A halo phenomenon describes a skin neoplasm that is surrounded by a hypopigmented or white halo. Halo lesions have been observed in association with an epithelial neoplasm (seborrheic keratosis), a fibrous lesion , a keratinocyte malignancy , melanocytic neoplasms, and vascular lesions. Benign lesions (café au lait macules and nevi) and malignant tumors (primary and metastatic melanoma) are melanocytic neoplasms that have developed perilesional halos. Halo nevi are a commonly occurring manifestation of a halo phenomenon; however, perilesional hypopigmented halos have also been observed around nevi in patients following treatment with antineoplastic drugs, acquisition of COVID-19 (infection and vaccine), the occurrence of a visceral tumor , surgery (such as the excision of a primary melanoma), and Turner syndrome. A halo phenomenon has also been observed in patients with congenital  or acquired  vascular lesions. In summary, a halo phenomenon can occur in association with primary lesions of various embryologic derivations. Most commonly, they have been observed in around nevi and vascular tumors. Halo lobular capillary hemangioma can be added to the list of acquired vascular lesions with the potential to develop a halo phenomenon. The preservation of melanocytes with loss of melanin pigment expression in the reported patient suggests the possibility that a post-inflammatory etiology may be responsible for the genesis of her halo lobular capillary hemangioma. A halo phenomenon in a lesion often refers to annular hypopigmentation that surrounds the tumor. Leukoderma acquisitum centrifugum was previously used to describe the occurrence when the halo is hypopigmented. However, this terminology has not persisted in literature, and the observation of a cutaneous hypopigmented halo is more commonly designated as Sutton’s phenomenon -3. In thLobular capillary hemangioma is a benign vascular lesion that can occur on the skin or mucosa. One of the designations originally used to describe the tumor was granuloma telangiectodes; yet, this terminology was subsequently replaced by pyogenic granuloma. However, since the neoplasm was neither infectious in etiology nor granulomatous in pathology, its name was changed to lobular capillary hemangioma ,5.A woman presented with an acquired red tumor that was surrounded by depigmented, white-appearing skin on her lower extremity. The clinical and pathological features of her lobular capillary hemangioma with halo phenomenon are discussed. In addition, other cutaneous neoplasms in which the halo phenomenon has been observed are reviewed -3,6-20.A healthy 50-year-old Caucasian woman with a history of seasonal allergies presented for evaluation of an asymptomatic red lesion on her distal right leg. She did not recall how long the lesion had been present but noted that it would occasionally become irritated. Indeed, it had previously spontaneously bled on two occasions.She was a healthy woman. Her family history was significant for her father having had melanoma and several basal cell carcinomas. A mild compound dysplastic nevus had been excised from her left lower back 13 years earlier, and two irritated seborrheic keratoses had been removed from her left flank one month earlier.Cutaneous examination showed a non-tender, 5.0 x 4.0-millimeter erythematous nodule with superficial scaling and a white epithelial collarette on her distal medial right leg proximal to the ankle Figure . A hypopA tangential excision, using the shave technique, was performed. The biopsy removed the entire lesion and some of the hypopigmented surrounding halo. The wound was allowed to heal by the second intention; the patient applied mupirocin ointment 0.1% three times daily.Microscopic examination of hematoxylin and eosin-stained sections showed a nodular lesion Figure . The cenThe lateral portion of the lesion shows a collarette of epithelium surrounding the vascular tumor Figure . Mounds The Fontana-Masson stain is used to detect melanin pigment. Sections stained with the Fontana-Masson stain showed positive staining in the central portion of the vascular tumor, overlying the area of vascular proliferation Figures -8. HowevThe melanoma antigen recognized by the T-cell (MART-1) stain is used to detect melanocytes. Sections stained with the MART-1 stain showed uniform positive staining along the epidermal basal cell layer of the entire lesion and the epidermis adjacent to the lesion Figures -11. MelaThe pathologic findings observed on the hematoxylin and eosin-stained sections are those of lobular capillary hemangioma. However, the correlation of the clinical presentation of the perilesional hypopigmented halo also included the white-appearing epidermal collarette surrounding the central vascular lesion established the diagnosis of a halo lobular capillary hemangioma. The Fontana-Masson stain and MART-1 stain, demonstrating the loss of epidermal basal cell layer pigmentation but the preservation of melanocytes in the hypopigmented halo, are suggestive of a post-inflammatory process instead of an autoimmune disorder (such as vitiligo in which there is both melanin expression and melanocytes are diminished).The halo phenomenon is observed in several conditions that affect the skin. Some of these cutaneous disorders include granulomatous diseases (such as sarcoidosis), infection-related non-specific lesions (such as syphilis), and papulosquamous diseases (such as lichen planus and psoriasis) . In addiA seborrheic keratosis is a benign epithelial neoplasm; at least three individuals with halo seborrheic keratoses have been described. One of the elderly patients was a man with numerous seborrheic keratoses that had been present for 20 years; a depigmented halo surrounded five of the lesions. Another man’s appearance of whitish halos around his seborrheic keratoses coincided with the diagnosis of colon cancer; following colon surgery, all the halos disappeared .Peri-cicatricial hypomelanotic macules have been observed to develop around scars in melanoma patients; in contrast to postsurgical scleroatrophy, scleroatrophic alteration is not present in the cicatrix with surrounding hypopigmentation. The halo phenomenon has localized around the surgical scars in three specific areas: the primary melanoma excision site, the excision sites of cutaneous melanoma metastases, and the scar associated with the locoregional nodal dissection. In a study of 2954 melanoma patients, the peri-cicatricial halo phenomenon was observed around the melanoma surgical scar of 17 individuals .The halo phenomenon has been described, albeit rarely, in association with basal cell carcinoma. The initial patient was reported as