Endoluminal vacuum-assisted closure (E-Vac) therapy has positively contributed, in recent years, to the management of upper gastrointestinal tract perforations by using the same principle of vacuum-assisted closure therapy of external wounds. The aim is to provide continuous wound drainage and to promote tissue granulation, decreasing the needed time to heal with a high rate of leakage closureA series of two different cases with clinical and radiological diagnosis of esophageal fistulas, recorded from 2018 to 2019 period at our institution, is presented. The first one is a case of anastomotic leak after esophagectomy for cancer complicated by pleuro-mediastinal abscess, while the second one is a leak of an esophageal suture, few days after resection of a bronchogenic cyst perforated into the esophageal lumen. Both cases were successfully treated with E-Vac therapy.Our experience shows the usefulness of E-Vac therapy in the management of anastomotic and non-anastomotic esophageal fistulas. Further research is needed to better define its indications, to compare it to traditional treatments and to evaluate its long-term efficacy. Vacuum-assisted therapy is broadly used in the management of skin and muscular defects, the first report dating back to 1962 . The perThis paper reports the successful closure of two different kinds of esophageal fistula; the first one is a complex anastomotic leak, complicated by pleuro-mediastinal abscess, after esophagectomy for cancer, which is the most common indication for this treatment. The second one is a leak of esophageal suture, few days after resection of a rare bronchogenic cyst perforated into the esophageal lumen.9 system for different types of postoperative esophageal leak consists in a sponge, to be placed across the defect, connected by a tube to an external vacuum pomp and a reservoir (ESO-Sponge®). The application system includes an application tube and a pusher. The treatment is performed in the operating theater with the patient in a supine position, anesthetized and intubated. General anesthesia is required to facilitate insertion of the E-Vac device. First, the fistula cavity is explored and measured with a flexible endoscope. The application tube (overtube) is pushed over the endoscope and brought up to the distal end of the leakage cavity. The endoscope is retracted, and the sponge is pushed into the overtube to its end. A metallic marker placed on the skin may help in the positioning and avoid sponge displacement. The application system is then removed, and the sponge tubing is passed and exited through the nostril and connected to the vacuum pump. Continuous suction with a negative pressure of 125 mm Hg is applied. Figure The E-VAC18F-fluorodeoxyglucose-positron emission tomography (18F-FDG-PET), leading to the diagnosis of esophageal adenocarcinoma (cT3N+M0) localized from 34 to 37 cm from the incisors, with the EGJ at 39 cm (Siewert 1). After a multidisciplinary discussion, she received two cycles of neoadjuvant chemotherapy, consisting in docetaxel, cisplatin, and 5-fluorouracil, in 3-week intervals. Post-treatment CT scan revealed a partial response according to RECIST criteria, with a reduction in both primary tumor size and lymphadenopathies (post-treatment staging: cT2N+). The patient was then scheduled for an Ivor-Lewis esophagectomy, with peri-gastric and peri-esophageal lymphadenectomy and an intrathoracic, end-to-end, semimechanical anastomosis.A 62-year-old woman diagnosed with esophageal cancer was referred to our hospital. Her work up included esophagogastroduodenoscopy (EGDS), contrast-enhanced computed tomography (CT) scan and 3. Chest CT scan revealed a frank anastomotic leak with a periesophageal, loculated abscess formation with a maximum thickness of 14 mm × 78 mm  or the pediatric one (diameter of 5 mm). Now, we use E-Vac therapy also for defects smaller than 1 cm, shaping the sponge accordingly or placing it directly into the esophageal lumen. For the present case, E-Vac therapy was performed for a total of 22 days, while the sponge was changed 6 times. During the first three changes, the sponge was placed directly into the cavity and then shaped, retracted, and switched to intraluminal treatment. Excellent granulation of the tissues around the leak was achieved, together with a progressive reduction of the cavity size and of the pleuro-mediastinal abscess Fig. . Daily oA 25-year-old man was referred to our hospital in 2014 for a suspected right bronchogenic cyst. He underwent a trans-esophageal biopsy to confirm the diagnosis. After a few days, he started to complain of fever and acute chest pain. A contrast-enhanced CT scan revealed a massive pleural effusion in the right hemithorax with complete atelectasis of the ipsilateral lung  with acute chest pain. He underwent a CT-scan which revealed a suspected esophageal perforation with acute mediastinitis and right pleural effusion. A subsequent gastroscopy showed, at 35 cm from dental arch, an erosion of the esophageal wall 1,5-cm long and a 2-mm perforation with a small leak of purulent liquid. Furthermore, an endoscopic ultrasound revealed the presence of the bronchogenic cyst just outside the erosive area. Thus, a right thoracotomy with intraoperative endoscopy was performed. An esophageal perforation at the level of the cyst was found, so the right hemithorax was cleaned, the cyst was opened in order to better understand its margin, and then resected, while the esophageal wall was closed with two interrupted, absorbable stitches. A 24-Ch drain was left in place. After 2 days, salivary material appeared into the drain, so the patient underwent an EGDS, which revealed a 7-mm hole of the esophageal wall at the level of the previous suture ; parenteral nutrition should be established as a bridge strategy.Our initial practice in E-Vac therapy for treatment of esophageal postoperative fistula confirms some technical rules:Our cases confirm that the open-cell sponge together with the topical application of negative pressure helps sealing the leak while providing an additional and simultaneous drainage of the cavity distal to it. This transluminal drainage allows an effective and continuous drainage of the abscess cavity, which is often difficult to address radiologically, therefore controlling and reducing the sepsis.Both our cases, despite different pathologies and type of perforations, were successfully treated with the E-VAC therapy, confirming its promising prospects for all kind of esophageal perforations, combined with a correct treatment of sepsis and risk of malnutrition. Further analysis with a larger number of patients should be performed to determine the correct indications for E-Vac therapy.


Aggressive angiomyxoma is a rare pelvic neoplasm that arises commonly in middle aged women and usually has delayed presentation due to its typically silent and slow growing behavior.A 47-year-old woman presented at our hospital with recurrent attacks of pelvic pain and a perianal bulge. Her past medical history was relevant due to previous en bloc resection of a right ovarian angiomyxoma. Current cross sectional imaging, including computed tomography and magnetic resonance imaging, was performed and showed evidence of local recurrence. Various available treatment options were offered to the patient.The imaging features of the aggressive angiomyxoma were peculiar and showed a swirl and lamellate appearance in T1 and T2 weighted images.A locally aggressive tumor with benign histopathology and swirl sign in imaging is highly suggestive of aggressive angiomyxoma. Thirty-six months earlier she had presented with chronic pelvic pain and had undergone en bloc resection of a right ovarian angiomyxoma. The patient underwent an exploratory midline laparotomy under general anesthesia. Upon entering the abdominal cavity, a large soft pelvic mass was noticed filling the pelvis, which was adherent to the right adnexa right ureter, and right pelvic side-wall. Fortunately, the mass was not infiltrating the adjacent organs; therefore, it was successfully resected along with the right adnexa after a careful pelvic side-wall dissection. The uterus, left adnexa, and upper abdomen were all normal.A review of the operative pathology slides prepared 36 months earlier revealed hypocellularity, spindle-shaped myxoid cells without atypia, and intermediate thickened wall vessels with few mast cells. Immunohistochemical marker analysis tests were positive for estrogen receptor, progesterone receptor, and desmin and locally positive for SMA as well as for CD34. The tumor had a myxoid stroma and variable-sized vascular channels with different kind of cells, include ovoid, stellate, and spindle-shaped cells with scant cytoplasm .Current initial clinical assessment showed a partially defined, partially firm mass in the right perineal area. Cross sectional imaging revealed a large pelvi-abdominal, elongated, relatively homogenous hypo attenuated mass extending from the perineum to the mid abdomen. It measured 24 × 18 × 16.5 cm in the maximum dimensions, extending from the right ischioanal, ischiorectal fat planes and traversing the right levator ani muscle. Inferiorly, it had a mass effect on the rectum anteriorly and to the left as well as on other pelvic organs, mainly the urinary bladder . The mas3AAM is a rare pelvic tumor that grows slowly and mainly breaches the pelvic structures adjacent to its site of origin. Clinically, it is usually larger than 10 cm in size, arises from mesenchymal tissue, exhibits pathognomonic imaging features, and has a growth pattern that commonly involves the perineum and pelvic organs . In womeThe high recurrence rate is attributed to soft tissue mass extension, and it is often not accurately documented due to its position in the pelvis between multiple organs, where tumor extension is seen above and below the urogenital and gastrointestinal organs; this also leads to incomplete resection and a high probability of residual tumor cells ,9. The cAccurate preoperative assessment and detailed imaging analysis is crucial for treatment planning and also affects the selection of appropriate surgical treatment approaches—either perineal, abdominal, or both—to avoid residual tumor tissue in the surgical bed. Postoperative imaging is also mandatory to assess the usefulness of the surgery and to exclude recurrent disease or residual tumor.On magnetic resonance imaging (MRI), it usually presents with hyperintense T2 signal intensity secondary to rich fluid content and low intensity in T1WI; the signal intensity of the mass is similar to that of muscle . PathognOn gross assessment, the tumors usually present without a distinct outline and infiltrate the adjacent soft tissue, which has a grey to pink coloration and a rubbery, gelatinous surface. Histologically, they have low cellularity, cells with regular nuclei and absence of atypia or mitotic changes, and an edematous stroma with numerous blood vessels.Different immunohistochemically-detectable receptors can be observed in abdominopelvic pathologies, and analyzing the expression of these receptors is important for selecting the appropriate medical treatment. In this case report, the focus was on estrogen and progesterone receptors ; other iThe treatment options for AAM are varied, and a multidisciplinary approach, including wide local excision, preoperative embolization, hormonal therapy, and to a lesser extent chemo/radiotherapy, may be used ,19. Surg4AAMs are rare, histologically benign tumors with high potential for local infiltration to adjacent organs and show a unique characteristic “swirl sign” seen on MRI. A variety of treatment options are available to patients according to tumor stage, and subsequent long-term imaging is advised to exclude the possibility of commonly observed local recurrence.None.None.Available.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Ahmed Abduljabbar .Mohammad Wazzan .None.Ahmed Abduljabbar.Not commissioned, externally peer-reviewed.


