A solitary fibrous tumor develops from mesenchymal cells as a pleural neoplasm, but it is also occasionally reported in extrapleural sites. Retroperitoneal tumors are a group of neoplasms located between muscles and the fascia of the posterior abdominal wall and the parietal peritoneum. Their cytology differs from that of urinary tract organs or adrenals. This case report presents a rare solitary fibrous tumor incidentally found during an ultrasound examination. A 54-year-old male underwent urgent surgery for a tumor located in the left retroperitoneal space. The histologic examination confirmed a solitary fibrous tumor with a 5% Ki67 proliferation index, a 1 MF/10 HPF mitotic activity, and CD34-positive immunostains. A solitary fibrous tumor is a rare retroperitoneal tumor. Its symptoms and signs might resemble those of the classical triad of renal cell carcinoma, although the tumor's growth phase is typically asymptomatic. Intraoperative diagnosis of a solitary fibrous tumor strongly recommends radical excision. A solitary fibrous tumor (SFT) is a rare type of soft tissue sarcoma that develops from mesenchymal cells . It was This case report presents a rare solitary fibrous tumor incidentally diagnosed on an ultrasound examination.A 54-year-old male, suspected of a left kidney tumor, was urgently referred to our urology clinic. The ultrasound examination incidentally revealed the lesion and reported it as a 3 cm mass in the upper pole of the left kidney. The patient was asymptomatic: he did not report any weight loss, and the lesion was undetectable by palpation. He presented with several associated diseases, including intervertebral disc degeneration, but had no history of surgical treatment. Multiphase contrast-enhanced computer tomography  revealed2). At the preanesthetic assessment, the patient was classified as ASA1. Surgery was started using lumbar access. A 3.5 cm tumor was seen at the upper pole of the left kidney without apparent involvement of the kidney or other organs. Gross total resection was performed. The postoperative course was uneventful. The patient was discharged home in good condition three days after the surgery.The patient was admitted to our Urology Department for urgent surgical treatment of a left kidney tumor. Laboratory tests showed no abnormalities; creatinine level was 1.0 mg/dl  with a Ki67 proliferation index of 5%, a mitotic activity of 1 MF/10 HPF, and positive immunostaining for CD34. There was no histologic evidence of necrosis .A follow-up examination performed three months after the surgery consisted of laboratory tests, CT of the abdomen, and chest X-ray. No abnormalities were detected. The patient continues to come in for scheduled appointments at our urology clinic.Retroperitoneal tumors are located between the posterior abdominal muscles, associated fascia, and the parietal peritoneum. They are defined as neoplasms derived from tissues that are not typical of the urinary tract organs or adrenals. The tumors represent approximately 2-3% of all neoplasms , 7. The Retroperitoneal tumors might be asymptomatic. If symptoms occur, they tend to be noncharacteristic, depend on tumor size and location, and mainly result from displacement of the neighboring organs. Retroperitoneal lesions grow slowly and thus are detected at late stages . They arThe treatment of choice for retroperitoneal SFTs is radical excision. Since lymph node metastases are infrequent, there is no need for retroperitoneal lymphadenectomy to be performed simultaneously with surgery for the tumor . DistantThe final diagnosis is based on histology reports, as specific immunohistochemical staining of surgical specimens plays a critical role in the diagnostic process . RegularA solitary fibrous tumor usually develops as an asymptomatic or oligosymptomatic lesion. It is rarely diagnosed in the retroperitoneal space. It tends to be found incidentally during an ultrasound examination performed for some other reason. Radical excision is the treatment of choice, but a follow-up at a urology outpatient clinic is strongly recommended.


Mature cystic teratoma is the most common benign tumor of the ovaries in women of reproductive age. Dermoid tumor in an ectopic or aberrant location is usually detected incidentally. The occurrence of a dermoid tumor in the fallopian tubes is extremely rare, with only approximately 75 cases being reported worldwide in the English language literature. This report describes a left-sided fallopian tube dermoid tumor in a 27-year-old woman with chronic right tubal ectopic gestation and discusses its clinical implications. Inspection of the contralateral fallopian tube in a woman with a ruptured tubal ectopic pregnancy is required clinically to rule out bilateral tubal ectopic gestation. Although rare, the ectopic presence of a dermoid tumor in the fallopian tube may be an incidental finding requiring surgical removal. Mature cystic teratoma, also known as dermoid tumor or cyst, is the commonest benign tumor of the ovaries in women of reproductive age group, comprising 16% to 20% of all ovarian tumors . This caA 27-year-old woman presented with a history of continuous but scant bleeding per vaginum for the previous two months following a self-managed abortion with tablet mifepristone and misoprostol at six weeks of gestation. Her previous menstrual cycles were regular with average flow. She had a full-term normal vaginal delivery three years earlier. There was no history of contraceptive usage, and both her previous and family history were unremarkable. On examination, she was conscious and oriented, with stable vital signs but a mild degree of pallor. Her abdomen was soft with no rigidity, guarding, tenderness, or palpable masses. The speculum examination revealed a normal cervix with no bleeding per vaginum. Bimanual examination showed that her uterus was bulky, retroverted, and soft, with side-to-side mobility and fullness in the pouch of Douglas (POD). Her urine pregnancy test was positive, her hemoglobin concentration was 8.3 g/dl, and her serum beta-human chorionic gonadotropin concentration was 654 mIU/ml.Two-dimensional ultrasonography revealed a normal-sized uterus with a well-defined heterogeneous lesion in the POD measuring 9.0 x 8.5 x 6.4 cm Figure  with no Histopathology revealed that her right fallopian tube contained areas of intramural and transmural hemorrhage, trophoblastic cells, and occasionally degenerated chorionic villi, confirming a right tubal ectopic pregnancy. The solid lesion within the left fallopian tube showed the presence of epidermis with keratin flakes Figure , skin adDermoid tumors are common in the ovaries . Their dA review of 30 patients with tubal dermoid tumors showed that four were associated with ectopic pregnancy, whereas none was associated with chronic tubal gestation on the contralateral side, as in our case . Our patDue to the rarity, the actual incidence and histogenesis of primary fallopian tube dermoid tumors remain unclear . We perfPrimary fallopian tube dermoid tumors are rare, and usually asymptomatic and diagnosed incidentally. If associated with a chronic tubal gestation on the contralateral side, management is challenging, as salpingectomy for the chronic tubal gestation needs to be combined with fertility preserving salpingostomy for surgical removal of the dermoid tumor of the contralateral fallopian tube.


In patients with portal hypertension, the direction of the splenic venous flow is to-and-fro or hepatofugal (2). In this case, the characteristic ultrasound findings were helpful for the diagnosis of splenorenal shunt.A 46-year-old woman presented for screening ultrasound examination. She had no history to suspect encephalopathy. During abdominal sonography, the direction of splenic venous flow was observed to be completely inverted in accordance with respiratory movements, namely, being hepatofugal in the inspiratory phase and hepatopetal in the expiratory phase . SubsequNoneSK wrote the manuscript. AM edited the manuscript.We have obtained informed consent for this case report.


