Giant vascular eccrine spiradenoma is a rare variant of eccrine spiradenoma. It is different from the eccrine spiradenoma in its larger size and greater degree of vascularity. It is often clinically confusedwith a vascular or malignant tumor.Here, we report a case of a 67-year-old man who presented with a tumor in the scrotum scrotal region for 6 years. The tumor had begun as a small nodule and had grown slowly with over time. Surgical excision and pathological examination revealed that the lesion was the a giant vascular eccrine spiradenoma.This study reports the first case of giant vascular eccrine spiradenoma in the scrotum. Eccrine spiradenoma, which was first described in detail by Kersting and Helwig in 1956, is an uncommon benign skin adnexal tumor originating from sweat glands .Giant vascular eccrine spiradenoma is a rare highly vascular variant of eccrine spiradenoma, that might be mistaken for a vascular lesion or malignant tumor due to its florid vascularity and hemorrhagic features. The gross morphology of the tumor is not distincttherefore, the clinical diagnosis of giant vascular eccrine spiradenoma is very difficult, and biopsy is required for an accurate diagnosis.Our patient was a 67-year-old male who presented with a solitary mass in the scrotum. The lesion began as a small nodule that slowly increased in size over the past 6 years. The patient had no family history or traumatic history. He complained of no symptoms other than mild tenderness. Ultrasonography indicated a well-circumscribed oval mass in the dermis of the scrotum. Urological and dermatological examination revealed a single tender, skin-colored, firm dermis mass of the scrotum. The lesion was surgically excised and sent for histopathological examination.2 cm in size(Fig. Gross examination of the resected specimen showed that the skin surface of the scrotum was not different from other areas. The mass, which was located in the dermis of the scrotum, was approximately 3.2 × 2.3sizeFig. a. The cusizeFig. b.Fig. 1Histopathologic examination showed several sharply defined basophilic tumor nodules in the dermis, extending into the subcutis without any connection to the overlying epidermis. The overlying epidermis was almost intact and the surrounding connective tissue was normal. The tumor nodules comprising round-to-oval or basaloid cells arranged in acinar, sheet, cord-like, and trabecular patterns, were surrounded by a thin layer of fibrous tissue. At high magnification, three types of cells were observed in the nodules Fig. a. The fiImmunohistochemically, the large epithelial cells were strongly positive for CK, CK7 and CK8 Fig. aand negaThus, the diagnosis of giant vascular eccrine spiradenoma was supported by the histologic features and immunohistochemical findings.Eccrine spiradenoma is a benign adnexal tumor that usually appears as a small, solitary, painful and gray -to-pink nodule and occurs equally in both males and females. Multiple eccrine spiradenoma is an extremely rare presentation comprising less than 2 % of all cases . MultiplLauret et al.  first inFollowing the two cases mentioned earlier, 12 cases of giant vascular eccrine spiradenoma have been reported in the literature. The summarization of the previously reported cases was described in Table Most reported giant vascular eccrine spiradenomas appeared as deep dermis or subcutaneous solitary tumors, the surface of which showed red, gray, purple, blue, or pink color. The skin over the tumor can be smooth, ulcerated or bleeding. Yamakoshi et al.  reportedThe composition and differentiation of giant vascular eccrine spiradenoma are stilldebated. It is generally believed that the tumors are composed of two types of cells, large pale cells situated in the center and small basaloid cells located in the periphery , 13. HowThe considerable degree of vascularization is an unusual feature of giant vascular eccrine spiradenoma. At present there are two explanations for the marked vascularity. One is that giant vascular eccrine spiradenoma arises from a highly vascular region of the normal sweat gland , 15. SomThe differential diagnosis of giant vascular eccrine spiradenoma isvery important, and a variety of clinical diagnoses, including chronic expanding hematoma , calcifyAlthough malignant eccrine spiradenomas have been previously reported , 33, no According to previous literature and our case, we summarized the clinical and pathological features of giant vascular eccrine spiradenoma as follows. Age: Most patients were older than 49 years, but one caseeach occurred in men aged 41 and 31. Location: All reported cases were located on the trunk or limbs except one on the scalp, and our case occurred in the scrotum. Gross appearance: The lesions presented as well-defined solitary mass larger than 2 cm in diameter and exhibited spontaneous hemorrhage and may be painful. Histological findings: Tumors usually contain three types of cells and dense dilatedvasculature.Our patient showed no recurrence one year after the operation. It is crucial to take giant vascular eccrine spiradenoma into consideration when diagnosing scrotum subcutaneous lesions. The principle of treatment for giant vascular eccrine spiradenoma is complete surgical excision with clear margins. Postoperative follow-up is important to detect recurrenceIn summary, we have reported the first case of giant vascular eccrine spiradenoma in the scrotum. ITshould be taken into account in the differential diagnosis of scrotal tumors.


Tarsometatarsal coalitions have rarely been reported in published literature. The few reported cases presented with varying degrees of pain. Here, we describe the case of a 16-year-old female with multiple tarsometatarsal coalitions, the first of its kind in the reported literature. Descriptions in anthropology literature suggest that these lesions might be more common than previously thought and, in some circumstances, can become symptomatic enough to warrant intervention. Tarsometatarsal coalitions are rare in clinical practice, and not many cases have been reported in the published literature -5. The oTo our knowledge, our report is the only such case published in the orthopedic literature. We discuss the clinical relevance of this finding and the similarities with existing orthopedic and anthropology literature.A 16-year-old female presented to our outpatient clinic with complaints of pain on the outer aspect of her right foot for the past few weeks. She had suffered a twisting injury while walking downstairs and had been symptomatic since.Our examination revealed a normal foot, comparable to the left side, and no signs of instability of either the ankle or foot. There were no signs of peroneal spasticity, and her arches were normal. There was no significant orthopedic or medical history.Radiographs of her right foot were obtained, which revealed a coalition of the base of the fourth and fifth metatarsals with the cuboid bone, as well as a coalition of the third metatarsal with the lateral cuneiform Figure . X-rays A CT scan was subsequently performed to rule out more significant subtalar/calcaneonavicular coalition(s). The CT scan revealed the X-ray findings in greater detail; and confirmed another coalition between the second metatarsal and the intermediate cuneiform Figure .The patient was managed conservatively by a short course of non-steroidal anti-inflammatory drugs and shoe wear modification. She had been using a pair of slippers (common in the Indian subcontinent) for most of her long walks every day. Switching from slippers to low-heeled, well-fitting sports shoes considerably improved her symptoms in a few weeks. Because she showed a favorable response to treatment at her three-month follow-up, further interventions were deemed unnecessary at this stage.Tarsometatarsal coalitions have seldom been described in the orthopedic literature. The handful of reports published so far have described different variations of tarsometatarsal coalitions involving one of the cuneiforms -4, with A published report by Kobayashi et al.  describeFujishiro et al.  proposedThese osseous and non-osseous coalitions may be much more common than previously thought, although clinical presentation with a symptomatic foot is uncommon . Indeed,This description of articular surface defects in these tarsometatarsal coalitions bears a striking resemblance to the radiological picture of our patient. Her CT offered evidence of a similar morphology of tarsometatarsal coalition, as described by Regan et al. The cuboid and the fifth metatarsal were curved toward one another, and the tarsometatarsal coalitions were predominantly on the plantar side of the joints, just as they have been found in skeletal remains studied by anthropologists Figure .The frequent finding of such lesions in anthropology literature, as opposed to their rarity in clinical practice, suggests that most of them might be insignificant and probably do not alter the biomechanics of the foot, unlike the well-described calcaneonavicular, and talocalcaneal coalitions -10.To our knowledge, ours is the only case described in the literature with coalitions across four tarsometatarsal joints . The fifth metatarsal-cuboid coalition was not completely ossified and was a non-osseous coalition. The other three coalitions were osseous, as confirmed by CT scan images. Despite the non-osseous nature of one of the coalitions, our patient responded to conservative treatment and was satisfied at her three-month visit. However, as our patient is young (16 years old), a long-term follow-up would be needed to ascertain if the non-osseous coalition leads to secondary osteoarthritis of the fifth metatarsal-cuboid joint. Furthermore, as the tarsal coalition has been described as a non-metric trait , we had Tarsometatarsal coalitions have sporadically sprung up in the orthopedic literature over the past three decades, with all authors recognizing the rarity of its incidence in clinical practice. However, based on descriptions by anthropologists in the past, tarsometatarsal coalitions might be more common than previously thought. Its rare appearance in clinical practice points to the possibility that most of these lesions might be asymptomatic and inconsequential to normal foot biomechanics.While the possibility of an orthopedist facing a symptomatic tarsometatarsal coalition is rare in routine practice, it would be helpful to be aware of such lesions when confronted with an innocuous midfoot sprain unresponsive to conservative treatment. Persistent pain from a non-osseous coalition often resolves following arthrodesis of the involved joint.


