Herein, we discuss the case of a 26-year-old male patient with a diagnosis of giant cell tumour (GCT) of the patella which is an exceptionally uncommon condition. The motive of reporting a rare case such as the giant cell tumour of the bone (GCTB) relies on its diagnostic incidence. Since the symptoms of this tumour overlap more common etiologies than GCT, the diagnosis of such a devastating malignant tumour is usually missed and hence delayed, which leads to poor treatment strategies and ultimately an irreversible fatal outcome.  The tumors of the patella are rare and more commonly of benign morphology rather than the malignant variant . StatistThe motive of reporting a rare case such as GCTB relies on its diagnosing statistics. GCTs are rare, affecting 1 in 1,000,000 people per year, and 50% of those tend to localize around the knee joint .Management criteria of GCT are poorly defined due to its scant diagnosis. However, it includes surgery with curettage or wide excision of the tumor . OngoingA 26-year-old male patient, employed at a garment factory, with no known co-morbidities presented to our orthopedic out-patient-department (OPD) with the chief complaint of pain in the left knee of 5 years duration. As per the patient’s history of presenting illness, he was in a usual state of health previously when he initially experienced sudden, throbbing, non-radiating, exertion-related, moderately intense left anterior knee pain which progressively increased with the passage of time. Pain aggravation was witnessed more specifically in routine activities at work and during exercise, partially relieved by over-the-counter analgesics taken as needed. No family history of any infantile or adolescent musculoskeletal disorders was present.On physical examination of the lower extremities, mild left anterior knee swelling was noted along with size discrepancy between left and right lower extremity, with left lower extremity atrophy. The sensations were intact overall in the affected limb with no skin color changes, discharge, nodules, lymphadenopathy, or alopecia. Powers of the left lower limb were compared with the right and exhibited moderate leg pain during knee flexion and extension against resistance. General physical examination of the skin, nails, and mucous membranes remained unremarkable. Baseline investigations including Complete Blood Count (CBC), Liver Function Tests (LFTs), Urine Detailed Report (D/R), Urea, Creatinine, and Electrolytes (UCE), Chest X-ray (CXR), and viral markers were performed and were found to be unremarkable. To further investigate comprehensively, imaging radiography of the patient was performed including a lateral radiograph of the left knee joint initially Figure . BilaterAn active giant cell tumor of the left patellar bone was finally suspected with a differential diagnosis of a solitary benign bone cyst, chondroblastoma, and aneurysmal cystic disease of bone kept into consideration. Biopsy was planned with patellar de-roofing and curettage. Results revealing multi-nucleated giant cells were obtained. The patient could not be followed up further due to his non-compliance to further treatment after the results of his biopsy.Giant cell tumour, a rare benign neoplasm, accounts for 5% of the primary bone tumours with 40% to 50% of cases reported entailing the knee joint , 8. The Manifestations of the GCT of the patella preside to evolve as knee pain is associated with swelling. Joint erythema, tenderness on palpation, effusion, and crepitus on physical examination have also been classified as a part of the disease symptomatology . The magImaging techniques have been reported to play a pivotal role in the diagnosis of morbidity . MRI andSurgical excision of the tumour has been considered the recommended treatment where intralesional curettage and partial patellectomy have been explained as the two dimensions of the surgical approach . No striAlthough patellar tumours are rare, they should always be kept in mind as a differential diagnosis in cases of anterior knee pain and discomfort. It is imperative to note that giant cell tumours of the patella can be difficult to distinguish from other GCTBs on the basis of routine diagnostic imaging and hence warrant histopathological investigation of the specimen obtained post surgery. Surgeons must be careful in mapping the treatment options that heavily rely on the staging of the tumour.


We report a rare case of an incidental pancreatic lesion that proved to be Castleman disease in a peripancreatic lymph node, which mimicked a high-grade pancreatic neuroendocrine tumour (PNET) based on findings on positron emission tomography (PET). The disease was discovered as an incidental finding on CT imaging of the abdomen and was investigated and managed as PNET. Surgical resection was performed with distal pancreatectomy and splenectomy, however, histology revealed the lesion was a lymph node affected by Castleman disease. Often termed the great mimic, Castleman disease is a rare lymphoproliferative disorder that is often mistaken for other primary lesions of the organ due to its location and should be considered a differential of fluorodeoxyglucose (FDG)-avid PET lesions on imaging. Pancreatic lesions discovered incidentally after imaging are a frequent event and are occurring more often as the number of scans being performed is increasing. Accurate pre-operative diagnosis of these lesions is challenging due to the position of the pancreas in relation to the gastrointestinal and vascular structures surrounding it.Preoperative lesion characterisation is becoming essential, as our understanding of the biology of pancreatic incidentalomas increases. Lesions with a more benign-behaving trajectory can be managed by active surveillance, whereas more aggressive lesions should be managed by surgery.Of the malignant pancreatic neoplasms, 85% of lesions are of exocrine origin, the most common being adenocarcinoma . BecauseNuclear medicine scans have become critical in differentiating and staging PNETs . PNET tuThe stakes for the pre-operative diagnosis of incidental pancreatic lesions are further raised by the morbidity and potential mortality associated with Whipple’s procedure or distal pancreatectomy to treat these lesions ,7. SimilWe describe a case of pancreatic incidentaloma that proved to be Castleman disease involving a peripancreatic lymph node where the pre-operative diagnosis and treatment were directed by the PET scan results.The patient was a 61-year-old female who presented to the emergency department with a 10-day history of vomiting, diarrhoea and abdominal discomfort. The symptoms were attributed to a viral illness but a CT scan of the abdomen was performed to exclude other pathology. This revealed an incidental 2.8 cm lesion abutting the superior border of the pancreas and adjacent to the lesser curvature of the stomach  of the stomach, PNET, lymphoma, pancreatic adenocarcinoma and splenunculus were all considered. The patient was sent for an EUS that demonstrated a 21x11 mm lesion that appeared separate from the stomach and arising from the pancreas. The lesion was homogenous and hypoechoic without the classical appearance of a gastrointestinal stromal tumour. Fine-needle aspirate biopsies were suggestive of a lymph node with no other specific features. Given the patient’s gastrointestinal symptoms, serum vasoactive intestinal peptide, chromogranin A and gastrin levels were tested, all of which were within normal limits.Given the difficulty in determining the tissue origin of the lesion, a diagnostic laparoscopy was performed. The mass was a 2 cm, well-circumscribed lesion and appeared to originate from the superior border of the pancreas. Its macroscopic appearance was that of a neuroendocrine tumour. Pre-operatively, the patient was adamant she wanted the lesion removed if it was found to be arising from the pancreas. However, the laparoscopy occurred the day Australia locked down for coronavirus disease 2019 (COVID-19). We decided, because of the slow growth over a long interval and the uncertainty of the coming weeks, to not proceed to distal pancreatectomy or enucleation and perform a PET scan to further characterise.A D-PET scan was then performed Figures , 3, confFDG-PET was also performed Figures , 6, reveThese findings were discussed at the multidisciplinary team meeting and the diagnosis remained inconclusive. The provisional diagnosis was FDG-avid PNET. The patient proceeded to surgical excision of the lesion via laparoscopic distal pancreatectomy and splenectomy.The specimen was sent for histology and revealed that the lesion was Castleman disease affecting a lymph node embedded in the pancreas Figure .The patient has now had 12 months of follow-up and is recurrence-free.Often termed “the great mimic”, Castleman disease (CD) was first described in the 1950s in published case reports. Benjamin Castleman, after whom the disease is named, discovered this unusual lymphoproliferative disorder characterised by foci of hyalinisation in the follicles ,11.CD can be classified as either unicentric or multicentric, depending on how many lymph nodes are involved. The more common unicentric CD (UCD) is a disease with an unclear aetiology, and, unlike multicentric CD (MCD), is not associated with human immunodeficiency virus (HIV) or human herpesvirus 8 (HHV-8) . There aClinicians have attempted to describe the spectrum of appearance of CD on imaging. In one study, it is described as a well-circumscribed solitary lesion that enhances on contrast imaging . As we fDespite imaging findings similar to that described in the literature, there was a diagnostic difficulty in this case due to a number of factors. First, given the location of the node, the differential diagnoses favoured a pancreatic lesion rather than a nodal lesion. In the literature, it has often been described that CD lesions are found embedded within the parenchyma of an organ. Hence, this disease may emulate inflammatory or neoplastic lesions related to that specific organ on imaging, as seen in this case. For this reason, CD is frequently termed “the great mimic.” Additionally, even if a nodal lesion was suspected, the provisional diagnosis of FDG-avid lymph nodes without an obvious primary is usually B-cell lymphoma or other forms of lymphoproliferative disorders. Given the rarity of CD, it is seldom considered in the differential of such presentations -18.Fortunately, UCD has a high cure rate with complete resection, and surgery is the mainstay of treatment. The overall survival after 10 years with complete resections is greater than 95%, and failure to treat was associated with significant mortality . Often dTimely and accurate workup and management of incidental pancreatic masses is critical and remains a challenge for clinicians. Unicentric Castleman disease has often been misdiagnosed as a primary lesion of the organ of its location, largely because of the rarity of the disease, and this was also evident in the above-described case of pancreatic Castleman disease. Although rare, it should be considered a differential diagnosis in PET-avid lesions in the pancreas and other primary organ masses.