having basal cell carcinoma with annular leukoderma mimicking leukoderma acquisitum centrifugum in a 57-year-old man whose tumor was on his posterior neck. Subsequently, additional patients with halo basal cell carcinoma have been observed .Melanocytic neoplasms that can be associated with the halo phenomenon include benign lesions and malignant tumors Table  2,6-12],6-122,6-A halo nevus, also known as Sutton nevus, clinically appears as a zone of hypopigmentation surrounding a centrally located pigmented nevus. In a study of 124 cases, dysplastic nevi , benign acquired  nevi , and nevi with congenital features  were the most commonly associated lesions. However, melanoma , regressed nevus , Spitz nevus , and combined  nevus  were the other pigmented lesions observed ,11.Idiopathic halo nevi have been observed in approximately 1% of the population. They usually appear in children, at a mean onset age of 15 years; indeed, a study including 124 nevi noted that the lesions initially occurred in adolescents from 13 to 19 years old  and young adults from 20 to 35 years old . Therefore, the new onset of halo nevi in an older adult may be cause for concern ,11.The back is the most frequent location of halo nevi. The trunk, including the back , chest , and abdomen , were the sites of 91 (73.4%) of these nevi in a study of 124 lesions. They have also been observed on the extremities and the head and neck ,11.The clinical presentation of halo nevi is classified into four stages based on the evolution of the lesion: a central nevus with a surrounding depigmented rim (stage I), pigment loss within the central nevus which is surrounded by a depigmented rim (stage II), total nevus disappearance and a residual round patch, including the site of the prior nevus and the surrounding rim, of depigmentation (stage III), and repigmentation of the site to the color of the adjacent, normal-appearing skin (stage IV). Microscopic features of halo nevus typically consist of not only the primary melanocytic lesion but also an accompanying, often dense, inflammatory infiltrate of lymphocytes that can fill the papillary dermis and extend into the underlying reticular dermis. As the halo nevus becomes older, there can be a marked decrease or complete absence of the melanocytic lesion or the inflammatory infiltrate, or both ,11.The pathogenesis of halo nevi remains to be established; there are two theories. Less favored is the antibody theory since activated and cell-proliferating lymphocytes, after excision of the halo nevus, disappear. The second theory is related to cytotoxic T-cell response; the T-cell lymphocytes (which comprise about 80% of the infiltrate), have cytotoxic activity as demonstrated by Fas ligand, granzyme B, and perforin positivity .The eruptive onset of halo nevi has also been associated with antineoplastic drugs, coronavirus vaccine and infection, visceral cancers, surgery, and Turner syndrome. Angiokeratomas, hemangiomas, hirsutism, and halo nevi are cutaneous manifestations of Turner syndrome. Indeed, compared to vitiligo, there is an increased prevalence of halo nevi in patients with Turner syndrome .In patients with melanoma, the administration of not only immunotherapy  but also targeted therapy with v-raf murine sarcoma a viral oncogene homolog B1 (BRAF) and mitogen-activated protein kinase (MAPK)/extracellular protein kinase (ERK) kinases (MEK), such as dabrafenib and trametinib. Immunotherapy using atezolizumab was also associated with the onset of halo nevi in a patient with lung cancer. In addition, the appearance of halo nevi has been observed in patients with multiple sclerosis receiving interferon beta immunotherapy ,8.The virus-related onset of eruptive halo nevi has also been reported. In several patients, receiving a COVID-19 vaccine has been associated with the development of halo nevi. In addition, the new appearance of multiple halo nevi has also been temporally correlated to the occurrence of COVID-19 infection .The new onset of halo nevi has been observed in melanoma patients. A study of 16 consecutive patients with eruptive halo nevi revealed not only melanoma in six individuals but also other visceral tumors in three of the patients. The patients with melanoma either had melanoma in situ (two patients), malignant melanoma of the skin (two patients), melanoma skin metastases without a known primary tumor (one patient), or primary cutaneous melanoma with pulmonary metastases (one patient). The other cancers included papillary thyroid carcinoma (two patients) and neuroendocrine  tumors of the lung (one patient) ,11,12.The development of halo nevi following the excision of a primary cutaneous malignant melanoma has also been observed; specifically, either a single (two patients) or multiple (three patients) halo nevi appeared after the individual had a melanoma surgically removed. In three of the patients, the halo nevi developed within one (one patient) or two (two patients) weeks after the melanoma was excised. The development of multiple halo nevi in another patient prompted the excision of a warty plantar tumor which was a melanoma. The interval of time between melanoma removal and the appearance of the halo nevus was not provided for one patient .Café au lait macules are benign flat pigmented macules or patches. The presence of six or more café au lait macules is one of the criteria for establishing the diagnosis of neurofibromatosis type 1 (von Recklinghausen disease). In Asian infants, congenital dermal melanocytosis  and halo nevi (six patients) but also focal hypopigmentation (21 patients), or generalized vitiligo (39 patients) was observed in 83 individuals. Four of the 21 patients with focal vitiligo had hypomelanotic lesions that developed around their cutaneous metastases .A primary melanoma was excised from the back of a 70-year-old man. Within seven months, axillary metastases were discovered; three months later, multiple cutaneous metastases appeared as light blue nodules on his trunk and thigh. He received six months of chemotherapy; however, zones of leukoderma around several of the blue cutaneous metastases appeared six months after treatment has finished. New white halos continued to develop around the cutaneous metastases during the subsequent eight months. He died two years and seven months after his melanoma diagnosis; he had widespread visceral metastases not only to his skin but also to his heart, liver, pituitary, spleen, stomach, and testes .Several benign vascular lesions have been associated with the halo phenomenon Table  13-20].