Atypical fibroxanthoma (AFX) is a tumor that commonly presents on the head or neck in older individuals. Making a definitive diagnosis of AFX is challenging, and frequently, it is hard to distinguish from pleomorphic dermal sarcoma (PDS). There are no clear recommendations regarding the treatment of AFX, but an extensive surgery is actually considered the best option. Electrochemotherapy (ECT) is a novel therapeutic modality of local treatment in which the application of electrical pulses, enhancing cell membrane permeability, allows greater intracellular accumulation of chemotherapy drugs in the skin or subcutaneous tumors.  We report a case of a 78-year-old male affected by a red, ulcerative, dermal, scalp nodule, which was treated with ECT with a complete clinical response. We have also reported literature data on this topic.  In this case, ECT showed to be an effective and safe treatment for recurrent neoplasms of the head and neck, considering the complete response obtained and the absence of disease relapse after two years.  To the best of our knowledge, this is the first case report that shows great clinical results using ECT after surgery in relapsed AFX/PDS. However, more studies are needed to confirm our results. Electrochemotherapy (ECT) is the most developed electroporation-based cancer treatment, and it demonstrated to be highly effective, with complete response rates between 60% and 70% and objective response  rates of 80% in primary or relapsed cutaneous tumors , 2. ThisIn February 2015, a 78-year-old white male was referred to us for the occurrence of a solitary rapidly growing nodule on the scalp. The nodule measured 3 cm in diameter. Clinical examination revealed a subcutaneous, bright red, ulcerated, dome-shaped lesion with irregular margins. The patient underwent an intervention of surgical excision, covering the substance loss with a skin graft from the left inguinal region. Subsequently, accurate haemostasis and suture were performed, and a compressive medication was applied. The histopathology described an ulcerated undifferentiated malignant neoplasm consisting of cells of different dimension and high mitotic index. The immunohistochemistry showed a weak positivity for S-100 and HMB45, negativity for CK, and moderate positivity for CD68. The surgical margins were clear and the diagnosis was atypical fibroxanthoma.After a month from the surgery, in May 2015, a new nodule appeared in correspondence of the graft, suggesting a recurrence . Conside2 of body surface area, diluted in 100 cc of physiologic solution in 15 minutes). The patient underwent a general anesthesia (deep sedation with Propofol—Diprivan®) before an adjustable linear needle electrode by IGEA® was introduced into the tumor mass at a depth of 15 mm; also, a safety margin of 1 cm was treated around it. A series of 8 pulses of 1000 V/cm was delivered at a frequency of 5 kHz and duration of 100 microseconds, as recommended by ESOPE guidelines [A local treatment with ECT has been performed in the region of the cutaneous relapse using a Cliniporator® EPS-02 produced by IGEA®. The procedure is reported following the guidelines by Campana et al. . ElectroAtypical fibroxanthoma (AFX) is a tumor correlated to sun exposure that most commonly presents as a solitary red or pink papule or dermal ulcerative nodule of the head or neck of elderly people . AFX affECT is a local antitumor therapy that started being developed in the 80s . StartinECT use is approved for skin and subcutaneous tumors, independently of their histological origin . HoweverTo the best of our knowledge, no data concerning ECT as a locoregional treatment for AFX/PDS are available. Therefore, we present the first case in literature and hence the importance of this report in order to expand the application field of this new promising treatment also for AFX and PDS, considering its effectiveness and safety in elderly patients. However, further studies are needed to confirm our results.


The Landscape Montage Technique was originally developed by Hisao Nakai, a Japanese psychiatrist, to pursue the possibility and application of a psychotherapeutic approach using drawing for patients with schizophrenia. Drawing was initially adopted to evaluate patients with an impaired ability for verbal expression, particularly for the diagnosis and treatment of patients with schizophrenia. Since its development, the Landscape Montage Technique has been utilized in various clinical settings throughout Japan. This study aimed to evaluate the psychiatric conditions of a patient diagnosed as having primary progressive aphasia using the Landscape Montage Technique at a 3-year follow-up.We present the case of a 64-year-old, right-handed Japanese woman initially diagnosed as having logopenic variant primary progressive aphasia or logopenic aphasia. At a 3-year follow-up, logopenic aphasia progressed to behavioral variant frontotemporal dementia or frontotemporal dementia. According to her husband, she began to have speech difficulties approximately 5 years before her first visit. The results of neurocognitive tests suggested mild cognitive impairment or early stages of dementia. Her clinical dementia rating score was 0.5, suggesting a diagnosis of mild cognitive impairment. She had a Raven’s Colored Progressive Matrices score of 31 out of 36, which indicated a nonverbal cognitive ability that was greater than the 90th percentile for her age. The Japanese Standard Language Test of Aphasia, which was performed at two points during the follow-up, indicated the possibility for a diagnosis of primary progressive aphasia given the progression of her aphasia. Based on her clinical symptoms and Japanese Standard Language Test of Aphasia results, a diagnosis of logopenic variant primary progressive aphasia was established. Magnetic resonance imaging revealed severe predominant left frontal and anterior temporal atrophy, as well as bilateral parietal atrophy. Amyloid beta deposition was negative. At the 3-year follow-up, logopenic variant primary progressive aphasia had progressed to behavioral variant frontotemporal dementia. However, the Landscape Montage Technique allowed for the diagnosis of behavioral variant frontotemporal dementia only 2 years after baseline.The present study showed that the Landscape Montage Technique can be useful for diagnosing behavioral variant frontotemporal dementia that starts as logopenic variant primary progressive aphasia at earlier stages. The Landscape Montage Technique (LMT) was originally developed by Hisao Nakai, a Japanese psychiatrist, to pursue the possibility and application of a psychotherapeutic approach using drawing for patients with schizophrenia , 2. DrawPPA is defined as “slowly progressive aphasia” and can be classified into three types: nonfluent/agrammatic variant PPA (nvPPA), semantic variant PPA (svPPA), and logopenic variant PPA (lvPPA) . The imaWe report a case involving a 64-year-old, right-handed Japanese woman with PPA, which could have been diagnosed as lvPPA or logopenic aphasia . At a 3-We present the case of a 64-year-old, right-handed Japanese woman initially diagnosed as having lvPPA. At a 3-year follow-up, lvPPA had progressed to bvFTD, with results from the Neuropsychiatric Inventory assisting in the diagnosis , 9.et al., 2011) [Her medical and family history were noncontributory. According to her husband, her speech difficulties began approximately 5 years before her first visit, which he thought was due to psychogenic factors considering that his business had not been doing well during those days. Her husband stated that she had difficulty recalling words . Althouget al., 2011) [et al., 2011) [et al., 2011) [et al., 2011) [et al., 2011) [et al., 2011) as shown by magnetic resonance (MR) images revealing predominant left frontal and anterior temporal atrophy at baseline and bilateral parietal atrophy, although parietal atrophy was lesser compared to frontal and temporal atrophy  [During the first visit, she appeared to recognize what was said. Therefore, cognitive function tests could be performed . Her cog., 2011) : observa., 2011) ; subtest., 2011) ; and no ., 2011) . The afo., 2011) . Moreove., 2011) .Table 1et al. (2011) [et al. (2011) [At baseline and 30 months later, our patient’s findings satisfied the criteria for imaging-supported lvPPA diagnosis . However. (2011) . In addi. (2011) , 8. More. (2011) . TherefoLMT has been longitudinally performed to evaluate the clinical features of patients in a nonverbal manner. Since its development, LMT has been utilized in various clinical settings across Japan. The basic procedure for LMT is as follows. First, the therapist draws a border with a black felt-tip pen on a white A4-sized sheet of paper in the presence of the patient. The therapist then tells the patient, “Now, I would like you to draw a landscape” and “I do not aim to evaluate your drawing ability.” The client is then asked to draw ten items in a predetermined order. The items are described as follows: “a river or rivers,” “a mountain or mountains,” “rice paddy,” “a road or roads,” “a house or houses,” “a tree or trees,” “a person or persons,” “a flower or flowers,” “a creature or creatures,” and “a stone or stones.” Finally, the therapist asks the client to color the images –3.et al., 2011), which could be called “clonic perseveration” [et al., 2011) were obvious from observation, thereby satisfying the criteria for possible bvFTD [et al., 2011) [In the present case, LMT was performed on our patient with logopenic aphasia to reveal her clinical features, both at baseline and 2 years later. From baseline until 2 years later, her speech, which stopped after a sentence, was fluent but sparse. She barely managed to answer closed questions 2 years after baseline. Although her listening comprehension and nonverbal expression at the 2-year follow-up seemed similar to those at baseline, LMT results indicated otherwise. The drawing she created at baseline is shown in Fig. eration” . Moreovele bvFTD . Both th., 2011) .Fig. 2aThe timeline has already been presented in Table S1 of our previous report . To outlThis case demonstrates the potential of LMT for diagnosing bvFTD at an early stage, particularly among patients whose bvFTD started as lvPPA or other types.FTD can be diagnosed according to established criteria, which are now predominant and include both observational and neuroimaging findings . UsuallyHowever, LMT can induce abnormal behaviors among patients with bvFTD. This is why the current patient had been diagnosed as having bvFTD a year earlier than usual. This striking discovery regarding the utility of LMT could help in the early diagnosis of bvFTD and the establishment of a care plan.Some limitations of the present report need to be considered. First, the current report discusses only one case. Therefore, future studies should investigate more cases, particularly other types of PPA. Second, although SLTA was used to evaluate aphasia, this test has been made especially for Japanese-speaking individuals. However, other language versions of SLTA remain to be established and validated in the future. Third, the current report could not demonstrate the utility of LMT for treatment, which should be investigated in the future. Lastly, instructions and procedures for LMT in languages other than Japanese have been quite limited. However, this could change following worldwide spread of LMT in the future.