Reports of osteoblastic tumours are limited to a few case reports in veterinary medicine. Osteoblastoma-like osteosarcoma has been accepted by the World Health Organization as an intermediate form between an osteosarcoma and osteoblastoma. This type of tumour indicates an osteosarcoma, that may resemble osteoblastoma clinically, histologically, and radiologically and have the capability for metastasis. Osteoblastoma-like osteosarcoma has not been described in veterinary medicine so far.An eight-year old cat was presented due to progressive ataxia and paraparesis of the pelvic limbs. Imaging confirmed a well-defined, extradural mass originating from the spinous process of the second thoracic vertebra (T2) leading to severe compression of the spinal cord. Decompressive cytoreduction was achieved by removal of the mass after dorsal laminectomy of T1. After recovering from an acute worsening 3.5 weeks after surgery, the cat had an improved neurological status and the dorsal compression was resolved at follow-up 8 months later. A focal contrast enhancing lesion was still evident at the base of T2 spinous process and lung metastasis was additionally suspected. Based on histopathological, radiographic, and clinical features, an “osteoblastoma-like osteosarcoma” was suspected.To the best of our knowledge, this is the first description of this tumour in veterinary medicine. In addition, this case report highlights the difficulty in the diagnosis and definition of osseous neoplasia in cats and provides a literature review. Osteosarcoma represents the most common bone tumour in cats, encompassing 70–80% of these neoplasms –6. In onIn contrast, reports of osteoblastic tumours in the veterinary literature are limited, and include lesions reported in three cats , 8, two Interestingly, in human literature, intermediate types have been described that combines features of both, osteosarcoma and osteoblastoma and led to a complex definition or redefinition of different subtypes of osteosarcoma and osteoblastoma. Table aggressive osteoblastoma” was first introduced into veterinary literature describing a vertebral neoplasia in a cat by Kim et al. [Osteoblastoma-like osteosarcoma (OBLOS) has been defined in human literature as a malignant tumour, that share features between both, osteosarcoma and osteoblastoma and have the potential for metastasising , 23. Them et al. . Aggressm et al. . Althougm et al. –29, thisIn this case report, we describe an osseous neoplasia arising from a thoracic vertebra of a cat that histologically is compatible with an osteoblastoma. However, the clinical and radiological behaviour suggest that the mass represents an intermediate form of osteoblastoma and osteosarcoma previously described in human medicine  butAn eight-year old female spayed domestic short hair cat was presented to the Small Animal Clinic of the Vetsuisse Faculty of the University of Bern, Switzerland, due to progressive gait abnormalities of both pelvic limbs. The first clinical sign, consisting of frequent stretching of the pelvic limbs, has been intermittently observed by the owner over the last 2 months prior to presentation. One week prior to presentation the cat acutely worsened, was reluctant to jump and to climb stairs and seemed to be painful. Additionally, an increased water-intake and periuria have been observed by the owner.9/l; range: 0–1.5 × 109/l), and an elevated aspartate transaminase  and creatinine kinase  activity. Serological testing for feline immunodeficiency virus and feline leukaemia virus were negative. Thoracic radiographs revealed no abnormalities of the intrathoracic organs, although a quite well defined, round geographic radiolucent lesion was identified at the base of the spinous process of the second thoracic vertebra (T2) expanding into the area of the vertebral canal  and restriction of exercise was recommended. A surgical exploration was suggested and performed fortnight after diagnosis.During surgery, a large dorsal laminectomy of T1 (modified Funkquist type B) was performed. A white, solid bulging mass originating from the lamina of T2 was identified on the excised bony segment see Fig. . After iRapid Decalcifier, J.T. Baker®), routinely processed, and stained with hematoxylin & eosin and van Gieson’s stain.For histological examination the resected material was fixed in 10% buffered formalin, decalcified , prednisolone  and omeprazole (1 mg/kg BID po during prednisolone-treatment). Strict cage rest for the first weeks after surgery and physiotherapy were recommended. At the first follow-up 2 weeks after surgery, the cat experienced a clear improvement of the neurological signs, showing only a mild ataxia in both pelvic limbs. Due to the temperament of the cat, physiotherapy could not be performed. Nevertheless, after initial improvement, the cat suffered an acute transient deterioration 3.5 weeks after surgery which improved with strict rest. At the clinical and imaging follow-up 8 months after surgery, the cat had only a mild ataxia in the pelvic limbs. CT revealed a well-defined, in respect to the spinal cord isodense lesion ventrally demarcated by a hyperdense rim within the base of the spinous process of T2, that was homogenously contrast enhancing, indicating recurrence of the tumour Fig. a, b. FurDue to the current good quality of the cat’s life, the owner denied further workup of the pulmonary changes, as well as any additional treatment. As metastatic lung disease could not be excluded, a histopathological re-evaluation including immunohistochemistry was performed. Primary antibodies used were COX-2, to differentiate osteoblastoma from low-grade osteosarcoma and Collagen I, to confirm osteoid matrix production and therefore osseous origin of the neoplasia. Heat induced epitope retrieval  for unmasking antigens was used. Negative immunohistochemical controls for each sample were prepared by omitting the primary antibody. For each primary antibody, a known positive control section was included in each immunolabeling assay.COX-2 immunostaining revealed strong positivity of few neoplastic cells entrapped in a focal area of woven bone, while the rest of the neoplastic cells were negative. Collagen I immunostaining was diffusely positive within the cortical bone next to the tumoral mass, the reactive woven bone and the extracellular osteoid matrix of the tumour Fig. . In summAt the date of submission  the cat was still alive and showed a good quality of life.Osteosarcoma is the most common vertebral tumour in the cat. In contrast, veterinary reports of osteoblastic tumours are restricted to a few case reports , 24, 30.Definitive diagnosis of primary bone tumours is challenging. These difficulties are highlighted in this case report, in which initially, based on the histopathological findings, a periosteal osteosarcoma was diagnosed. This diagnosis was based on the periosteal covering of the mass. The first diagnosis led to some concerns, since based on imaging, the lesion seemed to originate from the cancellous bone of T2, rather than its surface. Therefore, a pathologist specialized in bone pathology was consulted for a second opinion, who diagnosed finally an osteoblastoma arising from the cancellous bone of T2 with slow expansile growth that, in turn, has been resulted in encroachment of the border of the spinous process into the spinal canal causing clinical signs.After a lung metastasis was considered during the follow up, a further re-evaluation of the specimen via immunohistochemistry was performed, since an osteoblastoma would not be compatible with metastatic behaviour. Collagen 1-immunostaining confirmed the production of osteoid matrix by the tumoral cells, and therefore the osseous origin, being Collagen I the main fibrillar component of bone extracellular matrix. In human medicine, COX-2 positivity has been used to distinguish between aggressive osteoblastoma and low-grade osteosarcoma, being the first COX-2 positive and the second COX-2 negative . Kim et 4th WHO classification of tumours of soft tissue and bone (2013) listed OBLOS within the group of conventional (high-grade) osteosarcoma. Nevertheless, Gambarotti et al. [Based on the histological findings, the imaging features and the potential metastatic behaviour, an OBLOS was considered to be the most likely diagnosis. According to the grading by Gambarotti et al. , the Wori et al.  found ini et al.  and, thui et al. , 20, 33.No other potential neoplastic lesions were detected on the whole body CT-scan at the time of first presentation; thus, it was presumed that the lung lesion in the follow-up CT scan 9 months later might represent a metastasis of the vertebral bone lesion. The diagnosis of metastatic lung disease tumours is more challenging in cats compared to dogs . In contReasons for the difficulties in diagnosing primary bone tumours are on one side the overlap in histopathological and diagnostic imaging features among the different types  , 36–38 abiologically intermediate or indeterminate group”. Dorfman and Weiss [Rosado et al. in 2000  providednd Weiss  proposednd Weiss , 42–46.metastatic osteoblastoma” in a 15-year old boy; Kunze et al. in 1996 [In line with our findings, osteoblastoma usually presents as a well-defined, rounded, lytic, radiolucent lesion originating in the medullary part of the bone with varying amounts of surrounding sclerosis in radiographic analysis , 47. In  in 1996  and Wozn in 1996  describe in 1996 . Interes in 1996 . The spa in 1996 . In our Only few reports on osteoblastic tumours have been published in veterinary literature , 8–12. IEn bloc resection of cervical osteoblastoma has been reported to be the treatment of choice in human medicine –52. NeveIn conclusion and in respect to the entire radiological, clinical and pathological findings, the vertebral neoplasia in the reported cat, best resembles what in human medicine is mainly known as OBLOS  and it rOur case report depicts the challenges in diagnosing primary bone tumours and gives a wide overview about the current discussion on their definitions and classifications. The primary bone neoplasia in this cat clearly compounds features of both osteoblastoma and osteosarcoma and thus might represent a novel subtype of primary bone tumours. Additionally, it contributes interesting features to the sparsely existing literature on feline primary bone tumours. Malignant transformation of an osteoblastoma have not been described in veterinary medicine yet. This case report also reflects the almost daily clinical challenge in estimating the dignity of a lesion, which is in the end more important than the correct naming.