-20.13-20The capillary malformation is a congenital vascular malformation. It consists of dermal capillaries or venules that are increased in number or dilated or both. The vascular structures form a slow-flow, flat, pink-purple stain; as the child grows, the vascular malformation typically grows proportionately. Capillary malformations can occur as an isolated lesion; however, they can also be a component of various congenital syndromes .Capillary malformation-arteriovenous malformation is an autosomal dominant condition. Heterozygous mutations in RAS p21 protein activator 1 (RASA1), located on chromosome 5q13.3, are associated with this vascular malformation. Affected individuals with the condition have atypical capillary malformation associated with arteriovenous malformations, arteriovenous fistulas, or Parkes Weber syndrome; the latter is characterized by a large cutaneous vascular stain with subcutaneous and intramuscular arteriovenous fistulas and overgrowth of the affected limb .A clinical review of 45 patients with capillary malformation-arteriovenous malformation showed that all the patients had well-defined, round to oval, pink-purple or reddish-brown macules; these were located, in order of decreasing frequency, on the trunk (86%), upper extremities (71%), head and neck (64%), lower extremities (55%), palms (38%), oral mucosa (13%), soles (9%), and nasal mucosa (2%). The macules were surrounded by a white halo in 26 patients. In addition, pinpoint bright red macules with a surrounding pale halo were observed in nine patients; these were predominantly found on the face, forearms, and dorsum of hands .Congenital hemangioma is a vascular lesion that is fully developed and present at birth. It typically appears as a violaceous plaque or nodule with surrounding pale halo and telangiectasias. However, the surrounding pale pallor can be absent or its color can be grayish ,17.After birth, a congenital hemangioma does not have a proliferative phase. However, spontaneous involution of a congenital hemangioma may occur; the observed clinical progression has been used to classify the lesion. The vascular tumor can be a rapidly involuting congenital hemangioma (RICH), a non-involuting congenital hemangioma (NICH), or a partially involuting congenital hemangioma (PICH) ,17.A retrospective study of 10 congenital hemangiomas, consisting of six RICH, three NICH, and one PICH, was performed. One of the RICH and the PICH both developed a residual anemic halo at the site of the vascular tumor during their regression .Congenital hemangiomas have somatic activating mutations in GNAQ and GNA11. They also express vascular markers such as CD31 and CD34. However, they do not express lymphatic vessel markers such as podoplanin (D2-40) and lymphatic vessel endothelial receptor 1 (LYVE-1). In addition, they also do not express glucose transport-1 (GLUT-1) .Patients with RICH can develop vascular lesion-associated complications such as coagulopathy, high-output cardiac failure, and thrombocytopenia; therefore, treatment of their tumor may be necessary. In contrast, systemic complications do not usually occur in patients with PICH and NICH. Therefore, these latter types of congenital hemangiomas do not require further treatment or can be surgically resected .An infantile hemangioma is a GLUT-1 positive vascular lesion that usually appears one to two weeks after birth. It occurs in approximately 5% to 10% of all babies, making it the most common benign vascular tumor in infants. Clinically, it presents as an erythematous papule or nodule or plaque surrounded by a hypopigmented halo on the head and neck, followed by the trunk and the extremities; the lesion initially grows rapidly (during the first four months of age) and by five to seven months has stabilized in size .Less commonly, an infantile hemangioma is present at birth. In this setting, the lesion may appear as a pale patch; often, within the pale area, thread-like telangiectasias are present. This precursor patch of infantile hemangioma has been referred to as the pallor sign .In addition to the tumor expressing GLUT-1, infantile hemangioma not only demonstrate positivity for vascular markers such as CD31 and CD34 but also placenta-related biomarkers such as FcgammaRII, Lewis Y antigen, and merosin. They do not express lymphatic vessel markers such as D2-40 and LYVE-1 .Prior to the child reaching the age of five years, many infantile hemangiomas have spontaneously regressed or involuted. However, complete regression may take up to ten years. Beta-blockers applied topically or administered orally or both, such as timolol or propranolol, may be used to treat infantile hemangiomas that have not spontaneously resolved .There are several subtypes of infantile hemangiomas: focal, indeterminate, multifocal, and segmental. The latter, segmental infantile hemangiomas, occur in patients with posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, and eye anomalies (PHACES) syndrome and lower body infantile hemangioma, urogenital anomalies, ulceration, myelopathy, bony deformities, anorectal malformations, arterial anomalies, and renal anomalies (LUMBAR) syndrome. In addition, although considered to be benign, infantile hemangiomas have a 24% complication rate; adverse events associated with infantile hemangiomas include disfigurement, functional impairment, and ulceration .Angiomas are benign acquired vascular lesions; they are also referred to as Campbell de Morgan spots, cherry angioma, cherry hemangioma, hemangioma, senile angioma, and senile hemangioma. In affected individuals, they typically appear in the third decade of life. They present as small red papules, often less than four millimeters in diameter ,14.Halo angioma is the development of a depigmented to white halo around an angioma. There are only a small number of reports in the literature. However, the actual occurrence of the halo phenomenon around an angioma may be more prevalent than the paucity of published descriptions implies ,14.A study of angiomas was performed by inspecting the trunks of 488 patients, aged 40 years or older, who had at least one angioma. The investigator found that 5.1% (25 of 488) of the patients had at least one halo angioma; indeed, the halo phenomenon was discovered in 2% (69/3479) of the angiomas. Halo angiomas were more prevalent in patients aged 60 years and older, in individuals who had more than four angiomas, and in people whose angiomas were more than three millimeters in diameter .Rarely, halo formation around an angioma has been associated with a systemic disease. A 54-year-old man, with idiopathic lobular panniculitis that had been treated continuously with tocilizumab for four years, presented with persistent widespread yellow macules and halo angioma of one-year duration. His work-up