We report a case of vulvar clear cell adenocarcinoma in a woman presenting with a lump and pain in the right side of the labia majora. Three years prior to this visit, she underwent a total abdominal hysterectomy with bilateral salpingooophorectomy and excision of a labial mass. Pathological examination revealed adenomyosis and multiple leiomyomas in the uterus, endometriotic cysts in both ovaries, and foci of atypical endometriosis in the labial mass. The results of an incision biopsy performed before referral indicted only apocrine hidrocystoma. Physical examination revealed a hard mass at the right labia majora extending to the right groin. The mass seemed to be in continuity with the pubic symphysis that would require pubic bone excision and reconstruction with flap surgery to achieve complete resection. However, the patient refused such extensive surgery. Based on previous diagnosis of vulvar endometriosis, she had been treated with GnRH agonists and depot medroxyprogesterone acetate. However, the mass developed into an ulcer and increased in size. A second biopsy of the mass was undertaken, and the pathological diagnosis was clear cell carcinoma with coexisting atypical endometriosis. Computed tomography of the abdominopelvic region showed an ulcerative mass at the right labia majora and nodal metastasis at the external iliac and inguinal regions. Systemic chemotherapy was administered. The growth of the tumors stabilized during the first two cycles of chemotherapy but rapidly progressed thereafter. At 17 months after her initial presentation, the patient passed away due to the progression of the disease. Endometriosis is characterized by the presence of endometrial glands and stroma similar to that in the endometrial lining, but in a location outside of the uterine cavity . EndometWomen with endometriosis have an increased risk of certain histologic types of cancer, which mostly involve the ovary . In the A 46-year-old nulliparous woman presented after having experienced pain in the right side of her groin and labia majora for four months, as well as a lump that was increasing in size. She had previously undergone three laparotomies for ovarian cystectomy at 20, 10, and 8 years prior to this visit due to her severe progressive pelvic pain. The pathological examinations of surgical specimens obtained from all three operations indicated ovarian endometriosis.Three years prior to this visit, she experienced progressive pelvic pain and she also noticed a growing mass at the right labia majora. She underwent total abdominal hysterectomy (TAH) with bilateral salpingooophorectomy (BSO) and excision of a 3.7 × 2.5 cm labial mass. Pathological examination revealed adenomyosis and multiple leiomyomas in the uterus as well as endometriotic cysts in both ovaries. The labial mass contained focal atypical endometriosis on a background of benign endometriosis Figures  and 2. TOne month before this visit, she had undergone an incision biopsy of her labial mass at the provincial hospital, and the pathological report indicted only apocrine hidrocystoma. Nevertheless, the mass had increased in the size with accompanying progressive pain.Upon presentation at our hospital, there was a 7x4-cm hard mass at the right labia majora extending to the right groin area. The mass seemed to be in continuity with the pubic symphysis that would require pubic bone excision and reconstruction with flap surgery to achieve complete resection. However, the patient refused such extensive surgery. Based on the previous pathological diagnosis of endometriosis of the vulva in this patient, she was then initially treated with two doses of GnRH agonist followed by one 150 mg dose of depot medroxyprogesterone acetate. These medications were administered with the aim of shrinking the bulk of the mass. However, the mass developed into an ulcer and rapidly increased in size . A seconComputed tomography of the abdominopelvic region showed an ulcerative mass at the right labia majora and nodal metastasis at the bilateral external iliac and superficial inguinal regions that varied in size from 2.0 to 3.5 cm. Other CT findings were unremarkable.As this case was considered inoperable, systemic chemotherapy consisting of paclitaxel and carboplatin was administered. The growth of the tumors stabilized during the first two cycles of chemotherapy but rapidly progressed thereafter. Palliative treatment under multidisciplinary care was subsequently initiated. Pelvic radiation was administered to slow down the progression of disease and to alleviate pelvic pain. At 17 months after her initial presentation, the patient passed away due to the progression of the disease and severe sepsis.We report a case of vulvar clear cell carcinoma in a 46-year-old nulliparous woman who presented with a four-month history of a lump and pain in the right side of her groin and labia majora. Clear cell carcinoma of the vulva is a rare phenomenon and the most likely mechanism in this case is malignant transformation of residual foci of atypical endometriosis within the vulva.Although the pathogenesis of malignant changes in endometriosis remains unclear, hyperestrogenism has been proposed as one of the potential risk factors based on the fact that there are some reported cases in which endometriosis-associated adenocarcinoma developed in the large bowel, bowel mesentery, peritoneum, and vagina during estrogen replacement therapy after TAH with BSO –10. The Currently, the research is inconclusive with regard to the mechanisms that lead to the development of extrapelvic lesions. Extrapelvic endometriosis can occur via iatrogenic seeding of endometrial tissue at surgical sites during labor or an operation in which the endometrial cavity entered such as cesarean section or myomectomy. This accounts for the presence of endometriosis in previous episiotomy sites and abdominal wall scars from cesarean sections. Other explanations for the spread of endometrial tissue to the outside of the pelvis include (i) the transportation of viable endometrial tissue in circulating fluid within the abdominal cavity, (ii) lymphatic or hematogenous dissemination, and (iii) the direct extension from pelvic endometriosis to extrapelvic areas .In our patient, the lesion involved the right side of groin and labia majora. The occurrence of endometriosis in the vulva in our patient, which subsequently transformed to clear cell carcinoma, may have extended from the pelvis via the round ligament. Though extremely rare, the occurrence of endometriosis in the extrapelvic portion of the round ligament has been reported, and it involves the right side in more than 90% of cases –17. One Due to the rarity of endometriosis-associated cancers, the optimal treatment modalities in these cases are not yet known. Complete surgical removal remains the most common treatment in cases of malignant transformation of endometriosis. Neoadjuvant chemotherapy may allow for a complete resection to be performed in some cases . In caseClear cell adenocarcinoma of the vulva is a rare phenomenon and the most likely mechanism in this case was malignant transformation of residual foci of atypical endometriosis within the vulva. Our report confirms the relatively poor prognosis of this type of tumor, particularly in cases of later presentation.


Bilateral paranasal sinus diseases are rarely reported in horses. Treatment using a bilateral frontonasal bone flap on a standing, regular-sized adult horse has not been described previously. A 13-year-old Thoroughbred gelding was evaluated for bilateral mucopurulent nasal discharge. Radiographic, endoscopic and computed tomographic examinations revealed bilateral sinus pathological changes consistent with an ethmoid haematoma involving the maxillary and frontal sinuses. A bilateral frontonasal bone flap was created under standing sedation and local anaesthesia. A tracheotomy was performed initially to ensure a patent airway during the procedure. Additional analgesia had to be given to transect the dorsal part of the nasal septum while elevating the flap. The sinus masses were removed and communications with the nasal cavities created uneventfully. Small sequestra were removed transendoscopically from the left caudal maxillary sinus 4 weeks after the initial procedure. The horse made a complete recovery with an excellent cosmetic outcome. Histopathology revealed the mass to be a sinus cyst. We concluded that a bilateral sinus bone flap can be used in adult regular-sized horses to access the left and right paranasal sinuses simultaneously. Regional nerve blocks should be performed in order to increase analgesia. A temporary tracheotomy prevents any airway obstruction during the procedure. The post-operative cosmesis is excellent.frontonasal bone flap; sinus cyst; computed tomography; standing surgery; horse. Bilateral paranasal sinus diseases are rarely reported in horses. In previous studies reporting on large numbers of horses with sinonasal diseases, only 3.0% – 4.5% of cases had bilateral sinus involvement .A 13-year-old Thoroughbred gelding was presented to the Onderstepoort Veterinary Academic Hospital with a 1-month history of unilateral, mucopurulent nasal discharge that progressed to a bilateral nasal discharge 1 week prior to presentation. Upon clinical examination, all vital parameters were within normal limits. A moderate bilateral facial swelling was noted over the caudal maxillary sinuses as well as a bilateral purulent nasal discharge. Shallow infundibular caries were observed on teeth 109, 110, 209 and 210.Radiographic examination showed a round, well-circumscribed soft tissue opacity situated in the left ethmoid region extending rostrally into the nasal passages as well as towards the right side. Fluid was present within both caudal maxillary sinuses. The left ventral conchal sinus appeared enlarged with increased radio-opacity. Additional masses were visible in the dorsal conchal sinuses .Upper respiratory endoscopy revealed a reddish globulous mass, compatible with an ethmoid hematoma, extending from the left ethmoid region rostrally into the nasal cavity, occluding the left sinus drainage angle  . MucopurComputed tomography (CT) performed under standing sedation  revealed extensive bilateral fluid accumulation in the rostral and caudal maxillary sinuses, ventral and dorsal conchal sinuses, sphenopalatine sinuses as well as the left ethmoidal sinus, sometimes contained by mineralised walls. Discrete masses were obvious in the left dorsal conchal sinus. These findings were consistent with widespread bilateral ethmoid haematomata .µg/kg, intravenously [IV] as a bolus followed by an infusion at 0.5 µg/kg/minute initially and then titrated to effect) and morphine . Single dosages of flunixin meglumine  and intramuscular procaine penicillin  were administered. In anticipation of surgical haemorrhage and associated packing, a temporary tracheotomy was performed preoperatively to ensure a patent airway (Barakzai & Dixon Three days after presentation, the horse underwent surgery under standing sedation using detomidine (Domosedan, Zoetis, Sandton, South Africa; 10 Masses consistent with ethmoid haematomata were removed from both frontal and caudal maxillary sinuses using traction with sponge forceps and digital separation, and specimens thereof were submitted for histopathology. Maxillary septal bullae were digitally perforated and these communications enlarged with arthroscopic rongeurs (Dixon et al. The sinus packings were removed 48 h post-operatively through the nostrils with the horse sedated. Because a piece of stockinette was left behind, the right caudal maxillary sinus had to be trephined to extract the latter using arthroscopic rongeurs and sinoscopic guidance Nickels . Skin stHistopathological examination revealed the mass to be a sinus cyst with its wall being lined on both sides by a layer of pseudostratified respiratory epithelium. The connective tissue subjacent to the epithelium displayed various degrees of oedema and haemorrhage, both acute and chronic, granulation tissue formation and chronic inflammation. All cyst walls contained bony plates centrally that were made up of bony spicules that showed varying degrees of remodelling.Four weeks after surgery the horse was re-evaluated because of a mild left unilateral mucopurulent nasal discharge. The cosmesis of the surgical site was deemed excellent . The traNine months after initial surgery, no recurrence of the nasal discharge was observed. Satisfaction of the client was excellent.We described the successful surgical management of a bilateral sinus cyst using a large bilateral frontonasal bone flap on a standing adult Thoroughbred gelding.The frontonasal bone flap was previously reported as being the ideal approach to the paranasal sinuses because it allows access to the conchofrontal sinus, the caudal maxillary sinus and the ventral conchal and rostral maxillary sinuses after perforation of the maxillary septal bulla (Easley & Freeman The patient experienced discomfort when we sectioned the dorsal nasal septum in order to lift the bone flap caudally, and we had to inject local anaesthetic onto the sinus mucosa and into the dorsal septum. Previous authors also injected local anaesthetic into the sinus before making bone flaps (Easley & Freeman Although it was stated that because of the minimal blood loss occurring in standing sinus flap procedures, sinus packing may not be required (Easley & Freeman Sequestration is a well-known complication of sinus surgeries. It can occur on the margins of the flap or into the sinuses. We did not experience any healing complication, compared to other cases (Easley & Freeman No post-operative sinus lavage was performed in our case. This could have prevented dehiscence from occurring because early aggressive lavage could lead to fluid leakage into the peri-incisional tissues (Barakzai & Dixon Even if radiographs and endoscopy were useful in confirming the bilateral nature of the affection, CT allowed a thorough three-dimensional evaluation of all the sinus cavities and permitted elaboration of the surgical plan, as previously reported (Barakzai & Dixon Our initial diagnosis based on endoscopic and surgical findings was an ethmoid haematoma. However, histopathology revealed the mass to be a cyst. Sinus cysts generally carry an excellent prognosis, with only a single treatment usually required (Dixon et al. The aesthetic result of this large bilateral frontonasal bone flap was considered excellent and similar to what has been reported for unilateral maxillary and frontonasal bone flaps (Dixon et al. In conclusion, it appears that a large bilateral sinus bone flap can be used successfully to access left and right paranasal sinuses simultaneously without jeopardising the horse’s cosmesis. A temporary tracheotomy should be considered if substantial intraoperative bleeding is expected. Regional nerve blocks should be considered to ensure adequate analgesia.