High‐grade myofibroblastic sarcoma (HGMS) is a rare cancer that has high recurrence and metastatic rates. Here, we report the first case of HGMS originating from the pleura. Based on the findings of pleural biopsy, pathological examination and immunohistochemical staining, grade III myofibroblastic sarcoma (MS) was diagnosed. The patient underwent eight cycles of chemotherapy (epirubicin and ifosfamide), followed by radiotherapy. As of May 2020, the patient had been followed for six months and no tumor progression had occurred.This is the first report of high‐grade myofibroblastic sarcoma originating from the pleura. The patient was treated via nonsurgical strategies, including chemotherapy and radiotherapy. High‐grade myofibroblastic sarcoma (HGMS) is a rare disease. This is the first case diagnosed with HGMS originating from the pleura that was nonsurgically treated. Malignant sarcoma consisting of myofibroblasts or exhibiting myfibroblastic differentiation is diagnosed as myofibroblastic sarcoma (MS). MS is a rare disease that typically originates in the head and neck, extremities, and the trunk.In November 2018, a 57‐year‐old female patient presented to our hospital with a two‐month history of cough and shortness of breath. Her medical records revealed that she did not have a history of tuberculosis, malignancy, or chemoradiotherapy. Whole‐body positron emission tomography/computed tomography (PET/CT) scan revealed thickened right pleura  and a hypermetabolic mass (10.9 × 9.5 cm) with a clear boundary in the right lung (max SUV = 29) Fig . The levAfter consultation with a multidisciplinary team, the patient was administered eight cycles of chemotherapy with epirubicin (100 mg day 1) and ifosfamide (2 g day 1) every 21 days. CT evaluation after chemotherapy showed a partial response: the lesion had shrunk to 5.2 × 2.0 cm Fig . The patOne month after radiotherapy, an enhanced CT scan showed that the tumor had not progressed Fig . HoweverThis is the first report of HGMS in the pleura. HGMS is usually immunopositive for SMA, muscle‐specific actin, vimentin, and fibronectin,et al.et al.et al.Surgical excision with wide margins is the most effective definitive treatment for MS, but adjuvant therapies, including chemotherapy and radiotherapy, are also considered.There are no standard guidelines for the treatment of HGMS, as it a rare malignant tumor. Further, there is limited clinical information on the outcomes of nonsurgical treatment, such as chemotherapy and RT. Therefore, this case provides new information that could potentially be useful for similar rare cases.None of the authors have any conflicts of interest to report.


The drastic differences in treatment and prognosis of infantile hepatic hemangioma (IHH) and hepatoblastoma (HBL) make accurate prenatal diagnosis imperative. The retrospective comparisons of ultrasonic features between fetal IHH and HBL have been reported before, but clinically, the differential diagnosis in utero is very difficult and can lead to prenatal misdiagnosis.A 27-year-old woman at 30 gestational weeks underwent the routine prenatal examination. A heterogeneous solid mass of the fetus, with close relationship to the liver, was recognized by ultrasound.A diagnosis of HBL was highly considered.The fetus was aborted and the autopsy was performed.The histological outcome was IHH.The prognosis of fetal IHH and HBL is very different, so an accurate diagnosis prenatally is crucial and indispensable. The radiologist and clinician should differentiate between IHH and HBL, especially since the fetus can have serious complications. Giant IHH may cause life-threatening complications in utero, including fetal hydrops, anemia, thrombocytopenia, cardiac failure. IHH could regress spontaneously without treatment. The core treatment for IHH is conservative therapy (observation or follow-up).,5 However, the mainstay treatment for HBL are surgical resection, neoadjuvant chemotherapy, and even liver transplantation, and the prognosis depends on many factors and is not encouraging.Infantile hepatic hemangioma (IHH) is the most common benign mesenchymal tumor of the liver in fetus and infants, accounting for about 20% of all liver tumors arising in fetus, However, the overlap of imaging features between IHH and HBL may confuse the differential diagnosis in utero, and there is very few research about the prenatal differential diagnosis. The final diagnosis depends on the postnatal ultrasound, contrast-enhanced computerized tomography and the biopsy of the hepatic lesions.Since there exist differences in the treatment therapy and prognosis between IHH and HBL, how to make an accurate prenatal differential diagnosis is an issue. The ultrasound has become the main method for prenatal diagnosis, owing to its real-time assessment of the liver tumor in fetus, including size, location, the feeding vessels, even the relationship between portal and hepatic vein.In this case report and the review of literature, we tried to investigate if there were specific imaging features of ultrasound for the differential diagnosis between IHH and HBL in utero.2A 27-year-old woman, gravida 1 para 0, underwent the routine prenatal examinations in our hospital from 6 gestational weeks. Till 30 gestational weeks, the prenatal course was unremarkable, including systemic prenatal ultrasound diagnosis and the routine fetal cardiac screening . Fetal growth was within the normal process and the alpha fetal protein was 23.91 U/ml .At 30 gestational weeks, the B-mode ultrasound revealed that the liver of the fetus was enlarged with a heterogeneous solid mass located in the right quadrant of the fetal abdominal cavity, with a close relationship to the liver. The mass measured 5.8cmx4.7cmx5.2 cm and pushed the portal vein and gall bladder. The lesion seemed to have an ill-defined margin, irregular shape, partial capsule with multiple cystic cavities inside, and maximal diameter of 2.1 cm. The portal and hepatic veins were not dilated. In the color Doppler ultrasound, the mass appeared to be highly vascularized .,19–21 This could be significant imaging finding specific to IHH. However, in this case, the mass appeared to be a highly vascularized lesion, RI was 0.67 but the portal and hepatic veins of the fetus were not dilated, and according to the power Doppler ultrasound, the feeding vessels of the lesion was unclear. This is another reason for the misdiagnosis in our case.In the color Doppler ultrasound, studies showed that HBL appeared as arterial flow with RI > 0.7 and it may be that HBL mainly consists of numerous and disorderly hepatoblast-like cells and lack of arterio-venous anastomosis or draining veins.There exist difficulties in the differential diagnosis between IHH and HBL in utero by ultrasound. But according to some specific imaging features like necrosis or cystic cavity inside the tumor, ill-defined margin, polylobular, the type of the feeding vessels (arteries or veins), dilated hepatic arteries, veins, or artery-vein shunting, IHH could be diagnosed more accurately prenatally.Conceptualization: Ya Jin, Fan Yang.Data curation: Ya Jin, Fan Yang.Formal analysis: Ya Jin, Fan Yang.Funding acquisition: Fan Yang.Methodology: Ya Jin, Fan Yang.Project administration: Fan Yang.Resources: Lin Li.Validation: Lin Li.Visualization: Lin Li.Writing – original draft: Ya Jin.Writing – review & editing: Fan Yang.


Gynaecologic pelvic tumours are very common and they can present with a variety of symptoms depending on their size, location, pathophysiology and histogenesis. Infrequently, some pelvic tumours are found in the retroperitoneal space presenting with similar symptoms. Our objective is to present our experience and review of pertinent literature on miscellaneous retroperitoneal tumours.Four women with retroperitoneal tumours  cysts)) were encountered during routine laparoscopy (3 cases) and laparotomy (one case). Following multidisciplinary consultation and additional imaging, all tumours were removed by laparotomy with one case provoking litigation due to ureteral and bowel injury.Using these four cases, and additional cases from the literature, we highlight the potential pitfalls and provide an algorithm to minimize risks and adverse clinical and legal outcomes associated with unexpected retroperitoneal tumours. The algorithm includes resisting the impulse/temptation to remove or biopsy these tumours, requesting intra-operative consultation(s), obtaining additional detailed imaging to characterize these tumours, providing appropriate counselling to patients, obtaining informed consent, and consulting the appropriate surgical teams. At times, an interdisciplinary approach may prove to be the best course of action in order to optimize treatment and ensure patient safety.If a retroperitoneal tumour is unexpectedly encountered, it is imperative to have intra-operative consultation (if available), to not attempt excision or biopsy, and to subsequently obtain post-operative multidisciplinary consultations, specific imaging, and information gathering in order to treat these heterogeneous masses as safely as possible. Gynaecologic pelvic tumours are very common and can present with a variety of symptoms depending on their size, location, pathophysiology and histogenesis. Most of these are solid leiomyomas, followed by benign ovarian cysts. Infrequently, pelvic tumours can be found in the retroperitoneal space, separate from the uterus or the ovaries. Up to 30% of retroperitoneal tumours are asymptomatic and 50% will present with similar symptoms to intraperitoneal pelvic tumours; such as vague abdominal pain and distention, making distinction difficult . During Retroperitoneal solid lesions can arise from any of the mesenchymal elements including smooth muscle (leiomyomas), neuronal tissue (schwannomas), and others. We have previously reported a case and review of retroperitoneal leiomyomas and no further description will be provided here .Since retroperitoneal tumours are so rare, general gynecologists invariably encounter them very infrequently during routine laparoscopy or laparotomy performed for leiomyomas or benign adnexal cysts. Faced with such unexpected tumours, gynecologists are consequently challenged on what the next most appropriate action is. The options at this time are to either attempt to excise the tumour, or at least take a biopsy for histologic assessment, or abandon any further surgical intervention and arrange for interdisciplinary consultation, including additional imaging and investigation.Since the differential diagnosis of retroperitoneal tumours is fairly vast, the methods of treatment and their clinical implications can differ greatly depending on the pathology. Imaging modalities such as ultrasound, computed topography (CT) and magnetic resonance imaging (MRI) can be of benefit when attempting to narrow-down potential diagnoses. Characteristics such as lesion shape, size, location, and presence/absence of septa, calcifications or fat is information that can help the health care provider arrive at a diagnosis or at least help to rule out certain