Angiosarcoma is a rare subtype of soft tissue sarcoma with identifiable vascular differentiation. It can occur at any age and develop throughout the body, but it is most commonly found in skin, soft, and breast tissues. Primary retroperitoneal angiosarcoma is rarely reported in the relevant literature. This article reports a case of primary retroperitoneal angiosarcoma in a middle-aged man, with the relevant literature reviewed in detail. A 46-year-old male had experienced left waist pain for 2 months. An ultrasonic examination revealed a mass in the left retroperitoneum, and left retroperitoneal lesions were confirmed via computed tomography (CT) and magnetic resonance imaging (MRI). The tumor was removed surgically, and the CT scan revealed local tumor recurrence after 1 month when the first adjuvant therapy was performed. The patient died of a massive hemorrhage from a ruptured tumor. Angiosarcoma has high malignancy and a poor prognosis. Its early diagnosis and treatment significantly impact the long-term survival rate of patients. These tumors are rare, with an incidence rate of only 0.0027% . Among t2A 46-year-old male patient was admitted to The Second Affiliated Hospital of Fujian Medical University with the main complaint of left waist pain for 2 months. Previously, he had been in good health, with no underlying diseases and no tobacco or alcohol addiction. A physical examination revealed no bulges in either kidney region, the kidneys were not palpable, and the kidney areas were without percussion pain. Traveling along the bilateral ureteral area, there was no tenderness or palpable mass. Furthermore, there were no obvious abnormalities in the patient’s routine blood and urine tests and biochemical results, while the blood catecholamine, cortisol, and aldosterone readings were normal. A ultrasonic examination revealed no abnormality in the bilateral kidneys and adrenal glands. No obvious signs of metastasis were found in the lung and liver on computed tomography (CT) examination.The summary of the ultrasonic examination is as follows. The left posterior peritoneum, which was at the height of the left renal artery and abdominal aorta, had an irregularly shaped hypoechoic area (4.3 cm × 4.8 cm × 3.6 cm) with a non-uniform internal echo . The col-45, melan-A, podoplanin, CD117, discovered on gastrointestinal stromal tumor 1, thyroid transcription factor-1, chromogranin A, and smooth muscle actin). The antigen Ki-67 index was approximately 50% , and the tumor may rupture and hemorrhage . The CT This case supports the view of several studies that a tumor size of more than 5 cm is associated with a worse outcome . The labThe CT examination of angiosarcoma reveals the following: (i) soft oval tissue masses, some of which may be lobulated; (ii) an unclear boundary with a density much lower than that of muscle; and (iii) possible hemorrhage, necrosis, and calcification occurrence inside the mass.The MRI examination of angiosarcoma cases reveals the following: (i) T1 weighted imaging (T1WI) produces an uneven low signal, and (ii) T2 weighted imaging (T2WI) produces a high-signal imaging that shows facilitated water movement and a scattered low signal. However, the gold standard for the diagnosis of angiosarcoma is pathological immunohistochemistry, with the expression of platelet endothelial cell adhesion molecule-1 or recombinant cluster of differentiation 34 (CD34) and factor VIII-related antigen (+) characterizing the disease ,12. SincRetroperitoneal angiosarcoma needs to be differentiated from the following diseases: (i) pheochromocytoma ; (ii) retroperitoneal lipoma ; and (iii) neuroblastoma, which is more common in children; the echo of the mass is much lower than that of angiosarcoma.Surgical resection is the main method used in the traditional treatment of angiosarcoma. However, the median overall survival of patients with resection of the lesion alone is only 9 months, and the 5-year survival rate is only 20%. For patients supplemented with radiotherapy and chemotherapy, the median overall survival rate can reach 36 months, with a 5-year survival rate of up to 45%. The main causes of death with the disease are recurrence  and distant metastasis (rate: 34%). A multidisciplinary treatment approach is always warranted, but despite multimodality treatment, the recurrence remains high even for localized tumors . Recent In summary, angiosarcoma is a rare, fast-growing, and highly aggressive tumor with a very poor prognosis that can occur in any part of the body. The early diagnosis of retroperitoneal lesions is particularly difficult. Clinically, ultrasound, CT, MRI, and other imaging examinations should be integrated. An image-guided needle biopsy is particularly important since it can provide an early diagnosis. To improve the long-term survival rate of patients, a prompt surgical resection of the lesion should be combined with targeted radiotherapy and chemotherapy.4Angiosarcoma has high malignancy and a poor prognosis. Its early diagnosis and treatment significantly impact the long-term survival rate of patients. This study focused on the imaging manifestations of angiosarcoma; however, there remains a lack of understanding of the disease’s operation mode, radiotherapy, and chemotherapy. Therefore, additional similar cases must be identified and their characteristics summarized to inform further research.


GnRH-independent precocious puberty (GIPP) can be the presenting clinical picture experienced by patients with secreting germ cell tumor (GCT). Indeed, as luteinizing hormone (LH) and human chorionic gonadotropin (hCG) share identical α-subunits and similar β-subunits, an increased secretion of β-hCG may result in a precocious activation of Leydig cells. Though the co-occurrence of raised β-hCG levels and signs of precocious virilization usually prompts a complete oncological work-up, the diagnostic and therapeutic management of GCT-induced GIPP may be challenging. We report the case of a 6.2 year-old boy presenting with clinical and biochemical findings consistent with GIPP . Brain imaging detected a bilobed cyst of the pineal gland, while serum and cerebrospinal baseline assessment initially ruled out raised alpha-fetoprotein or β-hCG levels. Nevertheless, a strict biochemical follow-up highlighted a fluctuant trend of tumor markers, with a more aggressive behavior and recurrent erections occurring as a result of unpredictable phases of raised testosterone and serum/cerebrospinal β-hCG, followed by sudden spontaneous decrease. Accordingly, a secreting pineal GCT was suspected. Given the fluctuating trend of tumor markers, surgery was initially kept on hold and a combined treatment with bicalutamide (androgen receptor blocker) and anastrozole (aromatase inhibitor) was undertaken in order to prevent the patient from experiencing further virilization and excessive bone age maturation. Subsequently, a progression in the size of the pineal tumor prompted surgical resection and a diagnosis of secreting GCT was histologically confirmed. Accordingly, the patient was started on adjuvant chemo- and radiotherapy. Antineoplastic treatment was followed by persistent and remarkable decrease of tumor markers and by a complete pubertal arrest. We reported the challenging diagnosis of a secreting pineal GCT in a patient with GIPP and a fluctuating trend of tumor markers, testosterone levels and associated clinical signs, hence prompting the indication for a systematic assessment and a strict monitoring whenever a patient with GnRH-independent precocious puberty shows clinical or radiological markers potentially consistent with a GCT. GnRH-Independent Precocious Puberty (GIPP) is defined as the development of secondary sexual characteristics before the age of 8 years in girls and 9 years in boys as a consequence of the exposure to either endogenous or exogenous sex steroids, independently of hypothalamic-pituitary-gonadal axis activation.spectrum of GIPP includes congenital adrenal hyperplasia, familiar male-limited precocious puberty (FMPP), McCune-Albright syndrome, β-human chorionic gonadotrophin (β-hCG)-secreting tumors, sex steroids-secreting tumors, aromatase excess syndrome, hypothyroidism, and exogenous exposure to androgens  approximately account for 3% of malignant neoplasms reported in children’s cancer registries . GCTs reThe clinical expression of Central Nervous System GCTs depends on the secreting profile, the site and the size of the neoplasm and commonly includes symptoms consistent with raised intracranial pressure, visual disturbances and endocrine disorders. In addition, gender plays a central role, as GCT-induced GIPP does not occur in females.We hereby report the challenging diagnosis of a pineal GCT in a 6 year-old boy who presented with GIPP and fluctuating levels of β-HCG in serum and cerebrospinal fluid (CSF), demonstrating that also mildly raised tumor markers in the setting of a clinical picture consistent with GIPP should raise the clinical suspicion of a secreting GCT and hence prompt a strict monitoring.A 6.2 year-old boy with an unremarkable previous medical history presented to his local hospital with persistent headaches and a single episode of vomiting. A brain MRI showed a 5 mm bilobed cyst of the pineal gland with a contrast-induced enhancement of the cystic walls and with radiological signs of communicating hydrocephalus.Four months later the patient was referred to our Pediatric Endocrine and Neuro-Oncology services due to the