Pancreatic acinar cell carcinoma (PACC), a rare variant of pancreatic malignancy, is generally managed the same way as pancreatic ductal adenocarcinoma (PDAC). Surgical resection is the gateway to curing it; however, once it metastasizes , systemic chemotherapy has been the only option, but with unfavorable results.A 67-year-old man with symptoms of loss of appetite and weight underwent surgery for malignancy of the pancreatic tail extending into the entire pancreas. The pathological diagnosis was PACC following total pancreatectomy. Twenty-four months after the pancreatectomy, a solitary liver metastasis was treated by partial hepatectomy, and, subsequently, 4 months later, he presented with melena. Further examination revealed a type-2 rectal tumor. Histological examination following biopsy revealed it to be rectal metastasis of PACC, and it was treated by abdominoperineal resection. Subsequently, the patient did not have tumor recurrence as of 40 months after pancreatectomy.This is a rare case of PACC presenting with metachronal metastases in the liver and rectum, and we successfully treated them by surgical resections. Since the malignant behavior of PACC is usually less than that of PDAC, surgical resection could be an option even for metastatic lesions when the number and extent of metastases are limited. Pancreatic acinar cell carcinoma (PACC) is relatively rare; it accounts for 1–2% of pancreatic malignancies . The cliDespite these differences between PACC and PDAC, the clinical management is usually same in both. Surgical resection is the only potentially curative treatment for primary PACC, and chemotherapy or radiotherapy has been performed for locally advanced or metastatic PACC . HoweverHere, we report a case of PACC with unusual metachronal metastases in the liver and rectum after 24 and 28 months of the initial curative pancreatectomy, respectively. Metastases of PACC are commonly seen in the liver and lymph nodes, whereas colorectal metastasis is atypical and extremely rare. We chose surgical resection for both liver and rectal metastases with satisfactory outcomes.A 67-year-old man was admitted to the hospital with symptoms of loss of appetite and weight. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a pancreatic mass extending into the entire pancreas, splenic vein, and inferior mesenteric vein Fig. . The patPACC, a rare pancreatic cancer, demonstrates different behaviors and outcomes in comparison with PDAC. PDAC is the most common type of pancreatic cancer and is fatal in almost all cases. Frequently, it extends and/or metastasizes to the vessels, perineural tissue, adjacent organs, lymph nodes, liver, lungs, bones, and adrenals –10. In cResection of metastases in PACC, as performed in this case, is not routinely performed . We chosThe colon and rectum are quite rare sites of metastases from pancreatic cancer, as only a few such cases have been reported –23. As aWe presented a rare case of metachronous hepatic and rectal metastases of PACC treated with surgical resection. Our case indicates that aggressive and curative surgery can be an option in metastatic PACC. The therapeutic strategy against metastases from “less invasive” PACC should include surgical resection in addition to chemotherapy to improve the prognosis.


Intermuscular hemangioma (IH) usually occurs in the muscles of the limbs and trunk, but can rarely occur in the occipital region. IH in the occipital region is easily misdiagnosed as arteriovenous malformation (AVM).A 31-year-old woman had a right occipital mass for 5 months without pulsation.Head computered tomography angiography (CTA) and digital substraction angiography (DSA) examinations showed that the lesion was mainly vascular, approximately 3 × 5 cm in size, and supplied by occipital arteries and the muscular branches of vertebral arteries. The venous drainage of the lesions communicated with the suboccipital vein plexus and the paravertebral vein in the venous phase, indicating AVM. Postoperative histological investigation showed the lesion was a hemangioma.It was recommended for surgical removal. The surgery was carried out under general anesthesia. The lesion showed a clear boundary. The occipital artery touched the anterior margin of the lesion, was exposed and ligated, and was removed around the lesion. The lesion consisted of massive blood vessels, and the surrounding muscles were swollen, indicating IH.After the lesion was removed, the normal muscle tissue around the lesion was also removed.The patient achieved a good recovery after surgery, and pathology confirmed IH. A postoperative 1-year CTA review was performed and showed partial residual, then the radiotherapy was recommended. She refused further radiotherapy, follow-up 2 years later showed no enlargement of the lesion.Although IH rarely occurs in the occipital region, this can occur. Due to the complexity of the drainage veins in the occipital region, these IH are prone to misdiagnosis as AVM. The case was characterized as an arteriovenous malformation (AVM) on imaging due to its abundant blood transport in the posterior occipital area. The performance and treatment of this case are reported, and the related literature is reviewed.Intermuscular hemangioma (IH) is a type of benign endothelioma that often occurs in the muscles of the limbs and in the body, but can rarely occur in the head and neck. IH occurring in the head and neck accounts for approximately 15% of all IHs. While they mainly occur in the masseter, trapezius muscle and temporalis, they can rarely occur in the posterior occipital area.2The patient, a 31-year-old woman, was admitted to the department of neurosurgery at The First Hospital of Jilin University due to a “right occipital mass for 5 months”. The patient was in good health, and her family members did not have similar lesions. A physical examination showed that the mass was in the right occipital region at the upper margin of the sternocleidomastoid muscle and exhibited slight tenderness without obvious pulsation or vascular murmurs during auscultation.A head CTA examination at admission suggested that the lesion was approximately 3 × 5 cm in size, was mainly composed of vascular components, presented as a “wool mass”, was located in a subcutaneous region, involved the muscles, and showed obvious enhancement. The lesion was mainly supplied by the occipital and muscular branches of the vertebral arteries. The venous drainage of lesion communicated with the suboccipital venous plexus and the paravertebral venous plexus, and the venous drainage communicated with the subcutaneous cervical superficial veins Figs.  and 2.A further DSA examination revealed that the blood supply of the lesion was mainly from the occipital artery and less from the muscle branches of the vertebral arteries Fig. . The lesAfter the lesion was removed, the normal muscle tissue around the lesion was also removed. The specimen was sent to pathology, and postoperative pathology confirmed that the lesion was IH accompanied by thrombolytic recanalization. HE staining showed that the lesion almost purely consisted of capillaries with only rare cavernous components among the muscles. CD31 staining was positive in the vessel endothelium, indicating that the lesion was an IH Fig. . The pat3Being a Case Report, our institution does not require formal Ethical Approval. Written informed consent was obtained from the patient and her husband for publication of this case report.4,2 Based on its pathological classification, IH is divided into a capillary type, a cavernous hemangioma type and a mixed type, the capillary type the most common. The IH, in this case, occurred in the right occipital muscle and was capillary type. Because this location is rare, there have been few such cases reported in the literature. Our case enriches our view of IH and indicates one more type of disease that should be considered in diagnoses of opisthotic-occipital lesions.IH is a type of benign endothelioma. The first reported case of IH is attributed to Liston in 1843, who called this entity an “erectile tumor”. IH is rare and often occurs in the muscles of the limbs and the trunk but only rarely occurs in the head and neck.,6 In the posterior occipital region, the arteries mainly include the occipital artery and the muscular branches of the vertebral artery, and the drainage veins are the paravertebral venous plexus and the suboccipital venous plexus. Its extensive venous drainage introduces great difficulty when diagnosing vascular diseases in this region. The IH, in this case, was mainly supplied by the occipital branches and the muscle branches of the vertebral artery and drained to the suboccipital venous plexus, paravertebral venous plexus and subcutaneous superficial vein. These characteristics of its blood flow share similarities with those of AVM that occur in this area.IH is rarely found in the lateral occipital region, which is a very critical region, mainly due to its complex arterial and venous structures.However, postoperative pathology confirmed the lesion was an IH. A retrospective analysis of DSA images of the IH revealed differences between IH and AVM. Due to the tumor nature of the IH, different blood supplying arteries will supply different parts of the IH, and the IH can therefore only be partially clearly displayed on angiography of different blood supplying arteries. As shown in Figure  The treatments for IH include surgical resection, sclerotherapy, cryotherapy, and radiotherapy. Surgical resection is the best choice for treatment of this disease. Other methods can also be considered to alleviate pain, reduce depressive symptoms and reduce postoperative recurrence. IH have a rich blood supply. During surgery, the blood supplying arteries of the IH should first be controlled. In this case, the occipital artery was located at the anterior edge of the IH. It was ligated and removed around the IH. The IH was found to consist of massive blood vessels during the operation. The adjacent muscles were swollen, and postoperative pathology confirmed IH (capillaries).IH has a rapid growth rate and is prone to recurrence and therefore requires active treatment. Although radiotherapy and other methods are feasible for treating tumor recurrence after IH resection, as full a resection as possible should be achieved in the first operation. In this case, the IH was treated with radiotherapy because little residue remained.A postoperative 1-year CTA review of this case showed partial IH residues. Reports in the literature indicate that the recurrence rate of IH after surgical resection is 9% to 28%, with recurrence rate 28% in mixed type, 20% in capillary type, and 9% in cavernous hemangioma type.Therefore, although IH rarely occurs in the lateral occipital region, this can occur. Due to the complexity of the drainage veins in the occipital area, IH is prone to be misdiagnosed as a vascular malformation on imaging. A lateral occipital IH should be surgically removed, and if there is any residual, radiotherapy should be recommended for.Conceptualization: Hao Chen and Yunbao Guo.Methodology: Guangming Wang.Resources: Baofeng Xu.Writing – original draft: Yunbao Guo.Writing – review & editing: Kun Hou and Jinlu Yu.


