Most gastric lipomas are small, asymptomatic, and detected as incidental findings on radiologic evaluation or endoscopic examination of the upper gastrointestinal tract. We report a large lipoma which was noticed by its mass effect during a percutaneous endoscopic gastrostomy; the full evaluation prevented an unexplained failed procedure.2 lipoma. A resectional surgery was performed and combined with an open gastrostomy tube placement.A 65 year old man with a cerebrovascular accident (CVA) suffered from resistant vomiting and severe dysphagia. An endoscopic gastrostomy was indicated, but an incidental pedunculated submucosal mass in antrum terminated the procedure for further investigations, it was highly expected to cause an intermittent gastric outlet obstruction shortly after the procedure. The radiological evaluation found an 8 × 5 cmGastric lipoma is a very rare condition and mostly small and asymptomatic. Symptomatic ones could manifest by bleeding or obstruction. In our case, the obstruction was discovered incidentally by endoscopy. Computed tomography (CT) scan is an excellent diagnostic tool that confirmed the diagnosis. The treatment options range from open surgery to laparoscopic or endoscopic intervention.A full endoscopic evaluation for the upper gastrointestinal tract is recommended before gastrostomy. Our reported case gives an advantage of the percutaneous endoscopic gastrostomy procedure over the open gastrostomy tube placement to rule out a large asymptomatic gastric lipoma. Gastric22 with no other remarkable findings [A 65 year old man was referred to our clinic with a diagnosis of CVA, he developed multiple vomiting and sever dysphagia in every meal. The patient had been candidate to percutaneous endoscopic gastrostomy. Incidentally, the full upper endoscopy detected a gastric outlet obstruction; there was no intraluminal mass or ulceration on mucosa. This finding terminated the procedure without performing the gastrostomy. The patient underwent the full study, the CT scan of the abdomen showed a submucosal well encapsulated mass at the lower pyloric partition of stomach with a fatty like nature that measured 4.5 by 8 cmfindings . The patfindings  and a ga3Gastric lipoma is a very rare condition and mostly small and asymptomatic. Posterior wall of the antrum is the most site of origin . Most ca4Gastric lipoma is a rare condition mostly asymptomatic. However, it should be in mind when doing gastrostomy as it could fail the procedure and a resistant vomiting may be developed. We recommend a full endoscopic evaluation for the upper gastrointestinal tract whenever it is possible to rule out even a rare case of a large asymptomatic gastric lipoma.There are no potential conflicts of interest.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This scientific paper is correspondent with the policies and ethics that are mentioned in the Elsevier guides.Written informed consent was obtained from the patient and is available upon request. No patient identifying material was used in this manuscript.Mohamad Emad Abdulrahman: corresponding Author, wrote the manuscript.Anas Aji: the consultant who run the open surgery.Mhd Belal Alsabek: supervisor, contribute writing the manuscript.This case report has not been registered because it is for a rare case with new plan of management.It is not a research or a new surgical technique.Dr. Mhd Belal Alsabek.Not commissioned, externally peer reviewed.


Nephroblastoma (NB) is a malignant embryonal neoplasm derived from nephrogenic blastemal cells. NB usually forms a solid mass, but in extremely rare cases, it may show cystic changes.A six-month-old girl with persistent high fevers was found to have pyuria and bacteriuria. Ultrasonography revealed multilocular cysts in the right kidney. Right nephrectomy was performed with cyst wall rupture during surgery. An intraoperative rapid diagnosis, based on peritoneal fluid cytology, confirmed three components of blastemal, stromal, and epithelial cells. The blastemal cells were dyshesive, with scant to no cytoplasm and were the predominant cell type. The spindle-shaped stromal cells were arranged in fascicles. The epithelial cells demonstrated tubular structures. Macroscopically, the resected cystic tumor measured 80 mm in maximum diameter with a prominently thin cyst wall, but solid areas were also apparent. Histologically, the tumor was diagnosed as cystic NB  displaying a triphasic pattern. Hyperchromatic nuclei and apoptotic bodies were found. The clinical stage classification of Japan Wilms Tumor Study group was 3. The patient was treated with chemotherapy and radiotherapy. Tumor recurrence and metastasis have not been observed in the 8 months since surgery.This is an extremely rare case of infantile cystic NB. We diagnosed the NB cells that appeared in the peritoneal fluid by intraoperative rapid cytology. Cytological examination proved to be a very useful technique for determining the clinical stage of NB. Additionally, we propose that massive tumor degeneration and necrosis be considered as a pathogenic mechanism of cyst formation in NB. Wilms tumor, also known as nephroblastoma (NB) is a malignant embryonal neoplasm derived from nephrogenic blastemal cells. NB is the most common malignant renal tumor in children and 98% of cases occur under the age of 10. The mean age at diagnosis is 37 months and 43 months among males and females, respectively . GrosslyRenal cystic tumors in children can be benign, such as cystic nephroma (multilocular cyst), and malignant homologous tumors, such as cystic partially differentiated NB (CPDNB) . FurtherWe herein report an extremely rare case of cystic NB in an infant and discuss its pathogenic mechanism. In addition, we also describe the cytological findings of NB that appeared in peritoneal fluid.A previously healthy six-month-old girl who was born full-term following an uncomplicated pregnancy presented with persistent high fever and was found to have pyuria and bacteriuria. Prior to this, she had no significant medical history. No obvious gross malformations were observed on physical examination nor was any pertinent family history noted. Ultrasonography revealed multilocular cysts in the right kidney; of note, no abdominal abnormalities had been observed at her four-month medical examination.Abdominal contrast computed tomography (CT) revealed a multilocular cystic mass accompanied by septal wall formation pressing on the normal kidney parenchyma Fig. . The tumCytologically, the blastemal cells were dyshesive, exhibiting naked nuclei with scant to absent cytoplasm and were the most predominant cell type. The spindle-shaped stromal cells were arranged in fascicles, showing a fibroblast-like configuration. And epithelial cells formed tubular structures. Macroscopically, the resected cystic tumor measured 80 mm in maximum diameter involving the upper pole to middle portion of the right kidney Fig.  and the Histologically, the tumor was diagnosed as cystic NB  displaying a triphasic pattern. The blastemal cells showed a solid growth pattern, whereas the epithelial cells showed tubular structures Fig. . The spiThis case report describes an extremely rare case of cystic NB that developed in an infant with no family history or malformation. Furthermore, we describe the cytological findings of the tumor observed intraoperatively involving the peritoneal fluid.The differential diagnosis of cystic NB includes CPDNB. CPDNB also exhibits multiloculated cysts, as in this case, however these lesions should not have apparent solid or nodular mass formation. CPDNB contains mature or immature nephroblastic tissue, but conforms to the septum between cysts. In contrast, cystic NB has solid NB components expanding the cyst wall, which were easily identified in this case.To our knowledge, only three cases of NB with cyst formation have been reported in the English literature –6. HowevNB may be the cause of abdominal pain, anemia, and shock accompanying tumor rupture. According to the staging system of the JWiTS and the National Wilms Tumor Study (NWTS), diffuse peritoneal contamination associated with spillage of NB cells before and during surgery is classified as stage 3 –11. The We present an extremely rare case of infantile cystic NB. The diagnosis was able to be made on-site by intraoperative rapid cytology of peritoneal fluid. This technique of cytological examination proved to be very useful for determining the clinical stage of NB. Additionally, massive tumor degeneration and necrosis should be considered as a possible mechanism of cyst formation in NB.