sudden development of penile enlargement, appearance of pubic hair and growth acceleration in the setting of a pineal lesion. Tanner pubertal staging was genitalia 3, pubic hair 3 and axillary hair 1, with testicular volume of 3 (left) and 4 (right) mL and penile length of 7.5 cm. No skin stigmata suggestive for McCune-Albright syndrome were noticed on examination.The discrepancy between testicular volume and advanced genitalia staging raised the clinical suspicion of GIPP, which was confirmed by the finding of both baseline and stimulated suppressed gonadotropins with significantly increased testosterone levels . A normal genetic sequencing for the LCGHR (luteinizing hormone/choriogonadotropin receptor) gene ruled out FMPP.assay: 0.2 IU/L) prompted the indication of a complete oncological work-up, in order to rule out a secreting germ cell tumor: chest CT scan, testicular ultrasound, abdomen and head/spine MRI and ophthalmological evaluation were unremarkable, except for the previously known pineal bilobed cystic lesion (2O), in keeping with the radiological diagnosis of communicating hydrocephalus. Nevertheless, neither increased AFP or β-hCG concentrations nor tumoral cells were detected in the CSF.Alpha-fetoprotein levels were negative at diagnosis . However, marginally raised serum β-hCG of 15 IU/L  led oncologists to keep surgery on hold and to continue a stringent radiological and biochemical follow-up. Both β-hCG and testosterone levels then remained within the normal ranges for about 2 months.Surgery was arranged, but a reassessment of tumor markers immediately before the start of antineoplastic treatment showed an unexpected spontaneous and complete shrinkage of serum and CSF AFP and β-hCG levels and of serum testosterone . The comDuring this time lag, given the progressive development of pubertal signs and the radiological finding of advanced bone age , the patAt the age of 7.3 years, a repeat brain MRI showed further increase in the size of the cyst. The patient underwent a surgical excision of the lesion. The histology was consistent with a GCT with a predominant mature teratomatous component (>95% teratoma), as well as a small (<5%) germinoma component, with positive staining for CD117, placental alkaline phosphatase and β-HCG on immunohistochemistry. The tissue was reviewed by three independent pathologists who confirmed the diagnosis.Following histological confirmation, the patient continued treatment as per the SIOP CNS GCT II trial for localized germinomas with alternating Carboplatin/Etoposide and Ifosfamide/Etoposide. After four cycles of chemotherapy there was no residual disease and the patient received whole ventricular radiotherapy (24 Gy in 15 fractions). Serum levels of testosterone, AFP and β-hCG normalized during treatment , as wellAfter surgical resection, a remarkable reduction in height velocity was also noted .ratio>1). Gonadotropin-releasing hormone (GnRH) analog therapy was therefore started.Luteinizing hormone and FSH were monitored regularly and remained undetectable until the age of 8.0 years. At the time, the patient presented with a mild increase in testicular volume and a repeat LHRH test confirmed Central Precocious Puberty  stimulation test, that confirmed a diagnosis of isolated GH deficiency (GH peak after insulin tolerance test: 5.64 ng/mL). The patient was therefore started on recombinant human GH treatment, with a satisfactory catch-up growth.The patient has been now off-treatment for 3 years and 5 months. He is continuing regular oncological surveillance and shows ongoing complete oncological remission. He is still on GnRH analog and recombinant human GH treatment and he is showing regular height velocity .We reported the challenging process that led to the diagnosis of a pineal mixed GCT in a boy who developed signs of GIPP before the age of 7 years.Precocious virilization often represents the first sign of a secreting GCT. As hCG and LH share identical α-subunits and similar β-subunits, human chorionic gonadotropin can stimulate testosterone production by Leydig cells as well, causing GIPP.The therapeutic value of a compound treatment with anastrozole and bicalutamide has been described in patients with familiar male-limited precocious puberty (FMPP) , 5. ReitPineal cysts frequently represent incidental findings, with reported prevalence ranging from 0.6 to 57% in radiologic and autoptic series and hence requiring neither diagnostic nor therapeutic interventions in most cases . It is sIt is not uncommon that patients with CNS GCT experience a time lag before the tumoral source can be identified. For instance, Crawford et al. reported delays in diagnosis ranging between 7 months and 3 years in a series of 30 patients with GCT . SimilarThe formal diagnosis of germ cell tumor is based whenever possible on histology. However, when the radiological appearances are highly suggestive of a GCT  and the tumor markers are significantly elevated in blood and/or CSF, it is also possible to make a clinical-radiological diagnosis of a secreting GCT and proceed with anticancer treatment.Secreting GCT are generally accepted to be malignant NG-GCT . HoweverThe prognostic value of β-HCG levels in germinomas is also unclear. Some studies suggest that germinoma patients with elevated β-HCG levels are at increased risk of relapse and show worse survival rates than those with normal β-hCG levels, whereas, other Authors challenge this hypothesis , 15.Noteworthy, our case highlights the potential highly fluctuating trend of β-hCG levels in the setting of a GCT-induced GIPP. The diagnostic contribution of β-hCG levels at first biochemical evaluation, just slightly above the reference range, may have been underestimated. Superimposable conclusions have been drawn by Arya and Davies, who reported a similar fluctuating trend in tumor markers in a 5-year child with GCT-induced GIPP .In our case, the combination of clinical and biochemical findings consistent with GIPP and the radiological appearance of the pineal gland prompted a systematical monitoring program that allowed us to detect several phases of remarkable contemporaneous rise in testosterone and serum/CSF β-HCG levels, followed by an unexpected spontaneous decrease. We hypothesized that recurrent hemorrhages occurring within the tumor may have caused the sudden and unpredictable release of tumor markers in the bloodstream. In addition, as the CNS is an immunological sanctuary, hemorrhages may have disrupted the integrity of the brain-blood-barrier, resulting in the exposure of otherwise undetected tumor antigens. It is likely that the immune system may have played a contributory role in modulating tumor growth and, accordingly, the release of β-HCG in the CSF and blood stream, resulting in fluctuating circulating levels. This hypothesis is drawn from the experience gathered in patients with testicular germinomas. Testes are immunologically privileged sites, but the exposure of neoplastic antigens following orchiectomy or diagnostic surgery often results in an immunologic over-reaction promoted by CD8+ and CD4+ lymphocytes .Only repeated withdrawals allowed the detection of β-hCG peaks that finally supported the diagnosis of a secreting GCT. As the fluctuations of tumor markers in the case presented were completely unpredictable, no clear recommendations about the timing of biochemical monitoring can be provided. Nevertheless, β-HCG and testosterone flares were always followed by overt worsening of penile erections and aggressive behavior, hence supporting a clinically guided biochemical assessment.Finally, the onset of CPP following GIPP may have different explanations. Firstly, intermittently raised testosterone levels experienced during GIPP can trigger CPP, irrespectively of the endogenous or exogenous source of androgens. Secondly, the patient underwent cranial radiation, that has been demonstrated as the most effective therapy in patients with malignant germ cell tumors . As a reIn conclusion, we hereby documented the case of a child with histologically confirmed pineal teratoma/germinoma with markedly raised β-hCG levels which normalized spontaneously. Hence providing evidence to challenge the concept that β-hCG levels can effectively discriminate germinomatous and non-germinomatous GCT and prompting the indication for a strict monitoring of biochemical tumor markers whenever a patient with GIPP shows a clinical or radiological marker potentially consistent with a GCT.Accordingly, it is not uncommon that patients with CNS GCT experience a remarkable time lag before the tumoral source is identified and the diagnostic suspicion is verified. In the case presented, a combined treatment with anastrozole and bicalutamide was administered as a bridge therapy in the attempt to control pubertal progression before histological confirmation and thus before antineoplastic treatment was undertaken.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. Written informed consent to participate in this study was provided by the participants’ legal guardian/next of kin. Written informed consent was obtained from the minor(s)’ legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.AC and AA conceived the idea of describing the present case report and critically revised its contents. AC drafted the manuscript and produced the pictures. AA critically revised the contents of the manuscript and approved the final outcome. Both authors contributed to the article and approved the submitted version.