Visceral artery aneurysms (VAAs) and visceral artery pseudoaneurysms (VAPAs) are often found incidentally through imaging of a patient presenting with vague symptoms of abdominal pain, hematochezia, hematemesis, and melena. Due to the asymptomatic nature, the etiology is often unknown. However, suspicion for VAA and VAPA should remain high for those presenting with symptoms listed above after trauma or pancreatitis. Here we discuss a case of traumatic ileocolic pseudoaneurysm that has only been discussed a handful of times in the literature. Aneurysms or pseudoaneurysms occurring in visceral arteries and their branches such as the celiac trunk, superior mesenteric artery, and inferior mesenteric artery are termed either visceral aneurysms (VAAs) or visceral pseudoaneurysms (VAPAs). VAA and VAPA of the splanchnic arteries is an uncommon entity with the incidence ranging from 0.1% to 2% of people [An 80-year-old male presented to the ED with a chief complaint of only vague abdominal pain after suffering a fall off a ladder at home. He stated that the pain came on acutely, intermittent and colicky and severe in intensity with bouts of acute exacerbations accompanied by significant nausea and vomiting. Trauma surgery was consulted and he was worked up in the routine advance trauma life support (ATLS) protocol. Primary and secondary were essentially negative except for focal abdominal tenderness. His abdominal exam was significant for distension, upper abdominal tenderness to palpation, localized guarding, and no rigidity or masses.He denied any loss of consciousness, chest pain, numbness, tingling, hematochezia, and hematemesis. Upon examination, his vital signs were notable for a temperature of 97.6F, pulse of 86 beats per minute, and blood pressure of 167/84. His abdominal exam was significant for distension, upper abdominal tenderness to palpation, guarding, and no masses. Labs were significant for glucose of 278, lactic acid of 3.5, hemoglobin of 13.6, and hematocrit of 38.7.His past medical history includes atrial fibrillation, diabetes mellitus, essential tremor, hypertension, seizures, sleep apnea, and a thyroid disorder.Initial imaging at that time included a CT scan without contrast of the chest, abdomen, and pelvis. Significant findings on the CT showed few segmentally dilated loops of small bowel in the abdomen with air-fluid levels with a transition point. The findings suggested a small bowel obstruction as the bowel distally was more decompressed and normal caliber. He was diagnosed with a small bowel obstruction and admitted to the hospital. A nasogastric (NG) tube was placed; he was nil per os (NPO) and started on IV fluids. Serial abdominal examinations every six hours were ordered. A repeat CT scan with contrast was done which corroborated the earlier findings of a small bowel obstruction. However, in addition to the small bowel obstruction, findings on CT scan also included the incidental finding of a 1.6 cm x 1.4 cm rounded mesenteric structure in the right lower quadrant seen in Figure Vascular surgery agreed with the findings of the possible pseudoaneurysm and recommended a mesenteric angiogram and possible endovascular coil embolization due to the high risk of rupture. The symptoms improved with conservative management and the patient started passing flatus and stool on hospital day three. The NG tube was removed and his diet was advanced as tolerated. Due to the resolution of his small bowel obstruction, the patient opted to go home and agreed to elective angiogram by IR the same week. He was discharged home on short-term enoxaparin.Later that week, the patient was referred for a diagnostic angiography to allow determination of an appropriate treatment strategy. At the time of the procedure, access was established from the right common femoral artery under ultrasound guidance. A 5 French sheath was placed. An abdominal aortagram was obtained after which selective catheterization of the superior mesenteric artery was performed. Arising from the ileocolic artery was the known 1.7 cm aneurysm, into which a microcatheter was placed and can be seen in Figure During the subsequent angiogram, vascular supply from the ileocolic branch and ileojejunal branch surrounding the ileal branch aneurysm was found indicating adequate collateral flow for embolization. The decision was made to embolize the aneurysm sac itself after prolonged attempts of trying to embolize both proximal and distal branches of the vessel. Two 8 mm, 14 cm length Nestor coils, and three additional 6 mm, 15 cm length Nestor coils were advanced into the aneurysm. This resulted in successful thrombosis of the aneurysm with no distal flow seen here on both the early phase and late phase seen in Figure Visceral artery aneurysm and VAPA of the splanchnic arteries are an uncommon incidental finding found in relatively asymptomatic clinical settings. Visceral artery true aneurysms contain all three layers of the blood vessel wall, compared to pseudoaneurysms which occur due to damage to the blood vessel wall and subsequent accumulation of blood between the tunica media and tunica adventitia. Generally, VAA is much more common than VAPA. Visceral pseudoaneurysms, however, occur more frequently in gastroduodenal and superior mesenteric arteries compared to true aneurysm . The mosPseudoaneurysms can have a plethora of etiologies. SMA pseudoaneurysms, specifically, are most frequently caused by inflammation from pancreatitis or other etiologies, or trauma . Trauma  Currently, pseudoaneurysms are most commonly identified incidentally on CT scans of asymptomatic patients. Initial diagnostic steps for VAPA should include CT followed by angiography. Angiography allows for more specific localization of the suspected pseudoaneurysm as well as confirmation of collateral flow. Prompt diagnosis and management should be the top priority in anyone suspected of a VAPA because of the high rate of rupture and hemorrhage. According to a study by Pitton et al. pseudoaneurysms are far more likely than true aneurysms to rupture (76.3% vs. 3.1%) [Surgical technique involves an open technique in which the aneurysm sac is excised and vascular flow is regained. Indications for surgery of aneurysms include those patients that are symptomatic, aneurysms in pregnant women, and asymptomatic aneurysms greater than 2 cm. Elective surgeries for aneurysms have a mortality rate that is around 1.5% according to a study done by Pulli et al. . Less in New emerging technology in the management of aneurysms is a flow diverting stent. These are unique in that flow to collateral vessels will be preserved, as is not the case in other techniques. Flow-diverting stents are a type of flow modulator, meaning a stent with a certain porosity will be placed in the parent vessel allowing minimal flow to the sac itself. Minimal flow into the sac itself will then cause stagnation and eventual thrombosis of the sac, as seen in almost 88% of aneurysms using this method by Kallmes et al. . It has Pseudoaneurysms are a rare entity seen in clinical practice. With the advent of newer and more accurate technologies, more of these will be incidentally found. Prompt diagnosis and immediate management remain the mainstay of the workup for all identified pseudoaneurysms. The case itself identified abnormal symptomatology associated with the diagnosis as well as a different approach to endovascular therapy of pseudoaneurysms. We hope this case aids clinicians in the future care of patients with visceral artery aneurysms and pseudoaneurysms.


Scapular abscess is a rare clinical diagnosis. This is a report of an atypical case of extensive intramuscular scapular abscess involving the anterior and posterior aspects of the scapula with extension into the axillary region following minor trauma in a young healthy adolescent, describing a single posterolateral approach to the scapula to evacuate the abscess. Following surgical drainage and antibiotic treatment, patient recovered without any complication. Early diagnosis, urgent surgical intervention and antibiotic treatment are warranted for scapular abscess. Any delay in diagnosis or treatment may progress to septicaemia and may even lead to death1. A high index of suspicion is required to diagnose scapular abscess as it is rarely encountered in clinical practice. We report the case of a young healthy adolescent with extensive intramuscular scapular abscess extending into the axillary region following a minor trauma.Scapular abscess is an uncommon clinical diagnosis. A literature search yielded only 11 cases being reported so farA 13-year old girl presented with right shoulder pain associated with fever for nine days. The problem started when her right upper limb was pulled by her brother to drag her out of bed. Pain and swelling at her right scapular region  progressThree days later, her symptoms worsened and she started to have low-grade fever. She underwent traditional massage twice for her symptoms. She was prescribed oral cloxacillin by a general practitioner; however, her symptoms became worse, with loss of appetite and weight of 2 kg within a week.3/μL, erythrocyte sedimentation rate was 87 mm/hr, and C-reactive protein was 106 mg/L. Blood culture showed no growth.Upon admission, her pulse rate was 125 beats/minute, blood pressure was 110/69 mmHg with temperature of 37.4°c. Examination revealed, a swelling at the right scapular region, measuring 5cm x 7cm, tender, warm and fluctuant and she was unable to move her right shoulder due to pain. The white blood cell count was 27 x 10Ultrasound showed multi-loculated intramuscular collections around the right scapula. Computer Tomographic scan  revealedStaphylococcus aureus, sensitive to cloxacillin. She completed three weeks of oral cloxacillin and the wound closed by secondary intention. She had regained full range of shoulder motion and radiograph at three months showed no osteomyelitis changes of the scapula.A single posterolateral surgical approach  was used5 in the literatures and mainly located around the subscapular region. Our case demonstrates a more extensive abscess involving the entire periscapular region and extending to the thoracic space. Such an extensive abscess involving the scapula and axillary regions has not been reported in literature. We postulate that local pressure with massage had breached the anatomical barrier leading to extension of the abscess to a nearby compartment. Systemic infection, immune-compromised diabetes and trauma had been reported to cause scapular abscess5. Minor trauma in our case might cause hematoma formation in scapular region leading to formation of scapular abscess. Handorf et al1 reported a 19-year old male who had developed subscapular abscess after the shoulder was hit by baseball bat and Babayigit et al2 reported a case of subscapular abscess in a 7-year old boy after falling off a bicycle and sustaining trauma to the left shoulder. All the cases highlighted the possibility that minor trauma with soft tissue injury and hematoma could be the cause of the scapular abscess.The scapular region is not a common location for abscess formation. Thus far, there are only 11 cases of scapular abscess reportedet al1 reported the case of a 9-year old child’s shoulder was hit by a baseball bat, led to septicaemia and death, the subscapular abscess being identified at autopsy.Young patients presenting with painful shoulder swelling and fever can mislead with the diagnosis of septic arthritis or acute osteomyelitis of the proximal humerus, which are common in children. Tuberculosis of the joint should be considered in the differential diagnosis as tuberculosis is common in our country. It is challenging to differentiate all these differential diagnoses with scapular abscess, therefore a high index of suspicious with good history taking and physical examination are needed. In our case, ultrasound revealed multi-loculated intramuscular collection around the right scapula not involving the shoulder joint, thus the need for joint aspiration was excluded. Although scapular abscess is a rare diagnosis, delay in diagnosis and treatment may lead to septicaemia and death. Handort 2, anterior deltopectoral approach3, modified Judet approach4, and posterolateral approach to scapula with medial counter incision5. The single posterolateral incision was used in our case because the approach was easy and we were able to drain out the extensive periscapular abscess. This single approach did not need additional medial incision.CT scan helps to support the diagnosis of abscess and evaluate the anatomical area and extent of the abscess. Planning for surgical approach in this complex anatomical area is important to minimise the surgical damage and evacuate the loculated abscess completely. To our knowledge, only four case reports of subscapular abscess describe the surgical approach to drain subscapular abscess: vertical incision along medial border of scapulaIn conclusion, scapular abscess is rare and challenging to diagnose. The posterolateral approach to scapula is a simple approach and effective way to drain extensive scapular abscess. Early diagnosis, prompt antibiotic administration and surgical intervention to drain abscess is important in the treatment of scapular abscess, for the patient to recover without complications.