Lymphangioma of the gastrointestinal tract is very rare, 75 % of the lesions affect the neck and 20 % affect the axillary region, the rest are seen in the mediastinum, the visceral organs, the retroperitoneum, the mesentery, or in the bones. Lymphangioma of the gastrointestinal tract comprises less than 1 %. The majority of cases are asymptomatic but some cases presented with emergency presentations.A 31-year-old male presented to emergency department complaining of central abdominal pain for 2 days, with nausea, dyspnea but no vomiting.The abdominal examination revealed a mildly distended abdomen, with guarding and tenderness mainly in the right lower abdominal quadrant. There were no any palpable masses or organ enlargement. The white blood cells count was elevated .Abdominal ultrasound showed an evidence of 10*7 cm multiloculated thick walled lesion in the right lower abdomen, suggesting the possibility of an appendicular abscess. The patient was admitted for 2 days with little clinical improvement. Therefore, a laparotomy was performed at which there were 2 yellowish cystic lesions 60 cm from the ileocecal valve that were arising from each side of the small bowel and related to its mesentery causing luminal narrowing. Resection was done with end-end anastomosis. The histopathology results revealed a diagnosis of ill lymphangioma of the ileum.Complete surgical resection is the main form of therapy whenever possible, sometimes is impossible when there is local invasion. When complete resection is performed long term follow up is not recommended as the recurrence is very rare. In literature it is reported most lesions are diagnosed in children and there is no sex predominance, about 75 % of the lesions affect the neck and 20 % affect the axillary region, the rest of the lesions are seen in some very rare locations such as the mediastinum, the visceral organs, the retroperitoneum, the mesentery, or in the bones. Lymphangioma of the gastrointestinal tract comprises less than 1 % [Lymphangioma of the bowel usually arises from the lymphatic channels where the lacteals of the villi empty, lesions are thought to be hamartomatous lesions rather than tumors which enlarges gradually with time. Lymphangiomas are usually solitary and not connected to the lymphatic channels in that region resulting in stasis and proximal dilatation .The majority of cases are asymptomatic and are diagnosed accidentally, most patients present with painless palpable soft abdominal mass, which grows slowly, but in some cases it may cause acute abdominal pain or intestinal obstruction which may be caused by luminal obstruction, or cases reported to be caused by small bowel volvulus, in some rare occasions it may be caused by the traction effect of the mass over the bowel ,4,5.The characteristic findings on abdominal CT scan is the presence of oval submucosal well defined cystic lesions which show no enhancement when contrast is injected .The work of this report case has been reported in line with the SCARE 2018 criteria .2A 31-year-old male, who is an office worker presented to emergency department complaining of central abdominal pain for 2 days. d with nausea, dyspnea but no vomiting.The patients had many attacks of similar pain but of milder intensity for the last years which was diagnosed as irritable bowel syndrome, the surgical histories were unremarkable and the family history was negative for chronic illnesses and malignancies.2.1The patient had tachycardia (the pulse rate was 110 b/m) with normal temperature and blood pressure. Other parts of the general examination were normal.The abdominal examination revealed a mildly distended abdomen, with guarding and tenderness mainly in the right lower abdominal quadrant. There were no any palpable masses or organ enlargement. The bowel sounds were normal.2.2The white blood cells count was elevated  and the hemoglobin and the urinalysis were normal.Abdominal ultrasound showed an evidence of 10*7 cm multiloculated thick walled lesion in the right lower abdomen which was located lateral to the iliac vessels, suggesting the possibility of an appendicular abscess The patient was admitted for 2 days and he received broad spectrum parenteral antibiotics with little clinical improvement.2.3The decision was done for surgery. Therefore, a laparotomy was performed at which there were 2 yellowish cystic lesions that were arising from each side of the small bowel and related to its mesentery causing luminal narrowing. The lesion was located approximately 60 cm from the ileocecal valve. Resection of the affected bowel segment was done with end-end anastomosis by a slowly absorbable suture material. The other parts of the bowel were examined and there were no other pathologies detected The resected sample was sent for the histopathological examination which showed an evidence of numerous and variable sized lymphatic channels in the mucosa and the submucosa of the bowel, there was intense inflammatory cell infiltration and the sample was negative for malignant cells. The diagnosis was lymphangioma of the ileum.2.4The patient was well in the post-operative period and he was discharged home after 5 days with no post-operative events.3The majority of the lesions are diagnosed incidentally, but rarely the condition is diagnosed before surgery. The disease may be suspected with the typical radiological features, sometimes when endoscopy or enteroscopy is performed the lesions appear as elevated, yellowish or white submucosal polypoidal lesions with intact overlying mucosa, there is dilatation of the mucosal blood vessels over the affected part ,7.Lymphangiomas occur due to congenital malformation of the lymphatic channels, it is very important to differentiate these lesions from lymphangiectasia which is defined as dilatation of a previously normal lymphatic channels which is caused by lymphatic obstruction from various inflammatory or malignant conditions, histologically lymphangiectasia also lacks the endothelial lining and the investing smooth muscle components .In the pediatric age group, lymphangiomas tend to be larger in size than those in adults, and most arise from the mesentery, and clinically most patients have emergency presentation such as acute abdomen or intestinal obstruction, while in adults they usually discovered incidentally or present with abdominal mass .Intra-abdominal lymphangiomas usually occur in the retroperitoneum, but may affect the small bowel mesentery, liver and the pancreas. Lesions are usually confused with mesenteric cysts which are of mesothelial origin, lymphangiomas should be differentiated from mesenteric cyst because they have more aggressive behavior and tend to be locally destructive lesions with higher recurrence rate, other differential diagnoses may include congenital duplications cysts of the bowel, cystic tumors, and parasitic cysts especially in endemic regions of hydatid disease .Most cases are treated surgically, but the lesions tend to be diagnosed late when they become large in size due to the asymptomatic pattern of the disease. When the lesions cause complications they present earlier. Small lesions less than 2 cm may be managed endoscopically by submucosal resection, but this is done only for lesions which are accessible endoscopically .Complete surgical resection is the main form of therapy whenever possible, sometimes resection with normal surrounding tissue. Complete surgical resection sometimes is impossible when there is local invasion .When complete resection is done long term follow up is not recommended as the recurrence is very rare, but when the complete resection is not possible close follow up is recommended .3.1I was told before surgery that my appendix is perforated and I need an emergency surgery, after surgery I have been told that a part of my bowel was resected and will have no major concerns in the future.The author has no conflicts of interest to declare.None.Ethical approval has been exempted by my institution for reporting this case.Written informed consent was obtained from the patient for publication of this case report and accompanying images.The concept of reporting the case, data recording, and drafting the work done by Dr Dildar Haji Musa and Dr Ayad Ahmad Mohammed.Dr Dildar Haji Musa took the consent from the patient for publishing the case.Final approval of the work to be published was done by Dr Ayad Ahmad Mohammed.This work is case report and there is no need of registration.Dr Ayad Ahmad Mohammed is guarantor for the work.Not commissioned, externally peer-reviewed.


We present a case of primary eccrine porocarcinoma of the thumb in a 56-year-old male who ultimately developed multiple metastases. With so few cases of such lesions and their aggressive nature, accurate diagnosis and prompt surgical management is essential. Eccrine porocarcinomas are a rare malignant type of sweat gland tumor that were first described by Pinkus and Mehregan in 1963 . AlthougWe detail a case of eccrine porocarcinoma of the thumb that ultimately developed multiple metastases, with a subsequent discussion emphasizing the difficulties of initial identification and principles of surgical resection. All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation  and with the Helsinki Declaration of 1975, as revised in 2008. Informed consent was obtained from the patient included in the study.A 56-year-old male was referred to the plastic surgery clinic with a four-year history of a skin lesion on the dorsum of his right thumb . The lesOn physical examination the patient had a 1.8cm by 1.3cm well circumcised indurated lesion over the dorsum of his right thumb stump . The masThe patient subsequently underwent a surgical excision in August of 2017 of the eccrine porocarcinoma of the thumb with an amputation at the level of the distal interphalangeal joint, and fine needle aspiration of the ipsilateral enlarged axillary node. A wide margin of 2.0 cm was used to ensure margins were clear of the tumor on the thumb. Histologic examination of the thumb specimen showed persistent ulcerated invasive eccrine porocarcinoma that involved the epidermis, dermis and extended into the subcutis with no involvement of bone. There was extensive lymphatic involvement that extended beyond the breadth of the main tumor and closely approached the peripheral margin of resection. Histologic examination of the right axillary node was positive for malignant cells that were consistent with poorly differentiated porocarcinoma and highly suspicious for metastasis. Axillary lymphadenectomy was subsequently performed and histological examination revealed metastatic involvement in 2 out of the 42 excised lymph nodes. CT examination of the rest of the body at the time of axillary resection was unremarkable.2), carboplatin (area under the curve = 5), and intralesional interleukin 2 (IL-2) injections the metastases responded with near complete disappearance of the cutaneous lesions. After one year of follow-up the patient was still responding well to this maintenance treatment.Follow up initially was unremarkable and the wound healed well. However, four months after the operation the patient had a CT scan demonstrating a seroma in the axilla that required drainage. Seven months after surgery, a suspicious lesion was identified in the scar of the right amputated thumb . SubsequThere are two types of sweat glands: apocrine and eccrine. Apocrine sweat glands are typically only found in certain locations of the body like the axilla, areolas of the breast, ear canal, eyelids, perianal region, and certain parts of the genitalia . The remMalignant eccrine porocarcinomas are rare, with fewer than 250 cases reported in the literature ,8. A revEccrine porocarcinomas can appear as a nodular or verrucous, dome-shaped or plaque like, erosive or infiltrative growth, and are typically ulcerated . LesionsThe prognosis of eccrine porocarcinomas depends considerably upon the ability to metastasize. Based upon case series, approximately 20% recur at the primary site within a year of excision and 20% will metastasize to regional lymph nodes ,10,12. OTo our knowledge, this is one of the first reported cases of malignant eccrine porocarcinoma of the thumb in the plastic surgery literature. Studies have shown that eccrine porocarcinomas can occasionally occur on the hand, however only a single previous case report has shown occurrence on the thumb ,8,10,11.Eccrine porocarcinomas are rare and treatment planning can be challenging. In our patient, initial surgical excision did not eradicate the disease and even after multiple, more radical revisions the patient developed metastases to multiple distant sites. This patient fortunately maintained meaningful hand function despite thumb amputation, and has responded well to management of his metastatic disease with adjuvant treatments of chemotherapy and radiation therapy. Based on this experience, we would advocate for aggressive resection with wide margins to attempt to achieve local control this disease at initial surgery and hopefully prevent metastasis.


The causes of neonatal bowel obstruction are variable and dominated by malformations and the Meckel diverticulum must remain exceptional. We report a case of neonatal bowel obstruction in a six day old male neonate admitted on account of inability to pass stool, abdominal distension and bilious vomiting. The radiologic additional examinations are non-specific. Exploratory laparotomy found obstruction at the site of a Meckel's diverticulum. Meckel's diverticulum (MD) is the most common congenital anomaly of the gastrointestinal tract, with a complication rate of approximately 4%. Bowel obstruction, gastrointestinal bleeding, acute inflammation and umbilical abnormalities are the most common presentations of MD in childhood, whereas symptomatic MD in the neonate is quite rare. We report an interesting case of symptomatic MD in newborn, only a similar case was found in the literature.A six day old male neonate, was admitted on account of inability to pass stool, abdominal distension and bilious vomiting. Symptoms had started 4 days previously. He had passed meconium within 24 h of birth and had been opening bowel regularly till the onset of symptoms. Physical examination revelated dehydrated and hypotonic term neonate with abdominal distension. Blood chemistry showed hyponatremia at 133 meq/l. Plain Abdominal radiograph revealed multiple air fluid levels and dilated loops of small bowel . An ultrAlthough Meckel's diverticulum is the most common congenital malformation of the gastrointestinal tract, but neonatal revelation remains rare. Meckel's diverticulum originates from an incomplete obliteration of the omphalomesenteric or vitelline duct, which occurs around the fifth week of gestation , 2, it uMeckel's diverticulum is a frequent malformation but neonatal revelation remains exceptional. No investigation can be made to establish the diagnosis, but only a laparotomy can identify the lesion and the exact mechanism to propose an adequate treatment.The authors declare no conflict of interest.