pathologies.Herein, we present the management and clinical outcomes of four cases of miscellaneous pelvic tumours; one schwannoma, one granulosa cell tumour and two hindgut (tail gut) cysts, and briefly discuss the importance of pre- and intra-operative management of any retroperitoneal lesions, to minimize or avoid the risk of adverse or catastrophic events. It is imperative to have intra-operative consultation (if available) and subsequent post- operative multidisciplinary consultations, specific imaging, and information gathering in order to treat these heterogeneous masses as safely as possible.Informed Consent:Patients number 1, 2, and 4 have signed consent to publish their cases. Case number 4 is in the public domain , and ethA 59-year-old Caucasian woman presented with vague pelvic discomfort and was found to have a large pelvic mass, detected on routine physical examination by the family physician. Notably, the patient was menopausal as of age 48 and had been on hormone replacement therapy (HRT) since that time. Ultrasound imaging revealed an 8.6 x 7.6 x 8.3 cm solid pelvic mass, thought to be either of uterine or ovarian origin. MRI measured an 8.5 cm solid, heterogeneous mass behind the uterus, separated by a thin fat plane, and neither ovary was identified. Post-contrast imaging was suggestive of a pedunculated or subserosal leiomyoma. A biopsy and possible laparoscopy were recommended for definitive diagnosis of the mass. The patient was assessed by a gynaecologist who suggested an exploratory laparotomy, with possible total abdominal hysterectomy (TAH) and bilateral salpingo-oophorectomy (BSO).At laparotomy four weeks later, the mass appeared to arise from the sacrum and the rectosigmoid colon was noted to be adherent to the mass. The uterus, fallopian tubes, ovaries, and cul-de-sac were all normal. Intraoperative consults included a gynaecologic oncologist, who did not feel that the mass was of gynaecologic origin, and an orthopaedic surgeon, who proposed that the mass was most likely a chondroma and that further imaging should be obtained, prior to definitive surgical excision. At this time, no further surgery was performed and no biopsy was taken, given that the vascularity of the tumour was unknown.Computed tomography (CT) scan of the lumbar spine described the soft tissue mass as extending to the anterior cortex of the S1 vertebral body and to the anterior margin of the L5-S1 disc. There was direct continuity of the mass with the S1 vertebral body, but no erosive or destructive changes. CT of the abdomen and pelvis noted again the 9 cm soft tissue mass, separate from the bladder and uterus. The possibility of a sarcoma was questioned. A subsequent MRI of the lumbar spine described the pelvic mass as intimately opposed to the upper sacrum, but not involving the vertebral bodies.Two months after the first laparotomy, the patient had a second laparotomy, performed by a general and a vascular surgeon. The tumour was found to be adherent to the sigmoid colon, left ureter, left internal iliac vein  and presacral fascia and veins. Intraoperative blood loss was approximately three litres and the patient was transfused with six units of packed red blood cells. The final pathology revealed a Schwannoma measuring 9.0 x 10.5 x 9.0 cm and weighing 364g . Post-opA 49-year-old woman, P2G2, BMI 37 kg/m2, was referred to our clinic with chronic pelvic pain and a complex right adnexal mass measuring 8.2 cm, which had been followed for 4 years. She had a remote abdominal left salpingo-oophorectomy for ovarian torsion. On pelvic examination, a soft mass filled the posterior cul-de-sac which could not be dislodged from the pelvis. The CA125 was normal. The patient was scheduled for laparoscopic evaluation and possible right salpingo-oophorectomy and hysterectomy.At laparoscopy 3 weeks later, a normal sized uterus was displaced anteriorly by a retroperitoneal soft mass, filling the cul-de-sac, and intimately close to the sacrum, possibly arising from the rectosigmoid with significant blood vessels running over the mass. The right ovary and tube were normal and there was an absence of the left adnexa . After iOne month later, a CT scan reported a 9.0 x 6.9 x 6.9 cm complex cystic mass interposed between the anterior rectal wall and posterior margin of the uterus. This lesion was thought to be unusual for a tailgut cyst, since it was not centred in the retrorectal space. Six weeks later, an MRI measured the mass as 7.4 x 5.2 x 8.4 cm, relatively well circumscribed, cystic, and with some solid components. A US guided biopsy was reported as neoplastic tissue, possible sex cord stromal tumour.At laparotomy four weeks later, the tumour was relatively easily dissected from the anterior wall of the rectum, posterior wall of the vagina and the left pelvic side wall. The final pathology was reported as granulosa cell tumour, adult type with lesional cells present at the resection margins . After rSix months later, the patient was seen with abnormal uterine bleeding (AUB). Endometrial biopsy indicated proliferative endometrium and the patient was treated with a LNG-IUS. At 3 years of follow-up, she remains amenorrheic and well with no evidence of recurrent disease.A 45-year-old Caucasian woman had a CT scan for vague pelvic discomfort, which detected a right presacral, hyperdense, smooth-appearing lesion measuring 4.8 x 3.8 cm. The possibility of a neurogenic tumour was raised. MRI described the mass as a right presacral, complex cyst with high signal intensity measuring 5.9 x 3.9 cm, and appeared to be adjacent to the S1 foramen ovale, but separate from the nerve root. Ultrasound demonstrated a right posterior, complex mass measuring 5.5 cm, which was suspicious for a dermoid cyst.At laparoscopy three weeks later, the mass was clearly retroperitoneal and separate from the pelvic organs . No biopAs the patient was experiencing neurogenic-type pain, and there was a possibility of malignancy, the retroperitoneal cyst was excised by laparotomy 6 weeks later by a general surgeon who had specialized in pelvic surgery. At the time of surgery, the mass was noted to be soft, somewhat fluctuant, and fixed to the right pelvic sidewall. The mass was related to the right sympathetic nerve bundle and autonomic plexus, but did not involve major vascular structures.Microscopically, the cyst was lined by ciliated, respiratory-type epithelium with a smooth muscle wall with areas of focal squamous metaplasia. The diagnosis was a benign epithelial cyst, consistent with duplication cyst or hindgut cyst. Additional details of this case have been published previously  .This is a medicolegal case in the public domain .A 49-year-old, morbidly obese woman presented with left lower quadrant pain and a cystic mass identified on ultrasound measuring 7 x 8 x 13 cm. Past surgeries included a total abdominal hysterectomy and a subsequent bilateral salpingo- oophorectomy.At a laparotomy three weeks later, it was unexpectedly found that the mass was a retroperitoneal cyst. The gynecologist decided to dissect the cyst by creating a plane between the capsule of the mass, even though the ureters were not visualized. Dense adhesions made the dissection difficult and there was limited visualization of the operative field due to the patient’s large body habitus. Eventually, the mass was removed and a drain was left in the pelvis.On post-operative day #2, the smell of urine was detected in the drain and ureteric injury was confirmed with retrograde pyelogram. The patient subsequently underwent a laparotomy and repair of the left ureter. However, she continued to have symptoms and, due to fecal material present in the abdominal drain, a rectosigmoid injury was suspected. On post-operative day #7, she had a colostomy for a uretero-rectal fistula. The fistula healed spontaneously and the colostomy was reversed one year later. The final pathology was hindgut cyst.Expert opinion was that the gynecologist should have sought assistance from a urologist intraoperatively to help with identification of the ureters. The legal outcome was negligence on the part of the gynecologist for damage of the ureter, but not for damage of the bowel.As stated in the introduction, almost all these retroperitoneal tumours are encountered during routine laparoscopy or laparotomy to remove common pelvic tumours such as leiomyomas or adnexal cysts. Under these circumstances, gynecologists invariably are faced with several challenges including whether or not to excise an undiagnosed tumour without patient informed consent, or to at least take a biopsy of the mass, or to abandon any further surgery and re-operate at a later date after further evaluation and discussions with the patient and other health care professionals.Schwannoma: The present case of schwannoma, and review of similar published cases, indicate that a biopsy of solid tumours can result in excessive bleeding, disseminating/upstaging disease if the lesion is malignant and, occasionally, removing a perfectly normal ectopic organ such as a pelvic kidney. In our case, the schwannoma was found to be adhered to the left internal iliac vein which required ligation and resection and resulted in blood loss of at least 3 litres.Schwannomas are tumours that arise from the neural crest-derived Schwann cell. They are associated with neurofibromatosis type 2 and are mostly benign, with only 1% becoming malignant. Schwannomas are usually well-circumscribed, encapsulated masses that are usually attached to the nerve and symptoms are caused by local compression of the involved nerve . DependiUnder these circumstances, excision or biopsy may not be appropriate, and additional tests and imaging should be performed to determine the nature of the lesion. An image-guided biopsy can always be performed at a later date after further interdisciplinary consultations, detailed discussions with the patient, and complete informed consent have been completed.Granulosa Cell Tumours: In our case, we have no explanation for the origin of this granulosa cell tumour. In a previously reported case and review of the literature of granulosa cell tumors found on the uterosacral ligaments, we included the different theories on the histogenesis of ectopic granulosa cells  is the mainstay of treatment. Unilateral salpingo-oophorectomy coupled with staging and endometrial sampling to exclude metastatic disease and/or concurrent endometrial pathology can be offered to women of childbearing age with early-stage (stage Ia) disease. There is no consensus on whether radical surgery should be performed when these patients have completed their childbearing or when they reach menopause. Adjuvant chemotherapy is currently recommended only in those cases with advanced, recurrent or metastatic disease. Long-term surveillance is mandatory including clinical follow-up and tumor markers (i.e. Anti-Müllerian Hormone and inhibin B), as disease may recur many years after the initial diagnosis and treatment.Cysts that develop in the retrorectal space are histologically heterogeneous, as this space contains multiple embryological remnants as well as