Primary angiosarcoma is a rare clinical entity, it's typically located within theright atrium and known to be rapidly fatal. A 37-year-old female was presentedwith a history of recurrent facial paralysis and left hemiparesis. A cranialmass was identified at cranial magnetic resonance imaging and she underwentneurosurgery operation. The immunohistochemical examination was determined asmetastatic cardiac angiosarcoma. The tumor, as well as part of the rightpericardium, were resected. A piece of bovine pericardium was used toreconstruct the right atrial wall. Tricuspid valve was reconstructed with ringannuloplasty. Due to resection of right coronary artery with the tumor, coronarybypass surgery was performed successfully. The patient is currently healthfulwithout any recurrence and complaint 12 months after the diagnosis as followedup. It's typically located within the right atrium aslarge symptomatic masses and known to be rapidly fatal, with the diagnoses usuallydetermined only at autopsy. It frequently extends to the pericardium, vena cava, ortricuspid valve, causing tamponade and/or heart inflow obstruction. Early heart transplantation andnovel radiation therapy approaches may offer a survival benefit in non-metastatictumors, but up to 80% of the patients present with systemic metastasis atdiagnosis. Metastasesare common, and often widespread. Clinical diagnosis of angiosarcoma is oftendifficult because there are no specific symptoms associated with the disease.Symptoms can be either cardiac or systemic. The majority of the literature describesa uniformly dismal prognosis with a median survival of only 6 months for theseaggressive tumors,4. Additionally, with the aid of newer imagingtechniques, localization, biopsy diagnosis and resection of the atrial tumors arenow being achieved more often, with some improvement in survival.While primary malignant cardiac tumors, including the pericardium, are rare,angiosarcomas are the most commonly reported histologic type. Primary angiosarcomais also a rare clinical entity, with an incidence of 0.0001% in collected autopsyseries. We report a patient with a rightatrial angiosarcoma which first presented clinically as a cerebral tumor mimicking aglioma.Complete surgical resection confers the best long-term outcome but may becontraindicated because of anatomic considerations in advanced tumors2) and ifosfamide (7500 mg/m2) with a duration of 6cycles of combination treatment.A 47-year-old female was presented to a private hospital with a history of recurrentfacial paralysis and left hemiparesis. The patient had no other pertinent pastmedical history. After a marked right-sided visual field defect developed, variousinvestigations disclosed a tumor in the right parieto-occipital region. A cranialmass was identified at cranial magnetic resonance (MR) imaging  and she About 6 weeks after neurosurgery, she reappeared with shortness of breath andpericardial friction rub but no increased jugular venous pressure or dependentedema. She was transferred to our institution and underwent transesophagealechocardiography, which showed a homogeneous mass that involved the free wall of theright atrium. The patient then underwent computed tomography (CT), MR imaging, andangiography . FindingFull-arterial monitoring was performed under general anesthesia. Standard aorticarterial and bicaval venous cannulation were performed. Cardiopulmonary bypass (CPB)was achieved. Cardiac arrest was achieved with blood cardioplegia. Aftercross-clamping, surgical field was exposed with right atriotomy. The tumor, as wellas part of the right pericardium, were resected . Becauseth hour.Aortic cross-clamp time was 36 minutes and total perfusion time was 65 minutes.During the operation, mean arterial pressure was maintained at 70 mmHg. By the endof CBP, the patient was transferred to intensive care unit (ICU) with ahemodynamically stable condition. She was extubated on the postoperative7th day of the operation. Follow upechocardiographic controls revealed clean cardiac cavities. The control routinebiochemistry was normal and 9th month PET scan was also clean.Total drainage was 500 cc. After one night staying in ICU, the patient was dischargedin good condition after the 7In the present report, a patient with cardiac angiosarcoma and distant metastases wastreated with a combination of chemotherapy and surgical resections. The patientresponded well to neoadjuvant chemotherapy, and the tumor and metastases decreasedto an extent that made complete surgical resection feasible. Surgical resection wassuccessful in removing the mass and subsequent chemotherapy was used to destroy anyremaining tumor cells.Even up to 80% of the patients present with systemic metastasis at diagnosis, and inthe case reported on this article primary cardiac angiosarcoma with raremanifestation with cerebral metastasis was treated successfully with neoadjuvant andadjuvant chemotherapy and complete surgical resection of the tumor with thereconstruction of the right atrium. With the correct combination ofmultidisciplinary treatments for cardiac angiosarcoma, we hope that a cure for thisdisease is possible rather than treatment regimens being used as either palliativeor life-prolonging measures.. This patientwas presented with several systemic metastases, but with the combination ofneoadjuvant and adjuvant chemotherapy and complete surgical resection, the patientis currently alive and without recurrence 12 months after diagnosis with functionalclass New York Heart Association grade I. Multidisciplinary approach is mandatory toimprove long-term survival.While other authors have published case reports of long-term survival aftermultidisciplinary treatment for angiosarcoma, these were patients presented withoutmetastasesAlthough this patient has not experienced recurrence, this is an unusual case anddiligence in the surveillance for new tumor growth is, therefore, imperative.


Adenomyomatosis of the gallbladder (AMG) is characterized by mucosal hyperplasia leading to invagination through the thickened muscle layer, which is relatively common in adults, but is rare in childhood. We report a 12-year-old boy with adenomyomatosis of the gallbladder combined segmental and fundal type. This combined type is rare in adults and is first reported here in childhood. Although initial imaging with computed tomography (CT) suggested the presence of a circular solid mass-like lesion because of its rare morphology, repeated ultrasonography (US) was useful for leading to a correct diagnosis. Adenomyomatosis of the gallbladder (AMG) is a benign and acquired lesion characterized by mucosal hyperplasia leading to invagination through the thickened muscle layer, known as the Rokitansky–Aschoff sinuses (RAS) . While AA 12-year-old boy with acute abdominal pain presented to our hospital. The patient reported that pain sometimes occurred postprandially, and that the frequency and severity had worsened over the previous six months.A physical examination revealed normal findings, with the exception of the right upper abdominal tenderness. The laboratory data were within normal limits (data not shown). Contrast-enhanced CT in axial view in the first radiologic examination showed a circular solid mass-like lesion (30 × 23 mm in diameter) adjacent to the liver that was heterogeneously enhanced with a small luminal structure in the center . The assn = 10), magnetic resonance imaging (MRI) and/or MRCP (n = 6), and CT (n = 2). Symptomatic cases were treated by cholecystectomy in with favorable outcomes. Close observation was continued for the asymptomatic cases, and resolution of the lesion was noted eight months later in Case 5, with the same findings persisting for three months in Case 6.Our literature search revealed only 10 pediatric cases of AMG –11. The AMG has generally been morphologically classified into three types in adults: segmental, fundal , and diffused  , 12. SegThe pathogenesis of AMG has been suggested to be as follows: abnormal neurogenic muscular contraction may induce glandular proliferation and hyperplasia of the smooth muscle, leading to RAS formation. There are rare reports of AMG accompanied by PBM in adults , 15 and The reported pediatric cases of AMG included a neonate and a 4-month-old infant (Cases 5 and 6). This indicates that pediatric AMG might in part be developing congenitally. However, the former patient's lesion spontaneously resolved eight months later, and the US findings of the reported two cases showed several comet-tail signs on the thin wall in the literature, without gallbladder wall thickening . Thus, the lesions of very young asymptomatic children may have a different etiology from those in older children and adults.Surgery is mandatory when gallbladder lesions with the abovementioned radiological findings are encountered. Cholecystectomy is a treatment of choice for symptomatic AMG patients, and preoperative imaging studies, including US, CT, and MRI, are necessary for the precise determination of the anatomy. Surgeons should also be involved in the close follow-up of asymptomatic patients.In conclusion, we presented the first case of a child with combined fundal and segmental-type AMG. Because of the rare appearance of the lesion, it resembled a tumor with CT, but repeated US scans were useful as they led to a correct diagnosis.