Ovarian cancer has few subjective symptoms, so approximately 40%–50% of cases have already reached stage III or IV by the time of diagnosis. These are advanced stages of the disease and have poor prognosis. Among these cases, less than 3 % are reported to exhibit inguinal lymph node metastasis. This report documents a rare case of advanced ovarian cancer that we detected due to an inguinal metastasis in the canal of Nuck. The work has been reported in line with the SCARE criteria.The patient was a 43-year-old, married, premenopausal woman (G2P1). She was examined by her local practitioner for a chief complaint of a mass in the right inguinal region and was found to have a right inguinal mass. Magnetic resonance imaging (MRI) scans revealed a left ovarian tumor, and she was referred to our department. Rapid intraoperative diagnosis showed a highly atypical serous carcinoma present in both the left ovary and the right inguinal region mass, where the tumor had extended into the canal of Nuck.In this case, the right inguinal mass was ovarian cancer that had metastasized to a cyst in the canal of Nuck via the round ligament of the uterus. Though, many adult women with these types of inguinal hydrocoeles sometimes undergo fine-needle aspiration.This finding may highlight the need for a careful search for metastasis to the inguinal region in cases of ovarian cancer. With ov2The patient was a 43-year-old woman  with a body mass index of 17.5 kg/m2. She was examined one year earlier by a gynecologist, who detected her uterine myoma. No ovarian mass was observed. In 2016, She noticed a right inguinal mass without any spontaneous pain. She had her gynecologic check-up again. Then she was found to still have multiple uterine myomas, although ultrasound examination revealed an internally heterogenous cyst measuring 32 mm in size in the right inguinal region, as well as an internally heterogenous cystic tumor of the left ovary measuring 60 mm in size. Then she checked blood tests for tumor marker: Carbohydrate antigen (CA) 125 = 139 U/mL, CA 19−9 = 12.4 U/mL, carcinoembryonic antigen (CEA) < 0.5 U/mL.Her menstrual history included menarche at 12 years of age. She had not reached menopause. Her menstrual cycle was 28 days. She had dysmenorrhea and menorrhagia. Her medical history was unremarkable otherwise. She had no surgical history, allergies, oral medications, or history of alcohol consumption or smoking. Her family history was unremarkable. Her cervical cytology results were negative every year.Contrast-enhanced magnetic resonance imaging (MRI) performed 2 weeks after presentation revealed a potentially malignant tumor of the left ovary complicated by an endometrial cyst. The cyst in the right inguinal region was a hydrocoele of the canal of Nuck .Fig. 1PrThe patient was referred to our hospital for further examination. Endometrial cytology was performed, and the result was negative. A complete blood count and blood biochemistry were performed, and all values were within the normal ranges. Contrast-enhanced computed tomography (CT) from the neck to the legs revealed no evidence of distant extra-abdominal spread. Gastrointestinal endoscopy was performed as part of a whole-body screening exam, which revealed no abnormalities. The preoperative diagnosis was a left (lt.) ovarian tumor, a right (rt.) inguinal tumor, and multiple uterine myomas. The lt. ovarian tumor appeared to be a borderline malignant or benign tumor.3We performed an open total hysterectomy, bilateral adnexectomy, and resection of the rt. inguinal tumor and disseminated tumors. We observed 50 ml of ascitic fluid within the peritoneal cavity, which we submitted for rapid cytological diagnosis. The left ovarian tumor  was surr4Ascitic fluid cytology was positive h, revealHematoxylin and eosin (HE) staining revealed tumor cells with highly atypical nuclei and prominent nucleoli in the left ovary. The tumor cells were solid and proliferating in a papillary pattern, and we observed several mitotic figures. Similar nuclear atypia was observed in the right inguinal tumor, while some areas exhibited a slit structure and glandular formation, which we believed to represent a similar serous carcinoma a, b.Immunostaining: p53 = 100 % positive, ER = weekly positive, PgR = slightly positive, PAX8 = negative, WT-1 = positive (5Stage IIIC ovarian carcinoma (FIGO2014)6The patient was discharged from the hospital 9 days after surgery. 28days after the surgery, she received six courses of the TC regimen  every 3 weeks, as well as bevacizumab. The patient has not relapsed for two years.7We encountered a case of ovarian serous carcinoma believed to be a metastasis to the right canal of Nuck. The canal of Nuck is the portion of the processus vaginalis that lies within the inguinal canal in women, and its presence indicates a patent processus vaginalis . A hydroThe relation between endometriosis and hydroceles in the canal of Nuck is interesting. Some Nuck hydrocele cases associated with endometriosis have been reported . NotablyMeanwhile, Noguchi et al. reported a case of ectopic pregnancy that occurred in the canal of Nuck in a patient with a closed inguinal ring due to the presence of an endometrial lesion . This caWe searched PubMed and Japanese medical abstract databases for cancer cases with a hydrocoele in the canal of Nuck and found four reported cases  [[12], . All repWe presume that the mechanism of metastasis to a hydrocoele in the canal of Nuck—which is considered to be a closed cavity—is as a result of dissemination based on the cytological and histological evaluations performed in the present case of highly atypical serous carcinoma. Investigating the process of the onset of ectopic endometriosis and ectopic pregnancy may provide new insights into evaluation of ovarian cancer dissemination.8We encountered an advanced case of ovarian cancer with metastasis to an inguinal cyst that arose in the canal of Nuck, which is a rare site for metastasis to occur. Future studies are required to further clarify the mechanism of ovarian cancer metastasis to the canal of Nuck.There is no source of funding.Our ethics committee approved this report . The work has been reported in line with the PROCESS criteria .Fully informed written consent has obtained which should be documented in the paperKazue Togashi contributes the paper with writing the paper and data analysis.Noriaki Ooyama contributes the paper with data collection, data analysis and interpretation.Katsuhiko Enomoto contributes the paper with data collection, data analysis and interpretation.Hirokazu Sato contributes the paper with data analysis, interpretation and whole management.researchregistry5025.Noriaki Ooyama is most suitable for the Guarantor.Not commissioned, externally peer-reviewed.There is no conflict of interest.


Angiosarcoma is a cancer that is derived from endothelial cells that line blood vessels and lymphatic channels. Cutaneous angiosarcoma can appear anywhere on the skin and the clinical presentation is highly variable. Most cases appear on the scalp and face de novo. Our case describes a 91-year-old female with cutaneous scalp angiosarcoma. Our case serves to remind physicians that an abnormal skin finding in older adults should raise their index of suspicion for angiosarcoma and an early biopsy should be performed. Angiosarcoma is a highly invasive tumor with a poor prognosis. Ten-year survival rates of scalp angiosarcoma have been reported to be as low as 13.8% with metastatic disease and as high as 53.6%, if the disease is localized [A 91-year-old female was admitted to the hospital after suffering a burn to her forehead by a salon hair dryer approximately four months prior to diagnosis. Following the development of the burn, she reported pruritic red patches appearing on the scalp. Over the four months, several clinical diagnoses, including eczema, cellulitis, and hematoma, were rendered by her primary care physician. For her presumed diagnoses, courses of therapy including topical hydrocortisone, moisturizers, and antibiotics were prescribed. While she was compliant with the outlined regimen, her symptoms did not improve. She was then referred to the hospital after she reported mild but persistent bleeding from the lesions. On initial examination, a large purplish mass on the skin of the frontal scalp was identified . An inciWide local excision of the lesion for histological tumor-free margin is the treatment of choice for angiosarcoma . SystemiCutaneous angiosarcoma has been described to be clinically variable in literature. Previously published case reports have described lesions as raised purplish-red papules, which can be misdiagnosed as rosacea, eczema, and hematoma –6. Due tIn our case several clinical diagnoses, including burn, eczema, cellulitis, and hematoma, were mistakenly given prior to the correct diagnosis of angiosarcoma provided. A delay in the definitive diagnosis may have played a role in the malignant course and therefore limited her treatment options. Our case serves to remind physicians that an abnormal skin finding in older adults should raise their index of suspicion for angiosarcoma and an early biopsy should be performed.


Intra-articular osteoid osteomas are a rare cause of articular pain. Their diagnosis can be challenging due to their non-specific clinical presentation and imaging characteristics. We present the case of a young soccer player with a 12-month history of anterior knee pain that was first attributed to Sinding Larsen Johansson syndrome and then to Hoffa’s syndrome. A CT scan was performed that revealed the localization of an osteoid osteoma of the patella. The patient was successfully treated by percutaneous radiofrequency ablation under CT guidance without complications and returned to full sports activity. Although a rare entity, osteoid osteoma of the patella with its atypical clinical features could be included in the differential diagnosis of persistent anterior knee pain in the young adult. High clinical suspicion is necessary to avoid delay in diagnosis and irrelevant procedures for the patient. Osteoid osteoma (OO) is a benign bone lesion that constitutes 11% to 14% of all benign bone tumors and presents clinically with pain typically worsened at night, alleviated by nonsteroidal anti-inflammatory drugs (NSAIDs) or aspirin. The lesion is commonly found on the diaphysis or metaphysis of long bones, and its typical radiological appearance is a radiolucent zone surrounded by sclerotic bone (nidus) smaller than 1.5 cm in diameter [Intra- and juxta-articular OO are a diagnostic challenge for the orthopedist due to their rare appearance (13% of the lesions) and also their atypical clinical and radiological characteristics . MisdiagWe present the case of a patellar OO in a young soccer player that was treated by percutaneous ablation. The uncommon site in combination with the atypical clinical presentation caused a 13-month interval between the onset of the symptoms and final treatment.The aim of this paper is to report this rare case of patellar OO presenting as anterior knee pain (AKP) along with a review of the literature. The difficulties a clinician faces, in his effort to diagnose a rare entity presenting with a vague knee symptomatology and thus focus on crucial points in the diagnosis of intra- and juxta-articular OO, should be highlighted.A 17-year-old adolescent soccer player presented with a 12-month history of left AKP. The pain was initially mild and poorly localized around the knee joint and had been attributed to a direct blow on the patella he had sustained during training. However, there was no relief with conservative treatment and physiotherapy and the pain progressively deteriorated over months. The pain was present at day and night and partially relieved with NSAIDs. An x-ray of the knee had been obtained with unremarkable findings  a biopsy needle was transosseously inserted under CT guidance and a bony sample was obtained from the nidus. The tip of the RF electrode was precisely placed in the nidus Figure , keepingHistological study confirmed the diagnosis of OO, with the classic appearance of mineralized woven bone with regularly shaped nuclei containing little chromatin but abundant cytoplasm Figure .The patient remained hospitalized overnight and was discharged the day after the procedure with a mild discomfort on the ablation site, with full range of motion. He was advised to use crutches for the first two weeks and then proceed to full weight bearing. He returned to full sports activity one month after the procedure without any complaints. On his regular follow-up 1, 6, and 24 months following the ablation, he remained completely free of pain and satisfied with his sports performance.The unique imaging features of intra-articular OO combined with their common clinical presentation make them a diagnostic challenge for the clinician. Kattapuram et al. suggested that they should be considered separate clinical entities because of the difficulties encountered in their recognition . EspeciaThe first arduousness is that OO are a rare cause of joint pain. Approximately 5.2% to 13% of the OO appear in a juxta- or intra-articular position . Hip joiThe most common symptom when an OO arises around the knee joint is AKP. However, AKP is one of the most frequently met musculoskeletal disorders. Each year many young athletes seen in primary care setting complain of some degree of knee pain, which is usually attributed to chondromalacia patella, patellar tendinitis, mediopatellar plica syndrome, Hoffa's syndrome, patellofemoral malalignment, osteochondritis dissecans, meniscal tears, or ligamentous injuries . The paiImaging features of the intra-articular OO differ from the characteristics of the extra-articular OO. X rays are not diagnostic in 80% of the cases as at the time of the first symptoms no nidus or periosteal reaction is usually present ,4,17. OnMRI is often requested as first-line imaging when dealing with knee symptoms. In our case, three MRI scans were performed in 12 months, none of which revealed the lesion, showing diffuse bone edema over the patella and joint effusion. In the literature, it is reported that approximately 21% of intra-articular niduses are not identified and a further 29% poorly identified, on initial MRI . The incNewer techniques have shown promising results in the imaging of intra-articular OO. Especially the use of single photon emission computed tomography/CT and MRI gadolinium-enhanced imaging have proved to be efficient in detection of the nidus in intra-articular lesions, which may have been the reason for a relatively early diagnosis ,16. AccoThe average delay for diagnosis of intra-articular OO has been reported by Szendroi et al. at 26.6 months and by Rolvien et al. at 20.7 months ,17. For The aforementioned imaging difficulties accompanied by the persistent non-specific symptoms of the patellar OO can result in misdiagnosis, irrelevant surgical procedures, and great discomfort for the patient. In our case, three MRI scans and physiotherapies were performed and an arthroscopy was scheduled. Our patient presented with an ill-defined AKP that was first attributed to a mild injury during training and after that to Sinding Larsen Johansson and Hoffa's syndrome before final diagnosis. Other common misdiagnosis for patella OO across the literature includes chondromalacia patella in three cases: patellofemoral malalignment, osteochondritis dissecans, meniscal tear, and quadricept tendinitis -9,12,13.Although the standard of care for the treatment of OO is image-guided RF ablation, in subchondral lesions, as in our case, cartilage degeneration is a possible complication and needs attention, especially in weight-bearing joints ,19. A diAlthough a rare entity, OO of the patella with its atypical clinical features should be included in the differential diagnosis of persistent AKP in the young adult. Especially in adolescents presenting with long-standing non-specific complaints around the knee joint with non-conclusive MRI findings, CT scan seems a reasonable step in the diagnostic procedure. High clinical suspicion for this rare lesion is necessary for the orthopedist to avoid delay in diagnosis and irrelevant procedures for the patient. Percutaneous CT ablation seems a safe technique for the treatment of patellar OO, although attention is needed to avoid any cartilage damage.