neuroectoderm, notochord, and hindgut . For theHindgut Cysts: Tailgut and duplication cysts are types of retrorectal tumours. Their true incidence is unknown, but in one study, they were found to arise in approximately 1/40,000 hospital admissions , it might be wiser to plan for complete surgical excision. Although we have not encountered any neurogenic cysts in our practice, we summarize two cases reported from the literature to highlight their importance.Anterior Sacral Meningocele: An anterior meningocele is a herniation of the dural sac through a defect in the sacrum. The sac is in continuity with the subdural space and, therefore, contains cerebrospinal fluid.Berstock et al.  describeFollowing referral to a spinal surgeon, the patient was counseled with respect to the risks of bowel, bladder, and sexual dysfunction, numbness, and intraoperative coning of the brain stem before surgery. Ligation of the meningocele sac was performed at the level of the communicating stalk, with the patient in a gentle Trendelenburg position, and 1L of cerebrospinal fluid was aspirated from the cyst. Postoperatively, the patient had some minor transient neurological symptoms which subsided within one year. Additional anterior sacral meningoceles, previously misdiagnosed as ovarian cysts, have also been reported .Central to this case is an emphasis on advocating a cautious approach, when confronted with an incidental unknown pelvic retroperitoneal cyst, as deleterious consequences, including death from coning of the brain stem, may occur from puncture or biopsy of such a lesion .Tarlov Cysts: These cysts, also known as perineural cysts, were first described by Tarlov, in 1938, and are meningeal cysts that are filled with cerebrospinal fluid (CSF) . Tarlov A 72-year old woman presented with a short history of poor urinary flow. Pelvic ultrasound noted a left-sided cystic lesion, measuring 4.2 x 3.9 x 5.4 cm, with echogenic material and fluid of questionable ovarian origin. Tumour markers were normal. The patient was subsequently booked for a laparoscopic bilateral salpingo-oophorectomy and cystoscopy.At laparoscopy, the pelvic organs appeared normal, but a retroperitoneal cystic structure was noted on the left side, between the rectosigmoid and the lateral pelvic wall. The peritoneum was then divided, and the cyst punctured. The cyst drained clear fluid and a tiny biopsy was taken.Immediately post-operatively, the patient complained of excruciating pain in the left hip and buttock with radiation down to the back of the thigh and calf. The patient also complained of burning, stabbing, and electric shock sensations in the same region without relief with major analgesics. An MRI showed multiple cysts within the sacrum and a complex 5 cm cyst with multiple septation and evidence of haemorrhage in the region of the sacral plexus. Histopathology of the laparoscopic biopsy from the cyst indicated neural tissue elements consistent with a Tarlov cyst.The patient ultimately had a cerebrospinal fluid arachnoid drain placed at L2,3 to relieve the pressure and a laminectomy at L4,5 to remove clear fluid under high pressure. The nerve sheath root was narrowed with bipolar radiofrequency. The patient had good pain relief following these procedures, unfortunately, the voiding problems persisted .Tarlov cysts are fluid-filled sacral perineural cysts arising at the junction of the dorsal root ganglion and the nerve root. They display the characteristic of delayed filling on myelography (not meningoceles). Tarlov cysts contain nerve fibres and ganglion cells in the cyst wall and develop between the endoneurium and perineurium of the nerve root. They typically require the expertise of a neurosurgeon and pain specialist.The final point to highlight from these cases is that attempts to dissect and remove these tumours without appropriate preoperative evaluation, patient counselling and consent, planning, and multidisciplinary intra-and post-operative consultations can result in significant adverse events or sequelae, which may provoke litigation with unfavourable legal outcomes. This is evident in case No.4 (hindgut cyst) where a difficult dissection of the cyst resulted in ureteral and rectosigmoid injury, while in case No. 1 (schwannoma), although the dissection was more difficult, the use of appropriate planning and consultation resulted in uneventful clinical outcome.Limitations of this study include that it was conducted in a single center with a limited number of patients.Retroperitoneal tumours, while an uncommon entity, can pose both diagnostic and therapeutic challenges. With these four cases, and additional cases from the literature, we highlight the potential pitfalls and provide an approach to minimize risks and adverse clinical and legal outcomes associated with unexpected retroperitoneal tumours. Our recommendations include: resisting the impulse/ temptation to remove or biopsy these tumours, requesting intra-operative consultation(s), obtaining additional detailed imaging to characterize these tumours, providing appropriate counselling to patients, obtaining informed consent, and consulting appropriate surgical teams for definitive treatment as outlined in


Rhabdomyosarcoma is the most frequently occurring intrusive soft tissue sarcoma in the pediatric age group. Orbit is the most common location for a pediatric rhabdomyosarcoma, but it can occur in the oral cavity, pharynx, face and neck in the descending order of incidence. Rhabdomyosarcoma in the ear is extremely rare.A 5-year-old girl presented to the outpatient department of our tertiary care hospital with complaints of foul smelling, non-blood stained right ear discharge of one-month duration and deviation of angle of mouth to the left side of acute onset. Investigations revealed a diagnosis of embryonal rhabdomyosarcoma. Multimodal therapy was carried out, and the child was rendered disease-free after two years.Embryonal rhabdomyosarcoma of the head and neck mimics chronic otitis media. Early diagnosis is essential to deliver prompt treatment and prevent locoregional spread and metastasis. Rhabdomyosarcoma is the most frequently occurring intrusive soft tissue sarcoma in the pediatric age group -3. It isOrbit is the most common location for a pediatric rhabdomyosarcoma, but it can occur in the oral cavity, pharynx, face and neck in the descending order of incidence .Rhabdomyosarcoma in the ear is extremely rare . DiagnosA 5-year-old girl presented to the outpatient department of our tertiary care hospital with complaints of foul smelling, non-blood stained right ear discharge of one-month duration. There was a deviation of angle of mouth to the left side of 15 days duration. She did not have a history of decreased hearing, ear pain, itchiness, tinnitus and vertigo. The child did not have any other neurological deficit. There was no history of fever. On examination of the right ear, a pale polyp was visualised in the right external auditory canal with mucopurulent ear discharge without visualisation of the tympanic membrane. There was House Brackmann Grade 6 lower motor neuron paralysis of the right facial nerve. Rest of the otolaryngological and systemic examination was within normal limits. HRCT of the temporal bone revealed a soft tissue density in the right external auditory canal and middle ear with osteolysis. It also revealed erosion of the horizontal segment of the facial canal with thickening of the nerve distal to the second genu . A proviThe child underwent mastoid exploration under general anaesthesia. Intraoperatively, it was noted to be a friable mass with no evidence of cholesteatoma . Biopsy Histopathological examination revealed a subepithelial tumor composed of small round hyperchromatic, pleomorphic nuclei, inconspicuous nucleoli, abundant mitosis and spindle-shaped cells arranged diffusely in a fibromyxoid stroma with congested vessels, areas of haemorrhage and necrosis. Occasional cells with eccentric nuclei and eosinophilic cytoplasm (rhabdomyoblasts) were also visualized . Immunohistochemistry was positive for desmin and myogenin suggestive of embryonal rhabdomyosarcoma .The child was advised to undergo PET-CT to evaluate the extent of disease and metastasis. PET-CT revealed low-grade metabolic activity in the right mastoid and petroclival region. There was thrombosis of the right internal jugular vein and adjacent sigmoid sinus without any intracranial extension. There was no evidence of distant metastasis. The child underwent 14 cycles of chemotherapy with Vincristine, Actinomycin D and Cyclophosphamide. The initial five cycles of chemotherapy were followed by external beam radiation therapy of 50.4 Gy divided into 28 fractions administered over five and a half weeks. Following the radiation therapy, she underwent nine more cycles of chemotherapy with Vincristine, Actinomycin D and Cyclophosphamide over six months. Facial function improved and was normal six months after therapy. PET-CT done one year following treatment revealed no evidence of residual/recurrent tumor. The patient is on regular follow-up and PET-CT done two years after definitive chemoradiation showed no evidence of tumor . Pure toRhabdomyosarcoma is an aggressive malignant tumor that has a bimodal distribution in children with an incidence reported in the age group of 2-5years and late adolescence. More than 63 percent of tumors occur in the first decade of life . Four vaThe embryonal variant is the commonest, whereas, alveolar variant has been known to have the worst prognosis ,6. The eThe symptoms of rhabdomyosarcoma presenting in the ear are similar to chronic otitis media or otitis externa which include blood-stained ear discharge, reduced hearing and ear pain and related complications like facial weakness maybe present. Signs may include polyp or granulation in the external auditory canal ,3,4,6-8.Our patient presented with acute facial palsy and bloodstained ear discharge which mimicked chronic otitis media presentation and eventually proven to be embryonal rhabdomyosarcoma on histopathology. The most common site of distant metastasis is lungs followed by skeletal system, liver and brain . HRCT, MRI and histopathology with immunohistochemistry form the pillars of diagnosis in rhabdomyosarcoma. HRCT reveals destruction of bone with heterogeneous attenuation within the temporal bone ,5,7. PETTreatment of Embryonal rhabdomyosarcoma is multimodal. Recent trends suggest that chemotherapy and radiation work well concomitantly and increase the overall disease-free survival provided embryonal rhabdomyosarcoma has not metastasized ,8. The cEarly diagnosis of acute facial paralysis in childhood is essential to provide proper treatment and prevent long term morbidity in children. Embryonal rhabdomyosarcoma in childhood can present with facial paralysis and advances in the field of chemotherapy and radiation therapy have provided excellent remission rates for this tumor with a good quality of life. Surgery should be used as salvage therapy and the PET CT every year is a boon to detect recurrence which can produce a prompt treatment of the tumor.