The patient was diagnosed with primary extraskeletal osteosarcoma. It is necessary for multidisciplinary teams to diagnose malignant extraskeletal osteosarcomas.Extraskeletal osteosarcoma is a rare malignant soft-tissue sarcoma that is difficult to diagnose. Surgery is a common treatment, although chemotherapy and radiotherapy are also used. Patients at risk of bleeding can undergo embolization combined with resection. The occurrence of primary splenic extraskeletal osteosarcoma in humans does not seem to have been reported in the literature. A 50-year-old woman who complained of pain in the left upper abdomen for 1 day was initially diagnosed with “splenic hemangioma with a high possibility of rupture and bleeding” and urgently underwent digital subtraction angiography, combined with splenic arteriography and embolization. Abdominal pain worsened 2 days postoperatively, with a hemoglobin level of 106.0 g/L. Consequently, emergency laparotomy combined with splenectomy was performed. The clinical and imaging features, pathological diagnosis, and embolization treatment of this case were analyzed retrospectively. CT of the upper abdomen revealed splenomegaly, an irregular low-density shadow in the spleen, and a flake-like calcification in the lateral margin of the left kidney. Nuclear MRI of the upper abdomen showed splenomegaly and a mass (approximately 8.4 cm × 5.7 cm × 6.3 cm) below the spleen with clear boundaries—this exhibited an uneven signal, which was slightly low in T1-weighted imaging (T1WI) and slightly high in T2-weighted imaging (T2WI). Several small cystic lesions or cystic cavities were observed in the mass, which exhibited a longer T2 signal. During the enhanced scan, the signal of the lesion showed progressive enhancement, and the enhancement range increased in the delayed phase scan, as well as a hematoma below the spleen capsule and calcification below the lesion . Postoperative biopsy pathology showed splenic soft tissue tumor: at low magnification, the multinucleated giant cells were scattered; at medium magnification, osteoclast-like multinucleated giant cells were observed; and at high magnification, lace- or grid-like tumor osteogenesis was detected. Immunohistochemistry showed that the expression of CD31, CD34, F8, s-100, desmin, SMA, and CD99 was negative, whereas the expression of β-catenin, BCL-2, SATB-2, and P16 was positive. CD68 and MDM-2 showed low expression, while 50% of the cells were positive for Ki-67 expression. No abnormal concentration of radioactivity was found on the bone scan with  Extraskeletal osteosarcoma (ESOS) is a rare malignant soft tissue sarcoma, which is considered a subtype of osteosarcoma . ESOS haClinical features lack specificity, while some patients may be asymptomatic , 7. Whil9/L , the percentage of neutrophils was 86.9%, hemoglobin level was 114.0 g/L , C-reactive protein level was 60.82 mg/L , total protein level was 55.45 g/L, albumin level was 29.25 g/L, D-dimer level was 3.806 mg/L, and procalcitonin level was 0.28 ng/ml . CT of the upper abdomen showed a small amount of fluid effusion in the bilateral pleural cavity, splenomegaly with a low-density shadow in the splenic space, and a flake-like high-density shadow of the lateral margin of the left kidney, which was considered calcification.On September 2, 2021, a 50-year-old woman presented with left upper abdominal pain for 1 day. Physical examination showed that the left upper abdomen was slightly distended, abdominal breathing movement was present, the left upper abdomen had tenderness without rebound pain, the liver and spleen were untouched under the ribs, Murphy’s sign was negative, and no abnormal mass was palpable. Laboratory examination showed that the white blood cell count was 21.54 × 10Two days later, a nuclear MRI of the upper abdomen showed splenomegaly with a mass below the spleen. The spleen occupied a space of approximately 8.4 cm × 5.7 mm × 6.3 cm, with clear boundaries. A slightly low signal was observed in T1-weighted imaging (T1WI) and a slightly high signal in T2-weighted imaging (T2WI), and the signals were uneven Figure 2Intraoperative abdominal exploration showed the following: approximately 300 ml of blood accumulated in the abdominal cavity; the spleen was closely adherent to the periphery; the splenic hilum and the tail of the pancreas formed a mass and were adherent; and the lower pole of the spleen was enlarged, bleeding, and adherent to the splenic flexure of the colon, with no other abnormalities observed.Splenectomy was performed, in which the splenic flexure of the colon was fully freed from the lower pole of the spleen, and a hard mass could be palpated between the lower pole of the spleen and the left renal capsule. After dissection and ligation of the short gastric vessels, the splenic hilum was carefully separated, and the splenic artery and vein were successively dissected and doubly ligated proximally. The splenic hilum was dissected, and the spleen and the hard masses were removed.99mTc-MDP, further ruling out the occurrence of other bone tumors. Accordingly, the patient was diagnosed with primary ESOS.The splenectomy specimen showed a spleen size of 13 cm × 8 cm × 4 cm. Bleeding was observed on the excision of the spleen. Another free mass of 5 cm × 4 cm × 3 cm with partial calcification was observed, as well as a free clot of 18 cm × 10 cm × 4 cm with partial graying that showed a splenic soft tissue tumor  is the primary solid malignancy of the bone, which produces osteoid and/or immature bone from malignant mesenchymal cells . The mosCurrently, there is no clear indication of ESOS etiology . Its patESOS is usually treated by surgery, although chemotherapy and radiotherapy are being used. However, owing to the scarcity of cases, there is currently no standardized treatment plan. For tumors without distant metastasis, surgical resection is appropriate. For massive hemorrhage due to rupture, such as in the patient in the current report, DSA can also be used to identify the rupture and perform arterial embolization to stop the bleeding. Splenectomy should also be performed to ensure patient safety if bleeding cannot be completely stopped. According to a retrospective study of 370 ESOS cases, tumor size  and depth of tumor  were associated with systemic recurrence, and depth of tumor  and margin status  were associated with risk of local recurrence. Surgery combined with radiotherapy can reduce the risk of local recurrence , but chemotherapy cannot . Further, routine chemotherapy is not recommended for ESOS . For patThe primary ESOS of this patient showed a blood-rich mass of extraskeletal soft tissues with mixed signals and bleeding, but hemorrhage and hematoma are not uncommon in ESOS . AccordiReportedly, ESOS at rare sites has unusual presentations. Hunjan et al. reported a 76-year-old man with ESOS on his parietal scalp with heavy calcification. His clinical presentation resembled that of squamous cell carcinoma, and the lesion was largely asymptomatic . ShankarPrimary ESOS needs to be differentiated from the following diseases: i) myositis ossificans, which usually has a history of trauma and is considered to be related to abnormal repair after trauma; therefore, it is difficult to distinguish it from extraosseous osteosarcoma. Ectopic bone is formed in the soft tissue or muscle, and calcification can be observed. Lesion progression is often divided into early (<4 weeks), middle (4–8 weeks), and late (>8 weeks). Calcification is more obvious in the late stage and is more common in young men, often at the depths of soft tissues , 21. ii)In conclusion, although calcification in soft tissues is an important sign of this disease, bleeding, degeneration, necrosis, other tumors, tuberculosis, parasitic infections, and vascular disease in soft tissues can also lead to calcification. Therefore, it is important to explore the mechanisms of osteosarcoma and ESOS. ESOS is a rare malignant soft tissue sarcoma occurring in extraskeletal organs and soft tissues, with histologic similarities to primary bone osteosarcoma except for attachment to the bone or periosteum. The etiological mechanism is unknown, and the diagnosis is difficult, which mainly depends on a multidisciplinary comprehensive diagnosis confirmed by pathological biopsy. Surgical resection remains the primary treatment option.The original contributions presented in the study are included in the article/Supplementary Material. Further inquiries can be directed to the corresponding authors.The studies involving human participants were reviewed and approved by the Ethics Committee of Fujian Provincial Hospital. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.XP, H-LW, S-ML, J-LL, and D-DR acquired, analyzed, and interpreted the clinical data. XP drafted the manuscript. J-HZ, TC, J-WL, and Z-TF critically revised the manuscript. S-ML, J-WL, and Z-TF designed and supervised this study. All authors have read and approved the final manuscript.This work was supported by the Fujian Province Natural Science Fund Project , the Fujian Province Medical Innovation Foundation , and the Special Research Foundation of the Fujian Provincial Department of Finance , China.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.