Primary broad ligament carcinoma is a very rare occurrence with 28 reported cases worldwide, to date. The epidemiology, treatment strategy and prognosis are still uncertain because of the scarcity of cases. Currently, all broad ligament carcinomas are managed similar to epithelial ovarian cancer. We report the case of a 43-year-old female with a prolonged complaint of abdominal pain and intermittent urinary retention, requiring frequent catheterization. She was diagnosed with obstructive right hydroureteronephrosis. The abdominal Contrast Enhanced Computed Tomography (CECT) revealed a well-defined heterogeneous lesion of 2.1х3х3.2cm size in the right lateral and posterior wall of the cervix. An ultrasound (USG)-guided Fine Needle Aspiration Cytology (FNAC) of the mass was done and it was suspected to be malignant. The patient underwent total abdominal hysterectomy, right salpingo-oophorectomy, pelvic lymph-nodal sampling, and peritoneal washing. Histological examination depicted an endometrioid adenocarcinoma of the broad ligament. She received adjuvant chemotherapy, followed by hormonal therapy. It has been five years since her surgery, and she is now alive and disease free. CECT: Contrast Enhanced Computed TomographyFATWO: Female Adnexal Tumor of probable Wolffian OriginFIGO-IC: International Federation of Gynecology and Obstetrics - stage ICOPD: Out-patient DepartmentSTIC: Serous Tubal Intraepithelial CarcinomaUSG: UltrasonographyA 43-year-old female sought medical attention due to her complaints of frequent urinary voiding dysfunction requiring intermittent catheterization, and lower abdominal pain during micturition for a period of two years. She was first diagnosed with right hydroureteronephrosis and treated with the placement of a ureteric stent, without any relief of the urinary symptoms. Her past surgical history was significant for the exeresis of an endometrioma of the right ovary, 18 years before.Her general physical examination as well as her per-abdominal, and per-vaginal examinations were normal. An abdominal CECT showed the right moderate hydroureteronephrosis with a double J (DJ) stent in situ. The uterus was bulky and heterogeneous, with the presence of few hypodense lesions within the uterine wall. A well-defined heterogeneous oval lesion of 2.1х3х3.2cm in the right lateral and posterior wall of the cervix, along with few foci of calcifications were found. The bilateral adnexa were normal. She subsequently underwent trans-vaginal USG-guided FNAC of the uterine cervical mass that was suspected to be malignant. A Papanicolaou smear test from the cervix was normal.With the working diagnosis of a complicated uterine leiomyoma having chances of suspicious malignant transformation, the patient underwent total abdominal hysterectomy (TAH), right salpingo-oophorectomy (Right SO), pelvic lymph-nodal sampling, peritoneal cytology and ureteric stent replacement. Intraoperatively, a tumor with size of 4x4 cm was identified in the right broad ligament, posterolateral to the cervix. There was no ascites. Both the ovaries as well as the fallopian tubes were grossly normal. No lesions consistent with endometriosis or disease dissemination were found.A gross examination of the surgical specimen showed a normal appearance of the uterus measuring 8х6х6 cm. However, a bulging mass of the right broad ligament measuring 2.5x1.5x2cm was attached to it. Post serial slicing, a single well-circumscribed, grey-white, firm tumor was identified in the broad ligament. The endometrial cavity, the endocervical and ectocervical mucosal surfaces, the right ovary and the fallopian tubes were all unremarkable. On microscopic examination the broad ligament mass showed adenomyoma with focal areas of malignant transformation. The tumor cells were arranged in the form of nests, glands and trabeculae, infiltrating the smooth muscle and reaching just beneath the ectocervix . Three iAn immunohistochemistry (IHC) showed positivity for CK7 in the cytoplasm of the tumor cells, membranous positivity for vimentin and nuclear positivity for Estrogen (ER) and Progesterone Receptors (PR). IHC results for p16, CEA, CK20 and WT1 were negative  and 3.The peritoneal washing done intraoperatively was suggestive of metastatic adenocarcinoma. The final histopathological diagnosis was endometrioid adenocarcinoma, grade I, arising from broad ligament adenomyoma, FIGO-IC.The patient received adjuvant treatment with six cycles of paclitaxel and carboplatin chemotherapy, followed by hormonal therapy with tamoxifen 20mg daily. She completed five years of hormonal therapy during her latest visit, and is completely disease free clinically and radiologically. She is currently on a six-monthly clinical follow-up routine with yearly ultrasonography of the abdomen and the pelvis.We conducted a literature search in ‘PubMed’ and ‘Google Scholar’ using the keywords “broad ligament carcinoma and broad ligament endometrioid adenocarcinoma”. Additional papers were found through references in the primary articles . In thisAmong all types of neoplasms arising in the broad ligament, leiomyoma and its variants are most common. Most of these lesions, either benign or malignant, clinically present with vague symptoms and are often encountered during a routine gynecological examination or an abdominal surgery for other reasons.A primary broad ligament tumor was defined by Gardner et al..Most of the epithelial malignancies of the broad ligament are derived from the Mullerian remnants, including (i) serous, (ii) mucinous, (iii) clear cell, (iv) endometrioid, (v) borderline tumors and (vi) endometrial stromal sarcomas. Among all the reported carcinomas, serous adenocarcinoma, clear cell adenocarcinoma and endometrioid adenocarcinoma are the most common histologic variants,The precise origin of broad ligament tumors is not known, but there are several hypotheses concerning its histogenesis. It is suggested that the broad ligament serous carcinomas arise from Serous Tubal Intraepithelial Carcinoma (STIC) lesions, similar to the ovarian high-grade serous carcinomas.The Mullerian-origin broad ligament tumors and ovarian cancers can be confirmed by IHC. WT1, the most specific marker, is positive in 90% of the cases. IHC for CA-125 has a low specificity of 35%. The Mullerian-origin tumors also show positivity for ER, whereas the Wolffian-origin tumors show positivity for CD10, vimentin and calretinin, and negativity for ER. An IHC analysis by Brady et al.As the broad ligament tumors are encased within the two layers of ligament, they tend to be asymptomatic during the early stages. Similarly, their rupture, progression and metastasis are delayed because of the lack of their own vascular supply, in contrast to the ovarian tumors.There is a dearth of clinical experience in the management and prognosis of these tumors. Since the broad ligament is anatomically close to the ovary and the histological features of these two types of tumors are similar, their staging and management follow the epithelial ovarian cancer approach.While up to 25% of the ovarian carcinomas are bilateral, the borderline and malignant lesions of the broad ligament are unilateral, and similar tumors do not accompany them in the ipsilateral ovary. Therefore, in a young woman who opts for preservation of ovarian function and in cases where the reproductive function is desired, fertility-preserving surgeries are justified.,,,,-,,,-The exact role of the postsurgical treatment is not a consensus. Surgery alone,


A dermatofibroma (DF) is a common, benign tumor composed of fibroblastic and histiocytic cells. DF presents clinically with several different reported variants. One rare variant is hemosiderotic DF (HDF), which is made up of small blood vessels and hemosiderin deposits. HDF can be indistinguishable, clinically, from melanoma, making the use of other pathological tools crucial in the diagnosis.We report the case of a 25-year-old male medical student from the Caribbean who presented to our clinic with a single asymptomatic pigmented cystic lesion on his left posterior calf. The cystic lesion was excised surgically. Histopathology examination of the excised mass revealed a moderately cellular, poorly demarcated, dermal, fibrohistiocytic proliferation. Pathology consultation confirmed a diagnosis of HDF. A dermatofibroma (DF) is a common, benign tumor composed of fibroblastic and histiocytic cells . DFs preWe report the case of a 25-year-old male medical student from the Caribbean who presented to our clinic with a single asymptomatic pigmented cystic lesion on his left posterior calf Figure . The lesThe patient is otherwise healthy and has reported no fevers, chills, nausea, or vomiting. As medical student in Antigua with decreased access to medical care, he decided to seek surgical intervention due to recent mild discomfort.Further clinical examination confirmed a circumference of 1 cm for the cystic lesion. Physical exam revealed no fluctuance, pericystic erythema, or any tenderness to palpation. Excision was planned for one week after the initial consultation.The procedure was performed in an outpatient setting under local anesthesia. The patient was injected with 4 ml of local 1% lidocaine with epinephrine subcutaneously surrounding the 1 cm lesion. An elliptical incision was marked and carried out to excise the cystic lesion in a single specimen piece. The entire cystic lesion was subsequently sent for pathological analysis. The patient tolerated the procedure well.The pathology report confirmed an excision of tan-brown skin lesion, measuring 1.0 x 0.8 x 0.7 cm. The cutaneous surface was remarkable for a central 0.8 x 0.6 cm gray-tan, slightly raised, firm nodule. Histopathological examination of the excised cystic lesion revealed a moderately cellular, poorly demarcated, dermal, fibrohistiocytic proliferation made primarily of fusiform to stellate cells with unvarying, oval nuclei and amphiphilic cytoplasm Figure , associaDFs are relatively common skin lesions, approximately 3% of all skin lesions, appearing most often in the lower extremities of young adult females ,6. AtypiMelanoma is a cancer with malignant potential, and any suspicious lesion should be treated with WLE with negative margins. However, HDF is a rare tumor that looks identical to melanoma in the clinical setting. Therefore, HDF should always be included as part of the differential diagnosis, especially when the patient is a young adult and the lesion appears on a limb. Dermatoscopy and clinical evaluation are insufficient in confirming HDF or melanoma due to their similar nature, and expert histopathological examination should be performed to achieve a final diagnosis.