Low-grade adenosquamous carcinoma (LGASC) is a rare invasive tumor that occurs in breast parenchyma. It has previously only been reported in females. Herein, we describe the case of a 52-year-old male who presented with a palpable mass in his right axilla that he reported had been present for 20-years. This is the first report of a male patient with LGASC. Core needle biopsy pathology revealed a benign mass of mammary origin, but its type was initially misdiagnosed. It was only correctly identified via postoperative pathology after local excision, which indicated that the mass exhibited the typical pathological characteristics of LGASC. Immunohistochemical analysis revealed positive expression of estrogen receptor, which was inconsistent with the typical “triple-negative” immunophenotype of LGASC. After resection of the mass the patient was advised to participate in regular outpatient follow-up. In conclusion, LGASC should be considered in male patients with a mass lesion in their breast or axilla, even when core needle biopsy indicates a benign mass of breast origin. One-stage local resection is recommended for the treatment of male patients with LGASC, but it is crucial to ensure that the margins are negative and postoperative adjuvant radiotherapy is not recommended. Low-grade adenosquamous carcinoma (LGASC) is an extremely rare neoplasm that occurs in breast parenchyma. It is classified as a subtype of metaplastic carcinoma . LGASC oA 52-year-old male presented with a palpable mass in his right axilla that he reported had been present for 20-years. Sonographic examination revealed an irregularly shaped hypoechoic mass with unclear borders, uneven internal echoes, and dimensions of ~3.4 × 3.4 × 1.9 cm in the right axilla . Color DPathology analysis using ultrasound-guided core needle biopsy detected a small amount of breast tissue with duct hyperplasia, consistent with fibroid adenoma. The patient subsequently underwent right axillary mass resection under general anesthesia. Pathology analysis of intraoperatively acquired frozen sections revealed small sweat glands and sly cysts in the fibrous interstitial spaces, which was suggestive of syringomatous adenoma. This finding was inconsistent with the postoperative pathology report, which suggested LGASC . PatholoLGASC is a rare type of breast cancer, and hitherto it has only been reported in females, usually those of middle age , 4. PhysImaging of LGASC does not depict specific manifestations , and it Macroscopically LGASC tumors present as firm masses with ill-defined borders and tan or pale-yellow cut surfaces –3. MicroThe progression of LGASC is very slow , and itsLGASC is an extremely sporadic type of breast cancer that has previously only been reported in females. The current report is the first of LGASC diagnosed in a male patient who presented with a palpable axillary mass. LGASC should be considered in male patients with mass lesions in the breast or axilla, even when core needle biopsy indicates benign lesions of breast origin. One-stage local resection is recommended for the treatment of male patients with LGASC, but it is essential to ensure that the margins are negative. Postoperative adjuvant radiotherapy is not recommended after the excision of male LGASC.All datasets generated for this study are included in the article/supplementary material.The studies involving human participants were reviewed and approved by Ethics Committee of Taizhou Hospital of Zhejiang Province. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.XY and FZ acquired the data and prepared the manuscript. QW performed histological examinations with the assistance of FC. ZW and LM performed data analysis and interpretation. LC and YZ analyzed the ultrasonography images. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


External ear melanoma is rare, and early diagnosis and treatment are paramount for the patient's survival. Four clinical cases are reported, emphasizing the importance of the routine clinical examination of the ears in the dermatological consultation. The study included male and female patients, aged 60 to 81 years old, with melanocytic lesions in the outer ear, evaluated with detailed physical and dermoscopic examination, leading to the identification of lesions suggestive of melanoma. The cases were treated surgically with excision of the lesion, and the diagnoses were confirmed by histopathological study. The therapeutic approach was instituted early as most cases were diagnosed at an early stage, which directly impacted global survival. External ear melanoma is rare, corresponding to 1%–4% of cutaneous melanomas, among an incidence of 76,380 cases in 2016 in the United States. It affects individuals preferably in the sixth decade of life, being predominantly observed in white men, in the auricular helix, and of the extensive superficial subtype.A 60-year-old white woman, farmer, with xeroderma pigmentosum, reported skin cancer since the second decade of life, for which she had previously undergone multiple surgical and non-surgical treatments. The patient underwent clinical follow-up at regular appointments, but was lost to follow-up and resumed after two years. Upon dermatological examination, a brownish macule with irregular color and dermoscopy suggestive of melanoma was observed on the right ear lobe . The les2. Tomographic examination revealed an infiltrative lesion located in the subcutaneous area close to the left ear, probably corresponding to the remaining lesion, in addition to regional lymph node involvement. There was no evidence of distant metastatic disease.Male, 66 years old, white, administrative manager, with medical history of hypertension, dyslipidemia, diabetes, and severe coronary artery disease, sought medical attention due to the onset of a friable tumor on the left ear helix three months prior. The clinical and dermoscopic examination of the lesion was suggestive of nodular melanoma . The patDue to the patient's high cardiovascular risk, surgical treatment of the primary tumor with enlargement of margins and sentinel lymph node search was considered unfeasible. Adjuvant radiotherapy was indicated for local control. The tumor was classified as stage IIIC and the patient has been followed-up for ten months, with no signs of disease progression.A 81-year-old white man, driver, noticed the appearance of an asymmetrical pigmented lesion, with irregular borders, on the helix of his left ear, which had evolved for a year and six months asymptomatically. Upon clinical and dermoscopic examination, he presented findings suggestive of melanoma . An exci2. Ulceration and vascular invasion were not observed. Then, surgery to expand the safety margins by 1 cm was performed. The tumor was classified as early stage (AI) and the patient is being followed-up.A 69-year-old white man, farmer, underwent regular annual dermatological follow-up after invasive squamous cell carcinoma on his left forearm was excised six years ago. In one of the evaluation consultations, a clinical and dermoscopic alteration was detected: an asymmetric pigmented area on the lobe of the right ear, with findings indicating melanoma . An exciExternal ear melanoma (EEM) represents 1% of all primary cutaneous melanomas. The mean age at EEM diagnosis is around the sixth decade of life, and there is a significant predominance in males (86.5%) and fair-skinned individuals.EEM predominantly affects the regions of the helix and ear lobes; the most common subtypes are the superficial spreading (40.1%), followed by the lentigo maligna 33.7%) and nodular (16.4%).3.7% and Dermoscopy assists the diagnosis and some findings can predict the severity of the lesion, as observed in Case 2, in which the atypical vascular pattern was correlated with the deeper invasion of the tumor in the histopathologic examination.2The treatment of EEM does not differ in relation to other cutaneous melanomas; however, due to the anatomical peculiarity of the region and the lack of specific surgical protocols, there is a tendency to perform excision with reduced safety margins, preserving the cartilage and perichondrium not affected by the tumor.It is concluded that the early diagnosis and treatment of EEMs are definitive for survival; the importance of routine clinical examination of the ears in dermatological consultations is reinforced.None declared.Lara martins Fiorio: Design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; critical review of the literature.Lucia Martins Diniz: Design and planning of the study; drafting and editing of the manuscript; effective participation in research orientation; critical review of the literature; critical review of the manuscript.Karla Spelta: Approval of the final version of the manuscript; design and planning of the study; drafting and editing of the manuscript; collection, analysis, and interpretation of data; effective participation in research orientation; intellectual participation in propaedeutic and/or therapeutic conduct of studied cases; critical review of the literature; critical review of the manuscript.Bruna Anjos Badaró: Design and planning of the study; drafting and editing of the manuscript; intellectual participation in propaedeutic and/or therapeutic conduct of studied cases.None declared.


Ependymoma is a circumscribed glioma composed of uniform glial cells with bland nuclei in a fibrillary matrix. It is characterized by the presence of perivascular pseudorosettes. Unusual histopathological findings have rarely been reported in ependymomas, 0.5% of all diagnosed cases. Such unusual and exceedingly rare histological findings include osseous or chondroid metaplasia. To the best of our knowledge, only 15 cases of osseocartilaginous ependymomas have been reported in English literature. We report a 3-year-old boy who presented with ataxia, vomiting, and headache for three months. Radiological imaging revealed a posterior fossa lesion. Histopathological examination of the lesion confirmed a posterior fossa ependymoma with chondro-osseous metaplasia. The present case outlines the clinical presentation, histopathological findings, and outcome of chondro-osseous metaplasia in ependymomas. To date, the etiology of chondro-osseous metaplasia in ependymomas remains uncertain. Further research exploring such phenomenon is of paramount importance to explain how these tumors develop. Ependymoma is a noninfiltrative glioma usually arising near the ventricular system or central canal. Half of all ependymomas, particularly those in the posterior fossa, occur in children, making them the second most frequent solid brain tumor in the pediatric population after medulloblastoma . EpendymTraditionally, according to the WHO grading scheme, ependymomas are divided into three types. These include myxopapillary ependymoma (WHO grade I), conventional ependymoma (WHO grade II), and anaplastic ependymoma (WHO grade III). Unusual histopathological patterns such as pigmented (melanotic) ependymomas, giant-cell ependymomas, ependymomas with extensive tumor cell vacuolization, and chondro-osseous ependymomas have been reported in the literature. These rare patterns are noted in only 0.5% of all diagnosed ependymomas .The presence of bony or cartilaginous differentiation in gliomas is extremely rare and has been recognized in fourth-ventricular ependymomas and midline astrocytomas –9. A revA 3-year-old boy presented with a 3-month history of ataxia, vomiting, and headache, quickly followed by signs of increased intracranial pressure (ICP). Investigations and radiological imaging in private hospitals identified a posterior fossa space-occupying lesion . ConsequThe histopathological examination of the posterior fossa tumor showed mostly a moderately cellular glial tumor with perivascular pseudorosettes . There aAfter four months of the STR, the patient underwent gross total resection to remove the residual tumor. Afterwards, the patient completed radiation therapy. Routine follow-up after two years revealed that the boy's speech was coherent with no oropharyngeal deficits. The VP shunt was compressible, filling promptly on 2.0 pressure, with no signs of malfunction. He was able to ambulate independently with minimal residual gait ataxia noted when running. The patient was otherwise grossly intact.Subsequent radiological imaging revealed no evidence of residual tumor, recurrence, or drop metastasis. The cerebrospinal fluid and cytopathological analyses were negative for malignant cells. The patient is currently followed up in the pediatric neurosurgery clinic.N = 11; 68.7%) were diagnosed in the pediatric age group . The youngest patient who was diagnosed with ependymoma with chondroid metaplasia was one year old (age range: 1–61 years).Chondro-osseous metaplasia in ependymomas is exceedingly rare. An extensive review of the literature revealed 15 prior cases of chondro-osseous ependymomas . We repoN = 14; 87.5%) with the remaining tumors in the frontal lobe, temporo-occipital lobes, and cerebellopontine angle cistern.Of the fifteen reported cases, six depicted both chondroid and osseous metaplasia with no gender predilection. This makes the present case the seventh of its kind. The majority of the tumors were localized in the posterior fossa  had conventional ependymoma (WHO II), six patients (40%) had anaplastic ependymoma (WHO III), one patient had subependymoma/ependymoma, and one had myxopapillary ependymoma. As far as the outcome is concerned, six patients (37.5%) passed away within three years of the diagnosis, one patient (6.2%) was lost to follow-up, and two patients (13.3%) had an uneventful outcome. The boy in the present case had no recurrence of the tumor after two years of the diagnosis.According to Wang et al., the presence of chondro-osseous metaplasia in ependymomas is associated with a dismal prognosis despite aggressive therapy, concluding that such tumors may behave aggressively . Boukas Several mechanisms have been postulated to explain chondro-osseous metaplasia in ependymoma . Two of Gessi et al. identified four patients with cartilaginous ependymomas in his case series. They were not able to establish any relationship between the tumor grade and the presence of chondro-osseous metaplasia. In his case series, there were no high-grade features. They postulated that chondro-osseous metaplasia could have resulted from a simple transformation of mesenchymal stromal tissue within the tumor .The capacity of neoplastic cells to produce cartilage in gliomas may be related to their ability to secrete and synthesize basement membrane material which, upon condensation, may become a chondroid substance . HoweverWe report a case of posterior fossa ependymoma with chondro-osseous metaplasia. Although rare, such metaplastic changes can be identified in ependymomas and the prognostic significance is uncertain.