Background and objectives: Dermoscopy is a useful tool for the early and non-invasive diagnosis of skin malignancies. Besides many progresses, heavily pigmented and amelanotic skin tumors remain still a challenge. We aimed to investigate by dermoscopy if distinctive morphologic characteristics of vessels may help the diagnosis of equivocal nodular lesions. Materials and Methods: A collage of 16 challenging clinical and dermoscopic images of 8 amelanotic and 8 heavily pigmented nodular melanomas and basal cell carcinomas was sent via e-mail to 8 expert dermoscopists. Results: Dermoscopy improved diagnostic accuracy in 40 cases. Vessels were considered the best clue in 71 cases. Focusing on the diameter of vessels improved diagnosis in 5 cases. Conclusions: vascular diameter in addition to morphology and arrangement may be a useful dermoscopic clue for the differential diagnosis of clinically equivocal nodular malignant tumors. Several lines of evidence demonstrate the value of dermoscopy in the diagnosis of pigmented and non-pigmented skin tumors ,2. BesidWe recently noticed by reviewing our database that heavily pigmented BCCs commonly exhibit blue-to-blue-black structures in addition to some large caliber vessels, whereas such vessels were never seen in heavily pigmented nMELs.The latter observation may be explained by another feature we noticed, namely, that amelanotic nMEL in contrast to nBCC or other types of low-risk nodular carcinomas  typically reveal dense, short and fine, polymorphic small caliber micro-vessels, which may be easily covered by the presence of heavy pigmentation in the case of nMEL. In contrast, even large amounts of pigmentation do not completely hide the classical large superficial macro-vessels of nBCCs.We tested our hypothesis by sending a collage of 16 challenging clinical images of 8 amelanotic and 8 heavily pigmented nodular lesions (8 melanomas and 8 BCCs) and their corresponding dermoscopic images . Only in 3 cases, a diagnosis of melanoma was associated with the observation of small diameter vessels as best dermoscopic findings. Prominent large caliber vessels were correctly considered best clue for the diagnosis of BCC in 4 cases. Focusing on the diameter of vessels improved diagnosis in 5 cases.The results of our survey are shown in Dermoscopic vessels features considered best diagnostic clue are listed in The use of dermoscopy in clinical practice has been shown to increase diagnostic accuracy both for pigmented and nonpigmented skin tumors ,6.While specific dermoscopic features are easily recognizable in most pigmented tumors, when assessing nonpigmented or hypopigmented skin tumors, reaching out for a diagnosis can be a challenge and the visualization of vessels, usually not visible to the naked eye, with the identification of a characteristic morphology can be the only key to the dermoscopic diagnosis.Moreover, regardless of pigmentation, the dermoscopic recognition of nodular malignant skin lesions is also difficult, due to the fact that the tumor often lacks the specific criteria for malignancy ,7.In 2001, the Board of the Consensus Net Meeting on Dermoscopy discussed a two-step algorithm for the classification of pigmented skin lesions .In 2010, a revised two-step algorithm has been described, taking into consideration polarized dermoscopy and vascular architecture .In the same year, a three-step algorithm for dermoscopic diagnosis of non-/hypopigmented skin tumors, including vascular morphology, architectural arrangement of vessels  and other optional criteria  has been proposed . Among tTo date, six morphologic categories of vascular patterns have been described in literature, including comma-like vessels, dotted vessels, linear irregular vessels, hairpin vessels, glomerular vessels and arborizing vessels . Except Moreover, it is necessary to remember that clinically flat and superficial tumors usually show different vascular morphology compared with their thick and nodular counterparts .Sometimes highly aggressive nMEL may be difficult to distinguish from low-risk nodular keratinocyte cancer, such as nBCC.Surgical excision is the first-choice treatment for both entities. Nodular basal cell carcinoma usually doesn’t need an urgent referral, whereas in case of nMEL, early recognition and surgical removal is key for maximizing survival outcomes of patients.Based on our personal clinical experience and according to our preliminary results, the observation of vessels caliber and density by dermoscopy may help in distinguishing tumors with a longstanding progressive and indolent growth  from tumors with aggressive behavior .When dermoscopy of nodular non-/hypopigmented lesion reveals small caliber vessels, usually with high density, a suspicion of highly aggressive malignancy should be considered in order to perform a biopsy and obtain a prompt histological diagnosis. Indeed, we found that non-/hypopigmented nodular melanoma typically shows densely packed vessels of small diameter , whereasA similar situation can be observed confronting nodular poorly differentiated squamous cell carcinoma (SCC) with keratoachantoma, that are typically raised non pigmented skin lesions. Hairpin-like vessels occur more frequently in well differentiated SCC (such as keratoacanthoma) . They usIn case of pigmented nMEL, structureless blue pigmentation, areas of pink and black colors and/or ulceration can be the clues for the diagnosis. Vascular structures, when visible, are located at the periphery of the lesions as polymorphous vessels and milky-red globules  Figure .Pigmented nodular BCCs are instead characterized on dermoscopy by blue-grey ovoid nests or blue-grey dots/globules usually associated with arborizing vessels . At the In both cases, based on our personal experience, in the absence of any specific dermoscopic features, an aggressive behavior  should be hypothesized when on dermoscopy the lesion exhibits higher density of vessels of small diameter. Conversely, an indolent course  should be assumed for tumors with lower density of larger caliber vessels, typically visible also in presence of heavy pigmentation.These considerations could be useful in the triage setting of equivocal and suspicious hypo-/hyperpigmented nodular lesions, in order to identify those requiring prompt biopsy.These preliminary results suggest that vascular diameter in addition to morphology and arrangement may be a useful dermoscopic clue for the differential diagnosis of clinically equivocal nodular malignant tumors.Based on our experience, dense and small caliber vessels in nodular lesions are suspicious for high malignancy , whereas larger superficial vessels with low density suggest a locally aggressive behavior.These preliminary observations on the role of vascular diameter as a new diagnostic dermoscopic clue of malignancy should be validated in a prospective study, collecting a large series of nodular lesions. The differentiation of clinically and/or dermoscopically equivocal pigmented and non-/hypopigmented nodular BCCs and melanomas is indeed important especially in order to expedite surgical treatment of aggressive tumors and provide the best care, as early as possible.


Trichilemmal carcinoma (TC) is a rare skin malignant tumor with pillar differentiation. TC presents along with other malignant hair follicle tumors and accounts for only 1% of all adnexal carcinomas. TC usually occurs on sun-exposed skin in elderly people, nevertheless, it can occur at any age. We report a case of trichilemmal cyst carcinoma in a 54-year-old woman presenting with an increasing occipital cyst. A histological examination confirmed the diagnosis and a large excision was performed. Despite the absence of a well-defined consensus on the management of TC, surgical excision with adequate margins seems to be safe in the absence of metastatic lesions. However, in the case of second localization, chemotherapy could be initiated, but again, in this case, no consensus on the appropriate protocols exists.  Trichilemmal carcinoma (TC) is a rare tumor that usually occurs on sun-exposed skin, especially on the face, scalp, neck, and back of the hands. They are usually seen in elderly subjects (commonly between the 4th and 9th decades of life) but can develop at any age . The exaA 54-year-old White North African woman, with no medical history, reported a nodule on her scalp which was discovered by herself six months ago. A partial cyst exeresis was performed with a pathological diagnosis of TC with positive lateral and deep surgical sections. The patient was then referred to our oncological and general surgery department for further care. The clinical examination noted a tumefaction of the scalp located in the occipital region with the absence of a second location Figure . CerebraA large tumor exeresis was performed two months after the first excision Figure . The hisTC is a rare tumor that usually occurs in sun-exposed areas in elderly persons with no apparent predilection for either sex . Though Histologically, TC develops from the external epithelial sheath of the hair root with a well-limited and infiltrating lobule proliferation comprising large cells with a clear cytoplasm related to the accumulation of intra-cytoplasmic glycogen. Areas of hemorrhage and/or necrosis can be seen in large size tumors. Therefore, the mitotic activity of the cells is intense with several signs of atypia, and they also colorize very well on periodic acid-Schiff (PAS) stains . On immuThe treatment is exclusively surgical, although no consensus on managing TC is available . Simple TC generally has a good prognosis; reports of local recurrence cases are uncommon ,14. The Despite the absence of a well-defined consensus on the management of TC, surgical excision with adequate margins seems to be safe and effective in the absence of metastatic lesions. In the case of second localization, chemotherapy could be initiated, but again, in this case, no consensus on the appropriate protocols exists.