Pituitary metastasis of cervical adenocarcinoma is an exceedingly rare phenomenon. The authors present a case of a 66-year-old female with cervical adenocarcinoma who was discovered to have a rapidly growing intrasellar mass in the work-up of adrenal insufficiency and hypothyroidism. The patient underwent subsequent endoscopic endonasal subtotal resection of the mass. Histopathological analysis of the resected lesion demonstrated features consistent with metastatic mucinous adenocarcinoma of the cervix. While initially neurologically asymptomatic following surgery, the patient developed an oculomotor nerve palsy several weeks following surgical debulking, at which time neuroimaging revealed marked regrowth and suprasellar extension of the metastatic lesion. While metastatic cervical adenocarcinoma to the sella is rare, it should be considered in the differential based on the history of the patient. Metastatic lesions to the sella are believed to comprise only 1% of all pituitary lesions, of which the most common sites of primary tumor origin are the breast and lung . CompresA 66-year-old female with a history of mucinous adenocarcinoma of the cervix presented to the neurosurgery outpatient clinic for evaluation of a sellar mass found during workup of adrenal insufficiency and hypothyroidism. The patient did not have unusual headaches or vision problems. Three months prior to discovery of the sellar mass, she was diagnosed with stage IIb mucinous adenocarcinoma of the cervix and was treated with chemotherapy. At the time of neurosurgery clinic presentation, she was neurologically intact, including full visual fields. Laboratory work-up demonstrated pituitary insufficiency with central hypothyroidism.The initial magnetic resonance imaging (MRI) revealed a 1.8 × 1.1 cm contrast-enhancing mass within the sella, with extension to the suprasellar cistern and optic chiasm abutment. Preoperative imaging obtained the following month in preparation for surgery demonstrated that the mass had grown to 2.2 × 1.5 cm .The patient underwent an endoscopic endonasal approach for resection of the intradural sellar mass. Intraoperative findings demonstrated a very firm, infiltrative, vascular mass with dense adherence to surrounding structures, including the dura, medical cavernous walls, and diaphragma. Intraoperative frozen section pathology was consistent with metastatic carcinoma. The tumor was debulked until normal appearing pituitary tissue was identified and the margin of tumor adherence to the diaphragma was reached. Postoperatively, the patient did well without new hormonal deficiencies or vision problems. A subtotal resection (>80%) was achieved . Gross hThe patient's immediate postoperative course was unremarkable. Given the diagnosis and intraoperative/postoperative findings of subtotal resection, adjuvant chemoradiation therapy was encouraged but the patient refused additional treatment. She was discharged home two days after surgery. She developed decreased left eye visual acuity and ptosis one week after surgery. A CT of the head at this time did not show any intracranial hemorrhage and a repeat MRI showed new enhancement suggestive of tumor recurrence within the sellar and suprasellar regions. The patient was started on steroids but declined any further treatment, including repeat surgery. Her ophthalmic symptoms ultimately progressed to a complete left cranial nerve III palsy four weeks after surgery. Follow-up MR imaging at five weeks after surgery revealed significant progression of the tumor to 2.9 × 2.4 cm with significant suprasellar extension . Though Metastases to the sellar region have been hypothesized to occur either hematogenously, such as from the pituitary stalk or cavernous sinus, or via leptomeningeal spread . PituitaPituitary adenomas and metastatic lesions to the parasellar region are nearly indistinguishable on neuroimaging. On plain film X-ray images, sellar enlargement or deformity and erosion of the sellar floor may be seen in both metastatic and benign lesions . On MRI,Cervical carcinoma typically disseminates locally via the lymphatics to the retroperitoneal lymph nodes; distant hematogenous metastasis most commonly occurs to the liver, lung, and bone . CerebraFor brain metastasis from cervical cancer, combination therapy of either chemotherapy or stereotactic radiosurgery followed by whole-brain irradiation has been shown to extend median survival to up to 4.6 months , 14. TheWhile cerebral metastases of cervical malignancies have been described, metastasis of well-differentiated cervical malignancy to the pituitary region is rare. However, this should be considered in the differential diagnosis of patients with this history and based on the clinical presentation. The development of rapid endocrinopathies, oculomotor dysfunction, and quick radiographic progression suggest a more aggressive neoplasm. Maximally safe resection should be performed, including decompression of the optic apparatus, followed by efficient consideration for adjuvant therapy.


Preoperative diagnosis can be difficult in pauci-symptomatic cases. The Clinical signs, Abdominal-pelvic CT scan and biology are the steps of the preoperative diagnosis.•TREATMENT: Treatment is surgical. Preoperative medical preparation is aimed at reducing risks of peroperative hemodynamic instability. The anesthetist should therefore prepare himself to manage blood pressure peaks during the tumour’s dissection, but also the possible low blood pressure at the end of exeresis. Surgery remains the key element of treatment and consists in exeresis of the paragaglioma.•PROGNOSIS: paraganglioma of the ZUCKERKANDL organ is often a benign tumor. The resuscitation determines the patient’s prognosis. Paraganglioma of the ZUCKERKANDL organ are rare. Diagnosis is based on clinical, radiological and biological arguments. We report a case to describe our surgical procedure and insist on the necessity of preoperative diagnosis.BA, 52-years-old male patient was seen in consultation for left hypochondrium pains. The clinical examination had revealed a painful tumefaction in the left flank and the left hypochondrium. A deep mass was observed, but was difficult to be assessed, due to pain. Abdominal-pelvic CT scan with contrast injection had revealed a tissue mass, suggesting a tumor of the tail of the pancreas. Laparotomy showed this mass was not attached to the tail of the pancreas, and was along the abdominal aorta up to the aortic bifurcation. Upon touching the mass, blood pressure raised up to 240 mmHg. A least mobilization of the mass and the use of nicardipine helped maintain blood pressure below 180mmhg. Dissection was carried out from the aortic bifurcation to the TREITZ’s angle and the mass was removed. The follow-ups were characterized by low blood pressure a few minutes following the resection of the mass.Pheochromocytoma is rare. The Clinical signs, Abdominal-pelvic CT scan and biology are the steps of the preoperative diagnosis. The surgery consists a lumpectomy. The resuscitation determines the patient’s prognosis.Pheochromocytoma is an unusual mass. Preoperative diagnosis can be difficult in pauci-symptomatic cases. One should consider this in the face of any abdominal mass, so as to improve planning of resuscitation which determines the patient’s prognosis. It pr2The patient is 52-years-old and a shepherd living in Dori, was seen in cancer consultation on April 2017, for left hyponchondrium pains for the past 3 months. He was known to be hypertensive, but not diabetic. The clinical examination had revealed a painful tumefaction in the left flank and in the left hypochondrium. A deep mass could be observed, but was difficult to be assessed due to pain. Abdominal-pelvic CT scan with contrast injection had helped find an inhomogeneous tissue mass suggesting a tumour of the tail and body of the pancreas, measuring 86 × 83 mm .Fig. 1CTPreoperative workup revealed a sinus tachycardia. The indication for surgery was corporo-caudal spleno-pancreatectomy. Laparotomy showed a mass which was separated from of the tail of the pancreas, multicoloured, lying against the duodeno-jujenal angle through some small vessels, and along the abdominal aorta up to the aortic bifurcation . Upon co3Described for the first time by Félix Frankel in 1884, pheochromocytoma remains a rare tumour . It secrImaging helps localize the tumour and give its features. It holds an important place in the diagnosis of pheochromocytomas and paragagliomas ,10,12,13Preoperative medical preparation is aimed at reducing risks of peroperative hemodynamic instability ,16. PreoIn the literature, there is almost no mortality, thanks to the progress of medical imaging and biology, which allow preoperative diagnosis ,19.4Pheochromocytoma is a rare tumor. The Menard Triad which is the typical clinical observatory. This explains why the diagnosis was an operative surprise. A preoperative diagnosis based on clinical, radiological and biological arguments would help better reorganize resuscitation, the only guarantee of good prognosis. It is therefore important to publish this case in the English literature.The authors declare that they have no competing interests regarding the publication of this manuscript.No sponsors to declare.Ethical approval is not needed for this case report as patient consent and we are not trialing a new device.Written and signed consent by the patient to publish a case report has been obtained.Case report concept and design: Zongo N, KOAMA A, Kambou/Tiemtoré B.Acquisition of data: KOAMA A, Zongo N, Nde/ Ouédraogo NA, LOMPO OM.Statistical analysis and interpretation of data: KOAMA A, Sanou A, Diallo O, Lougué /Sorgho C, Cissé R.Drafting of the manuscript: KOAMA A, Sanou A, Lougué /Sorgho C, Cissé R, HOURY S.Critcal revision of the manuscript for important intellectual content: KOAMA A, Zida M, Ouédraogo MNL, Ouangré E, Sanou A, Lompo OM, Sanou A, Diallo O, Lougué /Sorgho C, Cissé R.All authors approved the final version of this publication.It is not a clinical trial.Dr Nayi Zongo.Not commissioned, externally peer-reviewed.


The textilome or still called gossybipoma is a very rare but well known and not insignificant post-operative grave lesion in the abdominal and gynecological surgery. It is a foreign body compound of gauze or surgical drape omitted in the operation site. The difficulties of diagnosis delay generally the discovery of abdominal textilome. The clinicals symptoms are: chronic disorders of the transit in sub-occlusive or occlusive syndromes. Erect Abdominal X-ray has little contributory in the diagnosis, but the ultrasound remains more reliable. Abdominal CT Scan allows a precise topographic diagnosis; certain teams propose explorations by MRI. We report a case of intra-colic textilome (sigmoïde), presenting acute abdomen in forme of an occlusive syndrome in patient who was operated 3 years ago for uterine fibroid.