Mucinous adenocarcinoma of the appendix is a rare neoplasm with a low propensity for lymph node metastasis. The present case refers to an appendicular mucinous adenocarcinoma with inguinal lymph node metastasis. A 71-year-old woman underwent an appendectomy due to a clinical presentation of acute appendicitis. However, the histological examination of the surgical specimen revealed a mucinous adenocarcinoma of the appendix. After staging, the patient underwent a right hemicolectomy and was proposed for adjuvant chemotherapy. At the 3rd year of follow-up, inguinal lymphadenopathy was diagnosed, which biopsy confirmed inguinal node metastases from primary colorectal cancer, with areas of extracellular mucin. Restaging revealed liver and peritoneal metastasis, and the patient was proposed for palliative chemotherapy. Appendicular neoplasms, due to their rarity, represent a diagnostic and therapeutic challenge. This clinical case depicts an unusual metastasis pathway for an unusual neoplasm. Appendicular lesions are divided into neoplastic and non-neoplastic.Appendicular neoplasms are rare, with an incidence of 0.12 per million people per year.The classification of appendix adenocarcinomas into mucinous type is given by extracellular mucin in more than 50% of the lesion in a microscopic section.The median age at diagnosis of MACA is 59 years, with a slightly higher incidence in women (55%). At diagnosis, one-third of these tumors are staged as T3, half as T4 and M1, and the majority (80%) are N0.A 71-year-old woman with a history of total hysterectomy and bilateral salpingo-oophorectomy by adenomyosis with benign final pathology, 20 years ago, came to the Emergency Department for abdominal pain in the lower quadrants, of sudden onset, associated with anorexia and nausea over the last 36 hours. On the physical examination, she had hemodynamic stability, apyrexia, and abdominal right lower quadrant pain without signs of peritoneal irritation. Analytically, she had increased inflammatory parameters, and ultrasound revealed uncomplicated acute appendicitis .The patient underwent an uneventful laparoscopic appendectomy. Pathological examination revealed well-differentiated invasive mucinous adenocarcinoma of the appendicular middle third, 2 cm in length, with subserosa invasion, without appendicular wall rupture, with overlapping evidence of acute appendicitis and appendicular mucosa of the proximal top with low-grade dysplasia lesions .Further staging by computerized tomography (CT) failed to identify metastatic disease. The carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA 19-9) were normal. No other lesions were identified in the lower digestive endoscopy (LDE); thus, the patient was staged as pT3cN0M0 and underwent laparoscopic radical right hemicolectomy. Histological examination revealed lymph node substage (12 lymph nodes) without signs of malignancy, and adjuvant chemotherapy (QT) with capecitabine was proposed.In the third year of follow-up, the patient presented a right inguinal swelling. The ultrasound depicted two suspicious lymph nodes . The truAs in the present case, the adenocarcinoma of the appendix is usually masked by the picture of acute appendicitis.Diagnosis and staging overlap with the other colic neoplasms. Tumor markers CEA and CA 19-9 are elevated in most advanced mucinous tumors,ad initium. In elective surgery, diagnostic appendectomy is indicated for mucinous lesions whenever the preoperative neoplastic diagnosis is not available. In situations with preoperative confirmation, right hemicolectomy should be performed in localized disease, coupled with cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (HIPEC) in metastatic disease. In cases of contained rupture, right hemicolectomy should be the norm, and in free rupture, this should be associated with peritoneal lavage and peritoneal biopsies of suspect lesions.The treatment of appendix adenocarcinoma depends largely on the diagnostic timing  or the surgical regimen (urgent or elective). In an emergency context, the gold standard is therapeutic appendectomy for patients with a clinical picture of acute appendicitis. Given the suspicion of intraoperative neoplasia, the extent of surgery should be dictated by the ability to obtain macroscopic free margins, ranging from resection of the caecum to ileocolectomy, or right hemicolectomy. If peritoneal disease is suspected, cytoreductive surgery is not indicated Well-differentiated MACA is associated with a low risk of lymph node metastasis , and there seems to be no significant difference in the survival of patients undergoing appendectomy or radical right hemicolectomy.The role of radiotherapy and adjuvant chemotherapy is not established. Extrapolating from the efficacy of adjuvant chemotherapy with fluorouracil (FU) and oxaliplatin for N+ colon adenocarcinoma cases, adjuvant chemotherapy appears to be a valid option in adenocarcinomas of the appendix with lymph node invasion.In the presented case, it is hard to say if the inguinal lymph node metastasis were an extension of the peritoneal involvement or if they already existed at the time of the appendectomy. Initially, the patient had only inguinal lymph node involvement, and the findings suggestive of peritoneal carcinomatosis, which was not confirmed, was related to a TC performed 5 months after the diagnosis of inguinal involvement.Appendicular neoplasms, due to their rarity, represent a diagnostic and therapeutic challenge. This clinical case depicts an unusual metastasis pathway for an unusual neoplasm.


A splenic lesion often represents a diagnostic challenge due to relative scarcity and the broad differential diagnosis. Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen is usually encountered only incidentally. Although benign, patients with SANT often receive splenectomy, due to its rarity, diagnostic uncertainty and sometimes intimidating imaging morphology and size. Imaging features on computed tomography, magnetic resonance and positron emission tomography have a high diagnostic value for SANT and help differentiate this entity from other splenic lesions. When the imaging parameters are matched with core needle biopsy tissue analysis, further watchful waiting can be recommended in order to avoid splenectomy. Sclerosing angiomatoid nodular transformation (SANT) is a rare benign, proliferative vascular lesion affecting the spleen with approximately 170 cases described in scientific literature. SANT is first described as a distinct pathology by Martel et al. in 2004 [Table 1. The time window of the reviewed literature ranges from 2004 until 2019 [1A systematic search was performed in the database Medline, using PubMed as search engine. The following search terms were used: (Sclerosing angiomatoid nodular transformation) OR (Splenic lesions) OR (splenic tumor) OR (Splenectomy). After the initial search we selected publications by title, subsequently by abstract. If the article did not contain enough imaging information to implement in this review, it was not included in the analysis. Ultimately 33 articles were considered eligible in this review in addition to our own case, containing 58 cases describing the imaging features of SANT, summarized in il 2019 1.SANT is an infrequent benign vascular lesion and often an incidental finding. There is a slight female predominance. The mean age in the study population was 44 years, correlating well with other, not included reviews 3. SANT iThe etiology of SANT remains unknown. In the literature there are several hypotheses. For instance, Martel et al. suggest a non-neoplastic stromal proliferative process in splenic red pulp tissue with accompanied nodular followed by vascular transformation . Weinreb5SANT can be distinguished from other vascular lesions of the spleen based on its distinct histologic findings and immunohistologic features. The most characteristic microscopic findings of SANT are multiple angiomatoid nodules separated by collagenous bundles . ImmunohNo specific symptoms are associated with SANT. Up to 62,3% of the cases, patients were asymptomatic. A minority of 13.5% of patients report non-specific abdominal pain or discomfort in the left upper quadrant. Only one report describes a patient with life-threatening intra-abdominal hemorrhage and severe abdominal distention, requiring urgent splenectomy .Table 1. Across the 33 studies the patients either underwent ultrasound, computed tomography (CT), magnetic resonance imaging (MRI) and/or fluoro-deoxyglucose-positron emission tomography (FDG-PET) investigations. Most articles contain imaging information on CT, where SANT presents as a hypodense solitary splenic mass in 94% (45/48) of cases and 93% (27/29) report peripheral enhancement and progressive filling in on the delayed phase . Some articles describe the enhancement as a ‘spoke-wheel’ pattern, corresponding to a central, stellate fibrous stroma with fibrous septa separating angiomatoid nodules [3910121314Fig. 2A). On T2-WI, 66% (21/33) of articles report abundantly low signal intensities . This is most likely due to hemosiderin deposition [Fig. 2C–D) [91015161819208101722Imaging findings of histologically proven SANT are summarized in  94% 45/4 of casesules 391012131415.position . The ste 94% 45/4 of cases 94% 45/4 of cases) 910151618192021.The sonographic characteristics of SANT was only mentioned in 14 articles. The most common presentation is a solitary heterogeneously hypoechoic mass 924252627.242526112526Fig. 3). Fourteen authors performed FDG-PET in the work-up. Only a moderate uptake was noted with a median maximal standardized uptake value (SUVmax) of 2.8 MBq/g (range: 2.0–4.7 MBq/g), as opposed to malignant tumors with higher average values such as lymphoma that has a median SUVmax of 6.9MBq/g [SANT cannot be distinguished from other benign splenic tumors based on FDG-PET findings alone, but it can be a helpful tool to differentiate between certain malignant tumors such as lymphoma, metastases and angiosarcoma 2930. In 296.9MBq/g .Primary splenic masses can be categorized into those of lymphoid and non-lymphoid origin . LymphoiLymphoma is the most common malignancy involving the spleen. Typical findings, not present in SANT, include splenomegaly, intra-abdominal lymphadenopathy and high FDG avidity [ avidity . On MRI  avidity .Hemangioma, or more correctly termed venous malformation, is the most common benign splenic lesion and may resemble SANT. Prominent hyperintensity on T2-WI and multiplicity may be helpful in the differentiating this lesion from SANT [rom SANT .metastatic disease explained by the parenchyma’s natural ability to resist metastases [Although richly vascularized, the spleen is an uncommon site for tastases . The mostastases .Angiosarcoma is an aggressive tumor arising from splenic endothelial cells with very poor prognosis, as distant metastases are usually present at the time of initial diagnosis [iagnosis . Spleniciagnosis .Littoral cell angioma (LCA) is a rare benign vascular tumor that may mimic SANT [mic SANT . Howevermic SANT 36.hamartoma is an uncommon non-neoplastic lesion composed of an abnormal mixture of white and red pulp. One key feature of a splenic hamartoma is its faint heterogeneous enhancement on immediate post-contrast images, with relatively uniform and intense enhancement on delayed images [Splenic d images 39. In add images .Extramedullary hematopoiesis (EMH) is defined as the production of blood cells outside of the bone marrow. The most common causes of EMH are myelofibrosis, diffuse osseous metastatic disease replacing the bone marrow, leukemia, sickle cell disease, and thalassemia [lassemia . EMH of lassemia 41.In 98% of reviewed cases the patient with SANT underwent splenectomy. The main reason for surgical resection is diagnostic uncertainty on imaging studies. However, it should be noted that the spleen is an lymphoid organ, with an important role for general immunity and splenectomy is not an innocent intervention . Intra- Moreover, in-depth splenic immune function studies suggesting unnecessary splenectomy for benign lesions leads to the loss of an important immunologic organ are widely accepted .Of the reviewed cases, only Gutzeit et al. and our own case obtained the final diagnosis of SANT through image guided core needle biopsy (CNB) .US or CT can both be used for image guidance and the choice is often related to the experience of the interventional radiologist. The advantages of US guided biopsy are the real-time needle visualization, the shorter time needed to reach the target, low cost and no radiation exposure for patient or radiologist. Color doppler sonography aids the radiologist in avoiding any large vessels and plotting a safe trajectory to the lesion. Usually three biopsies are performed, taken from the center and periphery of the lesion to assure enough tissue samples to acquire an adequate pathological diagnosis. Possible disadvantages include impaired visualization of deep lesions of the spleen caused by interposition of intestinal gases, lung parenchyma and overlying ribs. CT guided biopsy of the spleen has several benefits over the ultrasound-guided procedure: the ability to visualize all the abdominal organs and viscera; less risk of bowel transgression and better characterization of deep-seated lesions, collections and retroperitoneal structures 4748.47A common misconception amongst clinicians might be the possibility of a significant complication associated with splenic biopsy, due to the high vascular nature of the spleen. However a meta-analysis found the major complication rate was 1.3% for all splenic biopsies for fine needle aspiration cytology (FNAC) and CNB and to 1.9% for CNB alone, when biopsy needles of 18-gauge or smaller are used . The comImage-guided percutaneous biopsy of the spleen performs well and yields high overall diagnostic accuracy. For all biopsies, sensitivity was 87% and specificity was 96.4% . There aAbsolute contraindications to splenic biopsy are similar to those of any other solid abdominal organ biopsy: significant coagulopathy; severe cardiopulmonary compromise; pregnancy; hemodynamic instability; lack of a safe biopsy pathway, and an uncooperative patient .The imaging findings of SANT on ultrasound and CT are rather unspecific. The diagnosis of SANT can be suggested on MRI when encountering a lobulated lesion with smooth margins, which is iso- to hypointense on T1-WI and abundantly low on T2-WI compared to splenic tissue, shows peripheral arterial enhancement with delayed filling in or ‘spoke-wheel’ enhancement pattern and only moderate/heterogeneous uptake on FDG-PET. These findings combined with the absence of malignant characteristics such as wash-out, infiltrative growth and adenopathies, make the diagnosis of SANT very likely. When these imaging parameters are matched with core needle biopsy tissue analysis, further watchful waiting can be recommended in order to avoid unnecessary splenectomy. CNB in experienced hands is safer than splenectomy, yields diagnostic material in the majority of cases and should be the next diagnostic step after imaging.Typical imaging features of SANT are hypointense signal on T1-WI and T2-WI with peripheral enhancement in the arterial phase with a progressive centripetal filling in the delayed phase, displaying a characteristic spoke-wheel-like pattern in almost half of cases.Important discriminators between SANT and malignant splenic lesions are the absence of washout, moderate/heterogenous uptake on FDG-PET, infiltrative growth and adenopathies.Core needle biopsy shows high diagnostic accuracy, is safer than splenectomy and should be the diagnostic intervention of choice for solid splenic lesions.The additonal files for this article can be found as follows:10.5334/jbsr.2689.s1Patient characteristics and imaging features of Sclerosing Angiomatoid nodular transformation.This table is a summary of all the data acquired of SANT since this entity was first described in 2004. We divided the table in study name, age of patient, sex, clinical features, Computed tomography features, Magnetic resonance imaging features, Positron emission tomography features, Ultrasound features and treatment.