There are only 6 cases of intraductal papillary mucinous neoplasm (IPMN) complicated with intraductal hemorrhage have been reported in English literatures. All these 6 cases of IPMN occurred in the old people. The present rare case of IPMN complicated with intraductal hemorrhage occurred in a young woman, and mimicked a cystic solid pseudo-papillary neoplasm (SPN) on preoperative imaging findings.A 29-year-old young woman complained of a sustained mild right upper quadrant abdominal pain. CT and MRI showed a lobulated, partly ill-defined cystic lesion located in the pancreatic head. Spotted calcification within cystic wall was seen on CT. The lesion was demonstrated as predominantly homogeneous hyperattenuation on CT and homogeneous high signal without decreased signal on fat suppression sequence on T1WI. After contrast administration, the cystic wall and septa of lesion was showed gradually mild to moderate degree of enhancement over time both on CT and MRI. No communication between lesion and the main duct was found on MRCP and the main pancreatic duct and common bile duct were not dilated. Considering patient’s age, gender and manifestations of lesion on CT and MRI , the present case mimicked as a cystic SPN. The lesion was pathologically confirmed a branch type IPMN after surgical resection.We propose that IPMN may need to be taken into account in the differential diagnosis when pancreatic cystic lesions occur in young women with bleeding, calcification, progressive enhancement of cystic wall and no communication with the main pancreatic duct. Intraductal papillary mucinous neoplasm (IPMN) complicated with intraductal hemorrhage is rarely seen. To the best of our knowledge, there were only 6 cases of IPMN complicated with bleeding have been reported in English literatures and all of them occurred in the elder people  with a male gender predisposition 4 males, 2 females) , 2  on preoperative imaging findings.A 29-year-old young woman, who was 4 months later the end of her first pregnancy and during lactation, complained of a persistent mild pain in her right upper quadrant abdomen for 3 days. No fever, jaundice and weight loss. She had history of cholecystolithiasis, denied history of biliary colics and acute pancreatitis. She was Hepatitis B virus carrier, denied hypertension/pregnancy hypertension and history of diabetes, No surgery and injury history. No smoking and history of alcohol and drug use. Her menarche age was 13 years old, last menstrual period (LMP): 2015.07.05. Her marriage age was 28 years old. Natural birth of her first child. She denied the history of hypertension, diabetes, and tumors.Her Body Mass Index (BMI) was 26.3 , but her waist circumference was not obtained. Physical examinations suspected positive Murphy sign. Laboratory examinations results showed white blood cell count and amylase was normal. Tumor makers of carbohydrate antigen 19–9 (CA199), carcinoembryonic antigen (CEA), alpha-fetoprotein (AFP) were within the normal limits.Initially, she was arranged to perform an upper abdominal ultrasound (US) examination. Results demonstrated a hypoechoic mass with irregular shaped, ill-defined and no blood signals in the retroperitoneal space with uncertain origin Fig. , and somCT  and MR  images showed a lobulated, partly ill-defined cystic lesion located in the pancreatic head. The maximum cross-sectional size of lesion was 3.9 cm and 5.2 cm, the maximum cross diameter of three dimensions of lesion was 5.7 cm. The cystic wall and septa was thin, even thickness, about 1.5 mm. Spotted calcification within cystic wall was found on non-contrast CT images. The lesion was demonstrated as predominantly homogeneous hyperattenuation on CT  and homogeneous high signal without decreased signal on fat suppression sequence on T1WI, and slightly high signal compared to normal pancreas on T2WI, and hypo-intense signal on DWI b = 1000). After contrast administration, no solid component of lesion was detected, and the cystic wall and septa of lesion was showed a gradually enhancement from mild to moderate degree over time both on CT and MR images. Lesion was adjacent to the celiac axis, splenic artery, and the portal vein. Enlarged lymph nodes in the peripheral of the lesion and the retroperitoneal space were not found. Thickened wall gall bladder with some stones but without enlargement and exudation was seen. No communication between lesion and the main duct was found on MRCP images and the main pancreatic duct and common bile duct were not dilated  , 4, ductAs we know, there are only 7 cases, including the present case, of IPMN that have been reported complicated with intraductal bleeding. The causative factors of intraductal hemorrhage may be the epithelium denudation caused by high- pressure stress, resulting in the small vessels around the epithelium injury . The amoCommonly, hemorrhage within tumor manifested as a hyper attenuation without enhancement on CT images, and a high signal on TIWI, a high or low or mixture signals on T2WI. MR imaging, especially FS-T1WI sequence, has been reported that it can more effectively detect the hemorrhage within lesions of pancreas than conventional spin-echo imaging , 11. In Interestingly, we found that the IPMN complicated with intraductal bleeding may have a locational preference for pancreatic head. In 4 of the 7 reported cases (including the present case) had the above-mentioned feature, accounting for 57.1%. And, we believed that the subtype classification of these 4 cases were BD-IPMN according to the descriptions of tumors in their studies, although there was no definitive statements about the subtype classification on their reports. This may suggests that the intraductal bleeding is relatively more frequently occurred in branch duct IPMN than main duct PMN and mixed type IPMN, with a possible underlying mechanism of a higher incidence of more serious pressure overload caused by massive mucin production resulting in more easier and serious of epithelium denudation, and more frequent the small vessels injury, since the high-pressure within branch duct in the pancreatic head could not easily transmit to the main duct, especially for cases of branch duct IPMN with no obvious communication to the main duct.Calcification within IPMN was not commonly seen. The incidence of detection of calcification was approximately 20%, according to the Perez-Johnston’s study in a large cohort of 164 IPMNs . The shaIn our case, what extreamly rare is that the synchronous presence of calcification and intraductal bleeding within IPMN in a young woman were detected on CT and MR images. Considering the gender, age, calcification, intratumoral bleeding, gradually enhancement from mild to moderate degree of cystic wall and no obvious communication to the normal main duct, our case of IPMN mimicked a cystic SPN, and that is the reason for a preoperative misdiagnosis as a SPN.Therefore, we propose that IPMN may need to be taken into account in the differential diagnosis when pancreatic cystic lesions occur in young women with bleeding, calcification, progressive enhancement of cystic wall and no communication with the main pancreatic duct.


Soft tissue masses are fairly encountered clinical entities. Among the rare forms of soft tissues masses are non-pancreatic pseudocysts which are defined as encapsulated fluid collections not lined by epithelium. We are presenting a rare case of a pseudocyst of the back located within the paravertebral muscles at a tertiary care center.Patient presented with a progressively enlarging painless back mass. CT scan was done which showed a 10 × 10 cm cystic lesion along the paravertebral muscles of the back. Surgical excision of the mass was performed revealing an irregularly shaped multilocular cyst. Final pathology showed the lesion to be a pseudocyst.Pseudocysts can arise in a variety of locations such as omentum, adrenal glands, spleen or lungs. The etiology of these pseudocysts is highly variable and can be related to trauma, inflammation, or surgery. The diagnosis of these entities requires proper imaging and histopathologic examination.Pseudocysts arise due to a myriad of conditions and their diagnosis remains a challenge. Our case comprises the first reported case of idiopathic pseudocyst of the back. Although rare, pseudocysts should be considered in the differential of unexplained masses. The e2A 62-year-old non-smoker male patient presented to our hospital with a 6 months’ history of progressively enlarging mass along his back. It was first noticed 2 years ago upon palpation, with time it has gradually increased in size and became cosmetically noticeable. No history of pain, fevers, weight loss, or trauma.Physical exam revealed a 10 cm soft, immobile, painless mass along the right paravertebral with no overlying discoloration of the skin. Computed Tomography (CT) Scan  revealed3Pseudocysts are defined as fluid collections within a capsule not lined by epithelium ,5. PseudTrauma comprises another cause of pseudocysts. Traumatic pulmonary pseudocysts arise following blunt chest trauma . The forIn addition to traumatic causes of enlarging mass on the back, the possibility of soft tissue tumor should be considered. Soft tissue tumors are highly variable and often require imaging as an initial step . Imaging4Our patient represents an unusual case of a massive back pseudocyst. Whilst soft tissue masses are frequently reported complains, nonpancreatic pseudocysts remain rare entities. The etiology of these pseudocysts is variable and can be related to trauma, inflammation, or surgery. Our case comprises the first reported case of an idiopathic pseudocyst of the back. However, pseudocysts should be considered in the differential of such presentations. Identifying pseudocyts is a challenge. Thus, imaging is a valuable tool in diagnosing these lesions. Furthermore, histopathological examination is essential for tissue diagnosis .None.None.Exemption from ethical approval was provided.All patient identifiers were removed. Patient consented for the publication of this case report.WF & MK did the surgery. MM, JK, and IM did the literature review and wrote the manuscript. WF & MK edited the manuscript.1.Name of the registry: NA.2.Unique identifying number or registration ID: NA.3.Hyperlink to your specific registration (must be publicly accessible and will be checked): NA.Mohamad J. Khalife, M.D, FRCP, FACS, FRCS.Not commissioned, externally peer-reviewed.