Bowel perforation is a potential complication of colonoscopy, and colonoscopic polypectomy has a higher risk. Perforation may be responsible for abdominal compartment syndrome (ACS), which causes multiple organ dysfunction.Case presentation: Here, we have described the case of a woman who underwent colonoscopic polypectomy. Subsequent to the procedure, she developed progressive abdominal distension, dyspnea, drowsy consciousness, and hypotension. Her physical examination revealed increased abdominal circumference, coldness, and paleness of the lower limbs. ACS were accordingly diagnosed, most likely to have resulted from bowel perforation. Thus, decompression with a large-bore intravenous catheter and emergent laparotomy were performed. A 0.5-cm lesion over the cecum was discovered and repaired. The patient tolerated the procedure well and underwent intensive care. She was discharged on day 7 after operation, and she had completely recovered.ACS is a rare complication followed by perforation after colonoscopy. Multiple organ dysfunction may occur from ACS, affecting the cardiovascular, pulmonary, and gastro-intestinal system. In our case, we used a small-sized catheter for decompression but in vain due to the kinking of the catheter. ACS was eventually resolved via surgical intervention. Thus, when ACS occurred, emergent decompression with an appropriate size of catheter and surgical intervention are recommended.Colonoscopy perforation must be considered as a contributing factor to ACS; accordingly, early recognition and intervention may avoid deterioration of ACS. The risk of bowel perforation varies from 0.03% to 0.13%, based on whether intervention has been performed. Patients who receive polypectomy had an incidence rate of 0.08% perforation as compared with 0.04% for those without polypectomy . Most pa22) 100% in room air. Under intravenous general anesthesia, colonoscopy revealed a 1.5-cm cecal polyp at the cecum  72 beats per minute (bpm), blood pressure (BP) 139/74 mmHg, and oxygen saturation . After 10 min, her consciousness remained drowsy, with no response to stimuli and then gradually distended, tense abdomen was noted with dropped BP as 80/56 mmHg. She also showed rapid and shallow breathing, and mottled and cyanosis of the extremities and lips. Her physical signs revealed tympanic percussion and increased abdominal circumstances. Assisted ventilation was supported at that time. Under the suspicion of ACS, urgent needle decompression with a 20-gauge intravenous catheter was introduced at the right lower quadrant of her abdomen. An immediate rush out of air indicated the presence of IAH. However, kinking of the catheter resulted in incomplete drainage of free air, and the distended abdomen persisted. Concerning the impaired cardiovascular and respiratory functions, we performed emergent laparotomy and colon repair under general anesthesia with endotracheal tube intubation. The ventilation was extremely difficult, with diminished bilateral breath sounds and steady pulse oximeter reading. The initial peak airway pressure was approximately 30 cmH2O with a low tidal volume (approximately 200 mL) and improved dramatically while the pressure was relieved by laparotomy. The peak airway pressure became approximately 18 cmH2O and the tidal volume improved to approximately 450 mL. We repaired the cecal perforation, and the patient tolerated the entire process well. She was discharged on day 7 of the surgery with complete recovery. There was no functional impairment discovered at the outpatient clinic after 6 months of discharge.While arriving at the post-anesthetic care unit (PACU), she had not yet regained her consciousness; however, her vital signs were relatively stable . ), proced%. ), proColonoscopic perforation can be managed by conservative treatment, endoscopic clipping, and surgical intervention, including primary closure, bowel resection, and fecal diversion ,11. A prACS is a rare complication that may complicate colonoscopic perforation. A few case reports documented this complication with varied severity ranging from hypertensive pneumoperitoneum to cardiac arrest . ACS affTo normalize the IAP, IAH could be treated with diuresis, paracentesis, and/or decompressive laparotomy based on the severity . The WSA2) during the procedure. Compared to CO2, air is absorbed slowly through the bowel mucosa, causing more luminal distension and potentially greater abdominal pain. This fact may explain why the patient had a delayed onset of ACS at PACU.However, our patient failed to recover from paracentesis and, subsequently, received emergent laparotomy. The major difference is between the size of catheter used in our case (20 gauge) and that used in all previous case reports mentioned (14 gauge) for paracentesis. The inadequate size of catheter is insufficient for decompression and is prone to interruption due to kinking. Another difference is our patient suffered from progressive increase of IAP and deterioration of vital signs after cessation of the examination, while others reported sudden changes during the procedure. Since the effect of the insufflation type in sedated patients was conflicting ,18, we uThus, early recognition and intervention are essential whenever ACS occurs.4Colonoscopic perforation with ACS is a rare complication that should be kept in mind. ACS deteriorates rapidly; therefore, early diagnosis and management are crucial.There is no conflicts of interest.Ying-Chun Lin: Writing – Original DraftJen-Yin Chang: Writing – Review & Editing, investigationChen-Han Wu: Data Curation, Supervision, InvestigationJian-Syun Chen: Investigation, Writing – Review & EditingChien-Chuan Chen: SupervisionWritten informed consent was obtained from the patient. Upon request, copy of the written consent is available for review by the Editor-in-Chief of this journal.Our study is exempted from ethical approval in Institutional Review Board of our hospital.Not commissioned, externally peer-reviewed.This is a case report and not a human study. It is exempt from registering.Jen-Yin ChangYing-Chun LinChien-Chuan Chen


The diagnosis and management of upper gastrointestinal tract submucosal tumours can be challenging due to the difficulties with adequate pre-operative characterization and tissue sampling.A 61-year-old female patient underwent esophagogastroduodenoscopy because of epigastric pain and episodic vomiting. A submucosal tumour was found along the greater curvature in the antrum. On endoscopic ultrasound, the mass was suspected to be a gastrointestinal stromal tumour. The patient was referred for laparoscopic excision. The final histopathological examination confirmed an ectopic pancreas, not a gastrointestinal stromal tumour as thought preoperatively.Despite an adequate pre-operative diagnostic workup with endoscopic ultrasonography, the lesion was misdiagnosed as a gastrointestinal stromal tumour and surgery was performed to excise an ectopic pancreas, essentially a benign lesion.The case highlights the absence of firm diagnostic criteria to differentiate submucosal gastric lesions. DiagnosIn the stomach, GIST are the commonest submucosal tumours. They develop from the interstitial cells of Cajal in the muscularis propria and are usually seen in the fourth layer of the gastric wall on endoscopic ultrasound . AnatomiThe case is reported in accordance with the SCARE checklist .2A 61-year-old female patient was referred to our department for laparoscopic excision of GIST in the stomach. Prior to our treatment she underwent an esophagogastroduodenoscopy due to epigastric pain and occasional vomiting. Endoscopy showed erosive gastritis, a hiatal hernia, and a submucosal tumour located along the lesser curvature on the posterior wall of the antrum . The tumEndoscopic ultrasonography confirmed a 22 × 13 mm submucosal well-defined solid tumour arising from the fourth, muscular, layer of the stomach . The tumLaparoscopy was performed, and on initial exploration of the abdominal cavity, we discovered a tattooed submucosal tumour on the greater curvature of the stomach. Laparoscopic excision was performed and the specimen was recovered in a retrieval bag. The abdominal cavity was irrigated with saline and the fluid aspirated. An abdominal drain was inserted beneath the stomach. The fascia and skin wounds were sutured. The postoperative course was uneventful and the patient was discharged on the fifth postoperative day.Resection samples were sent for histopathological analysis. The histopathologic analysis report about 6.6 × 3.5 cm excision gastric wall. The mucosa and serosa have normal appearance, muscularis propria and submucosa include a gray yellowish node 15 mm in diameter. The tumour did not involve the resection margins. Histopathological analysis confirmed an ectopic pancreas, 15 mm in diameter .Fig. 3Ec3Ectopic pancreas is a congenital malformation of the pancreas, with an unclear pathogenesis – one of the postulated mechanisms is the separation and subsequent deposition at ectopic sites during rotation of the foregut ,7. Its fLarge GISTs have irregular margins, areas of necrosis and are ulcerated. About 50% of patients with GISTs have metastases in the liver or peritoneum . GISTs, The standard treatment for localized GISTs is complete R0 surgical excision, avoiding tumour rupture, and without dissection of clinically-negative lymph nodes . Althoug4It is often difficult to determine the aetiology of submucosal gastric lesions based on clinical, endoscopic and imaging findings. Despite using multiple diagnostic modalities, the diagnosis is often ultimately proven at surgery ,22.Neither ZŠ nor JH have any conflict of interest, relevant to the submitted contribution.The research, presented in the submitted contribution, was not funded.Case reports are exempt from ethical approval in our institution. The patient has provided written informed consent for the publication of this anonymised case report.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.ZŠ conceived the study and collected the data.JH wrote the paper.Both authors critically reviewed the manuscript and approved the final submitted version.The submitted contribution is a case report that was not registered.Zdravko Štor.Not commissioned, externally peer reviewed.


Clitoral neuromas occurring after female genital mutilation/cutting (FGM/C) can vary in presentation and may require surgical management. A 39-year-old East African female with a history of FGM/C presented during pregnancy with a progressively enlarging mass and worsening periclitoral pain. Postpartum surgical excision restored cosmesis and resolved the discomfort with pathology confirming the presence of a neuroma within the epidermal inclusion cyst. We present the first published case of a symptomatic clitoral neuroma within an epidermal inclusion cyst. This unique pathology demonstrates that complications of female genital cutting can present in complex and varied ways. Considering the prevalence of FGM/C and increasing rates of emigration from countries in which FGM/C is performed, complex clitoral neuromas are an important long-term complication which providers in Africa or internationally must be aware of. Female genital mutilation/cutting (FGM/C) is sociocultural practice defined by the World Health Organization as the “partial or total removal of the external female genitalia or other injury to the female genital organs for nonmedical reasons” . It is mIt is estimated that 100 to 140 million women around the world have undergone FGM/C, with approximately three million new cases each year .In 2007, the WHO published a revised classification system to systematically describe the extent of FGM/C based on parts of the genitalia removed. Type I includes partial or total removal of the clitoris (clitoridectomy) while type II also includes removal of the labia minora with or without excision of the labia majora. Type III, also known as infibulation, is the most extensive form and includes narrowing of the vaginal orifice by appositioning a portion of the removed labia minora or majora. Type IV includes any other nonmedical procedures to female genitalia such as piercing, nicking, or cauterizing. Further subclassifications of FGM/C do exist and can be found in the literature .Complications of FGM/C are many. Immediate complications include bleeding, infection, and pain , 6. The A 39-year-old G3P2002 female of Somali origin with two prior vaginal deliveries in Somalia presented with a periclitoral swelling. This had been slowly enlarging over the previous 10 years, but the change during this pregnancy was rapid an acute. She had a history of FGM/C Type IIIb  at seven years of age without reported complications. Past medical and surgical history were otherwise unremarkable. The patient had undergone prior defibulation at delivery, which was not noted to be protracted nor required operative delivery. She denied any urinary symptoms and had expectantly managed her symptoms throughout the pregnancy. Surgical management was deferred until following delivery despite the persistent periclitoral burning pain that worsened as the lesion enlarged throughout the pregnancy. The patient also noted worsening dyspareunia symptoms as the pregnancy progressed even before fetal size caused appreciable pressure. The patient delivered via cesarean section due to breech presentation and she represented during her postpartum course for surgical removal of the lesion due to persistent symptoms which negatively impacted her quality of life.Preoperatively, physical examination demonstrated the presence of a tender 4 cm x 3 cm x 2 cm periclitoral fluctuant lesion . SurgicaFollowing excision, the cyst was opened. Gross examination of the lesion demonstrated a dark grey stalk extending throughout two ends of the cyst with a glossy green-grey viscous material and no fluid. The widest diameter of the cyst measured 3.2 cm. Pathologic analysis of the cyst wall specifically demonstrated keratinizing squamous epithelium consistent with epidermal inclusion cyst. The stalk within the cyst wall was suggestive of a traumatic neuroma demonstrating proliferation of nerve fibers within a fibrotic tissue stroma . No maliThe patient's postoperative course was uncomplicated, and she reported resolution of both discomfort and dyspareunia.Relatively few clitoral neuromas have been reported as complications of FGM/C, but it is believed that this issue may be underrecognized and under-reported , 6, 9. CTreatment options include desensitization, steroid injections, and surgical excision, the latter of which is suggested to be the most efficacious therapy for resolution of traumatic clitoral neuromas. In such cases, recurrence of the traumatic neuroma is theoretically possible, and both providers and patients should be keenly aware of this possibility. However, to date, no cases have been published regarding such a complication . In simiAlthough few similar cases of neuromas have been published, none involved expectant patients . PregnanIt is important to note that clitoral neuromas after FGM/C may not always be palpable, and the patient's presenting symptoms may only be allodynia or hyperalgesia. This pain is neuropathic and thus often described as a burning, tingling, or shooting sensation . Althoug