Idiopathic retroperitoneal fibrosis (RPF) is a rare fibro-inflammatory disease, with a low incidence worldwide, which occurs around the abdominal aorta and the iliac arteries. It spreads through the retroperitoneum causing ureteral obstruction with associated renal failure and obstruction of other adjacent structures. RPF can be idiopathic or secondary to neoplastic processes, infections, or medications. RPF is considered part of the spectrum of the disease related to immunoglobulin G4 (IgG4) and other autoimmune disorders. Occupational exposure to asbestos and tobacco smoke are important risk factors for the development of idiopathic RPF. The clinical picture is nonspecific, from pain to symptoms due to ureteral compression, this being the main complication associated.Imaging studies are essential in the diagnosis; computed tomography (CT) and magnetic resonance imaging (MRI) are the most reliable imaging modalities. The goal of treatment is to stop the progression of the fibroinflammatory reaction. The first line of treatment is usually with medical management. Biological agents, such as rituximab and infliximab, have also been used, even with scarce data in the literature. Surgery is usually performed to improve a ureteral obstruction and should always be accompanied by systemic steroid treatment. The conservative approach given by systemic therapy and ureteral stent placement or nephrostomies has been recommended, thus reserving surgical treatment for refractory cases.We present two clinical cases of idiopathic RPF, one of them associated with IgG4. Retroperitoneal fibrosis (RPF) comprises a spectrum of rare diseases, characterized by the presence of aberrant fibroinflammatory tissue, which usually develops around the infrarenal portion of the abdominal aorta and in the iliac arteries. It often encompasses neighboring structures such as the ureters and the inferior vena cava. The first description of this disease was made in 1905 by the French urologist Albarrán, who reported the surgical treatment of an extensive retroperitoneal fibrotic process that was causing ureteral obstruction . The disIdiopathic RPF has an annual incidence of 0.1 per 100,000 people and a prevalence of 1.4 per 100,000 inhabitants, being more frequent between 40 and 60 years old, with male predominance 3:1) [:1 [3]. IClinical case 1A 54-year-old man presented with a 20-day clinical picture of colic pain in the left flank irradiated to the mesogastrium, which did not improve with outpatient treatment. As background, he revealed repeated urolithiasis, with a report of renal and urinary tract ultrasonography, of a simple renal cyst. CT urogram ruled out lithiasic pathology, showing thickening of the abdominal aorta before bifurcation, and CT reported a simple left renal cyst and retroperitoneal mass in contact with the abdominal aorta, above the bifurcation, with a diameter greater than 4 cm, with no infiltration to the vessel wall and no evidence of distant involvement. The patient underwent laparotomy with findings of a retroperitoneal mass, attached to deep planes, which circumferentially involves the infrarenal abdominal aorta and the intrahepatic vena cava. An incisional biopsy was performed, given the unresectability of the lesion. Surgical pathology reported a hypocellular fusocellular proliferation without atypia, mitosis or necrosis, with trapped fat, with obliteration of vessels with abundant lymphocytes and plasmocytes Figure .The above was complemented with a genetic study of CDK4 12q13), MDM2 (12q15) gene amplification by fluorescence in situ hybridization (FISH), for suspected dedifferentiated liposarcoma, which was negative. The requested immunohistochemistry found negativity for ALK, Desmin, P.16, S100, CD34, AE1/AE3, HMB45, CD117, CD31, and CAM5.2, and frequent IgG4 plasmocytes . But complementary immunohistochemical studies for CAM 5.2, CD34, CD117, Desmin, S100, and HMB45 were negative. With the previous findings, a definitive diagnosis was made of a retroperitoneal fibro-plasmacytic lesion compatible with IgG4 disease. The patient continued postoperatively, with good clinical evolution, so he was treated in an ambulatory manner with endocrinology follow-up.Clinical case 2A 48-year-old male patient presented with no relevant history and a clinical picture of two months' evolution, characterized by a predominantly left abdominal pain, with the sensation of a mass. CT of the abdomen showed a retroperitoneal mass of infrarenal location, compromising the para-aortic, interaortocaval space in front of the aorta, extending to the aortoiliac bifurcation, with well-defined contours, surrounding the vascular structures without infiltrating them, 99 x 38 x 55 mm, and associated with left hydronephrosis . Cyclin D1 is negative, AE1/AE2 cytokeratins do not show epithelial cells, and smooth muscle actin is positive in myofibroblasts. Additional studies with desmin, beta-catenin, and ALK are negative. Ki67 in the fibrous component is less than 5%. It is concluded that due to morphological findings and immunohistochemistry, correlating with imaging and intraoperative data, they lead to mass-forming fibroinflammatory disorders of the idiopathic RPF type. Medical treatment was initiated with corticosteroids at a dose of 1 mg/day and clinical follow-up without associated progression or morbidity episodes.The term retroperitoneal fibrosis (RPF) commonly refers to a clinical-pathological entity characterized by a mainly sclerotic tissue that develops in the periaortic and peri-iliac retroperitoneum and often encloses structures such as the ureters and the inferior vena cava . RPF covIdiopathic RPF is part of the spectrum of the disease related to immunoglobulin G4 (IgG4), an immune-mediated disorder that can affect different organs  and that is histologically marked by a lymphoplasmacytic infiltrate rich in plasma cells carrying IgG4, fibrosis, tissue eosinophilia, and obliterating phlebitis and is also characterized by elevated serum IgG4 levels in a significant proportion of cases; some cases of idiopathic RPF have such histological and clinical characteristics and, therefore, can be classified as related to IgG4 , althougDifferent mechanisms have been proposed to explain the pathogenesis of idiopathic RPF. Starting as a manifestation of a systemic autoimmune disease and that which may arise as a primary aortitis that subsequently causes a periaortic fibroinflammatory response. Clinical findings, such as the presence of systemic symptoms, association with other autoimmune disorders, as well as the good response to immunosuppressive therapies, conform to this systemic theory mediated by the immune system . MultiplIn our case, they did not present associations with environmental, infectious, or medicinal factors. Histologically, fibrous and inflammatory tissue predominates in idiopathic RPF, which is composed of an extracellular matrix with type I collagen fibers. IgG4-related RPF is characterized by plasma cell proportion > 40% in addition to eosinophil infiltration .The correct diagnosis is made only when obstructive uropathy or renal failure occurs (42%-95%). In most patients, the presenting symptom is dorsal, flank, and/or abdomen pain. Other rare symptoms include fever, testicular pain, abdominal angina, intermittent claudication, edema, or macroscopic hematuria. Urinary tract obstruction can lead to acute hypertension in some patients, possibly due to an increase in renin release . In our Inflammatory markers, such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), are elevated in more than half of the patients and do not help differentiate idiopathic or secondary RPF . Unlike Imaging studies are essential in the diagnosis and treatment of RPG and can help differentiate between idiopathic and secondary disease. Ultrasound should be done as the first line of study, especially when evaluating a patient with renal failure. On ultrasound, idiopathic RPF presents as a hypoechoic or isoechoic mass, which can involve the ureters and, therefore, cause unilateral or bilateral hydronephrosis. CT and MRI are the most reliable imaging modalities for the diagnosis of idiopathic RPF .In CT without contrast, it usually appears as a homogeneous, isodense plate with muscle, which surrounds the lower abdominal aorta and the iliac arteries, wrapping the ureters and the inferior vena cava. In contrast, RPF secondary to malignant diseases tends to displace the aorta anteriorly and the ureters laterally. Magnetic resonance provides a better definition. Idiopathic RPF is hypointense in T1-weighted images; in weighted T2, its intensity is high in the early or active stages of the disease due to tissue edema and hypercellularity and low in the last stages . PositroThe goal of idiopathic RPF treatment is to stop the progression of the fibroinflammatory reaction in order to inhibit or improve the obstruction of the ureters or other retroperitoneal structures, the acute phase reaction, and its systemic manifestations. Corticosteroids are the first-line therapy, suppressing the synthesis of most cytokines involved in the acute phase reaction, reducing the inflammation and inhibition of collagen synthesis and maturation . CorticoWhen there is a contraindication for glucocorticoid therapy, a good alternative may be the use of tamoxifen . ImmunosSurgery is usually performed to improve ureteral obstruction; Open ureterolysis with intraperitoneal transposition and omental envelope of the ureters is considered the best surgical approach . SurgeryRetroperitoneal fibrosis is a rare disease that is difficult to diagnose and constitutes a therapeutic challenge for retroperitoneal diseases. The diagnosis is histopathological, where an inflammatory infiltrate is initially shown by lymphocytes, plasmatic cells, and macrophages. The possibility of a disease related to IgG4 should always be ruled out and its serological ratio established for confirmation. The diagnostic images of choice are CT and MRI, which show the classic compromise surrounding the aorta and ureter. The first-line management is medical with glucocorticoids; when there is a contraindication, tamoxifen, immunosuppressants, and biological agents are proposed. Surgical management has been reserved for refractory cases; conservative treatment with systemic therapy being the standard treatment. RPF is a benign entity with biological aggressiveness and low incidence but high morbidity, so it always needs to be managed in a center of high complexity, with experience in retroperitoneal tumors.


Small bowel volvulus is a rare entity and it is even rarer for the ileum to undergo torsion without any known predisposing factors. It presents as acute abdomen with features of intestinal obstruction. As it is a life-threatening condition, it should be kept as a differential for small bowel obstruction despite its rarity. Therefore, we report this case.A 60-year-old gentleman presented to our emergency department with a 2-day history of worsening abdominal pain, vomiting, abdominal distension and obstipation. Exploratory laparotomy was done which revealed ileal volvulus with no predisposing factors. Derotation of the segment was done. The postoperative period was uneventful and on follow up after a month, he had a satisfying recovery.Though primary ileal volvulus is a rare diagnosis, it should be kept in mind in any patient with small bowel obstruction with pain out of proportion and resistant to opioid management. Early diagnosis and urgent surgical intervention is the key to prevent bowel necrosis and associated morbidity and mortality. Small bowel volvulus (SBV) refers to the abnormal twisting of a loop of small bowel about the axis of its mesentery –4. This A 60 years old Muslim gentleman with no known co-morbidities presented to our emergency department with a 2-day history of worsening generalized abdominal pain with abdominal distension and absolute constipation associated with few episodes of non-bilious vomiting. There was no history of previous abdominal surgery, fever, per rectal bleeding, unintentional weight loss or similar abdominal pain in past. However, he gave history of ingestion of a large fiber-rich diet after a period of fasting for religious reasons few days back. He had tachycardia of 110 beats per minute with the remaining vital signs within normal limit and on examination of the abdomen; generalized abdominal distension with tenderness and rebound tenderness was noted. Bowel sounds were sluggish and per-rectal examination was normal. Blood investigations revealed normal hemogram with hyponatremia. Plain X-ray of abdomen showed multiple air-fluid levels, dilated proximal small bowel loops and absence of gas in colon (Fig. A clinical diagnosis of complete bowel obstruction was made, the patient was resuscitated and exploratory laparotomy was performed with a midline incision. Minimal reactive ascitic fluid was present. It was noted that there was 720° anticlockwise rotation of a 20 cm segment of the distal ileum around its mesentery forming a volvulus about 35 cm proximal to the ileocaecal junction (Fig. Volvulus is a cause of dynamic intestinal obstruction resulting from torsion of a loop of bowel around the axis of its own mesentery , 2, 4. WOn the basis of etiology, SBV can be primary or secondary. Volvulus is considered to be primary in the absence of predisposing factors and is most commonly seen in children and young adults, whereas secondary SBV occurs in the setting of an underlying lesion upon which the mesentery can rotate. Various theories have been postulated to explain the occurrence of primary SBV such as long mobile mesentery with a shorter mesenteric base, strong abdominal muscles, increased peristaltic tone and ingestion of a large volume of fiber-rich diet after prolonged period of fasting. Volvulus may occur secondary to adhesions, fibrous band, Meckel’s diverticulum, congenital malrotation of gut, tumors, mesenteric lymph nodes, parasitic infestations, internal hernias, lipomas, pregnancy, endometriosis and hematomas , 9, 10. The most common presentation is that of an acute abdomen with central abdominal pain out of proportion to the physical findings and resistant to narcotic analgesia along with features of small bowel obstruction , 4, 10. Plain abdominal radiographs are nonspecific and fail to demonstrate the cause of small bowel obstruction , 4. ContEmergency surgery is the treatment to be undertaken with the aim of untwisting the volvulus and re-establishing the blood flow . If the Depending on the time delay before surgical intervention, the mortality rate of SBV ranges from 9 to 35% but with gangrenous bowel, rates as high as 20–100% have been reported .In spite of being a rare entity, primary ileal volvulus should be kept in differential for small bowel obstruction. Early diagnosis and urgent surgical intervention remain the cornerstone for prevention of bowel necrosis and associated increased morbidity and mortality.