revealed diffuse plane normolipemic xanthomatosis associated with monoclonal gammopathy .Lobular capillary hemangioma, also known as granuloma telangiectodes or pyogenic granuloma, is a benign vascular tumor. Similar to congenital hemangioma, it demonstrates positivity for CD31 and CD34 and does not express GLUT-1. It clinically appears as an erythematous, possibly pedunculated, papule that is surrounded by a collarette of the epithelium; the surface of the lesion may be ulcerated and bleeding may occur spontaneously or after minor trauma .Pathologic features of a lobular capillary hemangioma demonstrate a proliferation of small blood vessels and capillaries, arranged in distinct lobules, in the dermis. In the central aspect of the lobules, a larger (feeding) vessel may be present. The surrounding stroma can be edematous or fibrotic or myxoid ,5.A dense infiltrate of neutrophils is often present throughout the dermis; this may be especially prominent if the lesion is ulcerated. However, in older lesions, the inflammatory infiltrate may be diminished or absent. Indeed, no inflammation was observed in the dermis of the lobular capillary hemangioma of the woman described in this report ,5.The overlying epidermis of a lobular capillary hemangioma may be intact or atrophic and ulcerated. The collarette of epithelium observed clinically can be observed in the lateral portion of the lesion. Elongated epidermal rete ridges extend into the dermis and surround the vascular lesion ,5.The halo phenomenon of a lobular capillary hemangioma, to the best of my knowledge, has not previously been described. The woman in this was not aware of the hypopigmented annular ring that surrounded the lesion on her leg. The skin adjacent to the halo lobular capillary hemangioma was hyperpigmented secondary to chronic sun exposure; the solar elastosis in the lateral aspects of the biopsy specimen from her leg correlates with her clinical history of extensive exposure to ultraviolet radiation.Clinically, the white halo surrounding the woman’s lobular capillary hemangioma included the epidermal collarette and extended into the adjacent skin. The Fontana-Masson stain, which demonstrates melanin pigment, showed an absence of staining that began medial to the collarette, included the collarette, and extended laterally to the collarette. However, the MART-1 stain, which demonstrates melanocytes, showed preservation of melanocytes along the entire basal layer of the epidermis, including the areas of hypopigmentation.Therefore, in the woman with halo lobular capillary hemangioma, the etiology for the loss of pigmentation surrounding the vascular lesion was associated with an acquired absence of melanin pigment with preservation of the melanocytes in the affected area. In contrast to vitiligo, in which the melanocytes would be diminished or absent in the location of depigmentation, the pathogenesis of halo formation associated with the lobular capillary hemangioma may be post-inflammatory; however, this lesion may be of prolonged duration such that not only the dermal stroma is fibrotic but the inflammatory infiltrate has completely resolved. Future reports of halo lobular capillary hemangioma may be helpful to either confirm our hypothesis for the development of the depigmented halo in the reported woman or establish an alternative mechanism of pathogenesis.The term eruptive pseudoangiomatosis was coined in 1993; however, the condition was initially described as acute hemangioma-like lesions in children who had experienced a recent infection from either ECHO 25 virus or ECHO 32 virus. The condition was also observed in adults, and several other viruses have been associated with its development such as adenovirus, COVID-19, cytomegalovirus, Epstein-Barr virus, human immunodeficiency virus, and parvovirus B19. Other trigger factors associated with eruptive pseudoangiomatosis include acute lymphoblastic leukemia, chemotherapy, COVID-19 vaccines, Hodgkin lymphoma, insect bites, and non-Hodgkin lymphoma ,19.Eruptive pseudoangiomatosis presents clinically as small (1 to 4 millimeter) discrete cherry-angioma-like blanchable papules; the lesions are asymptomatic or slightly pruritic. A characteristic feature is that each vascular papule is surrounded by a white halo. The lesions occur on the face, neck, extremities, and trunk; in some of the patients, halos are not present on facial lesions ,19.The skin lesions are typically preceded by a prodrome of mild fever, sore throat, and/or gastrointestinal symptoms. They resolve spontaneously in about six days in children and 14 days in adults. If symptomatic, topical corticosteroids or oral antihistamines, or both, may be used ,19.The lesions are not usually biopsied. In a six-year-old boy, the lesion showed many dilated capillaries, lined by endothelial cells with a hobnail appearance, in the papillary and reticular dermis. The number of blood vessels was not increased; however, a mild-to-moderate perivascular lymphohistiocytic infiltrate was present ,19.Investigators have postulated that eruptive pseudoangiomatosis is either a viral exanthem  or a paraviral eruption to an antigen union at the vascular site. Some researchers considered the lesions to be similar to those of erythema punctatum Higuchi that have been noted in Japan and resulting from bites by Culex pipiens pallens. In addition to a viral exanthema, the clinical differential diagnosis includes Leukocytoclastic vasculitis and popular urticaria ,19.The halo phenomenon refers to a hypopigmented or white halo surrounding a skin neoplasm. Cutaneous tumors with a perilesional halo include basal cell carcinoma, benign and malignant melanocytic neoplasms, seborrheic keratosis, surgical scars, and vascular lesions. A MART-1 stain was used to determine the presence or absence of melanocytes, and a Fontana-Masson stain was used to assess the expression of melanin pigment in the perilesional white and hypopigmented areas of a halo lobular capillary hemangioma. The epidermal basal layers of the entire specimen showed a normal number of uniformly distributed melanocytes, and the white collarette and hypopigmented halo revealed an absence of melanin pigment expression. In conclusion, halo lobular capillary hemangioma can be added to the congenital  and acquired  benign vascular lesions that have been associated with halo phenomenon. A pathogenesis that involves post-inflammatory hypopigmentation may be responsible for the etiology of the halo lobular capillary hemangioma in the reported patient since the white epidermal collarette and hypopigmented perilesional halo revealed a loss of melanin pigment expression with the preservation of melanocytes.