Ovarian torsion after hysterectomy is a rare event. The diagnosis of ovarian torsion is challenging because symptoms are nonspecific. We present a case of ovarian torsion 2 years after laparoscopic hysterectomy (LH). Furthermore, we performed a literature review about ovarian torsion after hysterectomy. This case shows that, in cases of acute onset pelvic pain in patients with history of hysterectomy, the adnexal torsion must be kept in mind in the differential diagnosis, especially in those women who had undergone LH. Ovarian torsion accounts for 2-3% of all acute gynecological emergencies. It remains a clinically difficult diagnosis as the symptoms are usually nonspecific. Ovarian torsion can occur at any age and also after hysterectomy . AlthougA 41-year-old woman, gravida 3, para 3, was admitted to our institution with a 12-hour history of acute onset pelvic pain, nausea, and vomiting. She had undergone total laparoscopic hysterectomy 2 years previously. The abdominal exam revealed mild distention and tenderness over the right lower quadrant. Vaginal examination revealed exquisite pain in the right vaginal fornix and the finding of a painful adnexal mass in the rectovaginal pouch of Douglas. Transvaginal ultrasonography showed a 60-mm cystic lesion in the right ovary with moderate ascites. We performed an exploratory laparoscopy and found a right adnexal torsion  and a riOvarian torsion after hysterectomy is a rare event with a prevalence of 7.91/1000 hysterectomies . AlthougThis case shows that, in cases of acute onset pelvic pain in patients with history of hysterectomy, the adnexal torsion must be kept in mind in the differential diagnosis, especially in those women who had undergone LH. In addition, we encourage that, during LH with ovarian conservation, the fenestration of the broad ligament should be performed in the direction of the uterine artery and not towards the infundibulopelvic ligament, in order to keep the ovary more fix to the pelvic sidewall.


Waldenström's macroglobulinemia (WM) and multiple myeloma (MM) are two distinct forms of mature hematologic B-cell malignancies. A missense somatic mutation in MYD88 gene (MYD88L265P) has been found in hematologic B-cell malignancies. The simultaneous presentation of Waldenström's macroglobulinemia and MYD88 mutation with multiple myeloma in the same patient is very rare and only a few cases have been reported in the literature. Waldenström's macroglobulinemia (WM) is a rare, indolent B-cell lymphoproliferative disorder characterized by a lymphoplasmacytic infiltration in the bone marrow or lymphatic tissue and a monoclonal immunoglobulin M (IgM) protein in the serum -2. It deA 67-year-old male with a past medical history significant for diverticulitis and hypertension presented to the emergency department for a complaint of back pain that started two days prior to admission. The patient described the pain as severe, sharp in nature and aggravates with movement. A skeletal survey reported multiple small lytic lesions. A computed tomography (CT) scan without contrast of thoracic spine showed multiple myelomatous involvements of the T6 and T7 vertebra including compression deformity and ventral epidural extension at the T6 level Figure . MagnetiInterestingly, genomic data has led to the identification of a common missense somatic mutation within the MYD88 gene MYD88L265P) found in hematologic B-cell malignancies. Subsequent investigations confirmed MYD88 mutation occurrence at high frequencies in many NHLs including the rare lymphoplasmacytic lymphoma and WM . In factP found iIndeed, in our presented case, mutation detection has influenced the diagnosis of WM and the treatment initiation of bendamustine and rituximab therapy. The patient, however, did not endorse any improvement of his M-protein concentration post five cycles Figure . This reThe simultaneous occurrence of WM and MM was present in this case. In fact, this presentation is very rare in clinical settings. Furthermore, MYD88 L265P mutation, which has been recognized as a molecular marker for WM, is not usually found in the presence of MM.Our case shows that this rare co-existence of WM and MM may occur; and molecular investigation for MYD88 mutation, on top of the clinical findings, can facilitate the recognition of such infrequent association. We, therefore, suggest that further research investigations should study this phenomenon and clarify the significance of molecular data in determining the simultaneous presentation of both diseases.


Hepatocellular carcinoma (HCC) with right ventricle metastasis without inferior vena cava and right atrium involvement is very rare and the prognosis of HCC with RV metastasis is generally poor. The mass in the cardiac chamber may lead to lethal instability of hemodynamics, however, the initial symptom is probably non-specific, which means that diagnosis timely becomes even harder.We present a 63-year-old male with isolated metastasis of HCC in the right ventricle which caused inflow obstruction. Moreover, we reviewed a series of studies of isolated metastasis of hepatocellular carcinoma between 1980 and 2018, and summarized the relative outcomes.Isolated metastasis of hepatocellular carcinoma in the right ventricle is extraordinarily rare. It may damage cardiac structure and broke hemodynamic balance. Multimodality imaging plays an important in accurate pre-operation assessment. Nowadays, palliative treatments could relieve fatal symptoms to some degree, however, standard treatment has not been well established. Right heart cavity occlusion secondary to metastatic hepatocellular carcinoma (HCC) with cephalad extension via the inferior vena cava (IVC) is rarely reported. The incidence is less than 6% in an autopsy series according to literature review . FurtherA 63-year-old male with a reported history of primary HCC treated with surgery and interventional chemotherapy 2 years ago was admitted to our hospital. He suffered from shortness of breath, abdominal distention and palpitation for 1 month. On physical examination, a grade 3/6 systolic murmur over the pulmonary area was heard. Edema in both lower limbs was observed. ECG demonstrated sinus rhythm, ventricular premature contraction and complete right bundle branch block. Laboratory findings showed that his NT-proBNP level was 1769 pg/ml, CKMB was 27 U/L and TnI was 0.249μg/ml. He had no history of hepatic virus infection. Other findings were albumin33.7 g/L, GGT97U/L, BilT26.4umol/L, BilD10.7umol/L, uric444 umol/L and CRP114mg/L.Positron emission tomography CT (PETCT) in other hospital showed that there was no nodule in the liver but some sporadic nodules in the lung. Large amount of pericardial effusion could also be observed. Transthoracic echocardiography (TTE) revealed a huge mass with moderate echogenicity in right ventricle (RV) which caused severe inflow obstruction. The mass blocked almost the whole cavity and showed little degree of mobility along with the cardiac cycle. The right ventricle outflow tract (RVOT) was also partially occupied, however, the speed of pulmonary artery (PA) remained normal 1.0 m/s). Contrast echocardiography (CE) demonstrated higher perfusion in the mass than in the myocardium after the administration of contrast agent. Cord-like vascular perfusion was observed in the mass. Color Doppler imaging showed fine and high velocity flow with the speed of 2 m/s in RV. Enlarged RV, RA and PA were also noted. Massive pericardial effusion was detected with enlarged IVC.(Fig. .0 m/s. CThe incidence of secondary tumor metastasis to heart is reported to be 1.5–21%. The metastasic tumors include melanoma which is of the highest incidence, renal cell carcinoma, breast, lung, thyroid and thymic carcinoma [In all 13 patients with the diagnosis of isolated RV metastasis HCC were identified. A summary of the patients is presented in Table In the diagnosis of metastasis HCC to heart, multimodality of cardiac imaging is of vital importance. The diagnosis might be neglected because of non-specific symptoms. According to previous cases, the patients lacked evident symptoms after a segmental hepatectomy or interventional chemotherapy and were found to have a large mass invaded in the cardiac cavities already. Yet, there may be no tumor recurrence in the liver. In our case, PETCT in other hospital didn’t provide information about the mass but only pericardial effusion. TTE, as the first means to find the mass, illustrated the contour, nature and mobility of the tumor. The high velocity flow of color Doppler also indicated inflow obstruction in RV. Moreover, higher enhancement of the mass and vascular perfusion in CE highly suggested malignancy. The huge mass in RV occupying nearly the whole cavity which caused inflow obstruction was confirmed by the surgery. The combination of TTE and CE displayed the mass in a relatively accurate and vivid view. Cardiac MRI provided more detailed information of the mass afterwards, which further confirmed and supported the diagnosis. Based on multimodality cardiac imaging findings, the patient underwent a successful operation of cardiac neoplasm. Surgical findings showed the tricuspid valves were severely blocked and the mass had partially infiltrated into the myocardium. The histopathological findings showed tumor cells growing in distorted version of normal architecture of various sizes. Tumor cells gathered in cords or nests with huge round nucleus, markedly pink-stained cytoplasm. Intercellular sinusoids and necrosis could also be clearly observed. The immunohistochemical findings proved that the mass was hepatocyte-derived and highly malignant.Fig. .Fig. 3HHCC with RV metastasis without IVC and right atrium involvement is exceedingly rare and it may result from hematogenous spread. The prognosis of HCC with RV metastasis is generally poor. Surgical intervention may be palliative treatment to relieve lethal hemodynamic instability, however, the general consensus also believed that the effect of surgery was limited with untreated primary focus . .In our