Here, we discuss a relatively uncommon presentation of a hepatocellular carcinoma and discuss its preoperative planning and surgical intervention required to reach complete resection. Here, we discuss a relatively uncommon presentation of a hepatocellular carcinoma and discuss its preoperative planning and surgical intervention required to reach complete resection. Although rare, patients may present with large, pedunculated hepatocellular neoplasms that extend into the intra‐abdominal space. Here, we present a case report on a 66‐year‐old male who was found to have such a mass that required careful pre‐operative planning to complete a challenging, yet worthwhile, surgical intervention with R0 resection. A 66‐year‐old Caucasian male with a history of Hepatitis C presented with 4‐5 months of abdominal pain, distention, and weight loss. Imaging revealed a large heterogenous mass attached to the left lobe of the liver, with compression of the stomach, transverse colon and abdominal wall. Biopsy revealed hepatocellular carcinoma with extensive necrosis and ultimately the patient required surgical intervention, which required resection of omentum, partial gastrectomy, hepatectomy, and extensive dissection of the mass to separate the tumor from the remaining intra‐abdominal structures and the retroperitoneum. Peduncular hepatocellular carcinomas vary in presentation and difficulty of resection. The prognosis and probability of successful surgical intervention depends on the level of differentiation, early staging, size of the neoplasm and invasion into surrounding structures. In this case, R0 resection was successfully performed.Hepatocellular carcinoma is associated with hepatitis viral infection, alcohol use, metabolic syndrome, and non‐alcoholic fatty liver disease. Pedunculated Hepatocellular carcinoma is a rare subtype, which can be further categorized by its presence or absence of a pedicle attaching it to the liver. Its histological classification, as well as its clinical staging, determines patient prognosis. We report a case of pedunculated hepatocellular carcinoma, which required careful pre‐operative planning and extensive surgical intervention to achieve R0 resection.2®) initially presented to the Medical Oncology team in Hackensack University Medical Center, NJ with abdominal discomfort and distention for 45 months associated with an approximate 15 pounds of weight loss. He underwent CT scan of the abdomen with contrast and was found to have a large 15.0 × 11.0 × 13.0 cm heterogenous mass attached to the left lobe of the liver, with compression of surrounding intra‐abdominal contents including the stomach and transverse colon, as well as the abdominal wall , a foley catheter was reinserted. He had also developed an ileus requiring a nasogastric tube, which was removed four days later. The patient developed serosanguineous drainage from his incision site after a bout of heavy coughing. A Prevena VAC was placed but was discontinued one week later when the output was reduced to zero, and he was discharged from the hospital later in the day.3Hepatocellular carcinomas (HCC) are aggressive tumors prone to metastasis and make up approximately 75% of primary hepatic malignancies,Hepatocellular carcinoma is histologically classified into four grades using the Edmonson‐Steiner Classification. Grade I carcinoma includes a mostly‐differentiated tumor which is arranged in a thin trabecular pattern. Grade II shows enlarged nuclei with abundant cytoplasm, with cells maintaining trabecular pattern. Grade III carcinoma demonstrates large, hyperchromatic nuclei with less bile and acinar formation. The hallmark of grade III disease is the abundance of tumor giant cells. Grade IV carcinoma is poorly differentiated, with tumor cells varying in cytoplasmic abundance and quality, along with near absence of bile. These cells are disorganized, and cohesion molecules are often absent.Pedunculated HCC (P‐HCC) presents as two subtypes either with or without a pedicle.Hepatocellular carcinomas causes chronic inflammation and persistent cytokine releaseSurgical resection has been shown to be curative and is considered first‐line therapy if R0 resection is possible to achieve. Such interventions depend on the size and location of the tumor, as well as the pre‐operative liver function and these patients require evaluation for post‐resection liver volume.4We report a 66‐year‐old male presenting with a large, peduncular intra‐abdominal hepatocellular carcinoma extending into the retroperitoneum with satellite lesions requiring an exploratory laparotomy, resection of the tumor, partial gastrectomy, and hepatectomy. Surgical intervention was ultimately therapeutic despite the technical difficulty of achieving R0 resection.None declared.The DO, JD, XC, HZ and DAM all contributed to the manuscript, review of literature, acquisition of data, analysis and interpretation of data. All authors have given final approval of the version to be published and agree to be accountable for all aspects of the work.


Renal metastases are very rare condition in the clinical practice. The treatment is individualized and it depends on general status of the patient, involving of other organs and also the control of primary tumor. We are presenting a 64 years old woman, who had episodes of intermittent hematuria for two weeks. CT scan showed a large heterogeneous left kidney mass. The patient underwent open transabdominal radical left nephrectomy and the final diagnosis was isolated lung adenocarcinoma metastasis of the kidney. Renal metastases are very rare condition in the clinical practice. Moreover, isolated metastases in the kidney are extremely rare. The primary localizations of the tumors that metastasize most common in the kidney are from the lung, breast, digestive tract, melanomas and lymphomas, but also some rare cases with other etiology have been reported. The treatment is individualized and it depends on general status of the patient, involving of other organs and also the control of primary tumor.We are presenting a 64 years old woman, who was admitted in our Urology Department with symptoms of recurrent left lumbar pain, irradiating towards inguinal area for one week. The patient also complains from nausea, without vomiting and weight loss. She had episodes of intermittent hematuria for two weeks. Upon physical examination no abdominal mass was palpated and the bowel sounds were diminished in all quadrants. All laboratory results were in normal ranges.The patient has a history of right upper lobectomy for right lung adenocarcinoma (pT1aN0M0) before 1 year and the histological examination showed adenocarcinoma of the lung with no metastasis found in the dissected lymph nodes. The patient underwent chemotherapy and had 1 year of disease-free interval on postoperative follow-up examinations.The contrast computed tomography (CT) scan of the chest and abdomen showed a large heterogeneous left kidney mass around 8 cm in diameter, engaging the upper pole and enhanced its density after the contrast no other pathological findings . There wThe patient underwent open explorative transabdominal radical left nephrectomy with preliminary diagnosis of renal cell carcinoma. The whole tumor was removed using sharp and blunt dissection. Histopathology results showed a metastatic adenocarcinoma, which coincided with the histological findings of the previous surgery of the left lung . The finSince the renal blood flow accounts for approximately 20% of cardiac output is assumed that the kidneys may be affected by hematogenous metastases.The most frequent symptoms of the metastatic tumors of the kidney are abdominal or flank pain, hematuria, weight loss, sweats and fever. Also in most of the patients with renal metastases there are no specific symptoms.With regards to small cases reported in the literature, there is no clear guideline available for management of renal secondary tumors. Chemotherapy guided to the primary tumor can be useful, although results are usually poor. The role of solitary nephrectomy for metastasis in kidney has been unknown in detail because comparison of surgical versus nonsurgical treatment in the management of solitary metastatic disease in prospective randomized studies is almost impossible.Renal metastases are rarely seen in medical practice and that is way it is difficult to diagnose and choose the right treatment. Management of the disease is individualized and the nephrectomy can be a good option in selected cases.The authors declare that they have no competing interests.