Adult cases of retroperitoneal isolated enteric duplication cyst (IEDC) are rare, with only 17 case reports in the relevant literature. We herein present a case, which was characterized by changes in intra-cystic density on computed tomography (CT), which was safely resected by laparoscopic surgery.The patient was a 60-year-old male who received abdominal CT to investigate the cause of increased serum CA19-9 levels. CT revealed a unilocular cystic mass located in the lower right retroperitoneum. The size increased from 5 to 10 cm in three and a half years and the CT value decreased from 101 Hounsfield Units (HU) to 20 HU. We performed laparoscopic surgical resection, because the possibility that the enlargement of the lesion represented malignant transformation could not be denied. The large cystic mass firmly adhered to the appendix and its mesentery via the retroperitoneum, the appendix was resected en bloc with the cystic lesion. Microscopically, it had no communication with the appendix, and had an intestinal wall structure of muscularis mucosae and muscularis propria. The final pathological diagnosis was IEDC in the retroperitoneal space. There was no histological evidence of malignancy.When we encounter a retroperitoneal cystic lesion, we should consider the possibility of malignancy to determine the treatment strategy and perform a careful operation without breaking the cyst wall, irrespective of the preoperative diagnosis. Enteric duplication cyst (EDC) is a congenital anomaly that can be found anywhere along the alimentary tract –3. In alThe patient was a 60-year-old male who regularly visited a general practitioner for type 2 diabetes, who performed routine examination to rule out malignant disease. As his serum carbohydrate antigen 19-9 (CA 19-9) level was slightly elevated, abdominal CT was performed, which revealed a unilocular cystic mass of 8.0 cm in diameter, located in the lower right retroperitoneum. He was, therefore, referred to our hospital for surgical treatment.The patient had no relevant family history or past history of malignant disease in the abdomen or retroperitoneum. He was asymptomatic and in good health, with the exception of severe obesity with a body mass index of 40. An abdominal examination revealed no tenderness or palpable mass. Laboratory findings showed a high hemoglobin A1c level . The increased serum CA 19-9 levels, which was 43.3 U/mL in the previous hospital, had normalized to 16.9 U/mL at the time of the examination at our hospital. Ultrasonography identified a uniform hypoechoic cystic mass without septum and calcification in the retroperitoneum adjacent to the end of the ileum and appendix, which indicated a hyperechoic region with a debris-like appearance and no obvious nodular lesions or blood flow in the cyst. On enhanced CT, the retroperitoneal cystic mass appeared to be close to the appendix on the ventral side and to the right gonadal vessel on the medial side Fig. a–c. The Laparoscopic surgery was performed under general anesthesia. The operative procedure began with the insertion of a 1.2 cm umbilical port using Hasson’s technique. Four additional ports were then inserted under direct visualization Fig. a. The laThe resected specimens consisted of a unilocular cystic tumor of 10 cm in maximum diameter with a smooth inner surface and the appendix Fig. a, b. TheEDC is an uncommon congenital enteric malformation, the incidence rate of which is 1 in every 10,000 live births. Although most of patients with EDC are diagnosed in infancy or childhood, they can sometimes be seen in older patients . EDCs caIn 1961, Mellish and Koop reported that “enteric duplications” are defined histopathologically as spherical or tubular structures that possess a mucosal lining characteristic of 1 or more portions of the alimentary tract supported by muscular and serosal layers , pleuraln = 6) and non-malignant (n = 12) cases in Table To understand the clinical features of the disease, we searched the PubMed database cases of retroperitoneal IEDC. We identified 18 adult cases reports, including our case, between 1990 and 2020 and summarized these cases with the detailed clinical course (Table As described above, our case is noteworthy as we were able to observe the imaging changes of the lesion over time over a relatively long period. There were two characteristic changes. The first change was the decreasing CT value inside the cystic lesion. The second was the pattern of growth that occurred over time. Radiologically, intracystic hemorrhage can be distinguished from a high CT value of ≥ 100 HU. Fukuhara et al. reported a duodenal EDC complicated with intracystic hemorrhage. They reported that the presence of ectopic gastric mucosa could cause the development of complications, such as bleeding and peptide ulcer . AlthougThere are various differential diagnoses of solitary cystic lesions in the retroperitoneal cavity. Among them, non-epithelial tumors are the most common; these include cystic lymphangioma, bronchial cyst, cystic teratoma and cystic mesothelioma. Epithelial tumors include epidermoid cyst, mucinous cystadenoma and cystadenocarcinoma . ImagingWe reported a rare adult case of retroperitoneal isolated enteric duplication cyst, in which we could observe the changes of the image findings over time. It is difficult to obtain an accurate preoperative diagnosis of retroperitoneal cystic lesions. Therefore, we should consider the possibility of malignancy when determining the treatment strategy and perform a careful operation to avoid breaking the cyst wall, irrespective of the preoperative diagnosis.


This case describes the occurrence of basal cell carcinoma (BCC) and sebaceoma within a nevus sebaceous (NV), which has not yet been previously reported. This is significant to dermatologists as it emphasizes the importance of close monitoring of benign sebaceous nevi in the event that malignant transformation occurs, although such occurrences are rare. Prompt consideration for prophylactic excision of NS is warranted prior to malignant transformation.  Nevus sebaceous (NS) is a rare hamartoma with an incidence of 0.05%-1% diagnosed in childhood [A 65-year-old female presented to the surgery clinic with a six-week history of a pruritic, bleeding scalp mass in the left parietal-temporal region. The lesion had been asymptomatic since childhood but had grown over this six-week period. Examination of the scalp revealed a raised, fungated lesion measuring 4.7 x 4.0 cm with overlying alopecia secondary to the lesion Figure . The irr NS affect both surface epithelial and adnexal components of the skin and present as flat, hairless, and yellow-orange in color, with overlying alopecia . These cHRAS or KRAS mutations, confirming a strong correlation between activating RAS mutations and NS [While studies have been limited on NS and subsequent malignancy, a heterozygous mutation most often found in several independent NS tissues has been identified as either s and NS .It is uncommon for multiple tumor types to develop within a single NS, although multiple types of tumors can occur within NS lesions. BCC alone within NS has been noted in some cases predominantly affecting the trunk and scalp ,5. BCC aTreatment of NS typically consists of local excision secondary to the risk of malignant transformation or for cosmetic purposes . There iThis case discusses the malignant transformation of a nevus sebaceous. The patient presented to surgery with a fungated lesion that grew increasingly pruritic and friable over a six-week period. Excision and histologic examination of the lesion revealed basal cell carcinoma and sebaceoma embedded in the nevus sebaceous. This case is significant due to the rarity of sebaceomas and the presence of both BCC and sebaceoma within a single nevus sebaceous. Management of NS remains somewhat controversial as malignant transformation remains low risk. Some studies suggest conservative management through observation, while others prefer early excision. Excision of the lesions during childhood or adolescence is not required but may be beneficial as margins around the NS lesions can remain smaller than in the case of malignant transformation in adulthood. In the event of malignant change, although rare, more extensive procedures may be required, as seen in our case. This report serves as a reminder of the importance of close monitoring of NS and the recommendation of prophylactic excision of an evolving nevus sebaceous to yield optimal results.


