly elevated c-reactive protein of 12. Her carbohydrate antigen 19-9 was 252 kU/L.Her hepatitis and human immunodeficiency virus screen came back as normal.The computed tomography (CT) triple phase scan showed 3.0 x 2.9 cm hypo enhancing and hypovascular mass in the uncinate/head process. This was causing mild biliary and pancreatic ductal dilatation. A separate hypervascular mass was seen in the body of the pancreas measuring 3 cm causing pancreatic duct dilatation and gland atrophy.The magnetic resonance imaging (MRI) pancreas and magnetic resonance cholangiopancreatography (MRCP) showed the same findings as of the CT triple phase scan. However, the tumours could be further characterized. The mass within the uncinate process has imaging features more in keeping with pancreatic adenocarcinoma while the mass in the pancreatic body was more difficult to characterize but a NET was more likely.The CT thorax was completely normal showing no metastatic disease.We had a patient with two distinct masses in their pancreas. After the complimentary radiological investigations, our differential diagnosis for the tumour in the uncinate process was adenocarcinoma while our diagnosis hypothesis for the tumour in the pancreatic body either a NET or a solid pseudo papillary epithelial neoplasm or a malignant IPMN.After discussion at the hepatobiliary multidisciplinary team meeting, it was decided to go straight for surgery—in keeping with the patient’s wishes. The patient was consented for a total pancreatectomy with preservation of the spleen.An initial exploratory laparotomy was performed where no evident metastasis was seen. During the surgery, two tumours were clearly identified. There was also a large lymph node on top of the right hepatic artery around the superior mesenteric artery that proceeded behind the porta hepatitis up to the coeliac axis. This was removed and sent to histopathology. A pylorus preserving pancreatoduodenectomy along with total pancreatectomy was performed. For reconstruction, an end to side hepato-jejunostomy was performed and antecolic duodeno-jejunostomy on a single loop.The patient recovered on the intensive care unit for 4 days and was cared for 13 days on the ward before being discharged home. It took some time to get her diabetes under control.Histological analysis showed that the lesion in the uncinate process/neck of pancreas was an adenocarcinoma in the main pancreatic duct IPMN. There was focal squamous differentiation with some areas of clear cell appearance and there was mucinous secretion. It was 32 mm in maximum diameter and a Grade 2. There was peri-neural invasion and it infiltrated the peri-pancreatic fatty tissue, duodenal muscularis propria and mucosa as well as lymphovascular invasion. The large lymph node that was removed separately showed a metastatic focus in the sub-capsular focus. All transection margins were clear albeit it was only <1 mm from the anterior surface. Fourteen of the 15 nodes showed metastatic adenocarcinoma and there was background IPMN branch main duct focus. The tumour was graded as Grade 2 pT2 pN2 Mx R0.The second tumour was in the body. It was a circumscribed neuroendocrine tumour 36 mm in diameter and appeared to infiltrate the capsule at some sections. There was lymphovascular invasion and the Mib-1 index was 1% making it a grade 1 neuroendocrine tumour. It was limited to the pancreas and was T2 with no peri-neural infiltration. Five lymph nodes around this tumour showed no metastatic NET tumour. This was also thought to be an R0 tumour. There was some adjacent cell hyperplasia. The tumour was graded as Grade 1 pT2 NET No Mx R0.After surgery, she completed 6 cycles adjuvant chemotherapy with FOLFIRNOX. Unfortunately, 4 months later, a follow-up CT scan showed a large tumour recurrence. Enhancement pattern suggested it to be secondary to the adenocarcinoma with squamous cell differentiation rather than a NET. The tumour recurrence was seen within the portacaval space encasing the right hepatic artery extending into the small bowel mesentery and also involving the superior mesenteric artery and superior mesenteric vein. Enlarged lymph nodes were seen within the small bowel mesentery. The CA 19-9 had also risen to 191 from 37 previously.Synchronous primary tumours of the pancreas are extremely rare. The incidence of having concomitant adenocarcinoma and NET is even rarer , 8. The et al. [Usually, synchronous tumours of the pancreas are found in previously diseased pancreases with chronic pancreatitis or associated with premalignant lesions such as IPMN as presented in the case above. It is well known that there is relationship between adenocarcinoma and IPMN. Yamaguchi et al.  reportedet al. reported a case of three concomitant tumours of the pancreas: an adenosquamous, an adenocarcinoma and a NET. The adenocarcinoma and NET were incidental tumours found during the histologic process. However, Liu et al. were able to identify both the NET and the adenocarcinoma as well as a pseudocyst pre-operatively.A PubMed database search found just two cases with concomitant adenocarcinoma and NET . ConsideIn conclusion, the possibility of multiple primary tumours has to be considered when managing multiple tumours in the pancreas. The use of optimal imaging modalities allows for optimum preoperative diagnosis and planning, which result in the most appropriate treatment option available to the patient.