Disseminated peritoneal leiomyomatosis (DPL) is a rare condition that is characterized by the presence of multiple subperitoneal or peritoneal smooth muscle nodules of varying sizes on the omentum and peritoneal surfaces, grossly mimicking disseminated carcinoma. DPL usually develops in premenopausal women with a benign course, and it is often found incidentally during abdominal surgery. Malignant transformation is a rare clinical course of DPL. Only a few studies have focused on DPL transformation into a leiomyosarcoma. Herein, we describe the case of a 61-year-old woman with a history of recurrent leiomyoma of the uterus who presented with intermittent progressive abdominal pain. The imaging study revealed a huge heterogeneous density mass in the pelvic region with pulmonary and hepatic metastases. Exploratory laparotomy and debulking surgery were performed, and showed the coexistence of DPL and leiomyosarcoma. She died approximately one month after the diagnosis because of rapid progression of pleural effusion due to malignancy. This case highlights the clinical features of DPL and its malignant transformation and metastasis so physicians can make an early diagnosis and provide timely management. DPL is an extremely rare condition that is characterized by the presence of multiple smooth muscle, fibroblastic, and myofibroblastic nodules on the peritoneal surface of the pelvis and abdominal cavity . The truThis case study was approved by the Institutional Review Board (07-CR-085) of Taipei Tzu Chi Hospital, Buddhist Tzu Chi Medical Foundation.2 palpable pelvic mass was observed, and recurrent leiomyomatosis was suspected. A fourth laparotomy for tumor excision was suggested to the patient, but she refused. Hormone therapy with medroxyprogesterone (5-mg tablet daily) was performed until 2014 because of the progression of a thyroid nodular goiter and hypertension. After stopping hormone therapy, she reported intermittent, progressive lower abdominal pain, and a growing palpable mass was observed in the abdomen.A 61-year-old G2P1AA1 woman presented with intermittent, progressive lower abdominal pain for three years. She had a medical history of hypertension that was controlled with medication. In 1993, she was diagnosed as having a uterine leiomyoma with leiomyomatosis of retroperitoneal tumors. Because of her desire to bear children in the future, she underwent myomectomy at that time. Subsequently, recurrent DPL developed two years later due to an enlarged palpable mass in the abdomen with intermittent abdominal pain. She underwent abdominal hysterectomy with left-sided oophorectomy and right ovary wedge resection in 1996. Results of the pathological report showed a borderline mucinous ovarian tumor of the left ovary. In 1998, recurrent DPL with left ureter involvement was noted, and the third operation involving debulking surgery and reteroneocystostomy was performed. Results of that pathological report showed no significant malignancy. She was regularly followed up at gynecologic clinics. In 1999, a 9 × 9-cm2. On pelvic examination, a huge tumor was palpable in the pelvis. Transvaginal ultrasonography showed huge solid tumors measuring about 15.17 × 24.48 × 10.51 cm3 in the pelvic field without free fluid in the cul-de-sac region , carbohydrate antigen 19–9 level (10.20 IU/mL), and carcinoembryonic antigen level (0.649 ng/mL). The chest X-ray showed nodular densities in the right lower lung .3, 7.1 × 6.1 × 6.0 cm3, and 11.1 × 7.5 × 9.1 cm3, with multiple pulmonary nodules and multiple heterogeneous hepatic tumors. Therefore, distant metastasis was suspected. Severe hydronephrosis of the right kidney was obvious due to tumor compression . Immunohistochemistry staining showed that the tumor cells were reactive with desmin, negative with cytokeratin and CD117 (c-kit), and negative for estrogen and progesterone receptors. The immunohistochemical profile confirmed leiomyomatous differentiation in other small nodules that were seeding in the peritoneum and omentum, smaller pelvic masses with hypercellular spindle cell tumors, and minimal nuclear atypia, which were all indicative of leiomyomatosis. She was diagnosed as having a leiomyosarcoma arising from DPL at 61 years of age. Chemotherapy with doxorubicin caused the leiomyosarcoma.Unfortunately, she died approximately one month after the diagnosis because of rapid progression of pleural effusion due to malignancy.Benign smooth muscle cells are mainly involved in DPL. Because of its rare prevalence rate, the origin of DPL remains unclear. A couple studies have hypothesized that DPL may arise from submesothelial multipotential cells with multiple factor stimuli for cell differentiation, such as hormones and genes ,14. The The detailed mechanism of malignant transformation of DPL plays an important role in the ability of physicians to detect the progression of malignancy early and to provide timely intervention. Currently, a new concept of epithelial-to-mesenchymal transition (EMT) suggests that malignant transformation is induced by EMT-activating transcription factors, which regulate all stages of cancer progression, including tumor proliferation, invasion, migration, distant metastasis, and drug resistance ,21. In mIn previous clinical reports, DPL with malignant transformation was rarely discussed. In Our patient was diagnosed as having DPL at 61 years of age. She underwent nearly complete staging surgery. In previous studies, the surgery performed was based on the tumor size. The huge tumor was resected as complete staging surgery because we suspected malignancy. In our patient, we think that the several instances of recurrence were an indicator of malignancy and the need for advanced therapies. Atypia in the pathology report may have been another indicator of early malignant transformation. Therefore, we think that molecular and cytogenetic analyses of DPL should be routine. Herein, we highlighted the clinical and imaging features of leiomyosarcoma so that physicians can make an early diagnosis and provide timely medical and surgical intervention to improve outcomes. We also recommend that these rare cases be referred to specialized centers.


Neutrophilic dermatosis of the dorsal hands is an underrecognized entity, which is a distributional variant of Sweet's syndrome. It is often clinically misdiagnosed as an infectious process in overwhelming majority of the cases and the treatment is therefore delayed. Also, its association with underlying systemic and neoplastic disorders makes the need for an accurate diagnosis more crucial. We present a 45-year-old Caucasian woman who was initially diagnosed as having a hand infection with unsuccessful courses of antibiotic therapy. A later biopsy revealed a diffuse dermal infiltrate of neutrophils with leukocytoclasis, vasculopathic changes, and marked papillary dermal edema. Patient responded rapidly to oral prednisone treatment. By sharing a new case and comprehensive review of available published literature, we intend to raise awareness of this underreported entity and emphasize the role of timely biopsy of the lesions that will not only lead to an accurate diagnosis, but also avoid unnecessary antibiotic treatments, potentially aggressive management strategies such as surgical debridement or amputation, and referrals to wound care centers. More importantly, it will prompt a search to exclude any possible association, particularly hematopoietic malignancies. Pustular vasculitis of the hands was first introduced by Strutton et al. in 1995, to describe an eruption on the dorsal hands resembling Sweet's syndrome, but showing leukocytoclastic vasculitis histologically –4. NeutrA 45-year-old Caucasian obese woman presented with small painful ulcers on the back of her hands and fingers that had started three weeks prior to her visit. She first noticed small red flat discolored areas which gradually worsened by developing pain, swelling, and ulcers within two weeks. She did not recall a prior trauma. She had a history of previous laparoscopic gastric sleeve surgery for morbid obesity and a vague diagnosis of mild diabetes for which she was not on any medication. She denied taking a new drug. With the clinical diagnosis of an infectious process, bacterial culture and sensitivity were performed and she was given oral and topical antibiotics  along with wound care instructions. The patient started developing fever with malaise and was admitted to the emergency room, where she was placed on intravenous antibiotic (vancomycin) due to suspicion of sepsis, originating from her “hand infection”. After a few days, she returned to our clinic. Compared to the original visit, the condition appeared worse with development of erythematous ulcerated nodules and plaques, violaceous borders, and marked surrounding edema, present on the dorsal aspects of right index and left ring fingers along with proximal metacarpophalangeal joint of third digit. The fingers in the nonulcerated areas displayed a fusiform swelling Figures  and 2. SThe term neutrophilic dermatosis of the dorsal hands (NDDH) was first proposed by Galaria et al. in 2000, who considered it to be a subset of Sweet's syndrome . A thoroNDDH is best regarded as a distributional variant of Sweet's syndrome, where the lesions occur on the dorsal aspects of both hands in its typical presentation. In the majority of cases, the initial clinical diagnosis is an infectious process. In addition, patients are referred to wound care centers due to nonhealing wounds. Therefore, unsuccessful antibiotic treatments, failed surgical debridements, and even amputations could follow. By presenting a new case of NDDH and review of the existing published data, we intend to raise awareness of this clinicopathologic entity for dermatology practitioners and dermatopathologists. NDDH should be strongly considered in lesions occurring on the dorsal hands, particularly if suspicious of infection, and timely biopsy be performed. In addition, recognizing this disease should prompt the clinician for a thorough investigation to rule out any associated malignancy or systemic disorder.