Objectives: To investigate the clinical characteristics, treatment and prognosis of leiomyomatosis peritonealis disseminata (LPD) following laparoscopic surgery with uncontained morcellation and to summarize clinical features of iatrogenic LPD based on published literature together with our own experience.Methods: A cohort of 13 cases with iatrogenic LPD diagnosed and treated in Peking Union Medical College Hospital from 2011 to 2020 was reported focusing on clinical characteristics, treatment and prognosis.Results: All the patients had a history of laparoscopic myomectomy with uncontained morcellation. The average age was 35.6 (range 25–47) years. The interval between initial laparoscopic surgery and first diagnosis of LPD was 6.08 years on average (range 1–12). Most of the patients had no obvious symptoms. The accuracy of pre-operative diagnosis was low. Two patients had been treated with gonadotropin-releasing hormone agonist (GnRH-a) before surgery without obvious effect. The nodules of LPD are usually located in the lower half of the peritoneal cavity. The most commonly involved site was the pouch of Douglas. The number of nodules ranged from 3 to over 10, and they ranged in size ranged from 0.3 to 22 cm. All patients underwent surgical treatment: six patients underwent laparoscopy and seven underwent laparotomy. Pathology results confirmed LPD. The immunohistochemical profile indicated LPD tends to be positive strongly for desmin, caldesmon, ER, PR and SMA. Only one patient underwent post-operative treatment with GnRH-a. All patients were followed for an average period of 49 months without recurrence.Conclusion: Iatrogenic LPD is a relatively rare condition. Patients usually exhibit no hormonal stimulation factors. Surgery is the main method of treatment, and hormone suppressive therapy is only rarely used. The nodules are usually large and less numerous, and most involve the pelvis. The prognosis of iatrogenic LPD seems good. Leiomyomatosis peritonealis disseminata (LPD), also called disseminated peritoneal leiomyomatosis (DPL), is a rare gynecological disorder characterized by the dissemination of multiple smooth muscle nodules throughout the peritoneal surface. It was first described in 1952 by Wilson and Peale . IatrogeFrom January 2011 to May 2020, seventeen cases of LPD were identified in Peking Union Medical College Hospital. Thirteen cases were iatrogenic LPD while four cases were spontaneous LPD without operation history. Our study only focused on iatrogenic LPD. The clinical data collected from medical records were retrospectively analyzed and evaluated. SPSS 21.0 statistical software was used for analysis. The study was exempted from review by the Human Investigation Review Board (IRB).All patients were followed up regularly post-operatively with imaging. The plan of follow-up was that: once for 3 months for first year; once for 6 months for second year and once for 12 months for third year and afterward. Patients received gynecological ultrasound examination every 3 months and pelvic and abdominal MR image once a year.The clinical features of the 13 cases of LPD are summarized in Eleven patients initially sought medical treatment because of an incidental finding of pelvic mass or leiomyoma recurrence observed during routine examination. Case 4 had acute lower abdominal pain accompanied by fever while waiting for the scheduled surgery. Computed tomography and MR imaging indicated that there was a necrosis of nodules . After aThe average serum CA125 level was 36.77 U/ml . Six patients had an elevated level of CA125. Eight patients underwent MRI examination before surgery. Four of them indicated multiple nodules in the pelvic and abdominal cavity. In Case 13, MRI showed a nodule on the abdominal wall .The accuracy of pre-operative diagnosis was low. Four patients were suspected of malignant tumor before surgery while others were believed to have leiomyoma recurrence or broad ligament leiomyoma. Case 4 underwent CT-guided biopsy because of suspected malignancy. The pathological results of biopsy indicated leiomyoma.With a diagnosis of uterus leiomyoma recurrence at that time, Cases 9 and 13 were treated with gonadotropin-releasing hormone agonist (GnRH-a) before surgery to decrease the size of uterine myomas. Case 9 had a slight decrease of uterine myomas after 8 injections of Enantone. In Case 13, the size of one nodule that was considered a subserous myoma increased from 10 cm to 13 cm after 6 injections of Enantone.During the operation, multiple nodules were found distributed in the abdominal and pelvic cavities. The number of the nodules ranged from 3 to over 10 and the size ranged from 0.3 to 22 cm. Nodules were most commonly found in the pouch of Douglas (seven cases), followed by pelvic and abdominal peritoneum (six cases) , omentumOnly Case 9 showed coexisting ovarian endometriosis and the others had no evidence of pelvic endometriosis.All patients received individualized surgical treatments tailored to their age, symptoms, and desire for fertility. The main purpose of the operation was the complete removal of all nodules. The surgical procedures included the following: 1. Total abdominal hysterectomy and LPD nodule resection (Cases 1 and 8). 2. Abdominal LPD nodule resection . Case 2 underwent removal of both adnexa, omentum and right pelvic lymph nodes at the same time. Case 4 underwent repair because of the damage to the serosal surface of small intestine. 3. Laparoscopic removal of LPD nodules . Appendectomy was performed in Case 5 and bilateral adnexectomy in Case 12. 4. Laparoscopic removal of the uterus and LPD nodules (Case 6). 5. Laparoscopic myomectomy and removal of LPD nodules (Case 7). 6. Abdominal myomectomy and LPD nodule resection (Cases 9 and 10). All patients had uncomplicated post-operative course.On examination, the nodules were usually found to be round, firm, and well-defined. In some cases, the nodules had fused into a large mass. In Cases 7, 9, and 10, one nodule had cystic cavities with hemorrhage on the cut section.On microscopic examination, all specimens showed features typical of uterine leiomyoma, including proliferation of interlacing bundles of spindle cells without mitotic figures or cell atypia, or tumor cell necrosis. Histopathologic analysis in combination with the position of the detected nodules and the patients' history confirmed the diagnosis of LPD.The histopathologic analysis of some nodules from Cases 7, 9, and 10 showed these LPD nodules to be adenomyoma because the endometrial glands and stroma were present within the spindle cells .Of 13 patients, 8 underwent immunohistochemical staining. Their profiles are summarized in Only Case 9 underwent post-operative therapy. She received six injections of GnRH-a to prevent recurrence. All patients were on regular follow-up through ultrasound examination and MR image. As of May 2021, the average follow-up period was 49 months and no recurrence was observed.Case 9 and Case 10 had a desire for fertility. During follow-up, both patients were successfully pregnant and delivered by Cesarean section. No LPD nodules were found during the period of gestation or Cesarean section surgery.In this study, the surgical history of all the patients is laparoscopic myomectomy with uncontained morcellation. Morcellation can help fragment large specimen into small pieces granting removal through a small incision, which makes laparoscopic surgery possible. But the malignant and benign spreads of morcellated tissue have attracted more and more attention , 6. IatrThe etiology and pathophysiology of LPD remains unclear. Hormonal theory is the classical hypothesis for spontaneous LPD because it occurs mainly in the reproductive-aged females, and some cases are found after use of oral contraceptives and hormonal replacement therapy, or during pregnancy and in the presence of an estrogen-secreting tumor , 8. In oThe pre-operative diagnosis of LPD is challenging. Most of the patients had no obvious symptoms. Its lack of specific characteristics causes a very high rate of pre-operative misdiagnosis. In our case series, some cases were suspected of malignant tumors because imaging examination showed multiple lesions in the abdominal cavity. In suspected patients, image-guided FNA is a good choice to exclude the probability of malignancy and avoid inappropriate surgical treatment –14. UnclIn our cases, the sites of involvement are concentrated mainly in the areas below the umbilicus, in accordance with the movement of tissue fragments to the lower part of the abdomen because of gravity. The LPD nodules of Cases 7 and 13 were mainly located on the right side of the abdominal and pelvic cavity. This may be because the morcellator is usually placed on the right side in our institution. The nodules of iatrogenic LPD are usually large in size and less numerous, which differs from the carcinomatosis-like dissemination of typical LPD . An iatrThere is no consensus regarding the optimal treatment for LPD. Spontaneous regression of disseminated nodules has never been reported in iatrogenic LPD , so surgLPD coexisting with endometriosis within the same lesion has been occasionally reported. Toriyama et al. thought the presence of endometrial tissues within LPD lesions supports the hypothesis of submesothelial multipotential stem cells . In thatHormonal suppressive therapy is seldom used after surgery for iatrogenic LPD with only six cases reported –23. One Because of its rarity, it is not clear which factors are related to recurrence of iatrogenic LPD. In our data, only one case experienced recurrence of LPD but no evidence of relapse after treatment in our institution. In the literature, the longest follow-up was 96 months, and there was no recurrence . No maliIn addition to laparoscopic surgery, a case of LPD in a patient with a history of hysteroscopic myomectomy has also been reported . The patBased on published reports and series and on our own experience, we summarize some features of iatrogenic LPD. First, patients usually exhibit no hormonal stimulation factors, such as contraceptive pills, pregnancy, or estrogen-secreting tumors. Second, the effectiveness of hormone suppressive therapy in the treatment of iatrogenic LPD remains to be evaluated. Spontaneous regression of disseminated nodules has never been reported. So surgical resection is main choice of treatment. Third, the nodules of iatrogenic LPD, which are usually large and less numerous, are mainly located in the lower half of the peritoneal cavity below the level of the umbilicus, with the greatest concentration in the pelvis. Finally, from our experience, iatrogenic LPD usually has favorable prognosis but a regular follow-up is quite necessary.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.Written informed consent was obtained. The study was exempted from review by the Human Investigation Review Board (IRB).HL, HS, QF, DS, and JL diagnosed the patients. XC wrote the manuscript. All authors revised the manuscript.