Introduction. Ovarian carcinosarcoma (OCS), also known as malignant mixed Müllerian tumour (MMMT), is one of the rarest histological subtypes of ovarian cancer. It is an aggressive tumour with a dismal prognosis—the median survival of patients is less than two years. The rarity of the disease generates many controversies about histogenesis, prognostic factors and treatment of OCS. Histologically, OCS is composed of an epithelial and sarcomatous component. Case report. In the present case, a patient with bilateral ovarian cysts and bulky paraaortic lymph nodes is reported. Retroperitoneal paraaortic lymph node metastases were the only extrapelvic dissemination of OCS. The patient underwent comprehensive surgical staging procedures, including total abdominal hysterectomy and bilateral salpingo-oophorectomy, supracolic omentectomy and selective para-aortic lymphadenectomy. Histologically the ovarian carcinosarcoma was composed of an epithelial component (high-grade serous adenocarcinoma) and three sarcomatous components . Immunohistochemistry staining was performed. A postoperative complication  that has never been reported in the medical literature occurred. Conclusion. Carcinosarcomas are carcinomas with epithelial–mesenchymal transition and heterologous differentiation. Retroperitoneal pelvic and paraaortic lymph nodes should be carefully inspected in patients with ovarian tumours. Adhesions between the small bowels and abdominal aorta are possible complications after lymph node dissection in the paraaortic region. Ovarian cancer is the fifth most common cause of cancer death among women, and ninety percent of ovarian cancers are of an epithelial cell type, while sex cord-stromal tumours and malignant ovarian germ cell tumours are relatively rare . OvarianA 53-year-old postmenopausal woman  presented at the Department of Gynecology with a short history of abdominal pain and abdominal distension. She had no relevant history of urogenital malignancy and no vaginal bleeding. She did not have a medical history of gynaecological problems, hormone therapy, or any major medical diseases—diabetes mellitus, cardiovascular, or renal issues. Gynaecological examination revealed two large palpable, relatively mobile, non-tender masses in the pelvic region. Abdominal and vaginal ultrasonography showed two ovarian tumours of maximum diameter—left tumour 12 cm, right tumour—10 cm, composed of solid and non-solid masses with mixed echogenicity—hypoechoic and hyperechoic components. There was free fluid in the pouch of Douglas. According to the International Ovarian Tumour Analysis (IOTA), bilateral ovarian tumours were classified as malignant: irregular solid tumours, presence of ascites, and very strong blood flow ,9. The cComputed tomography and measurement of tumour marker levels were not performed. Laboratory data results were normal. Suspicion for ovarian malignancy prompted an exploratory midline laparotomy from above the umbilicus to the symphysis pubis . During The gross appearance of the ovarian tumours was soft and fleshy, with haemorrhage and necrosis. Both fallopian tubes were infiltrated, while the uterus was without macroscopic pathological changes. A histopathological examination revealed OCS in both ovaries, infiltrating both fallopian tubes. Histologically, the OCS was composed of epithelial component (high-grade serous adenocarcinoma) and three sarcomatous components . Immunohistochemistry (IHC) staining for cytokeratin shows diffuse intense staining of the epithelial element, while desmin staining exhibits rhabdomyosarcomatous components. Histologically, rhabdomyosarcomatous components were predominant at more than 25%. The expression of p53 protein was negative for both sarcomatous and carcinomas components. The number of para-aortic lymph nodes removed en masse during lymphadenectomy was 19—all with metastatic cancer spread from the epithelial component (high-grade serous carcinoma). Greater and lesser omentum and uterus were without metastases. Cytological findings from the ascites revealed adenocarcinoma cells. The patient was diagnosed with surgical stage IIIA1 according to the latest International Federation of Gynecology and Obstetrics (FIGO) classification, and pT2aN1bMx, according to the TNM classification . On postOn post-surgery day 15, the patient was admitted again at the Department of Gynecology with abdominal distension, abdominal pain, fever 38.2 °C, vomiting, and reduced intestinal peristalsis. Abdominal and vaginal sonography showed free fluid in the abdominal cavity. Computed tomography demonstrated postoperative ileus and a high amount of free fluid in the abdominal cavity. The patient’s laboratory results revealed hyperproteinaemia and hypoalbuminemia. The chest X ray was without abnormalities. After surgeon consultation, conservative treatment was initiated. Relaparotomy was performed as the patient’s symptoms persisted for 72 h. Abdominal wall dehiscence at the level of the umbilicus was observed. During the operation, up to 4 L of lymph fluid were evacuated from the abdominal cavity. A cytology and microbiology examination was obtained. Sigmoid and transverse colon were dilatated. Adhesion between the terminal ileum and the abdominal aorta just below the superior mesenteric artery was noticed. Adhesiolysis was achieved. Nasogastric intubation was obtained. Two drains were left in the abdominal cavity. The abdominal wall was closed and reinforced with synthetic mesh. Metal skin staples were also used during abdominal surgical wound closure. Postoperative empirical antibiotics—ceftriaxone , and metronidazole , and albumin infusion therapy was initiated. On the next three postoperative days, the patient was subfebrile with normal peristalsis and diuresis. The nasogastric tube was removed. Total blood loss was 300 mL. Microbiology and cytology examinations detected no bacterial flora and no tumour cells. On post-surgery day 7, drains were removed. On post-surgery day 10, the patient was discharged without complaints . On postOCS is a rare tumour, which represents one of the most highly aggressive cancers of the female genital tract with poor long-term prognosis . OCS is It is hard to distinguish MMMT and ovarian cancer preoperatively, as both malignancies have the same clinical and radiological findings . The cliThree theories have been suggested to explain the histogenesis of gynaecological carcinosarcoma: biclonal (collision) and monoclonal (combination and conversion) theories. The collision theory suggests that carcinoma and sarcoma are two independent tumours originating from a separate cell, which later merge. The combination theory assumes that sarcoma and carcinoma components are derived from a single stem cell and undergo divergent differentiation early in tumour evolution. The conversion theory states that an epithelial cell undergoes metaplasmic differentiation that initiates tumour genesis and gives rise to a sarcomatous component, and these tumours should be regarded as dedifferentiated carcinomas of the ovary ,12,14. LHistologically, OCS is composed of an epithelial , as well as of a sarcomatous component . Immunohistochemistry (IHC) staining for cytokeratin and epithelial membrane antigen showed diffuse intense staining of the epithelial element, while the mesenchymal component usually stains for vimentin, smooth muscle actin, desmin, and focal cytokeratin ,19. PosiThe rarity of the disease generates many controversies about the prognostic factors of OCS. Some studies conclude that older age, advanced stage at presentation, and suboptimal surgical resection are associated with poor outcome ,14. HighIn the present case, the patient had 7 favourable , and 6 unfavourable prognostic factors . Tests for Ca-125 levels, Ki67 reactivity, VEGF, and VEGFR-3 expression were not performed.The treatment suggestion of MMMT is based mainly on small retrospective studies or extrapolations from research on epithelial ovarian cancer or uterine carcinosarcoma . The iniEstablishing the most effective adjuvant chemotherapeutic regimen in OCS remains elusive ,35. Plat2/carboplatin (three to nine cycles) is the preferred adjuvant chemotherapy regimen for OCS stage I. Other recommended regimens are carboplatin/liposomal doxorubicin or docetaxel/carboplatin. In certain circumstances carboplatin/ifosfamide, cisplatin/ifosfamide, and paclitaxel/ifosfamide might be used [Currently, the National Comprehensive Cancer Network (NCCN) guidelines recommend that paclitaxel 175 mg/m be used . In stag be used .Neoadjuvant chemotherapy should be considered if optimal debulking cannot be achieved, or for patients who are poor candidates for surgery due to a high risk of operative morbidity or extra-abdominal disease . IntraveThe necessity for adjuvant radiotherapy  has not shown any overall survival benefit but has been reported to decrease local recurrence ,7. AlthoGeisler et al. examined nine cases of OCS and found receptors for oestrogen in six of the cases and no receptor for progesterone . In anotGiven the rarity of the disease, there are limited data regarding the relevance of targeted therapies. Overexpression of COX-2, HER-2/neu, C-kit in carcinosarcomas has been reported . ConsequIn the present case, the patient underwent optimal cytoreductive surgery with no macroscopically residual disease and started first-line platinum-based adjuvant chemotherapy paclitaxel/carboplatin/bevacizumab.The treatment of OCS is summarized in In the present case, optimal cytoreduction was performed. Metastatic para-aortic lymph nodes were the only extrapelvic tumour dissemination. Ligation of the inferior mesenteric artery (IMA) could be applied without any clinical consequences. Surgeons ligate IMA either directly at the origin of the IMA from the aorta (high ligation), or at a point just below the origin of the left colic artery (low ligation) . There aSmall bowel obstruction/ileus is a common reason for readmission after optimal cytoreductive surgery for epithelial ovarian cancer, especially after para-aortic lymphadenectomy ,50. The There are many studies suggesting prevention of postoperative ileus ,53,54,55Currently, enhanced recovery after surgery (ERAS) protocols were adopted in gynecologic oncology. ERAS was first used in colorectal surgery and later in urology, vascular surgery and gynecology. ERAS reduce the rate of postoperative ileus by series general measures, such as early feeding, avoidance of mechanical bowel preparation, opioid-sparing pain control, utilization of preoperative patient education, optimization of comorbid conditions, euvolemia, normothermia ,52,55.The strengths of the present case include: a brief review of OCS pathogenesis, prognosis, histology, and treatment summarized and illustrated in tables and figures. Retroperitoneal para-aortic lymph node metastasis is the only extrapelvic dissemination of OCS. The postoperative complication described has never been reported in the medical literature. The limitations of this case report include the following: levels of Ca125, preoperative computed tomography, clonality studies, patterns, genomic analysis, gene mutations and expressions were not performed.OCS is a rare disease with a dismal prognosis. Retroperitoneal, pelvic, and para-aortic lymph nodes should be closely inspected in patients with ovarian tumours. Retroperitoneal para-aortic lymph node metastasis could be the only extrapelvic dissemination of OCS. There are limited data on aetiology, diagnosis, prognostic factors and treatment of OCS. However, studies concluded that prognostic factors and treatment of carcinosarcomas are associated with epithelial components, as carcinosarcomas are carcinomas with epithelial–mesenchymal transition and heterologous differentiation ,44,45,46


Gastrointestinal (GI) xanthomas are rare entities found incidentally on endoscopy. There have been only a few cases where they presented with symptoms of bowel obstruction. We present a case of an 89-year-old woman with a history of short-gut syndrome due to partial colectomy who had multiple admissions with recurrent nausea/vomiting, abdominal distension, and bloating. She was found to have multiple, large, mass-like xanthomas in the rectosigmoid colon. The unusual location, mass-like lesions, and large size led to luminal narrowing causing the patient to have obstructive symptoms, which is a very unusual presentation of colonic xanthomas as most are discovered incidentally. Xanthomas are yellowish-white lesions found in different parts of the body especially on the skin. These lesions are due to the accumulation of fat tissue. Under the microscope, xanthomas consist of foamy histiocytes . The mosThis is a case of an 89-year-old woman, with a past medical history of short-gut syndrome secondary to partial colectomy at age 58, who presented to the hospital with complaints of nausea and vomiting of three days duration accompanied with generalized weakness. Upon presentation, her vitals were normal. On examination, her abdomen appeared distended with hyperactive bowel sounds. Blood work was notable for hyponatremia with sodium of 120 mEq/L. A CT scan of the abdomen revealed marked wall thickening of the rectosigmoid colon consistent with diffuse colitis Figure .A fasting lipid panel was obtained which was within normal limits. The patient's vomiting and electrolyte abnormalities resolved with bowel rest and IV fluids. Upon chart review, the patient had previously been admitted to the ED with similar complaints on multiple occasions. During each visit, imaging was significant for colitis, and colonoscopy had revealed sigmoid masses proven to be xanthomas on histopathology. The patient was given bowel rest which resolved her symptoms and she did not have to undergo surgical intervention. On discharge, she was advised to regularly follow up with a gastroenterologist and she was given a high fiber diet by a nutritionist to help prevent recurrence of her symptoms.Xanthomas have been seen in different parts of the GI tract. Although true incidence is unknown, these lesions occur more frequently in the stomach with relative sparing of other parts of the gut . The larA major study that looked at rectosigmoid xanthomas was reported by Miliauskas that described 13 cases . The stuThe largest case series about colonic xanthomas has been described by Nakasono et al. who has described 28 biopsy-proven colorectal xanthomas in 25 patients . In theiThe patient in our study is unique with respect to the clinical presentation and location of the lesions. While most of the above cases report incidentally found lesions, our patient presented with symptoms consistent with bowel obstruction such as nausea, vomiting, bloating, and abdominal distension. The sigmoidoscopy revealed that the xanthomas were much larger compared to previous studies, looked like masses, and might have had a mass effect Figure . The CT As discussed above, there have been rare reports of colorectal xanthomas in the past but the majority of those were incidental findings in routine colonoscopy without any symptoms. Furthermore, none of the xanthomas appeared as masses. Gastroenterologists should always keep this diagnosis as a differential in mind when they encounter lesions concerning for malignancy in a patient presenting with symptoms of bowel obstruction.The GI tract xanthomas are mostly found in the upper tract. Xanthomas of the rectosigmoid colon are rare. As discussed in the case above, a patient can present with symptoms of intestinal obstruction if the xanthomas are large in size or if they are present at ileocecal junction. Xanthomas should always be in the differential diagnosis of a mass-like lesion found on endoscopy or colonoscopy. A pathological evaluation of the mass-like lesion is recommended.