A rarely described condition known as branch retinal artery occlusion (BRAO) with concurrent obstruction of the central retinal vein (CRVO) is characterized by diffuse retinal hemorrhages, dilated and tortuous retinal veins, macular and disc edema, cotton wool spots, and a generalized delay in arteriovenous transit on fluorescein angiography, together with a retinal whitening in the area of the affected retinal arterial branch. Although BRAO and CRVO may share underlying systemic risk factors, the pathogenesis of combined BRAO + CRVO is still unknown. We present a BRAO + CRVO case report concerning a 63-year-old white male who came to our observation complaining of sudden vision loss in his right eye. An increased risk for thrombotic event was revealed in this case, and the patient improved only with systemic anticoagulant therapy and in the absence of ocular therapy. We also explain all the clinical findings that are detectable using different diagnostic devices and analyze the scientific literature for other, similar clinical cases. Conventionally, retinal vein obstructions are divided into central retinal vein occlusion (CRVO), branch retinal vein occlusion (BRVO), and hemi-central retinal vein occlusion, while retinal artery occlusions are classified into central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO), and cilioretinal artery occlusion (CLRAO). The concurrent artery and vein obstruction could be any permutation and combination of the clinical aforementioned entities .In particular, BRAO with concomitant CRVO is an uncommon event . This coDespite the fact that BRAO and CRVO share several underlying systemic risk factors, such as diabetes, coagulopathies, systemic arterial hypertension, and atherosclerotic cardiovascular disease ,3,4,5, tIt has been hypothesized that either CRVO followed by the external compression of a nearby branch retinal artery due to the optic disc swelling, or BRAO followed by a low-flow condition in the central retinal vein, could be the initial event in this condition ,5.Considering the pathophysiology of retinal vascular occlusive disease and its vision-threatening nature, early diagnosis is essential to try to best manage such a pathological condition and to ensure maximum visual recovery for the patient. For this reason, a multimodal imaging diagnostic approach with color fundus photography, optical coherence tomography (OCT), and fluorescein angiography is advisable for achieving this purpose .There are several papers in the published scientific literature reporting cases of BRAO + CRVO, managed differently and with different outcomes. In fact, some patients received no therapy ,6,7,8, oIn this case report, we present a BRAO + CRVO case report concerning a 63-year-old white male who came to our observation complaining of sudden vision loss in his right eye. We evaluated him using multimodal imaging, and he improved only with systemic anticoagulant therapy and in the absence of ocular therapy. Furthermore, we also explain all the clinical findings that were detectable with different diagnostic devices, and we analyze the scientific literature for other, similar clinical cases.A 63-year old Caucasian male came to our observation complaining of sudden vision loss in his right eye, after some slight visual defects in the previous two days. He referred to being affected by moderate chronic renal insufficiency and grade IV hydronephrosis in his right kidney. Moreover, he was under treatment for arterial hypertension with nebivolol and olmesartan/amlodipine, for hyperlipidemia with ezetimibe, and for benign prostatic hyperplasia with alfuzosin. On admission, his electrocardiogram was normal, and his blood pressure was 125/70 mmHg.His best-corrected visual acuity (BCVA), evaluated via Snellen chart, was 20/400 in the right eye and 20/20 in the left eye. The intraocular pressure was found to be 18 mmHg in both eyes, and the anterior segment assessment at slit-lamp examination was unremarkable in both eyes. In addition, the pupils were regular, eucyclic, and normoreactive in both eyes, without relative afferent pupillary defect.A pale region corresponding to the inferotemporal artery branch course was found during the fundoscopic examination of the right eye, together with numerous retinal hemorrhages, macular edema, increased vascular tortuosity, and optic disk swelling. The diagnosis of combined BRAO + CRVO was confirmed via color fundus photography  and OCT , which also showed subretinal fluid, intraretinal cysts, and a prominent middle-limiting membrane sign in the inner synaptic portion of the outer plexiform layer, corresponding to areas of paracentral acute middle maculopathy (PAMM).Anticoagulant therapy with subcutaneous injections of heparin 6000 IU twice daily was immediately started after a nephrological and cardiologic consult, and a fluorescein angiography was also scheduled. The results for homocystinemia and screening for vasculitis were all found to be normal. On the other hand, thrombophilic screening revealed methylenetetrahydrofolate reductase gene heterozygosis and clotting factor XIII gene homozygosis, both causing an increased thrombotic risk. The day after the start of anticoagulant therapy, the BCVA improved to 20/70, with a macular edema decrease at OCT.Three days after admission, the BCVA further improved to 20/50, and fluorescein angiography was performed  . The exaTwo weeks after the start of anticoagulant therapy, the BCVA further improved to 20/25, with the total resolution of the macular edema and the persistence of a slight hyper-reflectivity at the OCT corresponding to the area of branch retinal arterial occlusion.Six weeks after diagnosis, the BCVA was 20/20, with a further improvement in the color fundus photography and OCT, as can be seen in Combined vascular occlusion affecting the retinal vein and artery is a very infrequent occurrence .In particular, BRAO with concurrent CRVO is a rarely described condition ,18,19,20Concurrent BRAO + CRVO is a clinical condition different to CRVO linked with CLRAO ,3. UnlesSymptomatic, acute, simultaneous BRAO + CRVO, like BRAO, is an urgent ocular disease that requires early systemic assessment. Indeed, BRAO + CRVO may be a critical clinical event in an inflammatory, embolic, or other process necessitating a prompt and focused systemic medical examination based on the patient’s presentation and medical history. Overall, in younger patients (those under the age of 50), a thorough workup for hypercoagulability or vasculitis is required , while aIn this case report, we described a BRAO + CRVO that occurred in a 63-year old Caucasian male and that clinically improved only with systemic anticoagulant therapy, with no need for ocular therapy.In the scientific literature, there are some similar case reports published on this topic, showing different outcomes and therapeutic approaches, basically due to the lack of official guidelines ,18,19,20Concerning the diagnosis, color fundus photography, OCT, and fluorescein angiography were all demonstrated to be crucial in the identification and the monitoring of this rare ocular vascular syndrome . In partPAMM is an OCT finding that appears as a placoid, hyperreflective band at the level of the inner nuclear layer, with or without extension into the adjacent inner and outer plexiform layers, in individuals with retinal capillary ischemia and unspecific persistent scotomas ,24. ThisHowever, regardless of the usefulness of color fundus photography and OCT, fluorescein angiography remains a fundamental diagnostic examination in the diagnosis of retinal vascular and ischemic disease and, for this reason, it should always be performed in cases of BRAO + CRVO suspicion to confirm the diagnosis .In recent years, OCT angiography has gained an increasing usefulness in ischemic retinal diseases diagnosis and management, due to technological updates. In fact, OCT angiography has been demonstrated to correlate visual impairment with the extent of deep capillary plexus involvement in cases of PAMM related to CRVO . AlthougRegarding management and therapeutical approaches, only one major case series of combined BRAO + CRVO has been published, by Duker et al. in 1990 , who desIn addition, some authors ,7,8 descOn the other hand, the intravitreal dexamethasone implant (Ozurdex) could be considered a useful therapeutical approach, as it has been proven to be effective in the treatment of all subtypes of CRVO . In factNonetheless, the patient in this case report showed a great visual recovery in the absence of any ocular treatment and with only systemic anticoagulant therapy. This result agrees with several case reports already published in the scientific literature ,11,12,13In addition to the three patients examined by Ducker et al.  and treaRubio et al.  reportedOn the other hand, a similar case report was described by Jenisch et al. , who exaÖzdek et al.  evaluateFinally, Parchand  studied Regarding our case report, the patient showed an increase thrombotic factor due to methylenetetrahydrofolate reductase gene heterozygosis and clotting factor XIII gene homozygosis, in addition to arterial hypertension and hyperlipidemia, which were already being treated with drugs. Moreover, the patient was not taking any type of medication that could further increase the risk of vascular occlusive pathology, as demonstrated for interferon ,12. As pConcerning systemic anticoagulant therapy, its use in CRVO is controversial. In fact, as explained by Hayreh, the use of antiplatelet aggregation agents or anticoagulants could markedly increase retinal hemorrhages, which can be destructive to the retina . FurtherTo summarize, this case report confirms that simultaneous BRAO + CRVO is a separate and complex clinical entity that often manifests as abrupt, painless visual loss. In our opinion, according to other authors’ hypothesis ,5, a posTo establish the right BRAO + CRVO diagnosis, an ophthalmoscopic examination or color fundus photography combined with fluorescein angiography and OCT is required. Although there is currently inadequate information to suggest any particular treatment to enhance vision in concurrent BRAO + CRVO, starting systemic anticoagulant therapy as soon as possible, potentially combined with ocular therapy, appears to comprise a viable treatment approach, especially in case of thrombotic risk factors, as also demonstrated by previous case reports published in the literature ,18,19,20However, it is very important to consider that the CRVO component could also improve spontaneously or after the prompt treatment of extreme hypertension, so every single case should be accurately evaluated.In conclusion, acute, symptomatic and concurrent BRAO + CRVO, like BRAO, is an urgent ocular disorder that requires early systemic medical assessment to rule out microembolism, hypercoagulability, or vasculitis. Future research is needed to better understand this unusual retinal vascular condition and to draw up useful official treatment guidelines.