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Primary spindle cell sarcoma of the gallbladder is a rare condition.A 67-year-old woman was admitted to a local hospital with a chief complaint of abdominal pain in the right upper quadrant for the past 2 months.Surgical resection was performed following the diagnosis of primary gallbladder sarcoma with local hepatic metastasis. Histological examination confirmed a diagnosis of primary spindle cell sarcoma and hepatic metastasis with simultaneous cholecystolithiasis.Adjuvant chemoradiation therapy was not performed because the patient refused treatment. Three months after the surgery, a relapsed lesion was diagnosed. The patient underwent transcatheter arterial chemoembolization.The disease should be differentially diagnosed from gallbladder carcinoma or carcinosarcoma with hepatic metastasis. An aggressive surgical approach should be based on a balance between the risk of surgery and the outcome. Here, we report a case of primary spindle cell sarcoma with hepatic metastasis.Soft tissue sarcomas are rare solid tumors of mesenchymal cell origin that exhibit a heterogeneous mix of clinical and pathological characteristics. They account for 1% of adult cancers and 10% to 20% of adolescent and young adult cancers.2A 67-year-old woman was admitted to a local hospital with a chief complaint of abdominal pain in the right upper quadrant for the past two months. A multidetector computed tomography (CT) scan of the abdomen showed a thickened gallbladder wall with gallbladder stones and multiple hypodense tumorous lesions adjacent to the gallbladder in the lower part of segments IV and V of the liver. Emission computed tomography revealed no bone metastases. A biopsy of the liver lesion was performed at a local hospital. Histopathological examination revealed the possibility of a metastatic inflammatory myofibroblastic tumor originating from the gallbladder. The patient was then transferred to our hospital for further diagnosis and treatment.The patient had a history of high blood pressure for 2 years: which was controlled by oral administration of amlodipine and telmisartan. There was no remarkable family, medical, or genetic history. The patient was in good general health and had undergone a significant weight loss. Her vital signs  were within the normal limits. No positive signs were observed during physical examination. The complete blood count and serum biochemistry data on admission were normal. The patient also showed normal levels of tumor markers, including alpha-fetoprotein , carcinoembryonic antigen , and carbohydrate antigen19-9 .Magnetic resonance imaging (MRI) with perfusion-weighted imaging confirmed the presence of gallbladder stones (the largest measured 3.5 × 2.1 cm) and thickened wall in the enlarged gallbladder (13.5 × 9.9 cm), and hypervascular tumors in the liver . Exploration also indicated an 8 × 6 cm rigid lesion fused by multiple masses in liver segments IVb and V. All of these formed a mass of 14 × 10 cm. In addition, the tumor invaded the serosa of the colon transversum and duodenal bulb, as well as the pancreatic capsule. Enlarged lymph nodes were not observed. The patient underwent cholecystectomy, resection of liver segments IV and V, and regional lymphadenectomy.Postoperative histopathological examination confirmed primary spindle cell sarcoma and hepatic metastasis, with simultaneous cholecystolithiasis Fig. , which iAfter 10 days of recovery, the patient was discharged without any complications. Adjuvant chemoradiation therapy was not administered because of the patient's refusal to consent. Three months after the surgery, the patient underwent ultrasonography and MRI. Both examinations implied that there was a relapsed lesion (4 × 5 cm) in segment VIII. The patient underwent the treatment involving transcatheter arterial chemoembolization. The patient was discharged after 4 days. In the 11-month period since the second intervention, the patient was asymptomatic and had satisfactory general health, as confirmed by telephone interviews at 2-month intervals. More detailed information about the patient could not be obtained because the patient refused to consent to new imaging tests.3 A variety of tumors composed of sarcomatous elements include carcinosarcoma and 11 types of primary sarcoma   Tables . To date Nearly one-quarter of these types have been found in primary sarcomas of the gallbladder. Based on our data, this was more frequent in women with an M/F ratio of 1:2.42. It occurred at all ages, from 1.5 years to 91 years, with the highest frequency occurring between the ages of 50 and 90 years . Gallstones are invariably present in several types of primary sarcomas of the gallbladder low incidence and lack of experience,2)lack of obvious symptoms and typical radiological features, and3)non-specific tumor markers. We considered it difficult to differentiate between primary sarcoma, carcinosarcoma, and carcinoma of the gallbladder; therefore, we searched the literature and listed the main differences between the most common tumors of the gallbladder in Table –29 Recently, studies have shown that positron emission computed tomography scanning is useful in differentiating malignant from benign diseases when using various radiopharmaceutical agents to improve the detection and characterization of the pathophysiology of the disease. The fusion of positron emission computed tomography-acquired images with CT scans or MRI has significantly improved the overall diagnostic accuracy for sarcoma. Histopathology from biopsy or liver resection remains the gold diagnostic standard. If the lesions can be surgically resected, histological examination of the whole lesion is more reliable than a biopsy because insufficient, missed, or ample necrotic tissues may not be representative of the entire lesion. Needle-tract implantation and complications  are other reasons why preoperative biopsy of the liver is not recommended. The detailed diagnostic procedure is shown in Figure Apart from these cases, it might also have been due to the intrinsic complexity of sarcoma, technological complexity, and the lower influence of educational efforts. Based on the suggestions from the MDT, we decided to implement surgical therapy because curative resection, if possible, most effectively prolongs patient survival. Most of the patients with sarcoma of the gallbladder underwent surgical treatment, and several were further treated with adjuvant chemotherapy and/or radiation , which show that rodent MSCs are prone to spontaneous sarcomagenesis upon prolonged in vitro culture, and that MSCs have been used to establish several crucial experimental sarcoma models. The rarity of the disease means that clinical data are limited; therefore, the detailed etiology and mechanism still need further clarification. Recently, next-generation sequencing (NGS) has been recommended for application in oncology, spanning from diagnostics to prognosis and from prevention to treatability. NGS techniques have provided additional insights into the genetic diversity and complexity of sarcomas, including the potential therapeutic implications of some genetic alterations. In this study, the patients refused NGS and adjuvant chemotherapy because of economic limitations and doubts regarding adverse reactions to chemotherapy.The pathogenesis of sarcoma of the gallbladder has not been clarified, and some factors, such as germline mutations, radiation, and environmental exposure, are involved.In conclusion, primary sarcomas of the gallbladder are rare. Owing to the lack of apparent radiological features, it is not easily differentiated from other types of gallbladder tumors. Therefore, it is necessary to develop more accurate diagnostic techniques and administer more refined treatment strategies to diagnose and treat this disease.The authors thank MD Chao-Hong Yu for his assistance with language checking and revision.LX, CY and ZL designed the study and wrote the manuscript. CWX, WY, SJ, and CJ participated in study design, data collection, and drafting of the manuscript. LX, CY, and ZL participated in language checking. All authors have read and approved the manuscript.Conceptualization: Xin Long, Yan Chen, Lei Zhang.Data curation: Jia Song.Formal analysis: Jin Chen.Methodology: Wei-Xun Chen, Yu Wu, Jia Song.Supervision: Lei Zhang.Writing – original draft: Xin Long, Yan Chen, Lei Zhang.Writing – review & editing: Lei Zhang.


Navicular bone location of primary subacute hematogenous osteomyelitis uncommon. There are few cases reported in literature. We aim to describe the clinico-radiological features of primary subacute hematogenous osteomyelitis of the navicular bone in a 7-year-old child, to explain our management of this rare disease and demonstrate that medical treatment without surgery is enough.A 7-year-old child presented to emergency department. His chief complaint was fever, left limping and foot pain. The positive examination features of were painful palpation of the dorsal side of the foot and a swelling of the homolateral ankle. The erythrocyte sedimentation rate was high. The x-ray revealed a lytic lesion of the left navicular bone. The MRI findings led to the diagnosis of subacute osteomyelitis. Pain relief and normalization of inflammatory markers were obtained after 8 weeks of antibiotic therapy. Complete radiological healing was obtained after 9 months. One year after treatment, the patient was able to practice sports as previously.Subacute osteomyelitis of the navicular bone in pediatric population is a rare condition. This case shows the importance of early diagnosis thanks to MRI findings and appropriate antibiotic therapy based on the endemic bacteriological profile.The navicular bone may develop primary subacute osteomyelitis in immunocompetent child. Early diagnosis is important for prescribing effective conservative treatment. •Subacute osteomyelitis of the navicular bone is an etiology of lameness in children.•The penumbra sign on MRI is strongly suggestive of the infectious origin of the bone involvement.•Medical treatment without surgery can lead to a cure.•Antibiotic therapy should be sufficiently prolonged for at least 3 months. Its location in the foot small bones is uncommon. We present a rare case of primary subacute hematogenous osteomyelitis of the navicular bone in a 7-year-old immunocompetent child, treated medically without surgery. Few cases are reported in the literature . There iThis case report has been reported in line with the SCARE Criteria  Agha RA,2We report the case of a 7-year-old child, with no medical history. He presented to the pediatric orthopedic emergency department for lameness that has been evolving for a month and had worsened 3 days ago. The parents reported a history of a trauma to the left foot a month ago. Clinical examination confirmed left lameness, fever and a painful swelling of the back side of the foot without inflammatory signs. The ankle had painless full range of motion and there were no skin lesions. The x-ray of the foot showed an osteolytic lesion of the body of the navicular bone without cortical rupture . The bloThe patient undertook antibiotic therapy: Clavulanic acid/Amoxicillin 100mg/Kg/day or 800 mg three times a day intravenously for one week and Gentamycin 4mg/Kg/day or 90 mg once a day as an infusion for 4 days. Then Clavulanic acid/Amoxicillin per os for a total duration of 4 months. In association with the medical treatment, a cast boot was put for 6 weeks. The pain relief was obtained 8 weeks after antibiotic therapy onset. Biological monitoring did not show any abnormalities. Radiological monitoring showed calcification of the bone gap after 3 months of treatment. The patient was able to practice sports as previously after 1 year. At 5 years follow-up, there was no sequelae in the navicular bone .Fig. 5X-3Subacute osteomyelitis can be difficult to diagnose because the characteristic signs and symptoms of acute infection are often absent .SubacuteIn the current case, radiological healing was late according to the clinical one because the navicular bone is poorly vascularized. The duration of antibiotic therapy is not standardized. It ranged from two months for Cakmak Celik  et al. tSurgical treatment in subacute osteomyelitis has not been unanimous due to the low number of sporadic cases. Cakmak Celik et al.  have repThe most common and reported pathogen germ related to subacute osteomyelitis in children has is staphylococcus aureus . The juvThe immobilization of the foot in a plaster is an important part of the treatment. In fact, it allows bone to heal and to avoid pathological fractures ,4.4The navicular bone may develop primary subacute osteomyelitis in immunocompetent child. Early diagnosis is important for the onset of effective conservative treatment. Surgery is not necessary in the absence of a soft tissue abscess. Prolonged antibiotic treatment and the immobilization of the foot are the mainstream treatment.All data is available to readers.None.None.Mohamed Zairi Writing drafting the article. Ahmed Msakni conception and design, revising it critically. Rim Boussetta revising it critically for important intellectual content. Ahmed Amin Mohseni revising it critically. Mohamed Nabil Nessib final approval of the version to be published.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.1. Name of the registry:2. Unique Identifying number or registration ID:3. Hyperlink to your specific registration (must be publicly accessible and will be checked):Mohamed Zairi.Not commissioned, externally peer-reviewed.None.