Supernumerary Nostril, also called triple nostrils or accessory nostril, is a rare congenital nasal malformation.We report one conceal case of supernumerary nostril in a 19-years-old men which is misdiagnosed to a simple small nasal skin pit. Ordinary surgical excision led to recurrent infection of the lesion postoperatively, and was eventually required secondary surgery and the lesion was finally confirmed by pathological biopsy as a trinasal nostrils.Through this case, we stress the essential role in differential diagnosis, confirming the diagnosis and seeking for better solutions.Level of Evidence V Supernumerary nostril is a rare congenital defect, with approximately 60 related cases reported since Lindsay was first reported in 1906 [A 19-year-old man came to our department because of a small pit on the nasal dorsum which made him look different Fig. , but theWithin 1-year post surgery, the incision area was repeatedly infected with subcutaneous abscess. CT scan with contrast medium infused into the sinus showed that there was no obvious deformity in the nasal bone and skull base. The diameter of the sinus is about 3 mm wide and its depth is about 2 cm. And, the sinus and the nasal cavity are not connected (Gif.1). We concluded with the diagnosis of a supernumerary nostril.Design: The surgery was designed with an Inverted-V shaped incision on the columella and extended to the nasal cavity along the lower edge of the alar cartilage Fig. . The skiThe patient’s condition was generally good after the surgery, and the suture was removed on the 7th day, the incision was recovered well, and appearance of the nose was satisfying after half a year Fig. , and eveThis is an interesting case because the accessory nostril was observed only as a small pit on the nasal dorsum skin which differs in its appearance from all the other previously reported cases. Although, it was successfully treated finally. We still need to know about the multiple appearences of the supernumerary nostril for better treatment.Supernumerary nostril is a rare congenital defect that defined as the presence of additional nostrils when a pair of normal nostrils already exist, and may be manifested as asymmetric redundancy and differ from complete nasal duplication in horizontal or vertical . The exaSome of the cases are one of the manifestations of the multiple malformations, while others are simple multiple nostrils that show the presence of distinct cavities or localized bulges, totally different from the appearance of a normal nose –6. None First of all, pay attention to the personal history of patient, along with the symptom of the disease, as many of the cases are reported to be associated with multiple congenital malformations. Second, imaging examinations such as CT scan are necessary, helping us to exclude the presence of other problems . And if This case actually belongs to congenital nasal malformation, which must be distinguished from encephalocele, nasal dermal sinus, nasolacrimal duct obstruction, mid facial cleft and proboscis lateralis , 9. The Due to its rarity, the exact timing of the intervention and the treatment modality have reached no consensus. Mostly, the literature favors early surgery to ameliorate the medical risks it may pose such as psychosocial disorders or any derangement to the nasal , 11. In In this study, we presented a concealed case of supernumerary nostril.manifested as a small pit located on the nasal dorsum in a male adult. We made final diagnosis based on the clinical appearance, physical examination and histological features of the supernumerary nostrils. As described above, the cause and development of this disease is still unclear. However, we advocated early surgical treatment and suggested to handle similar situations with the columellar Inverted-V incisions. Early treatment is recommended to prevent any possible alar or nostrils deformity, as well as avoiding psychological problems.


Hypertrophic Pyloric Stenosis (HPS) represents a relatively rare occurrence beyond infancy. Here, we present the case of a barely 3-year-old boy diagnosed with late-onset HPS and successfully treated with extra-mucosal pyloromyotomy. We review the literature, challenging the principle that more aggressive surgical approaches should be preferred over less invasive ones. Hypertrophic pyloric stenosis (HPS) represents the most common cause of gastric outlet obstruction (GOO) in infants, being a rare occurrence beyond infancy.Here, we present the case of a barely 3-year-old child, who was admitted to the Emergency Department with symptoms of persistent non-bilious vomiting over the past 15 days. Abdominal ultrasound demonstrated thickening of the pylorus, while an upper GI series revealed obstruction to gastric emptying.Endoscopic balloon dilation (EBD), despite providing symptomatic improvement, failed to normalize gastric emptying. He was treated with extra mucosal pyloromyotomy, the gold standard of treatment in infantile forms, with a good outcome and fast recovery.After reviewing the literature, we found that conservative pyloric surgery, which represents the gold standard treatment for infantile forms, is also effective and safe in late-onset forms of HPS and should be preferred over more invasive surgical approaches whenever possible.A 2-year-and-11-month-old male was admitted to the emergency department for persistent non-bilious vomiting episodes, which had started 15 days before, and became more frequent over time. Episodes typically occurred 4–5 h after his meals and were preceded by hypersalivation and rumination. A weight loss of 3.5 kg had occurred over the last 4 weeks, while bowel function had remained regular. One month before, he had suffered from a self-limited upper respiratory tract infection. Past medical and family history was otherwise unremarkable.shoulder sign), a narrow string-like pyloric channel (string sign), and delayed gastric emptying, with only a modest amount of contrast medium passing through pylorus over the next 60 min. An urgent CT scan was performed to exclude extrinsic causes of gastric outlet obstruction (GOO), showing an elongated pyloric canal (approximately 20 mm) without a visible lumen and a thickened wall . These measurements were later confirmed by an abdominal MRI . Within 2 h of being admitted to the hospital, however, the patient presented some projectile, non-bilious vomiting. An abdominal X-ray showed significant gastric distension, with no signs of pneumoperitoneum, while an abdominal ultrasound (US) showed a thickened pylorus with a single-wall thickness of 7 mm and a length of 16 mm. An upper GI series  revealedinal MRI .Helicobacter pylori infection and showing no inflammatory infiltrate. The patient was started on PPIs and alginate, and 48 h after the procedure, a normal-caloric liquid formula was administered with good tolerance.A nasogastric tube was placed, and the patient was started on parenteral nutrition. Upper endoscopy was performed 48 h later, showing distal esophagitis with no evidence of gastric ulcers, mucosal webs, or foreign bodies. Endoscopic balloon dilation (EBD) was attempted, and biopsies were performed, testing negative for Despite significant clinical improvement, with no more episodes of vomiting reported, the post-operative GI series performed on the 5th post-operative day still showed delayed gastric emptying (complete emptying requiring approximately 4 h) and the persistence of a narrowed pyloric outlet. The patient was, therefore, referred to surgery. A laparoscopic extra mucosal pyloromyotomy was performed with prompt normalization of transpyloric transit time  and good tolerance to refeeding 24 h postoperatively.Over the subsequent 6 months, the patient has shown good clinical conditions with no more episodes of vomiting, or other relevant symptoms reported during follow-up evaluations. Weight loss was completely recovered over the first 30 days from hospital discharge.Gastric outlet obstruction refers to a group of heterogeneous conditions that prevent or delay the passage of gastric content into the duodenum . It occuHypertrophic pyloric stenosis is, by far, the most common cause of gastric outlet obstruction (GOO) in young infants, with an incidence of 1–4 in 1,000 live births . It is dcongenital ; secondary ; or primary acquired (Jodhpur disease), characterized by defects in pyloric motility with no hypertrophy . These ertrophy –9.HPS beyond infancy is a rare occurrence , 10, 11.If GOO is suspected, an early patient assessment should include blood gas analysis, electrolytes, and fluid resuscitation; placement of a nasogastric tube allows for prompt gastric decompression. Abdominal ultrasound is the first-line examination , allowinWhen it comes to late-onset HPS, the data in the literature are conflicting, and several different procedures have been reported  Table 1Table 1.Boybeyi et al. suggested a possible treatment algorithm, indicating gastrectomy followed by Billroth I reconstruction whenever two consecutive EBDs attempts have failed or in those circumstances in which EBD cannot be performed . The ratOther authors underline some other limitations of less invasive procedures, such as the inability to visualize the gastric and duodenal mucosa, or the impossibility to obtain large histologic specimens , 26, whiSome studies reporting on gastric emptying after conservative pyloric surgery seem to agree that conservative pyloric surgery produces no alterations in gastric emptying or intragastric distribution of meals and does not increase the risk of complications, such as gastric and duodenal ulcerations , 28.In our case, gastric emptying did not improve significantly after EBD but underwent complete normalization after pyloromyotomy, with no return of symptoms after 6 months from the procedure.The case report herein presented shows that a high degree of suspicion for HPS should be maintained even beyond infancy, to promptly identify and treat it.Endoscopic balloon dilation represents a relatively safe, minimally invasive first-line approach in late-onset forms of HPS. As to the surgical approach to be adopted in refractory forms, the evidence is still insufficient. Pyloromyotomy is the gold standard treatment in infants. However, its efficacy and possible complications in older patients are yet to be established. The adoption of more invasive strategies, including distal gastrectomy and subsequent Billroth I reconstruction, should be decided on an individual basis and should be reserved for those cases, in which: (a) there are doubts on adequate pyloric motility and functionality ; (b) pyloromyotomy/pyloroplasty is made technically difficult by anatomy or by the thickness of the pylorus; (c) full-thickness biopsies are required to exclude other diseases, such as neoplasms; or (d) more conservative approaches have failed, or relapses have occurred.The original contributions presented in this study are included in the article/supplementary material, further inquiries can be directed to the corresponding author.Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. Written informed consent was obtained from the minor(s)’ legal guardian/next of kin for the publication of any potentially identifiable images or data included in this article.OI and GV drafted the initial manuscript, and reviewed and revised the manuscript. GM and MW carried out the surgical procedure and critically reviewed the manuscript, providing important insights concerning the surgical aspects of the cases. SC and FC collected the data, conducted the endoscopic study, and reviewed and revised the manuscript. RF and PG contributed to the conception and design of the report and wrote sections of the manuscript. SP conducted the radiological studies, selected the images, drafted their description, and critically reviewed the manuscript. All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work.