Pediatric hepatocellular adenoma (HCA) is a rare tumor and often observed in abnormal livers. Despite advances in adults, there is little progress in understanding the origin and management of pediatric HCAs. Evidence of multifocality, malignant transformation and rupture in a subset of patients warrant a thorough understanding of this rare entity to guide optimum management. We present a concise review of the current literature on HCA, with a focus and comparative assessment on pediatric patients. Current understanding and classification of pediatric hepatocellular adenomas (HCA) are largely based on adult data. HCAs are rare in children and, unlike in adults, are often seen in the context of syndromes or abnormal background liver. Attempts to apply the adult classification to pediatric tumors have led to several “unclassifiable” lesions. Although typically considered benign, few can show atypical features and those with beta-catenin mutations have a risk for malignant transformation. Small lesions can be monitored while larger (>5.0 cm) lesions are excised due to symptoms or risk of bleeding/rupture, etc. Management depends on gender, age, underlying liver disease, multifocality, size of lesion, histologic subtype and presence of mutation, if any. In this review, we summarize the data on pediatric HCAs and highlight our experience with their diagnosis and management. Pediatric hepatic adenoma or hepatocellular adenoma (HCA) is a rare liver tumor that comprises approximately 4% of all liver lesions in children . While iIn adults, HCAs have an incidence of 4 per 100,000 patients and commonly arise from normal livers ,6. The nThe majority of HCAs lack symptoms and are incidentally diagnosed ,22. PatiClinically unstable patients are usually triaged to the operating room, intensive care unit (ICU), or interventional radiology for stabilization. While the latter requires immediate intervention, they both require establishing the diagnosis via a stepwise process. The differential diagnosis for a pediatric liver mass includes hepatoblastoma, hepatocellular carcinoma, focal nodular hyperplasia, benign hemangioma, hepatic cyst, and HCA.Ultrasound (US) examination of the right upper quadrant with or without Doppler is the most common initial modality for diagnosis and shows solitary or multiple discrete echogenic mass lesions . The appMRI is considered the gold standard for diagnosis in children with HCAs, with MR imaging features well described in the literature. Lesions can be heterogenous due to internal fat, hemorrhage, necrosis, and variable vascularity. T1-weighted imaging tends to show high signal with intra-tumoral hemorrhage ,24,25 FA,B. MR iWhile US and MRI are the currently recommended imaging modalities for detection and monitoring of pediatric HCA, we recognize that several institutions worldwide may still rely on computed tomography (CT). The utility of CT is valid in situations where there is a lack of other imaging tools. Regardless, MRI and US should be utilized when available. The downside with CT is the high radiation dose imparted, especially when multiphase CT imaging is performed for the evaluation of suspected hepatic adenoma. On CT, HCA appear without central scar and can show a heterogenous appearance given hemorrhage in the tumor  AdditionFemale patients or patients that are poor surgical candidates with asymptomatic HCAs less than 5.0 cm are recommended to have surveillance MRI at 6 months intervals, for the first year ,2,5,7,22On histopathologic examination, HCAs are well-circumscribed, un-encapsulated or partially/thinly capsulated tumors with uniform cut surface. Large or long-standing tumors can show secondary changes such as intra-tumoral hemorrhage or infarction A. They aLargely based on adult studies, HCAs are classified using histomorphology, immunophenotypic features, and molecular findings into the following subtypes:Inflammatory adenomas (i-HCA): These are the most common HCAs in adults and post-pubescent children [IL6ST) which encodes for glycoprotein-130 (gp130), which is a component of the IL6 receptor [IL6ST are seen in approximately 60% of inflammatory adenomas. Non-mutated inflammatory adenomas show similar inflammatory expression profiles including SAA and CRP reactivity by IHC, but the mechanisms are not clear [CTNNB1) giving rise to Wnt/beta-catenin pathway activation. This subset, in addition to SAA and CRP, can show nuclear reactivity for beta-catenin by immunohistochemistry and will have increased risk for malignant transformation.children ,30. Theychildren ,32. Theychildren ,34. Micrchildren ,36. Tumoreceptor . This acot clear . There iHNF1-alpha mutated adenomas (H-HCA): This is the second most common HCA subtype in adults and children [HNF1A  gene resulting in hepatocyte proliferation and increased lipogenesis by fatty acid synthesis and down regulation of liver-type fatty acid binding protein (LFABP), manifesting as lesional steatosis. In few cases, one mutation is somatic and the other germline. Jeannot et al. reported that heterozygous germline-inactivating mutations of the CYP1B1 gene might increase the incidence of HCA in women with TCF1 (HNF1A) gene mutations [HNF1A are associated with an autosomal dominant condition, maturity onset diabetes of the young type 3 (MODY3) [HNF1A gene mutation in these patients affects only one allele and leads to a primary defect in insulin secretion by the pancreatic β cells [HNF1A allele confers a predisposition to develop familial liver adenomatosis in patients with MODY3 [children ,22. Micrutations . Further (MODY3) . The HNF β cells ,40. Somath MODY3 . We haveth MODY3 . This haBeta-catenin mutated adenomas: They are shown to be the third most common in adults and most common in the syndromic subgroup among children [b-catenin, seen in this group lead to activation of b-catenin [children ,30. Micr-catenin . The mos-catenin . While e-catenin . These t-catenin , that is-catenin ,46. BothUnclassified hepatic adenomas: This category is created for tumors that lack characteristic features of any one subtype described above. Overall, they constitute 5–10% of HCAs. Some tumors that show near total necrosis, hemorrhage, or other secondary changes can be classified under this category.Well-differentiated hepatocellular tumors: These are HCAs with focal atypical morphologic features such as small cell change, pseudo acinar architecture, cytologic atypia, focally thick hepatic cords and/or focal reticulin loss and may not unequivocally satisfy the diagnostic criteria for HCC. They are typically seen in adults and called “atypical hepatocellular neoplasms” [oplasms” . It is iManagement of pediatric HCAs depends on underlying medical condition, surgical candidacy, symptoms, size and number of lesions, and histopathologic subtype. Most centers begin by ruling out underlying liver disease or other medical conditions. Literature also supports management based on sex, with less aggressive approach in females than males