We herein describe a case of laparoscopic surgery for an esophageal duplication cyst using a near‐infrared indocyanine green fluorescence system. A 64‐year‐old woman with a cystic tumor adjacent to the esophagogastric junction was referred to our hospital for treatment. Esophagogastroduodenoscopy and abdominal CT revealed a 70‐mm submucosal tumor derived from the abdominal esophagus. We performed laparoscopic resection and then evaluated the tissue perfusion of the abdominal esophagus by using a near‐infrared indocyanine green fluorescence system. A Dor fundoplication was performed to prevent postoperative gastroesophageal reflux disease and reinforce the mucosal layer defect. The postoperative course was uneventful, and pathological evaluation confirmed that the tumor was an esophageal duplication cyst. The patient did not develop recurrence in the 24 months after surgery. We have demonstrated that laparoscopic resection of an esophageal duplication cyst may be performed effectively with intraoperative assessment of tissue perfusion using a near‐infrared indocyanine green fluorescence system. Esophagogastroduodenoscopy revealed a submucosal tumor with no mucosal defects in the abdominal esophagus adjacent to the esophagogastric junction. Abdominal CT showed a 70‐mm low‐density area in the abdominal esophagus without distant metastasis Figure A,B. ThusA camera port was inserted through an umbilical incision using an open technique. Under pneumoperitoneum of 10 mm Hg, four ports (two 5‐mm ports and two 12‐mm ports) were inserted into the right upper, right lower, left upper, and left lower quadrants, respectively. Esophageal adventitia layer dissection was followed by muscular layer dissection along the tumor without injury. After enucleation, tissue perfusion of the excised lesion in the abdominal esophagus was evaluated using an NIR‐ICG fluorescence system , and hypoperfusion was found. A Dor fundoplication was performed to prevent postoperative gastroesophageal reflux disease (GERD) and reinforce the mucosal layer defect (Figure Histopathological analysis revealed that the cyst wall was composed of two smooth muscle layers and was lined by pseudostratified ciliated columnar epithelium; the pathological diagnosis was an EDC. The patient was still alive with no recurrence or complications 24 months after surgery.3Esophageal duplication cysts are rare congenital anomalies that require surgical intervention. They account for approximately 10% to 15% of all gastrointestinal duplication cysts, and they are usually located in the lower third of the esophagus. A few EDCs remain asymptomatic until adulthood, although most EDCs become symptomatic and are discovered in early childhood.The indications for treating EDCs include symptoms such as dysphagia, aspiration, hemorrhage, an increase in cyst size, and suspicion of malignancy.In conclusion, we have described our first case of laparoscopic resection for an EDC using an NIR‐ICG fluorescence system. An NIR‐ICG fluorescence system may be useful for preventing postoperative complications by intraoperatively evaluating tissue perfusion in the region of resection in the esophagus.The authors have no conflicts of interest to declare.M.M., K.S., and A.H. performed the operation and postoperative management. M.M. and K.N. drafted the manuscript, and C.K. and K.K. critically revised the manuscript. All authors read and approved the final manuscript.Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor‐in‐Chief of this journal.


Inflammatory fibroid polyps (IFPs) are infrequent gastrointestinal tract benign neoplasms. They mostly occur in the stomach especially the antrum. Signs and symptoms depend on their anatomic location and lesion size. Lesion biopsies are very challenging for accurate diagnosis in these lesions. Histopathological examination of resected tissue along with immunohistochemical studies is the perfect way to confirm the final diagnosis. In this paper, the authors present an unusual case of IFP in a 23-year-old female patient presented to the emergency room (ER) with a picture of intestinal obstruction and severe abdominal pain. Her investigations revealed a huge mass located at the ileocecal valve, with overall features mimic gastrointestinal stromal tumor (GIST) clinical presentation. Surgical resection is considered the most commonly used treatment method. The overall prognosis is good with a low risk of recurrence. Inflammatory fibroid polyp (IFP) was first described in 1920 by Konjetzny as polypoid fibroma . The exaIn this paper, we present a unique case of large IFP involving the ileocecal junction in a young female patient who presented to the ER with intestinal obstruction pictures. Her detailed clinical presentation, hospital course, investigations, and management with more detailed differential diagnosis are discussed below.A 23-year-old female patient with known case of multiple comorbidities, systemic lupus erythematosus (SLE), antiphospholipid syndrome, venous thromboembolism, old DVT, pulmonary hypertension, systemic hypertension, and vitamin B12 and D deficiencies is presented. She was controlled on her medications. She presented to the ER with a picture of intestinal obstruction complaining of a history of severe abdominal pain, sharp, progressive, and continuous, associated with nausea, vomiting, and diarrhea for the past 2 days since her presentation. At the time of admission, the patient looked ill, oriented, in pain about 3-4 out of 5 pain scale. Abdominal examination revealed a slight distended, mild tender abdomen in the right iliac fossa. There were organomegaly or palpable masses. Laboratory data showed no anemia, no elevated blood eosinophil ratio, nor abnormal globulin fraction. A computed tomography (CT) scan with contrast revealed ileocolonic intussusception measuring in maximum longitudinal axis about 7.4 cm with large polypoid soft tissue mass acting as a leading point measuring about 7 cm in its maximum dimension Figures . DiffereIFPs are uncommon benign mesenchymal lesions. IFPs are distributed almost equally between male and female populations. Some suggested that it shows a slight female predominance. The mean age of presentation is 41 years old (average between 19 and 46 years old) . IFP ranTo sum up, we present a case of unique clinical presentation as a giant ileocecal mass causing severe abdominal pain and clinical picture of ileocolonic intussusception, with clinical differential diagnosis of GIST. IFP is an unusual tumor of the ileocecal area and represents a common polyp in the gastric antrum and rectosigmoid colon. The underlying pathogenesis is still not well developed; however, PDGFRA activation is proved in these benign neoplasms. Patients are usually presented with vague sign and symptoms which are not specific. Thus, histopathological with the aid of immunohistochemistry studies is a must to confirm the diagnosis.


Nontraumatic and spontaneous intercostal and intrathoracic herniations are defined as protrusions of intra-abdominal contents through acquired or congenital defects of the abdominal and thoracic walls without any proceeding trauma and are sparsely reported in the literature with less than 35 detailed case reports reported in the literature worldwide. Most of these cases result from abdominal trauma and are considered surgical emergencies. The content of these herniations, as reported in the literature, have classically been lungs and intra-abdominal organs. We report a case of nontraumatic intercostal and intrathoracic liver herniation, which was managed conservatively given minimal liver injury and rapidly improving symptoms. The literature relies on computed tomography for diagnosis and for assessing damage to liver parenchyma, which guides management. Although intercostal liver herniations can be surgical emergencies, our case was managed conservatively.Nontraumatic and spontaneous intercostal and intrathoracic herniations are sparsely and separately reported in the literature. They are defined as a protrusion of intra-abdominal contents through acquired or congenital defects of the abdominal and thoracic walls. A nontraumatic adult liver herniation is sparsely reported with less than 35 case reports on a review of literature worldwide since 1956.A 58-year-old woman with a medical history of hypothyroidism and essential hypertension presented with severe dry cough and nasal congestion for 2 weeks. Three days before presentation, she had noticed a bruise on her right lateral chest wall which gradually increased in size and became painful with radiation to her back. The patient denied any recent history of trauma to the area. Her review of systems was positive for the presence of dry cough. Physical examination revealed a bulging, palpable, and tender ecchymotic lesion over the right ninth and 10th ribs in addition to clear lungs  with a concomitant intercostal herniation between ninth and 10th ribs Figure . Also re6 Based on a review of PubMed, Cochrane review, and Google Scholar, this seems to be a rare case of nontraumatic, spontaneous intrathoracic, and intercostal liver herniation. There have been cases reporting both these herniations separately as discussed below. Individual reports have linked sudden increase in thoracoabdominal pressure gradient such as violent coughing spell, heavy lifting, and parturition as a predisposing risk factor for herniation, with coughing being the most common of them all.9 Commonly, the symptoms of spontaneous intercostal herniation are thoracoabdominal pain, nausea, and vomiting, which are similar to intrathoracic herniation, where dyspnea may also be present and all of these symptoms were present in our patient.10 Ecchymosis or bulge or both over the hernia is usually seen in patients with concomitant intercostal muscle rupture as was seen in our patient.10Croce and Mehta first coined intercostopleuropertioneal hernia, whereas Cole et al first reported transdiaphragmatic intercostal hernia which resulted from violent coughing spells.11 Contents for 2 of these were intestinal loops, and 1 of them was liver, and all of them were treated with thoracotomy. A review of 20 cases of spontaneous diaphragmatic rupture of diaphragm  by Losanoff et al were also repaired by thoracotomy as compared to our case, which was managed conservatively.1 A case reported by Bendinelli et al, commented on the failure of conservative measures for a lacerated liver herniation, which led to liver necrosis, ultimately needing surgery.7Macedo et al reported 3 cases of transdiaphragmatic intercostal hernia secondary to cough.Surgical interventions in our case were deferred because there was no evidence of laceration and instead, a close follow-up was implemented. A key derivation from this review is to maintain a high suspicion of liver herniation in patients with poor abdominal muscle strength with sudden worsening of cough. The herniation may either be abdominal or transdiaphragmatic, both of which need evaluation. Given the rarity of such events, these patients can present as a diagnostic challenge, and delay in care can result in poor outcomes, especially in patients with organ damage. A consensus for management can be difficult because of the sparsely reported cases. Although an injured or strangulated liver herniation is a surgical emergency, as has been reported in the literature, we propose the possibility of a conservative approach for uninjured liver herniation. This would also include close medical and surgical follow-up after discharge from the hospital.Author contributions: All authors contributed equally to this manuscript. PS Harne is the article guarantor.Financial disclosure: None to report.Previous presentation: This case was presented at the American College of Gastroenterology Annual Scientific Meeting; October 25-30, 2019; San Antonio, Texas.Informed consent was obtained for this case report.