Erection transforms the penis from a safe, flaccid organ to a susceptible one. During an erection, the thick tunica albuginea thins and becomes fracturable. Penile fracture (PF) is a very uncommon ailment produced by a blow to the erect penis. Unphysiological bending of the erect penis during sexual activity or masturbation is the most often reported mechanism of trauma. The penis is made up of three columns of erectile tissue: one ventral corpus spongiosum and two dorsolateral corpora cavernosa, each enclosed by the tunica albuginea. The tunica albuginea is a bilaminar structure composed of collagen and elastin. The outer layer of the tunica determines its strength and thickness. It varies in several locations across the shaft and is thinnest ventrally. It has high tensile strength and can sustain rupture at intra-cavernous pressures of up to 1500 mmHg. The tunica albuginea's thickness decreases from 2 mm to 0.25 mm while the penis is erect, and a trauma-induced rise in intracorporeal pressure during an erection might easily cause rupture. PF with urethral bleeding is a very rare urological emergency. One of the common causes of PF includes vigorous sexual intercourse. Symptoms include a cracking, snapping, or clicking sound followed by an instantaneous detumescence. Additionally, the penis may exhibit acute discomfort, significant ecchymosis, rapid swelling, and noticeable eggplant deformity. This is a case report of a 30-year-old male who presented with a history of penile swelling and ecchymosis during sexual intercourse. There was blood spotted at the urethral meatus. A retrograde urethrogram showed complete disruption at the proximal third of the urethra. The patient was immediately taken for surgery, and extensive exploration was done. There was a significant defect of the tunica albuginea of the corpora cavernosa on the ventral side of the penis. Along with the defect and the PF, there was a sizeable urethral injury as well. The defect of the tunica albuginea of the corpora cavernosa was repaired with a Prolene 2.0 suture , and urethral reconstruction was done with Vicryl 3.0 interrupted suture  over a 14-Foley catheter. Ultrasonography (USG) can be used to assess patients who have suffered penile injuries as well as to determine the sort of incision that is necessary, saving time by preventing needless waiting. This instance emphasises the value of early diagnosis in cases of unique presentation and early surgical treatment for improved results. Penile fracture (PF), a rare urological emergency, must always be treated immediately. In 9% to 20% of cases, urethral trauma may be present. PF and urethral injury are related, particularly in a suggestive physical examination and medical history findings such as severe urine retention and urethral bleeding. Immediate primary surgery is the safest and most efficient method for treating urethral injury and PF simultaneously . The mosSurgery must be performed immediately. Typically, a catheter serves to recognize the urethral route and protect it from being injured by the dissection performed to treat the PF. In this case report, the patient had a PF and urethral bleeding, which required urgent surgical attention and a Foley catheter to stop the bleeding. A Foley catheter is a flexible tube that professional medical inserts through the urethra and into the bladder to drain urine ,3.A 30-year-old male patient presented to the emergency department with a history of penile swelling and ecchymosis during sexual intercourse in a "woman-on-top" position. He gave a history of clicking/snapping sound followed by abruptly sharp pain. He immediately lost his erection after experiencing pain. He had blood coming out of the tip of his penis. Moving and urinating were some of the factors that aggravated the pain. There was no history of hypertension, diabetes mellitus, or prior surgeries. He denied having any sexual disorders or using phosphodiesterase-5 (PDE-5) inhibitors in the past. On examination, eggplant deformity of the scrotum and penis was also noted. There was blood spotted at the urethral meatus. A retrograde urethrogram showed complete disruption at the proximal third of the urethra. All this confirmed the diagnosis of PF with urethral bleeding. All other investigations such as complete blood count (CBC), kidney function test (KFT), and urine analysis were normal. Urgent surgical treatment was required. The patient was immediately taken up for surgery. Extensive exploration was done using subcoronal degloving incision. This incision was used due to its better long-term recovery and because it is better cosmetically. On exploration, there was a large defect of the tunica albuginea of the corpora cavernosa on the ventral side of the penis. Along with the defect and the PF, there was a large disruption of the corpus spongiosum as shown in Figure The defect of the tunica albuginea of the corpora cavernosa was repaired with a Prolene 2.0 suture , and urethral reconstruction was done in two layers with a Vicryl 3.0 interrupted suture  and a 14-Foley catheter as shown in Figure During the hospital stay, low molecular heparin  and broad-spectrum antibiotics were administered. On the third postoperative day, nocturnal erections returned and were treated with diazepam. The patient improved well postoperatively without any complications. Penile oedema, a result of surgery and injury, subsided in three days, and the catheter, which was used to correct the deformity, was removed after three weeks. No recurrence has been noticed in the monthly follow-ups in the first year followed by quarterly follow-ups for the next two years.Erectile tissue is organized into a columnar pattern to form the penis. One ventral corpus spongiosum and two dorsolateral corpora cavernosa, each surrounded by the tunica albuginea, form the penis. Collagen and elastin make up the bilaminar structure known as the tunica albuginea. The tunica's strength and thickness are determined by its outer layer. It varies throughout the shaft at different places and is the narrowest ventrolaterally. It has exceptional tensile strength and can withstand rupturing up to an intra-cavernous pressure of 1500 mmHg. When the penis is erect, the tunica albuginea's thickness reduces from 2 mm to 0.25 mm, and a trauma-induced spike in intracorporeal pressure during an erection might easily cause rupture  7]..7]. In 50% of instances, the "woman-on-top" position was seen, followed by "doggy style." Contrarily, Barros discovered that bilateral fractures of the corpus cavernosum and urethral lesions were more commonly related to the "doggy style" (41%), and the "man-on-top" (23%) positions in coitus, which were the most prevalent causes of PF. These investigations showed that the relative risk of PF was affected by the sexual position. Coital dysfunction, urethral fistula, penile plaque, and erectile dysfunction were side effects of the damage . Prompt Up until the early 1980s, PF management had generated a great deal of controversy. Significant complications include delayed chordee, coital difficulties, arterial-venous fistulas, penile plaque, erectile dysfunction, penile curvature, nodules, and painful erections, which have been discovered to be substantially more common and can happen in up to 30-53% of patients and can be very severe. They were linked to conservative therapy. Direct incision, degloving incision, and inguinoscrotal incision are currently available surgical approach choices . ExcelleUltrasonography can be used to assess patients who have suffered penile injuries and determine the sort of incision necessary, saving time by preventing needless waiting. Patients who had delayed therapy had a residual fibrous area and a small curvature of the penis during erection. Regular sexual function was, nevertheless, achievable. Due to urinary extravasation and fibrous tissue, late or postponed surgery had less favorable outcomes. This fibrous tissue growth reduces the corpora's flexibility and extendibility, resulting in erectile dysfunction or penile curvature. Early diagnosis and treatment reduces the risk of postoperative erectile dysfunction. According to a study, 96% of patients who received surgical treatment and 50% of patients who received conservative treatment reported normal sexual activity with no penile curvature, the remaining 50% of the conservatively treated group experienced erectile dysfunction and penile deviation. This instance emphasizes the value of early diagnosis and surgical intervention over a conservative approach in cases of unique presentation and early surgical treatment for improved results .A highly uncommon occurrence called PF with urethral haemorrhage necessitates rapid surgical intervention. It most frequently happens during sexual activity. A snapping or cracking noise was the typical sign, followed by an instant detumescence and excruciating agony. An immediate surgical procedure that included penile degloving and surgical repair had a positive outcome. A Prolene 2.0 suture was used to repair the defect of the tunica albuginea of the corpora cavernosa, and urethral reconstruction was carried out with a Vicryl 3.0 suture over a 14-French Foley catheter. The patient recovered well after surgery, and no recurrence has been noticed in the monthly follow-ups in the first year followed by quarterly follow-ups for the next two years. Early surgical repair was carried out in our situation, resulting in a smooth recovery. This case report aims to further the field of medicine by offering guidance to doctors looking for experience treating this uncommon disease.