Hydroxyapatite crystal deposition disease (HADD) around the hip is typically described involving the gluteal tendons. However, HADD can occur in any location and result in varied clinical presentations. Even with small deposits, symptoms can be significant and imaging findings may appear aggressive, mimicking infection and malignancy particularly when in an atypical location.We illustrate cases of both common and rare locations of HADD around the hip, in particular presenting as greater trochanteric pain syndrome, piriformis syndrome and ischiofemoral impingement. The latter two manifestations have not been previously described in the literature.Low signal deposits were identified on MRI at the greater trochanter (gluteus medius tendon), proximal piriformis (adjacent to the sciatic nerve), and quadratus femoris , respectively. Associated inflammatory changes with tendinopathy, bursitis and oedema were also demonstrated. The patient with piriformis syndrome underwent steroid injections and shockwave therapy with significant symptom improvement.HADD should be within the differential diagnosis for hip pain and nerve compression syndromes. Knowledge of tendon anatomy and correlation with radiographs or CT, even after MRI, is crucial in recognising unusual manifestations and preventing unnecessary investigation. Therefore, we review the spectrum of imaging features of HADD, as well as the current evidence on its management, to confidently diagnose this condition. The most common manifestation is calcific tendinitis, which describes the deposition of hydroxyapatite crystals within tendons and occurs in up to 3% of adults, with peak incidence at 30 to 60 years of age.5 Although almost always described around the insertion of the gluteal tendons on the greater trochanter and/or gluteal tuberosity,6 HADD can occur in any tendon or muscle and hence have a variety of clinical manifestations.After the shoulder, the hip is the second most frequently involved site, seen in 5% of adults with calcific tendinitis.7 Furthermore, imaging may demonstrate associated bony changes and soft tissue oedema.7 HADD can hence mimic conditions such as infection, tendon rupture, myositis ossificans and malignancy, and pose a diagnostic challenge particularly when in an atypical location.Patients may be asymptomatic or present with: acute or chronic pain, tenderness, swelling, and restricted range of motion. The symptoms can be severe and are occasionally associated with low-grade fever and mildly raised inflammatory markers.Awareness of the anatomy of tendinous insertions and detecting calcific deposits on imaging is crucial to distinguish HADD from more aggressive pathology. We illustrate cases of both common and rare manifestations of HADD around the hip, in particular as piriformis syndrome and ischiofemoral impingement, which have not been previously described to our knowledge. We will review imaging features to facilitate a prompt diagnosis and the current evidence on management of HADD.A 46-year-old male presented with left lateral hip pain and stiffness, with focal tenderness over the greater trochanter. MRI demonstrated left gluteus medius insertional tendinopathy associated with a low signal focus at the left greater trochanter, confirmed to be a calcific deposit on a subsequent hip radiograph .8 and as in this case, gluteus medius calcific tendinitis. The pain can be anterior, superior or lateral depending on the facets of the greater trochanter affected.7Greater trochanteric pain syndrome is characterised by pain and tenderness over the greater trochanter. It may be secondary to a diverse group of conditions, such as gluteus medius and/or minimus tendinopathy, trochanteric bursitis due to rheumatological disorders,9 Piriformis syndrome may be due to several entities, including an accessory piriformis muscle, muscle hypertrophy, trauma or mass lesion, which results in compression of the sciatic nerve at the greater sciatic notch.10A 63-year-old female presented with a one-day history of acute severe left hip pain which radiated to her left calf, resulting in difficulty weight-bearing. The pain was worse on hip flexion and she was focally tender over the course of the sciatic nerve in the left gluteal region. Her symptoms were consistent with piriformis syndrome, which describes sciatic pain typically reproduced on internal rotation of a flexed hip. Other common features are: buttock pain; pain aggravated by prolonged sitting; tenderness near the greater sciatic notch; limitation of straight leg raise (positive Lasègue sign).A calcific deposit with surrounding oedema within the piriformis muscle was seen on MRI, closely applied to and likely resulting in irritation of the sciatic nerve due to inflammatory change . This deDue to the severity of her symptoms, she underwent a steroid injection around the deposits which provided significant immediate but short-term relief. She subsequently had low-energy shockwave therapy to this area with marked improvement in symptoms. The calcific deposit resolved on a follow-up radiograph one-month later .A 35-year-old male attended following a two-month history of right hip pain radiating to the lower back. On examination, he had reduced range of hip movement due to pain. MRI demonstrated a calcific deposit at the femoral insertion of the right quadratus femoris with surrounding oedema .Lastly, a 25-year-old female presented with three months of left hip pain on weight bearing, sitting on hard surfaces and stretching her left leg. On examination, symptoms were worse on extension and external rotation. MRI demonstrated a low signal deposit within the left quadratus femoris muscle, just lateral to the left ischial tuberosity, narrowing the ischiofemoral space and associated with considerable surrounding muscle oedema . Finding11 Other causes include anatomic variants of the ischium or femur, developmental hip dysplasia, ischial tuberosity enthesopathies, and mass lesions.12Symptoms can be non-specific but typically reproduced on hip extension, adduction and external rotation.Although it was not possible to obtain histopathological correlation as none of the cases proceeded to needle aspiration or surgery, the imaging features described are consistent with that seen in HADD. None of the patients had a relevant history of trauma or infective symptoms. They had normal inflammatory markers, as well as normal biochemical and bone profiles. In cases 1, 3 and 4, the patients’ symptoms resolved with conservative management indicating a benign process.13 and Bosworth1 postulated a degenerative process, whereby ischaemia or repetitive trauma results in hyaline degeneration and calcium deposition. This theory has, however, been contradicted by subsequent observations, namely, the relatively early peak incidence of HADD,1 complete resolution of HADD in certain cases4 and different composition of calcific deposits compared to degenerative tendinopathy.14 Benjamin hypothesised a process similar to endochondral ossification of fibrocartilage at tendon insertions.15Although the pathogenesis of HADD remains uncertain, several theories have been proposed. Bishop3 In the formative phase, impaired perfusion due to vascular or mechanical factors result in local hypoxia, triggering fibrocartilaginous metaplasia and deposition of calcium hydroxyapatite within extracellular matrix vesicles.16 This is followed by a resting phase for a variable period of time. During these initial stages, the calcific deposit is typically homogeneous and round/ovoid in shape. Patients tend to be asymptomatic or have chronic pain secondary to mechanical impingement.The most well-described pathogenesis is by Uhthoff et al, who proposed a reactive or cell-mediated process with progressive stages which have distinct imaging features and often correlate with clinical symptoms.17 This results in oedema and increased intratendinous pressure. The deposit resembles ‘toothpaste’ in consistency and can thus rupture into nearby tissues, including the musculotendinous junction and muscle, and induce an inflammatory reaction. The calcification appears ill-defined, heterogenous and fluffy, and often correlates with acute clinical symptoms.18 Finally in the reparative phase, fibroblasts incite new collagen fibre formation and restore the tendon collagen pattern.19During the resorptive phase, the deposit undergoes phagocytosis by macrophages and multinuclear giant cells.20,21A more recent theory is that HADD is caused by the erroneous differentiation of tendon-derived stem cells into calcium-depositing chondrocytes or osteoblasts instead of tenocytes, due to altered local conditions such as excessive mechanical loading and microinjuries.22 thyroid and oestrogen endocrine disorders,23 certain genes such as the HLA-A1 genotype,24 and with variations in tissue transglutaminase two and osteopontin.25 However, the exact correlation and underlying pathogenesis is still unclear.Increased incidence of HADD has been observed in patients with diabetes,26 Radiographs are helpful and cost-effective in characterising the contour and extent of calcific deposits and thus inference of the phase of the disease.Calcifications may range in size from a few millimetres to centimetres, but no relationship between the size and severity of pain has been shown.27 If there is a confounding history of trauma, HADD may be mistaken for avulsion fragments in the acute setting, or heterotopic ossification/myositis ossificans in chronic HADD. CT is particularly useful in this context as it can evaluate the morphology and consistency of deposits. This enables differentiation from ossification or avulsion fragments, which have corticated margins and are of higher density (100–400 HU for hydroxyapatite versus 700–1500 HU for bone).28 CT is hence also important if planning intervention, such as needle aspiration.CT is sensitive for the detection of hydroxyapatite deposits and a comet-tail appearance is characteristic.29 Increased vascularity on power Doppler may be seen in the acute phase, although in only a third of cases.30Whilst ultrasound is less useful for the diagnosis of HADD, it is commonly used to guide treatment. A range of sonographic appearances have been described; hyperechoic arc-shaped foci with posterior acoustic shadowing is suggestive of the formative and resting phases, whilst deposits can appear nodular, fragmented, or cystic, with or without acoustic shadowing, during the resorptive phase.7 Cortical erosion, periosteal reaction and marrow involvement have been described and may be confused for an infective or neoplastic process such as a chondroid lesion.27Calcific deposits appear as focal areas of low signal on all MRI sequences, typically near tendon insertions. Whilst MRI is the best modality for assessment of inflammatory changes and for other causes of hip pain, it is important to appreciate HADD can have an aggressive appearance. During the acute resorptive phase, associated oedema can be extensive and mimic trauma or infection.Detection of characteristic calcification near or within a tendon, in conjunction with the absence of a soft-tissue mass, is thus crucial for diagnosis. Small deposits may be difficult to visualise on MRI and hence if HADD is suspected, particularly in an uncommon location, radiographs can be obtained to confirm the diagnosis. Gradient echo pulse sequences could also be added to the protocol to utilise the susceptibility artefact to highlight calcification.26 and 80% of patients with calcific tendinitis of the hip31 with conservative treatment. If symptoms persist and/or significantly impact quality of life, steroid injection, ultrasound or CT-guided needle aspiration and lavage 32 and extracorporeal shockwave lithotripsy33 have shown to provide symptomatic relief and reduction in the size of deposits. However, there is little consensus on which minimally invasive therapy is more effective, and with the majority of trials evaluating calcific rotator cuff tendinitis. Meta-analyses showed only weak evidence that ultrasound-guided lavage is more effective than a subacromial corticosteroid injection,34,35 although needling combined with a corticosteroid injection has been shown to be more effective than injection alone36 or extracorporeal shockwave lithotripsy.37–39 In refractory cases, surgical resection may be considered.31Painful HADD is typically managed with nonsteroidal anti-inflammatory drugs and physical therapy. HADD is usually a self-limiting process, with studies showing clinically significant improvement in 72% of patients with calcific tendinitis of the shoulderHADD can occur in unusual locations, present with acute and severe symptoms, and imaging appearances can be aggressive with bony erosion and extensive soft tissue or marrow oedema. HADD can thus pose a diagnostic challenge, mimicking trauma, infection or malignancy. Radiographs can be especially helpful, even after initial imaging with MRI to visualise small deposits. Although an uncommon entity, HADD should be considered in the differential diagnosis of acute or chronic hip pain and nerve compression syndromes. Knowledge of tendon anatomy and the range of imaging manifestations of HADD is critical in recognising HADD when it presents in atypical locations, to promptly diagnose the condition, and prevent unnecessary workup or intervention. Further studies comparing the effectiveness of different minimally invasive therapies, particularly for HADD around the hip, is needed.Clinicians and radiologists should be alert to atypical manifestations of hydroxyapatite crystal deposition disease, which can present with acute pain and nerve compression syndromes.Imaging findings can appear aggressive and mimic trauma, infection, or malignancy.