given that males tend to have a higher risk for malignant transformation ,7,15. SiA subset of children with ruptured HCAs that present with peritonitis or hypotension require emergent resection, embolization or other temporizing measures. While these patients can be bridged safely, the outcomes are not well reported. In case reports, complications for ruptured pediatric HCAs include bile leaks, abscess formation, and respiratory complications . In contWhile pediatric HCAs are typically solitary lesions, up to 30% of patients can present with multiple tumors ,49. Hepan = 16, all females) with an age range of 29 to 48 years. There were no recurrent mutations in this subgroup. The subgroup was identified based on the overexpression of 6 genes  using unsupervised hierarchical clustering. There were no pediatric patients in this subgroup and this subgroup showed significantly higher rate of symptomatic bleeding compared to others.The advancements in the understanding of the underlying biology of HCAs have primarily come from adult patients ,52. In a9 Given the distinct presentation and outcomes in children, the molecular characterization of pediatric HCAs is likely unique. Likewise, the limited biological understanding of pediatric HCAs has impeded any translation to providing targeted therapies. One of the few clinical advancements in the last 20 years has been the implementation of transarterial embolization, allowing some patients to be temporized to permit surgical intervention in a less acute fashion. Nonetheless, the lack of targeted therapies has resulted in several patients requiring a partial hepatectomy, a high-risk operation. Further understanding of the tumor biology, key pathways, and overall tumor progression is warranted to create small molecule inhibitors that can help shrink or absolve these tumors.Advancements in subclassification have helped guide aggressive management for beta-catenin mutated adenomas and a cautious approach to manage bleeding in inflammatory and sonic hedgehog activated subtypes In summary, HCAs are rare benign liver tumors that have been historically linked to hormonal imbalance in adults and can be seen arising in abnormal livers in children. Molecular subclassification of adult HCAs has laid the foundation for our understanding of pediatric HCAs. Despite advances in molecular subtyping, progress in treatment and risk stratification in children is limited. Additional collaborative efforts are warranted for better diagnosis, classification, and management of these rare pediatric tumors.


Our patient had an extremely rare type of pediatric Diffuse Midline Glioma (DMG) with modified H3 K27 that occurred in the cervical spinal cord. Due to its location in the spinal cord, slow clinical presentation with torticollis for 7 months, and the non-specific radiological appearance of this tumour, it was initially considered to be a low-grade glioma. Based on imaging findings, the neurosurgery team performed a complete surgical resection, but the pathological features were consistent with a high-grade, diffuse midline glioma. Therefore, we are reporting a case of an altered high-grade DMG H3K27 glioma, which is difficult to diagnose due to its slow clinical symptoms which caused a delay in diagnosis, non-specific imaging, and with difficulty in accessing histopathological markers in low and middle income countries (LMIC). Spinal cord tumors, especially high-grade tumors in pediatric patients, are rare. We report a case of diffuse midline glioma (DMG) with H3K27M alteration, which is a rare and highly aggressive tumor according to the 2021 World Health Organization (WHO) classification [Patient information: a 6-year-old male patient with no notable personal or family pathological history presented with a neglected torticollis that had been present for seven months before admission. The evolution was marked by the aggravation of torticollis and the occurrence of left brachial monoparesis, which motivated consultation. The examination revealed altered left upper limb muscle strength, while muscle strength was preserved in the right upper limb and the lower limbs, and walking and standing were preserved without other neurological alterations. An MRI of the cervical spine revealed an intradural intramedullary tumor of the cervical spine. The patient underwent total tumor excision one month later. The patient was hospitalized in intensive care for three weeks, during which time he developed postoperative left hemiplegia and respiratory distress due to left diaphragmatic paralysis. This required a tracheotomy and progressive respiratory weaning, after which the patient was referred to the pediatric oncology department for further treatment.Clinical findings: on examination, the patient was stable with regular spontaneous breathing, conscious, well oriented in time and space, in a wheelchair with left hemiplegia. Sensitivity was preserved, with no impairment of the cranial nerves, sensory or sphincter functions, and no café-au-lait spots or other abnormalities were found during skin examination.Timeline and diagnostic assessment: at this stage, the diagnosis of low-grade glioma was evoked. The spinal MRI showed an expansive intramedullary intradural process, extended from C2 to C7, oblong, centered, well limited, in T1 iso-signal, heterogeneous hyper-signal in T2 and STIR, without diffusion restriction, enhanced peripherally after Gadolinium injection without central contrast, reducing the sub-arachnoid spaces, without bone lysis. Straightness of the cervical spine with beginning of inversion . The tumor appears hypo- to iso-signal on T1-weighted sequences and hyper-signal on T2-weighted sequences. In addition, the tumor margins are poorly defined, and slight peri-lesional edema may be notable. These radiologic features are consistent with high-grade glioma ,6. Some et al.  suggesteet al. .In the WHO 2021 classification, the DMG with H3 K27 alteration, which was described in our clinical case, is characterized by a loss of nuclear expression of H3K27me3. This is the physiological form that is trimethylated on lysine (K) at position 27 of the H3 protein. This loss of expression is associated with either an H3 K27 mutation, an EGFR mutation, or an overexpression of EZHIP. Regardless of histopathological aspects, these gliomas are classified as grade 4 . Our patThe new type of tumor, "diffuse midline glioma with H3K27 alteration," aims to establish a clearly defined and uniform diagnostic category that accurately describes the age, location, histology, and genetics of the tumor, with the ultimate goal of identifying this specific type of tumor. In our case, the discussion focused on the discrepancy between the slow clinical presentation with torticollis that resulted in a delayed diagnosis, the non-specific imaging for DMG, and the histological appearance, which indicates a high-grade tumor. However, characterizing tumors in LMICs is challenging due to the lack of specific markers.