An isolated descending colonic stricture is an unlikely complication of acute pancreatitis, with the nonspecific symptoms of colonic stricture making the overall diagnosis difficult. Crohn’s disease (CD) and tuberculosis (TB) are the two common etiologies of an isolated colonic stricture and may present similarly to colonic stricture related to acute pancreatitis. Unfortunately, colonoscopy and biopsy often cannot determine the etiology, and surgical resection may be needed to provide both symptomatic relief and confirm the diagnosis. As a result, descending colonic stricture may produce a diagnostic dilemma with CD and TB as all 3 conditions may be radiologically and endoscopically indistinguishable. We describe a young male with weight loss and abdominal pain. TB testing was negative, with radiography and ELISA (enzyme-linked immunosorbent assay) testing supporting a diagnosis of the CD. The patient was initiated on sulfasalazine but worsened over the next month. Further investigations revealed that the patient had descending colonic stricture without CD. Therefore, the stricture’s etiology was most likely related to an episode of acute pancreatitis the patient had 2 months before admission and was found to have left colonic segment adherent to the pancreas eventually requiring segmentectomy. Although the pathophysiology of colonic stricture after pancreatitis is unclear, we speculate that inflammatory injury to the colon is an important component. Finally, we emphasize that colonic stricture is a rare complication of pancreatitis. The anatomic relationship of the large bowel to the pancreas is a vital factor in the genesis and localization of these lesions. Strictures related to pancreatitis may occur after a significant delay following the resolution of pancreatitis.6 Benign colorectal strictures can develop after diverticulitis, ischemic colitis, radiation colitis, or colonic resection. Large bowel necrosis represents an early complication of acute pancreatitis, with mortality rates between 50% and 90%.7Large bowel obstruction is a relatively common condition, although it is a rare sequelae to acute or chronic pancreatitis and is associated with high mortality rates.A 32-year-old man presented with colicky abdominal pain in the left lower quadrant for 2 weeks duration. He had a significant weight loss of approximately 30 kg over 2 months associated with decreased appetite. He also has a history of chronic alcoholism and smoking. There was no associated nausea, vomiting, hematemesis, melena, or hematochezia. Review of symptoms was negative for fever, arthritis, skin rash, jaundice, and pruritus. Two months prior, the patient was discharged from another hospital following a 6-week hospitalization for alcohol-induced necrotizing acute pancreatitis, which was treated successfully by conservative measures. Admission vitals revealed an afebrile and normotensive patient with a heart rate of 92 beats per minute and oxygen saturation of 98% on room air. On physical examination, the abdomen was soft with mild tenderness to deep palpation in the left iliac fossa and lumbar region. There were no signs of peritonitis. Laboratory results and abdominal sonogram showed no significant findings. Abdominal CT scan from the episode of acute pancreatitis 2 months ago showed findings consistent with necrotizing acute pancreatitis .A colonoscopy was done because of the abdominal pain and significant weight loss. The scope revealed ulceration with a partial stricture at the descending colon approximately 30 cm from the anal verge . HoweverDue to tuberculosis (TB) being a possible differential diagnosis, a workup for TB was warranted. This included sputum for acid-fast bacillus, chest X-ray, purified protein derivative test, erythrocyte sedimentation rate, and pathological examination, all of which were negative for TB. Biopsy of the lesion revealed active focal colitis with ulceration, but no granulomas were seen. Barium enema and CT colonoscopy were done to assess the length of the stricture to plan an appropriate treatment strategy as shown in Based on the clinical presentation and barium enema, Crohn’s disease was high in the differential diagnosis. ASCA immunoglobulin G antibodies to saccharomyces cerevisiae was ordered with a result of 22.2 U/mL . The patient was thought to have had a CD at this point and was subsequently initiated on sulfasalazine. Despite treatment for CD, the abdominal pain persisted over the next month with worsening weight loss. Repeat colonoscopy was performed, which revealed further strictures requiring a segmentectomy with single layer extra mucosal end-to-end anastomosis. Anatomically, the left colonic segment was observed to be adherent to the pancreas. A surgical biopsy specimen revealed active focal colitis, and negative for TB and CD. A final diagnosis of colonic stricture due to acute necrotizing pancreatitis was made. Abdominal pain decreased postoperatively, and the patient was discharged on postoperative day 7. A follow-up colonoscopy 2 months later revealed healed anastomotic sites.8 However, stricture of the descending colon is exceptionally rare. A case report by Yoo et al showed an obstruction of the descending colon as a complication of acute pancreatitis, and the patient underwent surgery due to nonresolving stricture.4 Another case of descending colon stricture presented as an acute intestinal obstruction secondary to acute pancreatitis and during the subsequent laparotomy; a tumor of the left colonic flexure with a fistula to a retroperitoneal abscess and necrosis of the pancreatic tail was observed.5 In a case series analyzing strictures formed during pancreatitis including a total of 13 colonic strictures, 9 strictures were located at the splenic flexure; 2 were adjacent to the splenic flexure; and 1 was at the hepatic flexure.9 Another retrospective case series involving 296 patients with acute pancreatitis showed that only 6.1% of patients developed colonic complications. Of these, only 1 case had incomplete colonic stenosis, and no strictures being described as occurring in the descending colon.10 This makes our patient an atypical presentation, as none of the strictures noted in both of the pancreatitis case series occurring at the level of the descending colon.Stricture of the colon at the splenic flexure as a sequel to pancreatitis was first reported by Forlini et al in 1922.11Since pancreatitis rarely manifests into a descending colonic stricture, and because of the weight loss, imaging, and ELISA results being consistent with CD, we presumed our patient to have CD. Up to 20% of patients with CD may present with isolated colitis. The clinical presentation of the colonic CD is highly variable between patients. This was part of the initial diagnostic dilemma in our case and the initiation of sulfasalazine.12 Many pathological hypotheses have been suggested for the development of colonic obstruction following pancreatitis; acutely as a result of ischemia and necrosis due to acute pancreatitis.13 Additionally, the peritoneal reflections from the anterior surface of the pancreas provide a route for the spread of both pancreatic enzymes and inflammatory mediators within the transverse mesocolon and small bowel mesentery.1 This proximity of splenic flexure region to the pancreas explains the localization of these lesions, with the splenic flexure and transverse colon being the common sites for stenosis. Additionally, the splenic flexure is a watershed region between the areas of supply of the middle and left colic arteries and is particularly susceptible to periods of hypotension during acute pancreatitis, leading to an ischemic process leading to necrosis followed by stricture.6 Through-the-scope balloons may be used to reach and pneumatically dilate strictures.14 Through-the scope balloons have been used as a first-line treatment modality for benign colorectal strictures, but the success rate is variable in the literature.16 Predictors of a successful outcome include relatively narrow stenosis (<10 mm), a short segment stricture (<4 cm), and anastomotic strictures. Poor predictors of success include multiple strictures, complete obstruction, associated fistulas within the stricture, active inflammation around the stricture, recent surgery, a tight angulation, and malignancy.16 In our case, failure to dilate the stricture was likely due to more extensive involvement and a more severely stenosed segment. Postoperative histology provides valuable information as it can exclude primary large bowel pathologies such as inflammatory bowel disease including CD and neoplasia. In this case, histology confirmed the presence of scarring and peri-colonic inflammatory changes ruling out CD as well as other etiologies.Colonic obstruction can arise during both the acute stage of pancreatitis and also a few weeks after the resolution of acute pancreatitis.Clinicians should consider acute pancreatitis as one of the etiologies of descending colonic strictures, especially in patients with no history of CD or TB, and a recent history of pancreatitis. Acute pancreatitis may involve the descending colon and can cause bowel obstruction even after a delay following the resolution of pancreatitis. Bowel resection may be warranted after the failure of medical and endoscopic therapy. Postoperative histology may assist in confirming the diagnosis.