Ewing sarcoma is a rare poorly differentiated and highly malignant tumor primarily affecting the skeletal system. It most commonly presents during the first two decades of life and rarely might it be of extraskeletal origin. Majority of extraskeletal cases are reported in the paravertebral and lower limb, with very few cases reported in the cervical spine region. We report a case of extraskeletal Ewing sarcoma in a 42-year-old man who presented with a 1-year history of neck swelling associated with neck pain, diagnosed by computed tomography and magnetic resonance imaging scans, in conjunction with histological analysis. Very few cases of cervical EES presenting as dumbbell tumors have been documented in the literature especially in this age group. Ewing sarcoma (ES) was first described in 1921 by James Ewing as an undifferentiated small round cell tumor involving the diaphysis of long bones which regressed with radiation treatment, in contrast to osteosarcomas  in 1975.A 42-year-old male presented with a one-year history of a painless progressively enlarging mass in the posterior aspect of his neck. Examination showed a large bilateral nape of neck swelling measuring approximately 15 × 15 cm as one piece, firm in consistency and with smooth margins . LaboratFurther investigations included PET CT which revealed a hypermetabolic soft tissue mass at the posterior aspect of the neck measuring 144 × 124 × 75 mm “dumbbell-shaped soft tissue mass” and bilateral deep cervical lymph nodes at multiple levels with insignificant FDG uptake. Core biopsy revealed to have blue cell tumor most likely Ewing's sarcoma. The patient thereafter received 6 cycles of preoperative neoadjuvant chemotherapy: Vincristine, Ifosfamide, Doxoroubicin, and Etoposide (VIDE) regimen 3 doses per cycle. He did not receive any radiotherapy. Post cycles, the mass regressed in size and became softer in consistency. A postchemotherapy restaging PET CT showed interval decrease in metabolic activity of the preexisting hypermetabolic soft tissue mass in the posterior neck and the size measuring 139 × 116 × 75 mmand few bilateral cervical lymph nodes persistently seen with no significant FDG uptake. The CT cervical spine done preoperatively showed a huge bilobed intermuscular soft tissue mass occupying the whole posterior compartment of the neck from the c2 level down to the c6 level with significant stretching of both superficial and deep posterior neck muscles. The mass is semihomogenous in density with no evidence of central breakdown or calcifications. A very thin layer of fat is seen surrounding the mass all around separating it from the surrounding muscles. However, it appears very adherent to the deep posterior neck muscle layer (erector spinae muscle). The lesion invades the interspinous spaces between c2, c3, and c4 spinous processes minimally eroding them, but there is no evidence of deep invasion into the spinal canal or involving the posterior neural arches at other levels. On the 10th of January 2021, the patient underwent a major surgical excision of the mass under the care of surgical oncology, spine surgery, and plastic reconstruction surgery at our institution. The mass was massive and invading the outer surface of the spinous process; it was shaved off with the attached muscles . His immES and EES previously considered separate entities have been reclassified into the Ewing sarcoma family of tumors (ESFT) which includes in its umbrella Ewing's sarcoma of bone, extraskeletal Ewing sarcoma (EES), Askin tumors of the chest wall, and primitive neuroectodermal tumors of bone or soft tissues , 8. ThesEES tends to present more commonly in adult populations when compared to Ewing sarcoma which rarely presents past the third decade of life , 9. PatiImaging options used in the diagnosis of ESFT tumor status, local resectability, and distant metastases are achieved via the use of MRI, CT, and PET/CT imaging modalities , 10. PETThere are no specific radiological features for EES; however, they are usually described as well circumscribed noncalcific soft tissue tumors, which can demonstrate internal heterogeneity with areas of low attenuation secondary to necrosis or hyperattenuation secondary to hemorrhage on computed tomographic examination. Lymphadenopathy due to metastasis is very rare and is only reported in 0-12% of cases . DefinitEES is considered to be a poor prognostic factor to survival when compared to skeletal Ewing sarcoma, due to its propensity for invasion of local adjacent organs and metastasizing to the lungs , 5. AccoUpon diagnosis of ESFT, the initial treatment involves induction chemotherapy with Vincristine, Ifosfamide, Doxoroubicin, and Etoposide (VIDE) regimen for 6 cycles at 2–4-week intervals , 15. AftDumbbell tumors are defined as tumors with both intraspinal and anterior paraspinal components that communicate via an intravertebral foramen . About 1Due to the scant and limited amount of literature available on metastatic spinal dumbbell tumors (SDT) and especially that on EES presenting as a SDT, we believe our case is rare in its presentation.