Embryonal Rhabdomyosarcoma (ERMS) is a malignant soft tissue musculoskeletal tumor which constitutes about 0.06% of all malignancies affecting children. Biliary tract ERMS is still rare, though it is considered the most common cause of malignant obstructive jaundice in children.A report of a 2-year-old boy, who was presented with recurrent episodes of scleral icterus of three months duration, is added to the related literature. His labs went with obstructive jaundice and the radiological investigations were consistent with a diagnosis of choledochal cyst. The found mass was suspected to be an ERMS of common bile duct and turned out to be so by the histopathology. He was managed totally by laparoscope, both excision and hepaticojejunostomy reconstruction, which is an extremely uncommon entity.Common Bile Duct Rhabdomyosarcoma is rare and diagnosis at this anatomical site is difficult. Our case highlights the feasibility of laparoscopic resection and hepaticojejunostomy reconstruction with very good results at 16-month follow up and parents' gratitude as well. •Rhabdomyosarcoma (RMS) is a rare malignant tumor affecting children.•RMS is worth considering when dealing with a choledochal cyst.•Surgical approaches reported for biliary RMS were laparotomies.•Laparoscopic management is feasible for biliary RMS.•Our case is the least invasive surgical approach reported up to date. However, they are rare as they constitute about 3% of all malignancies affecting pediatrics , with meERMS looks like the radiological and clinical features of Choledochal Cyst (CC); diagnosis is usually made at surgery . The manWe are reporting a case of a 2-year old boy presented with attacks of jaundice because of CBD ERMS that was diagnosed initially as CC, managed totally with laparoscopic excision and Roux-en-Y Hepaticojejunostomy (RYHJ).This work has been reported in line with the Surgical Case Report (SCARE) criteria .2A 2-year-old boy, not known to have any medical or congenital diseases, referred to our tertiary Hospital by his family physician due to recurrent attacks of scleral icterus for 3 months, almost with same intensity, with no fever, abdominal pain, and change in stool nor urine color. He has no history of allergy and his parents denied regular or recent drug intake. The family and psychosocial history were unremarkable. His physical examination was unremarkable, except yellowish sclera bilaterally. Laboratory tests revealed mild elevation of white blood cells, elevated levels of total bilirubin, direct bilirubin, and alkaline phosphatase. Abdominal ultrasound showed fusiform dilatation of the Common Bile Duct with sludge consistent with CC type I, the liver and spleen were homogeneous with no focal lesions and the gall bladder was partially contracted with no stone inside. A diagnosis of CC was further supported by magnetic resonance cholangiopancreatography (MRCP). After informed consent signed by his father, the patient was booked for minimally invasive CC excision with Roux-en Y Hepaticojejunostomy (RYHJ) surgery.2 pneumoperitoneum was established at a pressure of 8 mmHg and flow of 5 L/min. Another 10 mm port was placed just below the left costal margin medially, two additional 5 mm trocars were inserted in the right lower quadrant and an adequate exposure was achieved by retracting the liver and elevating the gallbladder to allow dissection of the CC and freeing it from the surrounding structures. The CBD was 3 cm fusiform in shape, with the help of camera magnification meticulous dissection and resection of proximal part of common hepatic duct was done, almost up to the confluence of right and left hepatic that permit anastomosis, with small lymph nodes (LN) around, this approach gave us superior field view over open approach. CBD was divided after clip placement distally as close as possible to pancreaticobiliaery junction.This procedure performed by a consultant pediatric surgeon subspecialized in minimally invasive surgery in pediatric age group at one of reputable centers in Europe, has a good audit of laparoscopic CC excision and Roux-en-Y hepaticojujonostomy reconstruction. The patient was placed in a supine position, an infra-umbilical incision by open Hasson technique used for 10 mm trocar, 30° lens, and CoA grapelike soft tissue mass that appeared within the excised part of common hepatic duct lumen made a new diagnostic challenge that raised the possibility of biliary RMS, while the procedure went through same plan; the gall bladder was freed and excised with the sample en-block {On the first post-operative day, the general condition of the patient went uneventful, with 16.6 μmol/L total bilirubin and 14.2 μmol/L direct bilirubin, 1045 IU/L alkaline phosphatase, post-operative course went smooth and feeding began on day five post-operative. The Histopathologist confirmed biliary embryonal RMS tumor with cells at the resection margin and tumor free two Lymph nodes, the tumor cells were positive for Myogenin and Desmin immunostains {3RMS is a rare malignant tumor affecting children, forming about 2% present at birth , it is dAround one hundred cases of CBD RMS reported in literature , the larThe management of RMS is complicated and requires multimodal treatment that includes surgery, chemotherapy, and radiotherapy .Complete removal of the main tumor is the goal whenever possible. In some cases, surgery may be done even if it's clear that all of the cancer can't be removed “functional resection”. As re-operating on the patient due to incomplete resection was accompanied with poorer prognosis, meticulous dissection with good camera magnification i s crucial and offers careful intra-surgical assessment to choose the most suitable intervention in order of resecting the tumor and guarantee optimal outcomes. The safety of laparoscopic CC management and feasibility porta hepatis anastomosis has been proved in most recent reports ,11; the Chemotherapy is needed for every RMS patient, regimens vary by the risk group . MoreoveTo the best of our knowledge, there is only one report advocate meticulous dissection and excision of CC contains RMS using Robot, while the procedure converted to open for hepaticojejunostomy reconstruction . We are 4Rhabdomyosarcoma is worth considering when dealing with choledochal cyst. Surgical approaches reported for biliary Rhabdomyosarcoma were laparotomies. Laparoscopic hepaticojejunostomy reconstruction is feasible when facing a grapelike tumor inside the common bile duct. The report of Nakib et al. makes us believe that our case is the least invasive reported one up to date.Ethical approval, IRB and informed consent obtained from patient father had been taken under Ref. 28/127/2019, date October 02, 2019.Written informed consent was obtained from the patient father 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.1. Name of the registry: Research Registry.2. Unique Identifying number or registration ID: 5870.https://www.researchregistry.com/browse-the-registry#home/3. Hyperlink to your specific registration (must be publicly accessible and will be checked): Thekraiat Majed Al Quran.Written informed consent was obtained from the patient father 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.None.Not commissioned, externally peer reviewed.Al Quran Tm: Conceptualization, Writing - original draft, design and, Methodology, Supervision, Resources, collection, Writing - drafting initial manuscript, critical revision the manuscript, Agreed and approved the final draft for submission. Rousan LA: Supervision, Writing - original draft, Resources, collection, Critical revision the manuscript, Agreed and approved the final draft for submission. A.M. Aljaafreh: Supervision, Writing - review & editing, Resources, collection, Reviewing and editing the manuscript, Agreed and approved the final draft for submission. Bataineh Za: Conceptualization, methodology, Resources, Writing - original draft, Writing - review & editing, Supervision, Project Administratio, Writing - original draft, Writing - review & editing, Visualization, Supervision, Project Administration.All authors have NO financial, personal nor conflicts of interest to disclose.All authors disclose that NO funding to be declared.


Primary extranodal marginal zone B-cell lymphomas (MALToma) of the endometrium are rare tumors. We report a case of MALToma diagnosed within an endometrial polyp in a patient presenting with postmenopausal vaginal bleeding. The patient underwent a conventional hysteroscopic procedure for intrauterine polyps. There was no suspicion of malignancy during the preoperative investigations or at surgery. Conventional bipolar resection of the polyps was performed. The present case demonstrates that conservative management of endometrial MALToma may be considered as a safe alternative to hysterectomy. Further data on long-term follow up is needed to confirm the safety of this conservative approach. Extranodal lymphoma refers to a group of neoplastic disorders arising from sites which normally do not contain : mucosal-associated lymphoid tissue (MALT). It can develop anywhere in the body, the skin, stomach, brain, small intestine and the Waldeyer’s ring being the most common sites .In this report, we discuss an exceptionally rare case of MALT lymphoma (MALToma) in a woman who presented with postmenopausal bleeding.A 60-year-old postmenopausal woman was referred to our Institution  complaining of postmenopausal vaginal bleeding. Her BMI was 28. She had a history of hypertension and smoke approximately five cigarettes a day. Her obstetrical history consisted of two vaginal deliveries, a first trimester spontaneous miscarriage and an ectopic pregnancy treated with salpingectomy.The initial workup included a transvaginal ultrasound (TVS) that revealed a normal size uterus and bilateral normal adnexae; however, the endometrium appeared thickened with two focal hyperechoic, avascular areas of about 1 cm each, suggestive of endometrial polyps. The patient underwent an outpatient diagnostic hysteroscopy without anesthesia, using a 4 mm continuous-flow hysteroscope . However, the procedure was aborted due to intolerable pain. Following this, the patient was taken to the operating room for hysteroscopic polypectomy under anaesthesia. The procedure was performed using a 10 mm GYNECARE ® resectoscope with bipolar GYNECARE VERSAPOINT TM ; saline solution was used at 50 - 70 mmHg automated constant intrauterine pressure regulated by Endomat . The cervical canal was free of lesions, whereas in the uterine cavity two small polypoid lesions with a mean diameter of <1cm were identified. The remaining endometrium appeared atrophic. Both polypoid lesions were removed using bipolar electrical resection; the base of the lesion was sent to pathology as a separate specimen for analysis. Several random endometrial biopsies were also obtained .Macroscopic examination showed two small endometrial polyps (6 x 4 and 10 x 5 mm in size) with a normal vascular pattern. Microscopic examination revealed two glandular endometrial polyps with the larger one containing lymphoid tissue consistent with extra-nodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma) . MALTomaImmunohistochemistry demonstrated neoplastic cells positive for CD20, CD43 and BCL2, without displaying immunoreactivity for CD3, CD10, BCL6, BCL10 and CD138 . Ki-67 sTo the best of our knowledge, there have been only three cases of endometrial MALToma in polyps reported in the literature: two were treated with hysterectomy and one with hysteroscopic resection of the lesions . Hence, Primary malignant lymphomas of the genital tract are exceptionally rare neoplasms with only a few cases described in literature . To thisTo the best of our knowledge, this report is the fourth case of MALToma identified in the core of an endometrial polyp .Annibali et al.  first deIt’s important to note that, when considering all four cases of MALToma (including ours) reported inside an endometrial polyp, all have been reported in postmenopausal women. As such, two patients were asymptomatic, whereas the other two presented with postmenopausal bleeding. None of the patients suffered from other conditions which can be associated with MALToma such as autoimmune or infectious diseases. .Our choice of conservative management warrants some consideration. In two similar cases, hysterectomy was the treatment of choice for MALToma confined to endometrial polyps, whereas Annibali et al.  was the Since there is lack of specific recommendations for the management of endometrial MALToma, we decided to follow the National Comprehensive Cancer Network (NCCN) guidelines for non- gastric MALT lymphomas. These guidelines state that surgery might represent an appropriate initial treatment for MALToma and observation could be considered in case of negative margins after surgery . In thisAfter opting for conservative management of a rare tumor, an optimal follow-up strategy must be planned. In our case, the patient agreed to follow-up with office diagnostic hysteroscopy and endometrial biopsy every 6 months for the first five years after surgery and, if negative, an annual follow-up. She was also encouraged to immediately alert our team in case of recurrence of postmenopausal bleeding. This follow-up strategy is generally well-received by the patients, as diagnostic hysteroscopy can be performed in an office setting with miniaturized instruments without the need for anaesthesia . NeverthIn conclusion, MALToma is a rare condition within an apparently normal endometrial polyp. Its management must be based on the risk of residual disease and patient’s desire of preserving the uterus. When the base of the polyp as well as multiple endometrial samplings are negative for pathology, conservative management of this condition may be considered as a safe alternative to hysterectomy. If conservative management is elected, an individualized follow-up plan should be put in place to exclude disease recurrence. Overall, more data on long-term follow up of such cases is required in order to confirm the safety of the conservative management of this exceptionally rare condition.Informed consent was obtained from the patient to publish this case. The IRB  was consulted for approval to publish and advised that IRB approval was not necessary for this case report.