Subsequently, using a five-point Likert scale, the two radiologists evaluated the diagnostic performance of a pre-defined MRI system using features reported as characteristics of LGESS in previous case series: uterine tumor with high signal intensity (SI) on diffusion-weighted images and with either worm-like nodular extension, intra-tumoral low SI bands, or low SI rim on T2-weighted images. Area under the receiver operating characteristic curve (AUC), sensitivity, and specificity of the two readers’ Likert scales were analyzed. Intra-tumoral low SI bands (p < 0.001), cystic/necrotic change (p ≤ 0.02), absence of speckled appearance (p < 0.001) on T2-weighted images, and a low apparent diffusion coefficient value (p ≤ 0.02) were significantly associated with LGESS. The pre-defined MRI system showed very good diagnostic performance: AUC 0.86/0.89, sensitivity 0.95/0.95, and specificity 0.67/0.69 for R1/R2. MRI can be useful to differentiate uterine LGESS from rare leiomyoma variants.The purpose of this study is to evaluate utility of MRI in differentiation of uterine low-grade endometrial stromal sarcoma (LGESS) from rare leiomyoma variants. This multi-center retrospective study included consecutive 25 patients with uterine LGESS and 42 patients with rare leiomyoma variants who had pretreatment MRI. Two radiologists (R1/R2) independently evaluated MRI features, which were analyzed statistically using Fisher’s exact test or Student's  Low-grade ESS (LGESS), a less aggressive subtype of ESS, typically develops in premenopausal and perimenopausal women2. Although it is indolent compared to high-grade ESS and leiomyosarcoma, LGESS frequently shows malignant behavior, including extra-uterine involvement, metastasis, and recurrence2.Endometrial stromal sarcoma (ESS), a very rare uterine sarcoma entity composed of tumor cells resembling endometrial stromal cells, constitutes 7–25% of uterine sarcomas, second to leiomyosarcoma, but accounting for only 0.2% of uterine malignancies 5. Because of its rarity, nonspecific symptoms and preponderance among young women, LGESS can be misdiagnosed as leiomyoma or adenomyosis. It can lead to less-invasive treatment options for presumed benign indications such as enucleation with/without power morcellation, instead of hysterectomy and bilateral salpingo-oophorectomy as a standard treatment6. Since the US Food and Drug Administration issued a warning against its use in 2014, the safety of power morcellation has been debated, weighing patients’ benefits of minimal invasiveness and risks of occult cancer7. Reportedly, LGESS is a dominant type of unexpected uterine sarcomas after laparoscopic power morcellation4. It can be an unfavorable factor by increasing intraperitoneal spreading8. To perform minimally invasive procedures for leiomyoma safely, further improvement in pretreatment workup to detect LGESS is mandatory8.Differential considerations in the diagnosis of LGESS include leiomyoma, adenomyosis, endometrial cancer, and other uterine sarcomas. Among them, pretreatment differentiation from benign lesions and recognition of its malignancy is the most clinically relevant issue9. Regarding leiomyosarcoma, evidence has been accumulated supporting the utility of MRI. Lakhman et al. reported a classification model with high sensitivity and specificity using conventional MRI features to differentiate leiomyosarcoma from benign rare leiomyoma variants10. Recently, diffusion-weighted imaging (DWI) has been highlighted as a potentially useful tool for the differentiation of uterine sarcoma, reflecting the cellular density of tumor13. Compared to leiomyosarcoma, evidence of MRI about LGESS remains very limited because of its extreme rarity14. Characteristic MRI features of LGESS have been reported in small case series: worm-like nodular extension15, intra-tumoral low signal intensity (SI) bands on T2 weighted-images (T2WI)16, and low SI rim on T2WI17. Li et al. reported that 13 of 14 patients (93%) showed high SI on DWI18. Nevertheless, no report of the relevant literature describes the diagnostic performance of MRI in differentiation from leiomyoma, especially from rare leiomyoma variants.Pretreatment differentiation of uterine sarcoma from leiomyoma by magnetic resonance imaging (MRI) has persisted as a challenge. Most leiomyomas with typical MRI appearance are easy to diagnose, but some rare leiomyoma variants might mimic sarcomaThe initial purpose of this multi-center study was to assess different MRI features on LGESS and leiomyoma variants. The second purpose was to analyze if a pre-defined MRI system using features reported previously as a characteristic of LGESS could be used for differential diagnosis with leiomyoma variants.This multi-center retrospective Health Insurance Portability and Accountability Act-compliant study was approved and the need for informed consent was waived by the respective institutional review boards of seven institutions: Kyoto University Hospital, Kindai University Faculty of Medicine, Kobe City Medical Center General Hospital, Japanese Red Cross Osaka Hospital, Japanese Red Cross Otsu Hospital, Kitano Hospital, and Japanese Red Cross Wakayama Medical Center. All methods were carried out in accordance with relevant guidelines and regulations.We searched each institutional pathological database consecutively for patients who were histologically diagnosed as having LGESS based on surgical specimen or biopsy and who had pretreatment MRI. Between January 2007 and June 2019, 25 patients from seven institutions were identified (LGESS group).10 and focusing on the variants with high cellularity/ cytologic atypia / mitotic activity. Smooth muscle tumor of uncertain malignant potential was not included because of its uncertain malignancy. During the same period, 42 patients were identified, constituting the Rare leiomyoma variant group.We also searched pathological databases of two institutions consecutively for patients who were histologically diagnosed as having cellular leiomyoma, leiomyoma with bizarre nuclei or atypical leiomyoma, mitotically active leiomyoma, or intravenous leiomyomatosis based on surgical specimens, and who had undergone pretreatment MRI. These variants were selected, referring a previous paper about the differentiation of leiomyosarcoma from leiomyoma variants19. Patient enrollments and excluded cases of the two groups are described in Supplemental Fig. All cases were diagnosed histologically by board-certified pathologists of the respective institutions, according to the World Health Organization’s Classification of Tumours of Female Reproductive Organs 20142. Apparent diffusion coefficient (ADC) maps generated from DWI were available for 18 cases of the LGESS group and for 37 cases of the Rare leiomyoma variant group. Twenty-two cases of the LGESS group and 21 cases of the Rare leiomyoma variant group had contrast enhanced T1-weighted images (CE-T1WI) with/without fat saturation after administration of 0.1 or 0.2 mmol/kg gadolinium chelate contrast medium. Representative MRI protocols at the respective institutions are presented in Supplemental Table All MRI studies were acquired in supine position on 1.5 T or 3.0 T scanners using pelvic phased-array coils. At a minimum, each study included two planes of T2WI , axial or sagittal T1-weighted images (T1WI) with/without fat saturation. Twenty-two patients of the LGESS group and all patients of the Rare leiomyoma variant group had undergone DWI. The high b values of participating institutions were 800–1000 s/mm20. They were aware that patients had been diagnosed histologically as having either LGESS or rare leiomyoma variants, but they were blinded to all other clinical information. The following qualitative features were evaluated: on T2WI, (1) main site of the tumor , (2) nodular and/or irregular border15, (3) extrauterine involvement, (4) worm-like nodular extension15, (5) intra-tumoral low SI bands16, (6) low SI rim surrounding intra-myometrial tumor17, (7) speckled appearance (reported to be characteristic of leiomyoma)20, (8) cystic and/or necrotic change18, (9) higher SI than outer myometrium; on T1WI images, (10) tumoral hemorrhage , with worm-like nodular extension on T2WI defined as detached nodules from the primary tumor15, with speckled appearance defined as diffusely scattered high SI within the tumor on T2WI20. When DWI and CE-T1WI were available, the following features were assessed: (11) SI relative to sciatic nerve roots (iso or high vs low) on DWI and (12) heterogeneous tumor enhancement on CE-T1WI. Case examples of respective qualitative features were in Figs. Two board-certified radiologists with 11 years of experience  independently reviewed pretreatment MRI, after trained to identify MRI features of LGESS with previous reportsorder15,  extrauteorder15,  extrautelvement,  worm-lik2) were placed manually on solid parts of the tumor, carefully avoiding cystic/necrotic/hemorrhagic areas. Then, the ADC value of the tumor was measured as the mean value of three circular regions of interest. The normalized ADC value  was also calculated to reduce the effect of ADC variation from mixed DWI protocols21.The two readers also evaluated the following quantitative features independently. The maximum tumor size was measured on either the sagittal, axial, or coronal T2WI plane. When an ADC map was available, referring other MRI sequences, three circular regions of interest , the two readers (R1/R2) used a Likert scale for independent evaluation of whether the uterine tumor met the criteria, or not. Its scales were 1–5: 1, definitely no; 2, probably no; 3, equivocal; 4, probably yes; 5, definitely yes. Before the evaluations, two readers were informed that scales of 4 and 5 would be regarded positive, whereas scales of 1–3 negative, to calculate diagnostic performance, such as sensitivity and specificity.A pre-defined MRI system was created before evaluations of qualitative and quantitative MRI features above to avoid testimation (estimation after testing) bias. This system was based on T2WI and DWI features reported previously as a characteristic of LGESS: tumor with iso/high SI on DWIt-test for quantitative data. Concordance correlation coefficients (CCCs) were calculated for quantitative features between R1 and R2: maximum tumor diameter, the ADC value, and the normalized ADC value. Regarding CCC values, the following were inferred: greater than 0.99 were considered almost perfect; > 0.95 to ≤ 0.99, substantial; > 0.90 to ≤ 0.95, moderate; and ≤ 0.90, poor22.The associations between MRI features and LGESS were analyzed using Fisher’s exact test for qualitative data and Student 23. Then Likert scales were dichotomized with scales of 1–3 indicating leiomyoma and scales of 4 and 5 indicating LGESS to calculate sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of the MRI criteria for R1/R2.Receiver operating characteristic (ROC) curve analysis of the pre-defined MRI system was applied for each reader’s Likert scales. Area under the curve (AUC) with its standard error calculated using the DeLong method were computed: AUC greater than 0.90 was considered excellent diagnostic accuracy; 0.81–0.90, very good; 0.71–0.80, good; 0.61–0.70, moderate; 0.51–0.60, poor; ≤ 0.5, test not usefulκ > 0.80, excellent agreement; 0.61 < κ ≤ 0.80, good agreement; 0.41 < κ ≤ 0.60, moderate agreement; 0.21 < κ ≤ 0.40, fair; κ ≤ 0.20, poor agreement24. Reader agreement after dichotomization of Likert scales  was also calculated for the pre-defined MRI system.Reader agreement