One of the major disabling health conditions among elderly is back pain due to degenerative diseases. Less than 1% of spine disorders are caused by malignancies. Among the rare primary vertebral neoplasms the multiple myeloma and the plasmacytoma account for 26% of these cases.We are reporting a case of 64 year-old woman, who suffered from progressive upper back pain and intermittent neurological symptoms including lower limb weakness and voiding disorder under axial loading. Her medical history includes a Lynch syndrome (LS). Computed tomography (CT) and magnetic resonance imaging (MRI) detected a single malignant osteolytic process of the spine involving T5. Urgent surgery with laminectomy intralesional tumor removal and posterior stabilization (Th4–Th6) due to unstable pathologic fracture with spinal cord compression was conducted after interdisciplinary decision. Histopathological examination of the tumor revealed a solitary plasmacytoma.To our knowledge this is the first case report of a solitary plasmacytoma of the bone (SPB) that arise in a patient who suffers from LS. Similar DNA mismatch repair malfunction is existent in LS and SPB. Hence, a hereditary correlation might be imaginable.When detecting a lytic spinal tumor in a patient who suffers from LS a SPB should be taken under consideration. It isWe are reporting a case of a 64 year-old woman who suffered from a LS and a SPB involving thoracic vertebra 5. This work has been reported in line with the SCARE criteria .2A 64-year-old female presented with progressive back pain at our hospital. Previously 6 month of outpatient conservative treatment led to no recovery of the symptoms. On time of admission in the emergency room she suffered from worsening upper back pain and intermittent neurological symptoms including lower limb weakness and voiding disorder under axial loading. The patient reported that cancer surgery of rectum, colon and uterus due to LS had taken place 10 years ago. Sporadic oncological aftercare was conducted the past 5 years. The additional medical history includes a first degree relative who suffers from LS. Neither patient’s vital signs and blood tests nor urine analyses revealed any inflammatory processes. Weight loss, fever and night sweat were negated. CT and MRI detected a single malignant osteolytic process of the spine involving T5 with a pathologic fracture leading to segmental kyphosis . EpiduraDifferential diagnosis, causing patient’s symptoms, like osteoporotic fracture with posterior wall displacement, myelopathy, spondylodiscitis and other primary vertebral tumors were next to metastatic malignancies interdisciplinary discussed.The clinical symptoms increased rapidly. Hence, urgent surgery with laminectomy intralesional tumor removal and posterior stabilization (Th4-Th6) due to unstable pathologic fracture (SINS 13) with spinal cord compression was conducted after interdisciplinary decision with radiologist, oncologist and spine surgeon ,11. The In conclusion the diagnosis of SPB was made. After case presentation at our interdisciplinary tumor board the decision for radiotherapy (RT) with 46 Gy and bisphonsphonate therapy was made. The patient is self-sufficient and occasionally depending on oral non-opioid painkiller 18 months after surgery. Neurologic symptoms vanished. A follow up CT showed a steady segmental kyphosis and a clear epidural space.3Due to rapid neurologic symptoms and loss of spinal stability an urgent surgical therapy in the reported case was necessary from our point of view. A final preoperative determination of the dignity was not possible, which at the best wouldn’t require the surgical treatment but only single local radiation .The procedure of dorsal decompression (T5 laminectomy) and instrumented stabilization of T4-T6  was done after interdisciplinary decision and in advice of the spinal instability neoplastic score of 13 . The natPlain radiography, MRI and CT are standard imaging techniques in detecting spinal disorders. More often than primary malignancies are metastatic lesions of the axial skeleton . In the Our patient also suffered from LS. It is a dominantly inherited cancer predisposition syndrome and the most common cause of inherited colorectal cancer (2–4%) . To our After histopathological results and exclusion of a plasma cell myeloma oncologic treatment following the SPB therapy recommendations were initiated. Local RT was applied at the tumor site. Nevertheless according to literature and guidelines on this topic there seems to be no consensus about the optimal radiation dose ,21. The 4Back pain of the elder is a common everyday symptom. Early diagnostic imaging has to be considered in medical history of cancer. When detecting a lytic spinal tumor in a patient who suffers from LS a SPB should be taken under consideration.To our knowledge, this is the first case report of a SPB that arise in a patient who suffers from LS. A DNA mismatch repair malfunction, as cancer pathogenesis, is existent in LS and SPB. Hence, a hereditary correlation might be possible.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.This study was not applicable for ethical approval.I have obtained written consent for publication of this case report from the patient and I can provide this should the Editor ask to see it.Dr. med. Ekkehard Friedrich Röpke (corresponding author): Contribution to the paper: first author, data collection, data analysis and interpretation, writing the paper.PD Dr. med. Franz Theissig: Contribution to the paper: histopathological examination of the tumor, interpretation of the histological pictures.Dr. med. Carsten Bochwitz: Contribution to the paper: treatment and examination of the patient.Dr. med. Katharina Bäker: Contribution to the paper: treatment and examination of the patient.Dr.med. Alexander Grundig: Contribution to the paper: treatment and examination of the patient.Dr. med. Gerhard Ulrich: Contribution to the paper: bone scintigraphy examination and interpretation.Dr. med. Christoph Paasch: Contribution to the paper: data analysis and interpretation, supervising writing of the paper.The case report at hand is not a first-in-man case report of a novel technology or surgical technique, therefore a registration of these case reports according to Declaration of Helsinki 2013 is not required.Dr. med. Ekkehard Friedrich Röpke.Commissioned, externally peer-reviewed.None.


A 67-year-old woman presented with clinical symptoms, radiological findings, and preoperative work-up highly suggestive of advanced stage IV carcinoma of the gallbladder (CG). An extended cholecystectomy with the excision of adjacent liver segments and loco-regional lymphadenectomy was performed. Final pathology results revealed diffuse xanthogranulomatous cholecystitis (XG) with ruptured Rokitansky-Aschoff sinuses with tumor-resembling adenomyosis without atypical or malignant cells. There was a reactive inflammatory and fatty degeneration of the adjacent hepatic tissue and a nonspecific inflammatory reaction of the enlarged periportal lymph nodes. The main concern in the management of patients with mass-forming XG is that this benign condition shares strikingly similar clinical, imaging, biochemical, and intraoperative features with advanced CG, which has one of the poorest overall survival rates. Misdiagnosis is not uncommon, which causes significant distress for patients and their families and, in some cases, may result in erroneous treatment. Although the presence of some preoperative imaging findings and/or intraoperative frozen section biopsies may be helpful in suspecting XG, definitive diagnosis is usually delayed until the final pathology result that may come as a surprise. Increasing awareness of this rare, insidious disease will contribute to a better understanding of its biology and natural history and, eventually, help improve management. Xanthogranulomatous cholecystitis (XG) is a variant of cholecystitis characterized by an acute or chronic inflammation of the gallbladder with a focal or more-diffuse accumulation of fibrous tissue and lipid-laden macrophages in the gallbladder wall. The direct involvement of surrounding organs by the inflammatory fibrosis is not common; however, the inflammation in most severe, tumor-like cases of XG may extend into the adjacent structures, making this benign condition practically indistinguishable from advanced carcinoma of the gallbladder (CG) . Such caWe present a case of a mass-forming XG complicated by a severe, extensive inflammatory involvement of the adjacent organs with clinical signs and preoperative imaging highly suggestive of advanced carcinoma of the gallbladder.A 67-year-old woman was admitted to hospital for an evaluation of worsening right upper abdominal tenderness associated with episodes of nausea and vomiting for the last month. She described the pain as mild to moderate, continuous, radiating to the back, unrelated to eating, and without alleviating or exacerbating factors. She denied fevers, jaundice, or issues with bowel movements and urination. She admitted to a 10 kg weight loss over the last two months. Her medical history was significant for sinus tachycardia with good medical control on metoprolol. She admitted smoking and drinking on social occasions. Her family history was unrevealing.At initial evaluation, her vital signs were within normal limits with a body temperature of 98°F, a pulse rate of 61 beats per minute, a respiratory frequency of 12 breaths per minute, and a blood pressure of 130/85. The abdominal exam revealed a palpable, poorly defined, mildly to moderately tender tumor-like firmness in the right upper quadrant (RUQ) without rebound tenderness. The remainder of her physical examination was unremarkable. The laboratory evaluation revealed a white blood cell (WBC) count of 10.2×109/L, hemoglobin 135 g/L, glucose 6.1 mmol/L, creatinine 0.8 mg/dL, alanine aminotransferase (ALAT) 9 U/L, aspartate aminotransferase (ASAT) 18 U/L, total bilirubin 6.8 mmol/L (direct bilirubin 3.6 mmol/L), and an international normalized ratio of 0.94. Tumor marker cancer antigen 19-9 (CA 19-9) was 14.4 U/mL  and carcinoembryonic antigen (CEA) was 0.6 ng/mL .Abdominal ultrasonography (US) and computed tomography (CT) of the chest, abdomen, and pelvis revealed a large, ill-defined, heterogeneous mass completely replacing the gallbladder body and fundus with an extensive involvement of the adjacent liver segments, the duodenum, the head of the pancreas, and the hepatic flexure of the colon Figures -1B. TherAdvanced stage IIIb-IVa CG was the main differential diagnosis and the possibility of acute or chronic cholecystitis with diffuse mass-forming XG was lower on the list. A detailed discussion of the possible intraoperative scenarios and the extent of the surgical resection with the risks, benefits, and prognosis was carried out, and informed consent was obtained. Surgical exploration confirmed the preoperative imaging findings and revealed a mass occupying the entire subhepatic space adherent to the adjacent liver segments, omentum, hepatoduodenal ligament, the second portion of the duodenum, and proximal one-third of the transverse colon. No intra-abdominal collections, abscesses or lesions suspicious of metastatic malignant disease were seen. Intra-operative frozen-section biopsies were not performed. Despite the extensive fibrosis and adhesions, we were able to define and dissect through soft tissue planes and safely separate the involved structures to perform a resection of the entire mass with adjacent liver segments and loco-regional lymph nodes for proper staging and prognosis.Surgical pathology revealed an 8x11 cm XG tumor with diffuse, marked thickening of the gallbladder wall (up to 1.5 cm) and lumen filled with spongiform purulent matter. Histology showed deep, ruptured Rokitansky-Aschoff sinuses penetrating the muscle layer with areas of tumor-resembling adenomyosis Figure . There wThe postoperative course was uneventful, and the patient was discharged home on day seven after surgery. Six months after the operation, the patient remains asymptomatic and in good health.The incidence of early, focal XG in patients with symptomatic gallbladder disease may range widely depending on geographical region, studied population, and cohort size and can be found, on average, in 1.3% of patients with cholecystectomy in Europe, 1.5% in America, 1.9% in Japan and Korea, and in more than 9% in India . A severThe underlying pathophysiological mechanisms of XG have not been completely understood. One compelling theory supported by this case Figure  is that The presenting signs and symptoms of focal, less severe XG are usually that of chronic or acute cholecystitis, including postprandial right upper quadrant (RUQ) abdominal pain with or without positive Murphy’s sign, fever, nausea, and vomiting; however, the presentation of diffuse, tumor-like XG with severe inflammatory fibrosis, involving the surrounding tissues, as in the case presented here, may also include RUQ mass, abdominal tenderness unrelated to meals, jaundice with or without cholangitis, and weight loss that make the clinical picture virtually indistinguishable from CG .Establishing the precise diagnosis in such complicated cases could be a daunting task, and it is usually delayed until the final pathology results. The difficulty in defining the right diagnosis and management strategy comes from the fact that both XG and CG share similar clinical, imaging, biochemical, and intraoperative features. Although, some imaging findings on CT and ultrasound (US) such as diffuse wall thickening, intramural hypoattenuated nodules or hypodense bands, gallstones, and continuous mucosal line have been associated more often with XG in some studies, others have shown that these imaging features can be missing in XG and present in patients with CG -6. AnothAll these factors contribute to a considerable diagnostic and management uncertainty that may cause significant distress in patients and patients’ families, and potentially result in over- or under-treatment in some cases . The choIn the present case, the choice to limit surgery to an extended cholecystectomy with the excision of the gallbladder fossa and loco-regional lymphadenectomy was based on our current understanding of biology, natural history, and the prognosis of XG and advanced CG as well as the morbidity and mortality related to more extended radical procedures, including biliary tree resection and or pancreaticoduodenectomy.Aggressive multivisceral resection has been shown to be feasible in some cases of advanced stage III or IV CG despite increased mortality and morbidity; however, the true benefit of these procedures is yet to be clarified -9. LargeThe increasing awareness and growing number of reported cases of XG contribute to a better understanding of this insidious disease. Yet, distinguishing between the benign XG and the advanced CG prior to the final pathology results is extremely difficult, and defining the optimal management strategy remains a considerable challenge. Treatment decisions should be guided by a close collaboration between surgeons, radiologists, and pathologists with a thorough review of all patient-related and disease-related factors, such as disease extent and location, as well as patient’s comorbidities, symptoms, and performance status to ensure the most favorable outcome.