At a certain juncture, when clinicians are unable to gather information about a particular disease especially due to patient's unobtrusive findings, the presence of an aberrant connection might yield a diagnosis. Herein we present the findings of an unusual enlightening case of an 81-year-old Caucasian male with a history of bowel resection, poor appetite, generalized weakness, ptosis, and two weeks of weight loss. The computerized tomography scan revealed several sets of an abdominal intramuscular fluid collection with enhancements in the surrounding, indicative of several abscesses, and brain and spine magnetic resonance imaging indicated right-orbital metastasis in the superior rectal muscles. A biopsy of the cystic lesion of the anterior abdominal wall revealed poorly differentiated metastatic adenocarcinoma, most consistent with the primary pancreaticobiliary origin. This case report sums up this innovative portrayal of metastatic cancer as an intramuscular fluid collection. An estimated 57,600 adults, consisting of 30,400 males and 27,200 females, are predicted to be diagnosed in 2020 with pancreatic carcinoma in the United States (US) , 2. The Pancreatic cancer patients are estimated to have a five-year survival rate of 9% ,2. AmongTransabdominal ultrasound (US) is an appropriate first test in patients with obstructive jaundice, but typically it would not display the tumor. Helical computed tomography (CT) scanning for visualizing the pancreas and associated structures is usually required. Although its spatial resolution is inadequate to assess the degree of local tumor invasion, it is the most effective tool to rule out the existence of distant metastases . EndoscoAn 81-year-old Caucasian male with a past surgical history of bowel resection (post road traffic accident at a very young age) was admitted with poor appetite, generalized weakness, and weight loss for two weeks. The patient had 30 pack-years of smoking history, however, he denied alcohol use. On examination, he was cachectic and found to have right eye ptosis and multiple non-tender, non-erythematous lumps of about 2 cm in diameter on the left arm, left scapular area, and abdominal wall. His vitals were 98.5 F/36.9 C (temperature), blood pressure was 118/64 mm Hg, and 94/min pulse rate. Respiratory rate was 16/min, and oxygen (O2) saturation was 94% on room air. At the time of admission, laboratory data showed elevated white blood cells (WBC) of 19,000, hemoglobin (Hb) 12.6, platelet count of 708, and a bilirubin level of 10 mg/dl. Auscultation revealed normal respiratory and heart sounds. The abdomen was soft non-tender, with normal bowel sounds. A lump was found in the right periumbilical area with multiple small lumps in the left lower quadrant and bilateral inguinal adenopathy. The neurological evaluation revealed right-sided ptosis with normal power, sensations, and reflexes. CT scan of the lung showed medial right lower lobe consolidation. He was started on piperacillin-tazobactam for suspected abscess. An abdominal computerized tomography scan of the patient showed intramuscular fluid collections with surrounding enhancement indicating of abscesses  of the brain and spine showed right retro-orbital metastasis likely arising from the superior rectus muscle. A cervical and thoracic spine MRI showed widespread bone metastasis with pathologic fractures of the thoracic ninth (T9) and thoracic twelfth (T12) vertebral bodies. He underwent a biopsy of cystic lesion in the abdomen which on histopathology, reported as adenocarcinoma. Further, on performing immunohistochemistry (IHC), tumor cells were positive for CK7 and CK19 and tested negative for CDX2, favoring the diagnosis of metastatic adenocarcinoma of pancreaticobiliary origin.An oncologist continued to follow up with the patient in an outpatient setting regarding his treatment for the metastatic pancreaticobiliary adenocarcinoma. It was later discovered that the patient refused any further treatment and preferred to be managed by the primary care physician (PCP). Unfortunately, he passed away soon after the diagnosis.Pancreaticobiliary cancer consists of cancers of the ampullary, peri-ampullary, and bile duct. In our case, the patient had a sporadic presentation of underlying adenocarcinoma originating from the pancreaticobiliary region. Diagnosing pancreatic cancer in a patient without symptoms is complex. It is due to the lack of specific, cost-effective screening tests that could find early-stage pancreatic cancer efficiently, reliably, and accurately in individuals without symptoms. Delay in diagnosis often causes cancer infiltration to the surrounding structures and distant metastasis. Thus, making them a poor surgical-resection candidate .Periampullary tumors are neoplasms that occur in the vicinity of Vater's ampulla. The neoplasms that arise at this site can stem from the pancreas, duodenum, distal common bile duct (CBD), or ampullary complex. Ampullary carcinomas are classified in the ampullary complex as those that are distal to the juxtaposition of the pancreatic duct and the distal common bile duct. Due to their lower incidence relative to other periampullary malignancies, ampullary cancers are not usually suspected as a cause of obstructive jaundice ,2.Typical pancreaticobiliary cancer nonspecific signs include diarrhea induced by fat malabsorption (steatorrhea), fatigue, and mild weight loss . Our patThe symptom of jaundice is found in about 80% of the ampullary cancer patients caused due to tumor obstruction of the distal bile duct . A combiThe prognosis of an ampullary carcinoma in the absence of metastases depends primarily on two factors: the extent of local tumor invasion, as indicated by the T stage, and the prevalence of lymphatic spread, as expressed by the N stage. The tumor, node, metastasis (TNM) method of the American Joint Committee on Cancer (AJCC) and Union for International Cancer Control (UICC) is the most commonly used method for tumor classification. The latest eighth edition of the 2017 version includes a variety of reclassifications resulting in improved prognostic and clinical relevance compared to the previous edition. It includes revised interpretations of the traditional TNM staging as well as the introduction of various subsets . Our patMetastases to the abdominal wall can occur via numerous routes, including its ample arterial supply, lymphatic system, or veno-systemic circulation. The metastases are generally more prominent around the umbilical region. Besides, the literature has described umbilical invasion through embryonic residue, i.e., umbilical ligament. Also, abdominal wall metastasis can occur via direct peritoneal extension and postoperative seeding of the abdominal wall . Common Plaza et al. reported that most soft tissue metastases are poorly differentiated and show signs of advanced disease in a study conducted over 30 years in a large academic medical center. About 27% of patients presented with soft tissue metastasis as an initial manifestation, but none of these 32 patients out of 118 had pancreatic cancer . AbdominAddressing the complexities of pancreaticobiliary cancer is an arduous job, especially when the findings are atypical. Results found in this case are unique in the sense that a novel metastasis presentation was occurring in the anterior abdominal wall rather than metastasis to the liver and lymph nodes. Our findings suggest that using an abdominal cyst lesion examination in such a presentation might provide us a piece of evidence and information about the nature of the lesion, its origin, and also assist in management. Clinicians can use this unconventional finding to have an improved understanding of this varied presentation of pancreaticobiliary cancer.