on the Likert scales of the pre-defined MRI system was analyzed using a weighted kappa statistic: ® version 18.5; MedCalc Software, Ostend, Belgium). For all statistical analyses, a p value result of less than 0.05 was inferred as significant.Statistical analyses were performed using software , whereas benign disease was presumed preoperatively in ten cases . All patients were diagnosed as having LGESS by surgical specimen, except for one patient diagnosed by biopsy, who was not regarded as a surgery candidate because of multiple bone metastases.In the LGESS group, a malignant tumor was suspected before treatment in 15 cases , nine cases of leiomyoma with bizarre nuclei , two cases of atypical leiomyoma , four cases of mitotically active leiomyoma , and two cases of intravenous leiomyomatosis . All were diagnosed by surgical specimen.The two readers’ evaluations of MRI features are presented in Table p value, < 0.01/0.02 for R1/R2; CCC, 0.51, poor; optimal cutoff, ≤ 932 10–6 mm2/s / ≤ 776 10–6 mm2/s for R1/R2) and a normalized ADC value  of LGESS were lower than those of rare leiomyoma variants for both readers. With respective optimal cutoffs, sensitivity/specificity for R1 and R2 were as follows: the ADC value, R1 78%/76% and R2 89%/65%; the normalized ADC value, R1 72%/78% and R2 89%/70%. No significant association was found for the maximum tumor size .Regarding quantitative MRI features, an ADC value (κ = 0.59). After dichotomizing the Likert scale (1–3 vs 4 & 5), the inter-rater agreement was improved to be good (κ = 0.78). Details are presented in Table The diagnostic performance of the pre-defined MRI system was very good for R1  and R2 . With the pre-defined cutoff > 3 of a Likert scale, the system showed high sensitivity (95% for both readers) and relatively low specificity (67% and 69%). One LGESS case was diagnosed as leiomyoma with a scale of 3 assigned by both readers , which was a tumor located predominantly in the endometrial cavity with a small portion of myometrial invasion . A non-expert radiologist in gynecologic imaging obtained an AUC as good as a specialized radiologist. Our results suggest the ability of MRI to differentiate LGESS from rare leiomyoma variants and to optimize treatment selection. This MRI system would be clinically applicable to screen out LGESS in pretreatment workup for cases with higher suspicion of LGESS or leiomyomas with unusual appearances on MRI.16, Fig. 20, Supplemental Fig. 15 and/or intra-tumoral low SI bands  and NPV (97% for both readers). The system based on qualitative assessments would be intuitive for radiologists to use, although it could be subjective. After dichotomizing the Likert scale (1–3 vs 4 & 5), the inter-rater agreement was improved (κ = 0.78). The system would pick up LGESS from leiomyoma and provide information leading to prevention of misdiagnosis of LGESS and treatment optimization. Its relatively low specificity (67%/69% for R1/R2) and PPV (60%/62% for R1/R2) might be because the three T2WI features  could be also observed in some of rare leiomyoma variants (Supplemental Table 17. Koyama et al. described that low SI bands on T2WI was not apparent in the tumor confined to the endometrium16. LGESS with a very small part of myometrial infiltration can be a pitfall of the pre-defined MRI system; it might be difficult to differentiate from submucosal leiomyoma. In such a case, pretreatment biopsy represents an option when a tumor shows high SI on DWI with a low ADC value.It is noteworthy that one case of LGESS predominantly located in the endometrial cavity was not diagnosed correctly in both readers’ evaluation of the pre-defined MRI system Fig. . All preOur study has several limitations. First, the number of patients with LGESS was relatively small. Considering its extreme rarity, we conducted a multicenter study, and it included the largest number of LGESS patients in the previous studies of MRI features. Second, this retrospective study was conducted without validation. Selection bias should be taken into consideration. An external and/or prospective validation with more numerous patients must be done to introduce our results to clinical preoperative workup safely, especially for minimally invasive surgery. Third, the MRI protocols of respective institutions were diverse. They can affect evaluations of some features such as the DWI and ADC map. Regarding ADC values of tumors, we calculated normalized ADC values to reduce the bias. Fourth, the control group enrolled patients only from the two institutions and the ratio of LGESS and rare leiomyoma variants would be different from that in clinical settings. Also, the control group was heterogeneous and differentiations between LGESS and respective leiomyoma variants were not performed. The control group did not include other tumors mimicking LGESS, such as usual leiomyoma with atypical MRI findings, other leiomyoma variants, other uterine sarcoma, or endometrial cancer. Fifth, considering the rarity of LGESS, disease prevalence and a grade of suspicion would have influence on the results of the MRI systems. The diagnostic system would be appropriate to use in the pretreatment approach of cases with high suspicion while evaluating MRI rather than a screening approach.In conclusion, we presented characteristic MRI features of LGESS in comparison to those of rare leiomyoma variants. We also presented the MRI diagnostic system for LGESS with very good diagnostic performance. MRI can be a reliable tool for optimization of treatment strategies for uterine tumor, avoiding inappropriate less-invasive treatment options.Supplementary Figures.Supplementary Table S1.Supplementary Table S2.


We report two cases of patients with gastric linitis plastica (GLP), in which the histopathological diagnosis was made by endoscopic ultrasound‐guided fine‐needle biopsy (EUS‐FNB) using a Franseen‐tip needle. Esophagogastroduodenoscopy findings showed mucosal swelling and poor distensibility of the gastric antrum. Abdominal computed tomography findings showed significant thickening of the gastric wall at the antrum. Conventional endoscopic and bite‐on‐bite biopsy were attempted but resulted in failure to diagnose the lesions. We performed EUS‐FNB to obtain histopathological samples from a deeper site, which confirmed the diagnosis. We considered this method safe and effective for the diagnosis of GLP. Gastric linitis plastica (GLP), known as “leather bottle stomach,” is a type of diffuse infiltrating gastric cancer characterized by thickening and rigidity of the gastric wall. In such cases, tumor cells exist more abundantly in the submucosa than in the mucosa. When there are no tumor cells in the mucosa, mucosal ulceration or erosion is often absent.In this report, we present two cases of patients with GLP, in which endoscopic ultrasound‐guided fine‐needle biopsy (EUS‐FNB) with a Franseen‐tip needle was used to successfully confirm the diagnosis after a failed conventional endoscopic biopsy. All participants provided written informed consent to undergo the procedure. The Toyama University Hospital Institutional Review Board granted permission to review the patients’ records. The study was conducted according to the principles of the Declaration of Helsinki.Case 1 was of a 68‐year‐old woman who underwent esophagogastroduodenoscopy (EGD) at a local clinic because of epigastric discomfort. EGD findings showed mucosal swelling and poor distensibility at the gastric antrum. Conventional endoscopic biopsy was performed, resulting in an inconclusive pathological diagnosis. Therefore, the patient was referred to our hospital for further evaluation.The serum carcinoembryonic antigen and cancer antigen (CA) 125 levels were within the normal range, however, the CA 19‐9 level was elevated to 343 U/ml. Abdominal computed tomography (CT) findings showed thickening of the gastric wall at the antrum Figure . FluorodThe patient had Stage‐III cancer as per the Union for International Cancer Control (UICC) classification (T4aN1M0). Despite our recommendation, she was unwilling to undergo surgery; therefore, general chemotherapy was commenced later.Case 2 was of a 69‐year‐old woman who visited a local hospital because of anorexia. EGD was performed at the hospital, which showed mucosal swelling and poor distensibility of the gastric antrum. Conventional endoscopic mucosal biopsy was performed with histopathological findings yielding an inconclusive diagnosis. However, CT showed circumferential thickening of the gastric wall at the gastric antrum and multiple nodules in the abdominal cavity Figure . TherefoThe blood test findings were normal, including the carcinoembryonic antigen and CA 19‐9 levels, except for a slight elevation in the CA 125 level (73 U/mL). EGD findings showed stricture with rigidity without mucosal erosion and ulceration in the gastric antrum Figure . TherefoThe final diagnosis was gastric cancer UICC Stage IVB and general chemotherapy was commenced.The diagnosis of GLP is often difficult because of its pathological features presenting as submucosal proliferation. Endoscopic findings often show only thickening and rigidity of the gastric wall without any suspicious mucosal findings. In addition, definitive diagnosis of GLP is difficult based only on EUS findings. Although pathological diagnosis is required to establish the definitive diagnosis of GLP, conventional endoscopic forceps biopsy is often difficult to obtain adequate sample, as the tumor cells are found mainly in the submucosal or deeper layers with reactive fibrosis.p = 0.0746). However, the diagnostic yield of a MIAB was significantly higher than that of EUS‐FNA in patients with tumors <2 cm in size, lesions in the greater curvature, and non‐gastrointestinal stromal tumor SELs.For biopsy from deeper sites, several endoscopic biopsy techniques are currently used, such as the bite‐on‐bite technique, jumbo biopsy, endoscopic mucosal resection, mucosal incision‐assisted biopsy (MIAB), and EUS‐guided fine‐needle aspiration (EUS‐FNA).Recently, EUS‐FNB has gained popularity, as it helped us obtain more pathological samples and easily establish a more accurate pathological assessment. Several studies have already confirmed that the diagnostic ability of EUS‐FNB is superior to that of EUS‐FNA.In our reported cases, EUS‐FNB was performed because mucosal biopsy and biopsy with the bite‐on‐bite technique could not help in making a definitive diagnosis. Although the thickening of the stomach wall was thin , sufficient sample volume was obtained for histopathological and immunohistochemical examination without any complications. In addition, GLP often metastasizes to the lymph nodes and peritoneum. EUS‐FNB is also helpful in determining the TNM staging using the cytological and histological assessment of such lesions. In our second case, a small amount of ascites was also obtained by EUS‐FNB, and the diagnosis of carcinomatous peritonitis was confirmed using the cytological assessment. The 22‐gauge Franseen‐tip needle may be recommended for EUS‐FNB for GLP because of its easy manipulation and high diagnostic ability.While EUS‐FNB contributes to the diagnosis of GLP, needle tract seeding should be noted. Since the report of the first case by Hirooka et al. in 2003, 18 cases have been reported to date.In conclusion, EUS‐FNB using a Franseen‐tip needle is a safe and effective technique for the definitive diagnosis of GLP when it is being considered as a probable cause of gastric wall thickening.Author I.Y. is an Associate Editor of DEN Open.None.