Surgery which includes peritonectomy, visceral resections, and perioperative chemotherapy has been used extensively to treat peritoneal metastases from appendiceal mucinous adenocarcinoma. The results of treatment vary with the grade of the neoplasm, the extent of abdominal and pelvic disease and the completeness of tumor removal.The clinical features, surgical procedures and outcome of two patients with mucinous appendiceal neoplasms were reviewed. The decision to move to total pelvic exenterative surgery after prior treatment failure was critically evaluated.Patient 1 had three extensive surgical procedures prior to total pelvic exenteration and one surgical procedure after. Patient 2 had one 16-h surgical procedure prior to total pelvic exenteration. Patient 1 had 8 years and 1 month survival from onset of disease until death. Patient 2 is free of disease at 27 years from onset of pseudomyxoma peritonei. After recovery of the patients from the extensive surgery, their quality of life was acceptable.Total pelvic exenteration surgery is seldom used in patients with peritoneal metastases from appendiceal neoplasms. These two patients were studied in an attempt to determine the indications for this procedure with this disease. The combination of complete cytoreductive surgery with perioperative intraperitoneal chemotherapy is currently the standard of care . The mosIn this case series two patients are subjected to total pelvic exenterative surgery as treatment for recurrent appendiceal mucinous neoplasms with peritoneal metastases. Clinical features associated with success are critically evaluated.2The grade of the malignancy was assessed by an experienced pathologist as low, moderate or high grade . The perwww.researchregistry.com website with UIN 4834.Data on these two patients was prospectively recorded and then retrospectively reviewed at an academic institution. This research work has been reported in line with the PROCESS criteria . This st33.1In November 2008, this 46 year old woman noted a mass in the right lower quadrant of her abdomen. CT demonstrated a multicystic mass in the caecal/appendiceal region.In December 2008, the patient underwent exploratory laparotomy. A large mass in the area of the appendix was visualized. Numerous mucinous tumor nodules were seen beneath right and left hemidiaphragm and within the greater omentum. A right hemicolectomy was performed. Pathology showed a high grade mucinous adenocarcinoma of the appendix with 3 of 17 lymph nodes positive for cancer.In February 2009, a repeat CT scan showed an 8 cm right ovarian cyst and nodules within the greater omentum but was otherwise normal. A CEA was 10.1 ng/ml, CA19-9 was 50.7 μg/m, and CA125 was normal at 6.2 μg/ml. The patient returned to the operating room for cytoreductive surgery and perioperative chemotherapy. Procedures performed included greater and lesser omentectomy with splenectomy, right upper quadrant peritonectomy, and pelvic peritonectomy with hysterectomy [In July 2100, a CT scan showed 4 areas of disease progression. The CEA was 2.8 ng/ml and CA19-9 was 18.9 μg/ml. A third exploratory laparotomy was performed. Multiple thick fibrous adhesions were present. Three separate small bowel resections were required. A rectosigmoid colon resection was performed with low anastomosis. A solitary metastasis in the liver was resected. No perioperative chemotherapy was used. The procedure required 11 h. Metal clips were placed on a positive margin on the bladder. External beam radiation was used postoperatively in an attempt to control disease in the pelvis.In February 2013, despite bladder irradiation, CT showed gross disease progression within the wall of the bladder. Right hydronephrosis had developed. The CEA was 4.3 ng/ml and CA19-9 was 42.5 μg/ml. A fourth exploratory procedure was performed. The exploration allowed visualization of the parietal peritoneum, liver, small bowel and large bowel. Disease progression was limited to the bladder with disease extension to the rectum and vagina. The bladder was excised and an ileal conduit was created. The old colorectal anastomosis was resected back to the junction of sigmoid and descending colon and an end sigmoid colostomy created. The procedure required 10 h.In June 2015, the CEA had increased to 6.4 ng/ml and CA19-9 to 51.7 μg/ml. Enteric material was determined to be exiting from the vagina and CT showed a colovaginal fistula. The fistula was attributed to wide field pelvic radiation combined with extensive surgery. The colon in the pelvis which contained the fistula was excised and a more proximal colostomy created. Following recovery from this complication the patient gained weight and enjoyed a normal nutrition. Despite the colostomy and urostomy appliances, her activity level was satisfactory. She did not have a chronic pain syndrome.The clinical and pathologic features for this patient are shown in 3.2In January 1992, a 53 year old man noted increasing abdominal girth and constipation for several months. The CEA was 28.8 ng/ml and CA19-9 was 143 μg/ml. The CA125 was 30.8 μg/ml. CT showed ascites and omental thickening. Paracentesis produced mucoid fluid and cells consistent with mucinous carcinoma. Colonoscopy was normal and barium enema showed a filling defect in the appendix.In February 1992, with a diagnosis of pseudomyxoma peritonei the patient had a cytoreductive surgery. The procedure required 16 h. There was a greater and lesser omentectomy with splenectomy, peritoneal stripping of right and left hemidiaphragm and pelvis, right colon and rectosigmoid colon resections with anastomosis and distal gastrectomy with gastrojejunostomy. Tubes and drains were placed for early postoperative intraperitoneal chemotherapy (EPIC) . All speThe patient recovered well. He received 6 cycles of combined intraperitoneal 5-fluorouracil and systemic mitomycin C .In September 2008, CT showed approximately 8 tumor nodules within the small bowel regions and a confluence of tumor in the pelvis. The CEA was 6.6 ng/ml and CA19-9 was 39.3 μg/ml. The CA125 was 4.4 μg/ml. The patient required resection of tumor nodules from the posterior aspect of the stomach, resection of numerous tumor nodules from the small bowel, total proctocolectomy with end ileostomy and total cystectomy with an ileal conduit. Heated intraoperative intraperitoneal chemotherapy (HIPEC) was used.The clinical and pathologic features for this patient are shown in Hospital stay was 18 days. Rehabilitation was complete. The patient has no evidence of disease as of February 2019. He is now 27 years from his initial diagnosis and disease-free with an excellent quality of life. He maintains his urostomy and ileostomy on his own. His nutritional status remains normal. He does not have a pain syndrome and takes no pain medicine on a regular basis.4The decision to proceed with resection of both bladder and rectum  was not simple in either of these patients. In patient 1, the only palliative option for bladder recurrence with ureteral obstruction seem to be resection. This decision was reinforced by a negative exploration of the abdomen. It is possible, in retrospect that ureteral stents would have provided palliation. This exceptionally radical surgery did not result in a long-term disease-free survival. Also, a serious complication, probably as a result of extensive surgery that was performed on irradiated tissues, occurred 28 months later. This was a colovaginal fistula.The second patient recurred with a long free interval of 16 years. At exploration several sites of disease were documented outside of the pelvis. These were removed and treated with HIPEC with no recurrence . The decIn patient 1, the HIPEC plus EPIC did not control microscopic residual disease at the time of the first cytoreduction. Perioperative chemotherapy was not used in subsequent cytoreductive procedures. Perhaps a greater effort with perioperative chemotherapy should have occurred in an attempt to preserve the visible complete repeat cytoreduction.Appendiceal mucinous neoplasms may metastasize extensively to parietal peritoneal surfaces and the greater omentum. Origin of a malignancy in the right lower quadrant (appendix) is an unusual anatomic site for an extensive resection within the pelvis. Usually, the indication for this surgery is a pelvic malignancy such as cervical cancer , rectal The peritoneal metastases do accumulate and then progress within the pelvis in large volume with mucinous appendiceal neoplasms. With a large extent of cancer resected by the original cytoreduction and despite HIPEC plus EPIC, recurrence in the pelvis was seen. In both patients the pelvis was visibly free of tumor at the completion of the first cytoreductive surgery. The progression of gross disease at this site indicates that the mucinous tumor was not sufficiently responsive to the hyperthermic intraperitoneal chemotherapy. Alternatively, the distribution of HIPEC and EPIC was such that insufficient contact of chemotherapy solution and residual microscopic disease within the pelvis occurred. A major shortcoming of the intraperitoneal chemotherapy in both of these patients is the single application of the chemotherapy treatment. Recent attempts to repeat the intraperitoneal chemotherapy treatments by drug instillation in a large volume of fluid directly into the peritoneal space have been published ,14. PlanPerhaps the most important observations from this study regards the clinical, radiologic, pathologic and surgical features of these patients that led to total pelvic exenteration surgery. These two patients were both a performance status one and thought to be fit for a major surgery plus perioperative chemotherapy. Radiologic study by CT with both oral and intravenous contrast showed an absence of concerning radiologic features . There wThe surgical exploration must be complete if total pelvic exenteration is being considered. Unresectable disease in the abdomen would be a contraindication to such an extensive resection. To perform total pelvic exenteration and have residual disease at another site would defy established surgical principles. In patient 1, complete exploration revealed no disease outside of the pelvis. In patient 2, there were tumors resected from small bowel surfaces and from the posterior aspect of the stomach. Tumor on the bladder, colon and rectum was definitively resected by total proctocolectomy plus total colectomy.Finally, the mucinous adenocarcinoma, as expected, did not metastasize out of the abdominal space to distant lymph nodes, to liver or to systemic sites. The positive lymph nodes present in the right colon specimen in patient 1 was a warning that the disease may have an invasive capability difficult, perhaps impossible to eradicate by cytoreductive surgery and single cycle of perioperative chemotherapy with hyperthermic mitomycin C intraoperatively and normothermic intraperitoneal 5-fluorouracil immediately postoperatively. Multiple cycles of intraperitoneal chemotherapy may have been beneficial in both of these patients.Foundation for Applied Research in Gastrointestinal Oncology.Secretarial support and data management support funded by Local IRB-approval for this case report was not required:does not meet the DHHS definition of research (45 CFR 46.102(d)(pre-2018)/45 CFR 46.102(l)(1/19/2017)) or the FDA definition of clinical investigation (21 CFR 46.102(c)) and therefore are not subject to IRB review requirements and do not require IRB approval.MedStar Health Institutional Review Board has determined that a case report of less than three (3) patients This case report is only of 2 patients.Written and signed consents were obtained from the patients.Paul H. Sugarbaker, MD: study concept or design, data collection, data analysis or interpretation, writing the paper.www.researchregistry.com website with UIN 4834.This case report is registered as a case series on the Paul H. Sugarbaker, MD.Not commissioned, externally peer-reviewed.The author has no conflicts of interest to declare.