We present a case of a rare complication in a 10-month-old female referred to our institution for an anal stricture after primary cloacal repair as an infant. Multimodal imaging, careful physical exam, and endoscopic evaluation revealed her vagina had been pulled through to the location of her anal sphincter muscle complex. We describe the correction of this problem, including identification of her rectum. Anorectal malformations (ARMs) can have a wide spectrum of anatomic complexity, and clear preoperative imaging is key to planning and executing an anatomic reconstruction. Common postoperative complications of ARM repair include rectal prolapse, remnant of the original fistula, anal or rectal stricture, wound dehiscence, and recurrent fistula.A 10-month-old female was referred to our center for an anal stricture. She was diagnosed as an infant with a short channel cloaca. After initial colostomy and mucous fistula creation, she underwent a posterior sagittal anorectoplasty at an academic children's medical institution, with mobilization of what was thought to be the rectum. She reportedly did not require any surgical correction of the genitourinary sinus component of her cloaca.On our exam, the urethral meatus was hypospadic, 0.5 cm inferior to the convergence of the corpora. Cystoscopy showed a normal urethra and bladder, but we could not identify the vagina. The perineum had an opening within the muscle sphincter complex, previously described as the anus that was severely strictured for a length of 1.5 cm, accommodating only a size 4 Hegar dilator.A distal colostogram  showed Based on our preoperative studies, we started the surgical reconstruction with a laparoscopic mobilization of the rectum and dissected out the rectovaginal fistula but left it attached to the posterior vagina. Inspection of the pelvis revealed an atretic hemiuterus on the left side, which was resected with the Fallopian tube, while preserving the left ovary. The right side had normal anatomy. After sufficient length on the rectum was obtained, the patient was then positioned prone. Via a posterior sagittal incision, a full-thickness mobilization of the native vagina around the “anoplasty” was performed. The vagina, once mobilized, was then placed at the introitus abutting the posterior urethra and urethral meatus. The rectum was detached from the back of the vagina, mobilized, and brought down into the anal muscle complex to create the anoplasty. The perineal body was then reconstructed. The orthotopic urethral meatus was visible, allowed for easy catheterization, and did not require any mobilization. The accessory urethra appeared blind ending, as it approached the glans clitoris. This will be observed with consideration of excision if clinically symptomatic.Evaluation of an ARM patient who is not doing well postrepair starts with obtaining a detailed history including symptoms, current and past medical therapies, and surgical interventions.At our institution, endoscopy is routinely performed for patients with complex malformations to understand the anatomy of the bladder, ureters, urethra, vagina(s), and common channel (in the case of a cloaca), as well as to visualize the location and height of the distal rectum. Anomalies of the genital tract such as a vaginal septum, bicornuate uterus, or uterine didelphys may be diagnosed.Three-dimensional reconstructed fluoroscopy or cloacagram is performed to obtain the lengths of the common channel and urethra and to assess spatial relations of the rectal fistula and other structures relative to the pelvis.Two months after her reoperation, this patient's colostomy was closed. At her follow-up appointment 9 months postoperatively, she was experiencing mild constipation, which was well managed with stimulant and mechanical laxatives.This case demonstrates a rare complication that occurred during what was thought to be a cloacal reconstruction in which the vagina was pulled through as the anoplasty. Multimodal imaging allowed us to delineate the anatomy and plan a successful reconstructive strategy to ensure a repair that maximized the patient's potential for fecal continence as well as gynecologic function. This case is an important reminder of the need for correct and comprehensive preoperative imaging and anatomical evaluation prior to performing a reconstruction for an ARM.


Neuroendocrine tumors (NETs) are neoplasms that arise from cells of the endocrine and nervous system. NETs, often found in the gastrointestinal tract, can be found anywhere in the body, and have metastatic potential. NETs occasionally present with metastatic disease without an identifiable primary tumor.A 79‐year‐old female patient presented with an abdominal wall mass. Percutaneous biopsy was consistent with a NET. Preoperative endoscopy and PillCam were unremarkable. PET Dotatate demonstrated uptake in the abdominal wall as well as vague uptake in the pelvis. Intraoperatively, we identified a suspicious nodule on the sigmoid colon, which was consistent with a drop metastasis on final pathology.In this case report we present a patient with a NET metastasis to the abdominal wall without a known primary site. This case highlights the limitations of endoscopy and imaging in the workup of metastatic NETs. Additionally, this is a novel case report of a metastatic NET to the abdominal wall without an identifiable primary site. Chromogranin A, mTOR, CDKN1B, and circulating tumor cell levels have all shown prognostic value for patients with NETs.Surgery is the mainstay of treatment for resectable NETs and also has a prominent role in metastatic NETs as it can improve survival.Historically, exploratory surgery has been useful to ultimately identify the primary tumor in cases that were unidentifiable on initial workup.2A 79‐year‐old female patient was referred to surgery for evaluation of a suspicious periumbilical mass with vague abdominal discomfort. She had previously been seen by hepatology for liver cysts and was followed with surveillance CT, which incidentally identified a 4 cm abdominal wall mass. She had no other pertinent medical history.Follow up ultrasound, colonoscopy, and MRI further characterized a 3.8 × 4.1 × 2.5 cm hypoechoic abdominal wall mass  Dotatate imaging demonstrated uptake in the abdominal wall as well as uptake in the pelvis adjacent to the sigmoid colon  low grade histology, 2) primary tumor drained by the portal system , 3) tumor burden involving <50% of the liver, and 4) at least 6 months of documented stable disease prior to consideration for transplant.Treatment guidelines for metastatic NETs of unknown primary tumor location are not well‐established. When histopathology is consistent with well‐differentiated disease, treatment typically involves surgical resection. In such cases, management still often incorporates systemic therapy involving somatostatin analogues, immunosuppression, and/or chemotherapy.Multiple case reports have similarly characterized NET metastases to various intramuscular locations; however, in each of those cases a primary NET was identified. The site of metastasis in these cases was most commonly to the lower extremities and the primary tumor location was most frequently identified in the ileocecal region.The combination of preoperative imaging with PET Dotatate and exploratory surgery has dramatically improved identification of the primary NET tumor location.A recently developed gene assay has shown early promise in identifying NETs with an unknown primary site. The 92‐gene assay identifies a molecular determinant of NET tumor type/subtype. Specifically, the molecular assay classifies NETs into distinct subtypes, such as pancreas , skin , lung , thyroid  and adrenal gland  tumors.This case report describes a unique presentation of a metastatic NET with an unknown primary and highlights the limitations of PET Dotatate imaging in the workup of metastatic NETs. Long‐term follow up of our patient as well as other patients with unknown primary NETs will hopefully elucidate optimal management approaches for future such NETs patients.4NETs can present myriad clinical challenges due to diagnostic limitations as well as the variability of disease manifestation. Current diagnostic workup of NETs includes PET dotatate imaging, pan‐endoscopy, and surgery; however, this approach does not always localize the primary tumor. Our case report highlights such a clinical conundrum and describes how we proceeded with management. Our patient underwent complete resection of all identifiable gross disease followed by adjuvant treatment with a somatostatin analogue. Her ultimate outcome will likely be driven by her disease biology, as prognosis is highly impacted by histologic grade in NETs. The expression “Biology is King” is often invoked in cancer care. NETs are exemplary in this respect, as well‐differentiated NETs can often behave indolently when compared to poorly differentiated NETs. As diagnostic and therapeutic modalities continue to evolve, hopefully the prognosis of patients with NETs will continue to improve as well.The authors have stated explicitly that there are no conflicts of interest in connection with this article.The Piedmont Healthcare Institutional Review Board has determined this project does not meet the definition of human subject research under the purview of the IRB according to federal regulations.Olivia Jagiella‐Lodise: Data curation ; writing – original draft . Valerie Jagiella: Conceptualization . Evan Weitman: Conceptualization ; formal analysis ; supervision ; writing – original draft ; writing – review and editing .


Surgical treatment of gynecological disease with colorectal involvement is a challenge which requires complete surgical excision of lesions.Method: A step-by-step video demonstration of the techniqueResult: A 53-year-old woman with a history of dysmenorrhea had abdominal distention for 2 months. PET-CT showed that the uptake of fluorodeoxyglucose in the surrounding peripheral rim of soft tissue masses was elevated. Laparoscopy showed severe adhesions in the pelvic cavity, which might be caused by malignant tumor and endometriosis. It is difficult to expose the Douglas pouch due to the dense adhesions with poorly defined margins posteriorly to the uterus. Therefore, the surgeon decided to open the Douglas pouch finally. After coagulating the ligaments, pushing the bladder, and incising the vaginal wall, the outline and margin of the lesion was exposed so that the lesion could be cut off safely. Omentectomy, appendicectomy, partial peritonectomy, pelvic and para-aortic lymph node resection were performed. The neoplasm in the right ovarian cyst was clear cell carcinoma, and the lesion on the surface of the rectum was endometriosis. The patient recovered well without any complications and defecated 3 days after surgery. She is still alive up to now.Conclusion: Our video showed that a flexible change in laparoscopic surgical procedures could overcome difficulty safely and quickly. Compared with the traditional type, retrograde hysterectomy can recognize the margin of a lesion, and is a safe alternative for patients with dense adhesion around the uterus.