Neuroblastoma is the most common extra-cranial solid tumor in infants and young children, and accounts for approximately 8–10% of all childhood cancers. The International Neuroblastoma Staging System ) is based on the age of patient and preoperative imaging, with attention paid to whether the primary tumor is affected by one or more of specific Image-Defined Risk Factors (IDRFs). Patients are classified into the following groups: locoregional L1 and L2 (absent or present IDRFs respectively), M stage (a disseminated form of neuroblastoma) and Ms . This publication is aimed to present an unexpected complication associated with an accidental ligation of the celiac trunk during resection of a neuroblastoma tumor in a 2.5-year-old boy after initial chemotherapy, initially with vascular IDRFs, stage L2. The consequences of this complication were pancreatic and spleen ischemia and necrosis, and ischemia and perforation of the common bile duct, gallbladder, stomach, and duodenum. Despite detailed diagnostic imaging , the presence of vascular IDRFs may result in an unexpected complication in the surgical treatment of neuroblastoma in children. Neuroblastoma is the most common extra-cranial solid tumor in infants and young children, and accounts for approximately 8–10% of all childhood cancers .The clinical presentation of the tumor is affected by many factors. It depends on the tumor’s localization, size, stage, symptoms of catecholamine secretion, and the presence of paraneoplastic syndromes ,2,3. AboTo minimize complications associated with the surgical procedure, the Image-Defined Risk Factors (IDRFs) were introduced in 2009. They have been designed to guide surgical management of neuroblastoma at diagnosis, in particular, to indicate whether a biopsy or attempted resection is recommended as the first surgical procedure. The same system of risk factors can be applied later during treatment and follow-up ,7. SummaIn the International Neuroblastoma Staging System ), locoregional tumors are staged L1 or L2 based on the absence or presence of one or more of the Image-Defined Risk Factors (IDRFs), respectively assessed at the moment of diagnosis. Metastatic tumors are defined as stage M, except for stage MS, in which metastases are confirmed in children younger than 18 months of age to the skin, liver, and/or bone marrow ,7.Other factors which influence the prognosis and treatment results are the age of patient, the histopathological type of the tumor, and the presence of genetic markers  ,7.This publication is aimed to present an unexpected complication associated with an accidental ligation of the celiac trunk  during resection of a neuroblastoma tumor in a 2.5-year-old boy after initial chemotherapy, initially with vascular IDRFs and stage L2.A 2.5-year-old boy was admitted to the hospital because he had been lethargic for about two weeks, had experienced chest and abdominal pain, and had been vomiting.The boy had been born from a second pregnancy at 39 weeks of gestation, with a birth weight of 3790 g and an APGAR  score of 10. The pregnancy had gone without any complications. Our patient had a negative family history concerning cancer diseases. His parents, older brother, and the closest family were healthy.3. The lesion adhered to the pancreas, the right adrenal gland, the liver’s visceral surface, the right kidney, the duodenum, the right leg of the diaphragm, and the right psoas muscle, showing the features of a neoplastic infiltrate. The tumor modelled the inferior vena cava (VCI), which was about 2.5 cm away from the spine and segmentally narrowed to 4 mm. The tumor was in close proximity to large vessels—aorta, celiac trunk, common hepatic artery, superior mesenteric artery, VCI, right and left renal veins, splenic and portal vein.  anteriorly from the right kidney and the thoracolumbar spine showed a solid mass of the tumor, ranging from Th11 to L3 and undergoing significant contrast enhancement. Within the tumor, calcifications were visualized. The dimensions of the lesion were determined to be 4.9 × 4.6 × 7.3 cmal vein. . A neuro3). The tumor volume reduction exceeded 35% (Computed tomography (CT), magnetic resonance (MR), and MIBG (metaiodobenzylguanidine) scintigraphy, as well as bone marrow aspirate and biopsy, excluded the presence of metastases in the lungs, liver, skeletal system, bone marrow, and central nervous system. According to the The International Neuroblastoma Risk Group Staging System (INRGSS), the tumor was classified as L2 (IDRFs present). Subsequently, an open biopsy of the tumor was performed.  [+], Synaptophysin [−], CD56 [+] and S-100p [+] were analyzed. Ki-67 was positive in 20% of the cells. N-myc(-) gene amplification was absent. The vascular port was implemented and chemotherapy was applied. After two cycles of Vepeside and Carboplatin, a check-up CT was done in which the decrease of the tumor mass was detected , and ferritin levels indicate no active neoplastic process. A slight gastric fistula was closed. The child is fed through a jejunostomy tube. The drainage of the common bile duct (with the drain of bile to the outside) is maintained.Another procedure—anastomosis of the bile duct with the intestinal loop is planned. Due to the over four-month-long interruption in oncological treatment, the decision to continue chemotherapy and/or radiotherapy will be made only if the cancer comes back.The different ways to treat neuroblastoma include surgery, chemotherapy, radiotherapy, differentiation therapy, immunotherapy, and in selected cases careful observation only. The method of treatment depends on many factors, and both chemotherapeutic and surgical treatment play essential roles in patient therapy ,2,3.Since the introduction of Image-Defined Risk Factors (IDRFs) to the International Neuroblastoma Risk Group (INRG) staging system in 2009, their role in predicting surgical complications has been analyzed by many authors . AccordiIn order to facilitate the tumor resection, two cycles of chemotherapy were initially applied, resulting in a tumor volume reduction of over 35%. According to the publication by Barak et al., the role of surgery in the treatment of neuroblastoma is evolving . Most paCeliac artery in adults is a short, broad branch, about 1.25 cm long. The mean arterial diameter of the standard celiac trunk is 0.8 cm . The celIn the preoperative CT, the celiac trunk diameter in our patient was only 4 mm. The patient presented type 1 of the celiac trunk variation. A mistaken ligation of the celiac trunk may have resulted from the fact that the trunk was compressed and displaced by the tumor.There is no literature describing the consequences of the ligation of the celiac trunk during surgery in children with cancers. In the publication by Kronick et al., of 2017, information on 11 patients with damage to the visceral trunk due to a blunt abdominal trauma was collected. Most visceral injuries are reported to be the result of penetrating injuries. Overall survival in the described patients was 80%, with one intra-operative death and one death due to fulminant hepatic failure. The author suggests that open surgical ligation is recommended in a haemodynamically unstable trauma patient during damage control operation. The thriving open antegrade surgical bypass was described for hepatic ischemia with ligation alone . The stuIn the literature, possible ligation or occlusion consequences are ischemia of the liver, gallbladder, and spleen ,18. It iIn the available literature, no such extensive complication as the one that occurred in our patient was described. The only case found in the literature concerned a 75-year-old man with a celiac trunk injury following a motorcycle accident. A pancreatic leak complicated the prolonged hospital course with a pseudocyst formation necessitating ERCP (Endoscopic Retrograde Cholangio Pancreatography) and endoscopic cyst-gastrotomy, as well as with pneumonia, acute kidney injury, and splenic artery pseudoaneurysm managed by exclusion and embolization .To our knowledge, this patient is the first reported case of such an extensive complication after an accidental ligation of the celiac trunk, and the first reported neuroblastoma case to experience such a complication.The decision to undergo surgery must consider the operational risk associated with the extent of the tumor. The IDRFs play a significant role in predicting operational risk in the preoperative period in children with neuroblastoma. Detailed imaging diagnostics  are necessary before deciding on the surgery. Even so, the presence of vascular IDRFs may result in an unexpected complication in the surgical treatment of neuroblastoma in children.


Thymic tumors  are rare tumors. Thymic carcinomas are aggressive thymic epithelial neoplasms with a poor prognosis. Cardiac tamponade as a presenting complaint of malignant thymic carcinoma is rare.A 64-year-old woman presented to the emergency department with complaints of progressive exertional dyspnea and chest discomfort. On physical examination, she had diminished breath sounds at the left lung base. The chest x-ray showed a mediastinal widening, significant cardiomegaly, and pleural effusion. CT scan of the chest revealed a dominant mediastinal mass, left-sided pleural effusion, and pericardial effusion. Transthoracic echocardiogram showed 3 cm circumferential pericardial effusion, with evidence of cardiac tamponade. An emergent pericardiocentesis and thoracentesis were done. A core needle biopsy of the mediastinal mass revealed a high-grade non-keratinizing squamous cell thymic carcinoma. Immunohistochemistry staining was positive for pan-cytokeratin, high molecular weight cytokeratin, CK 5/6, E-cadherin, p63, epithelial membrane antigen (EMA), and BerEp4. The patient had repeated hospital admissions due to recurrent malignant pericardial effusion and left pleural effusion. The patient was planned for radiation and chemotherapy with oncology.In our review of literature, the primary squamous cell thymic carcinoma presenting initially as a cardiac tamponade was found to be a rare event. Early diagnosis and treatment are of utmost importance given the aggressive clinical course culminating in to poor outcome. Thymic neoplasms are exceedingly rare tumors with an estimated incidence of less than 1% of all the adult cancers. Thymic epithelial tumors (TETs) comprise thymomas and thymic carcinomas (TCs) . The iniThe patient is a 64-year-old woman with a past medical history of sarcoidosis and hypertension, who presented to the emergency department (ED) with complaints of progressive exertional dyspnea and chest discomfort going on for one week. In the ED, her temperature was 97.7 degrees Fahrenheit, blood pressure was 168/109 mmHg, heart rate was 115/minute, respiratory rate was 25/minute, and oxygen saturation was 96% on room air. On general physical examination, she was alert and oriented to time, place, and person, in no acute distress. No scleral icterus or conjunctival pallor was noted. Cardiac examination was significant for tachycardia and distant S1 and S2 heart sounds. She had diminished breath sounds at the left lung base, and no wheezes, crackles, or rhonchi were heard. The abdomen was soft and non-tender with normoactive bowel sounds. Lower extremity had no edema or calf tenderness. The initial laboratory studies are shown in Table An electrocardiogram (EKG) showed sinus tachycardia and low voltage QRS complexes Figure .A chest x-ray was done that showed a significantly widened mediastinum, cardiomegaly, left lung lower lobe pleural effusion, compressive atelectasis and/or pneumonia, and clear right lung Figure .CT scan of the chest revealed a dominant mediastinal mass with pathological appearing mediastinal lymphadenopathy, left-sided pleural effusion, and pericardial effusion Figure .A transthoracic echocardiogram (TTE) was checked that showed small hyperdynamic left and right ventricle with evidence of ventricular diastolic collapse, 3 cm circumferential pericardial effusion, and excessive respirophasic variation, consistent with cardiac tamponade. An emergent pericardiocentesis and thoracentesis were done. The pericardial fluid cytology showed malignant cells. Oncology was consulted. A tissue diagnosis to elucidate the exact nature of the mediastinal mass along with the positron emission tomography (PET)/CT scan for staging was recommended. The patient was discharged and had an outpatient mediastinal mass core biopsy. It revealed high-grade non-keratinizing squamous cell TC Figure . On immuFlow cytometry findings indicated a non-lymphoid nature of this malignancy. In the meantime, the patient was readmitted to the hospital with symptoms of worsening dyspnea, secondary to recurrent malignant pericardial effusion and left pleural effusion, and underwent pericardial window and pleural effusion drainage catheter placement. A PET-CT scan showed an increased size of the hypermetabolic anterior mediastinal mass (11.5 cm transverse and 6.1 cm anteroposterior dimension), with a maximum standardized uptake value (SUV) 16.9. It also revealed a new hypermetabolic (maximum SUV 16.9), marked pleural thickening throughout the left hemithorax periphery consistent with the pleural extension of disease  has given the classification of TC based on their histological appearance Table  1]..1].In 1977, TC was first described by Shimosato et al. in their study on thymic squamous cell carcinoma (TSCC) . HistoloTC typically presents as an anterior mediastinal mass noted incidentally on a radiographic imaging study, or as symptoms arising from compression of the nearby mediastinal structures. This can be cough, chest or neck pain, phrenic nerve palsy, and superior vena cava syndrome . PericarTreatment of the TCs is per National Comprehensive Cancer Network (NCCN) guidelines, and should typically involve a multidisciplinary team approach with oncology, cardiothoracic surgery, radiation oncology, and pathology doctors with expertise in the management of TCs. For surgically resectable tumor, defined as a well-defined anterior mediastinal mass in the thymic bed with no lymphadenopathy or continuity with the thyroid and negative tumor markers, a complete thymectomy with complete tumor excision is recommended. Also, since the recurrence rate is high for the TCs, it is important to give postoperative radiation therapy with or without chemotherapy, depending on the extent of resection . For locThe survival rates for TCs depend on the stage of cancer and the resectability. Survival for patients with a completely resectable tumor is longer than the ones who have a non-resectable tumor or have an incomplete resection of the tumor . Primary squamous TC presenting as cardiac tamponade and recurrent pericardial effusion is a rare phenomenon. As seen in our patient, despite having a diagnosis, the recurrent hospitalization for cardiac tamponade and pleural effusion lead to an overall delay in the therapy of this aggressive malignancy. TCs are morphologically and pathologically distinct from the thymomas in lacking the immature T cells and having the cytoarchitectural features similar to squamous cell carcinoma elsewhere. Given the highly aggressive course, early diagnosis and prompt treatment are of utmost importance. The prognosis is reported to be extremely poor if there is a delay in the administration of chemo or irradiation therapy.