Liver metastasis of an anal squamous cell carcinoma (SCC) with a pseudocyst is uncommon. There are many diseases which form hepatic cystic lesions and sometimes it is difficult to arrive at an accurate diagnosis.A 69-year-old woman visited our hospital with complaints of bloody stool and difficult defecation. A palpable mass in the anal canal was found on digital examination. The laboratory tests revealed anemia and raised levels of SCC antigen. On endoscopy a type 2 tumor was identified in the anal canal and biopsy revealed SCC. The patient was diagnosed with advanced anal SCC  as a result of the examination and underwent chemoradiotherapy (CRT); unfortunately the tumor persisted after CRT. A salvage abdominoperineal resection with D2 and left lateral lymph node dissection was conducted. Histopathologically, the tumor was diagnosed as poorly differentiated SCC. She was readmitted for fever 3 weeks after surgery, and the abdominal CT showed multiple low density areas with enhancement at the edge of the liver, suggesting abscess or metastasis. The metastatic SCC was revealed by cytology of liver drainage and liver biopsy.Anal cancers are sometimes encountered however; a pseudo cystic presentation of a liver metastasis is very rare which makes it difficult to distinguish from other diseases.We present a case of SCC in the anal canal with liver metastasis presenting as a hepatic pseudocyst. The lunMetastatic SCC in the liver presenting as a hepatic cyst is extremely rare. We present a case of liver metastasis from a SCC in the anal canal with a formed pseudocyst. This work has been reported according to the SCARE criteria .2A 69-year-old woman was admitted to the Department of Surgery at our hospital for complaints of bloody stool and difficult defecation. She also had hypothyroidism, Sjogren’s syndrome, and uterine myoma. She was a non-smoker, did not drink alcohol, and had no history of surgery. A palpable mass was found in the anal canal on digital examination. The laboratory tests showed mild anemia, elevated levels of SCC antigen, and normal levels of serum carcinoembryonic antigen and carbohydrate antigen 19-9. On endoscopy, a type 2 lesion was found in the anal canal , and on The patient was readmitted 8 days after discharge for fever, bilateral pedal edema, and perineal pain. White blood cell count and C-reactive protein levels were elevated. An abdominal contrast-enhanced CT showed low density areas in segment IV, V, and VII of the liver with enhancement at the edges. Multiple liver abscesses were initially suspected due to fever and elevation of inflammatory marker levels; however, whole body CT revealed melting of vertebral bodies, multiple lung nodule, and enlargement of the right inguinal lymph node a–e, whic3Hepatic cysts are often encountered in clinical practice, occurring in 2.5% of the general population , howeverBorhani et al.  developeThe reasons for the hepatic cystic changes were unclear. The pathological findings of this case showed poorly differentiated SCC and massive venous invasion; recurrence after surgery was extremely early and the disease progression was very quick. We theorize that the poor pathological findings led to rapid tissue necrosis, which was revealed as a liver pseudocyst on the abdominal CT with poor response to chemotherapy.We could not find any previous reports on liver metastasis from anal SCC forming a pseudocyst, and to the best of our knowledge, this is the first reported case.4We identified a rare case of liver metastasis from an anal squamous cell carcinoma with a formed pseudocyst. It is important arrive at a comprehensive diagnosis from the physical examination, imaging findings and detailed clinical history of patients.This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.E-477-3.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.All authors in this manuscript contributed to the interpretation of data, and drafting and writing of this manuscript. Tomoaki Bekki is first author of this paper. Yuji Takakura is corresponding author of this paper. Tomoaki Bekki, Yuji Takakura, Masatoshi Kochi and Hiroyuki Egi conceived and designed the study and drafted the manuscript. Tomoaki Bekki, Yuji Takakura, Masatoshi Kochi, Yoshihumi Teraoka and Hiroyuki Egi were engaged in patient’s care in our hospital including surgery. Kei Kushitani, Keiichi Mori and Koji Arihiro diagnosed SCC.Hideki Ohdan contributed to study concept, and review of the final manuscript and submission of the paper. All the authors read and approved the final manuscriptThe manuscript does not report the result of an experimental investigation or research on human subjects.Yuji Takakura.Not commissioned, externally peer-reviewed.None of the authors have any commercial or financial involvement in connection with this study that represents or appears to represent any conflicts of interest.


Mesenchymal hamartomas of the chest wall are unusual tumours diagnosed in neonates. They mostly resolve spontaneously hence conservative management has been advocated. Some compress vital structures in the thoracic cavity or bleed warranting surgical intervention. We present a neonate with mesenchymal hamartoma of the chest wall presenting as unilateral multifocal lesions with life threatening complications. He responded well to surgical intervention and was successfully discharged. They may present as innocuous mass lesions on the chest wall or with fatal compromise of intrathoracic structures.The male infant was born following spontaneous preterm labour at 34+3 weeks gestation with a birth weight of 2.35 kg. This was the mother’s first pregnancy and it had been uneventful with normal antenatal scans and protective serology. The only background of note is a maternal history of Haemophilia C. The baby had negative genetic screening and normal factor XI level. He was intubated soon after birth due to respiratory distress, and although he was extubated within 24 h, he was reintubated a few days later following an acute respiratory deterioration preceded by escalating continuous positive airway pressure and oxygen requirements.T1 weighted images with layering and fluid–fluid levels indicating internal haemorrhage. The lesion size was 4 cm × 6.5 cm × 7.3 cm displacing the heart to the right, almost contacting the chest wall. There was a significant mass effect with deviation of the trachea approximately 2.5 cm to the right with severe compression, particularly at the level of the carina and involving the bronchi. The left second to fifth ribs were abnormal and inseparable from the mass. Further lesions were noted on left 10th and 11th ribs posteriorly and left 3rd and 4th ribs anteriorly. There were concerns about the possibilities of tracheobronchomalacia and left lung hypoplasia. An echocardiogram showed a structurally normal heart but with severe dextroposition and compressed left ventricle.Shortly after birth he was noted to have an abnormal bony prominence of his chest wall. Chest radiograph  showed aThe baby was intubated, ventilated and monitored in the neonatal intensive care unit. Episodes of bradycardia and desaturation were noted when his head was turned, likely due to dynamic airway compression. Two of these episodes required cardiopulmonary resuscitation for less than a minute each time. Ultrasound-guided biopsy with aspiration of the mass and drainage of pleural effusion did not improve his symptoms. The parents were counselled that surgery entailed a high risk of catastrophic bleeding and mortality, and risk of ongoing respiratory distress with unknown development of the underlying lungs which could be hypoplastic. He underwent surgical excision of the mass along with two ribs, followed by chest wall reconstruction. The second small benign mass at 10th and 11th ribs was left behind. Histopathology of excised lesion confirmed the diagnosis as chest wall MH . He reco3 Diagnosis is usually made at birth but may be antenatal,4 or in later childhood, with one adult diagnosed and operated at 60 years of age. Clinically presenting as mass lesions on the chest wall with or without respiratory symptoms, they are not true neoplasms. These self-limiting tumours grow in early infancy and then regress after a plateau phase. Involution subsequently follows due to vascular compromise of the lesion. This natural course supports conservative management in many children, where the pressure effects are not life-threatening.3 Although the lesion is benign it can enlarge progressively and cause significant destruction to surrounding structures causing distortion and displacement of airways, lungs, heart, major vessels or erode ribs, as in this case. Radiologically, they present typically as a large expansile solid to cystic intrathoracic soft tissue mass arising from central position of ribs with destruction of adjacent ribs and secondary changes evident in other ribs and vertebrae. In our experience rib involvement and expansion, to a lesser degree of ipsilateral ribs remote from the major mass lesion as in this case, is commonly seen.MH of the chest wall is a rare entity, occurring with an incidence of one in a million.5 Histopathologically, it is characteristically a solitary well-circumscribed lesion arising from ribs with cystic areas composed of mucoid and bloody contents and solid areas comprising cartilage, collagen and bone.6 Histology is helpful to rule out Ewing’s sarcoma or peripheral neuroectodermal tumour which mimic MH in presentation. Surgical resection is offered in children with life-threatening symptoms. Surgery can be partial resection, with good recovery or a total resection of involved chest wall. Radiofrequency thermoablation has been known to offer benefit when surgery might be too risky or radical.7A unilateral multifocal mass lesion, as seen in this child, has been described in only five children in the literature to the best of our knowledge.MHs are rare chest wall tumours usually diagnosed by clinical and radiological manifestations and confirmed histopathologically. Considering the benign self-limiting nature of the lesion a conservative approach is generally adopted. However, in life-threatening situations with compression of vital intrathoracic structures, surgery becomes necessary and was live-saving in this child.Mesenchymal hamartomas of the chest wall in neonates can cause life-threatening clinical features secondary to pressure effects on vital intrathoracic structures.Imaging by MRI/CT provides valuable diagnostic information in mesenchymal hamartomas of the chest wallUnilateral multifocal lesions are uncommon but do occur.Surgery is life-saving when these tumours cause life-threatening features.