Vasoactive intestinal peptide (VIP) secreting tumors (VIPomas) are insidious functional neuroendocrine tumors originating mainly from pancreatic islet cells. Hepatic localization is considered exceedingly rare as only few cases have been reported in the literature. Diagnostic and therapeutic management of this tumor is still not clearly codified and therefore represents a real challenge for clinicians. Herein we report a unique case of a primary hepatic VIPoma recurrence in a female patient 22 years after curative resection. The patient had two sessions of transarterial chemoembolization. Complete symptomatic improvement was achieved since the first day after the first session. This case highlights that long-term follow-up for patients with hepatic VIPoma is mandatory as recurrence could occur several years after curative surgical treatment. VIPomas are rare tumors secreting a hormone that is vasoactive in the intestine causing severe diarrhea. The majority of VIPomas arise within the pancreas. The hepatic localization is extremely rare. We report a case of a very late recurrence of a primary hepatic VIPoma surgically treated 20 years ago. The case was managed with therapeutic radiology by blocking the blood supply to the tumors after administering anticancer drugs in the vessels near them. Vasoactive intestinal peptide (VIP) secreting tumor (VIPoma) is a rare and insidious functional neuroendocrine tumor (NET) first described by Verner and Morrison in 1958 .Due to their extreme rarity, no management guidelines of these tumors have been established and both diagnosis and treatment of VIPomas remain challenging. Prognosis is highly variable and is mainly dependent on metastatic extension, tumor growth speed and differentiation. Herein we report a unique case of recurrence of a primary hepatic VIPoma in a female patient occurring more than two decades after initial curative resection.A 58-year-old woman with a history of newly diagnosed diabetes mellitus and primary hepatic VIPoma treated with right liver hepatectomy in 1998 presented in September 2020 to the emergency department with a five-month history of multiple watery, non-bloody diarrhea of up to 20 loose bowel movements per day and a weight loss of 10 kgs. The patient denied nausea, vomiting or abdominal pain. The physical examination revealed no signs of dehydration. The laboratory work-up revealed a potassium level of 1.9 mmol/l, anicteric cholestasis and a normal level of albumin and hemoglobin. The clinical picture was very similar to the one she presented with 22 years ago when the hepatic VIPoma was diagnosed. A recurrence of VIPoma was immediately suspected. The plasma VIP level was 451 pmol/l (upper limit 40 pmol), the chromogranin A level was within normal range.An abdominal computed tomography (CT) scan was performed revealing four hepatic lesions of segments I, III, IV and the sectional slice. The lesions were spontaneously hypodense, intensely enhancing after injection of contrast product in the arterial phase with a wash out in the portal phase . The lesNeuroendocrine tumors (NETs) are a rare type of tumor, deriving from neuro-ectodermal cells . They coUnlike most PHNETs who are typically endocrinologically silent and diagnosed either incidentally or at advanced stages, VIPomas are mainly revealed by large-volume diarrhea due to VIP unregulated overproduction. VIP is a peptide hormone that has been studied in numerous organ systems including the nervous system where it is mainly produced, the gastrointestinal, respiratory, cardiovascular, and endocrine systems. VIP is however majorly localized in the myenteric and submucosal neurons and nerve terminals in the gastrointestinal tract where it mediates through its receptors a myriad of functions such as regulating gastric acid secretion, anion secretion, pancreatic enzyme release, vasodilation and intestinal and cellular motility . VIP alsThe diagnosis of primary hepatic VIPoma is based on three pillars with regard to watery diarrhea, elevated VIP serum level and imaging study. Although radiological imaging is crucial in determining the lesions number, locations, size and thus guiding the treatment strategy, ultrasound (US), CT, and MRI lack sensitivity when it comes to PHNETs imaging. Thus, distinguishing PHNET from metastatic hepatic N and more common hepatic tumors, chiefly hepatocellular carcinoma and cholangiocarcinoma remains very challenging. PHNET features may vary largely appearing solid or cystic with diffuse or well-circumscribed margins and possible presence of areas of necrosis. The enhancement patterns of the solid PHNET are reported to be similar to those of hepatic metastases of NETS of the pancreas and the GI tract showing hyper-enhancement in hepatic arterial phase and washout in portal venous phase which was the case for our patient. . As primIn addition to this case, we were able to identify only three other cases of primary hepatic VIPoma based on a PubMed and a Google Scholar search of the English literature. The radiological features of these cases along with the clinical and biological ones are summarized in Similarly to PHNET, the mainstay of treatment of HPV is surgery resection or partial hepatectomy. The resectability rate for PHNET was reported to be nearly 70% and surgery seems to be effective and safe even when major hepatectomy is performed . The 10-This case highlights that long-term follow-up for patients with hepatic VIPoma is mandatory as recurrence could occur several years after curative surgical treatment. In these patients, the immediate focus should be on rapidly controlling the diarrhea to restore losses, prevent further complications and improve the patient quality of life. While surgery remains the mainstay of VIPomas treatment, in unresectable cases, TACE seems to be a safe and efficient therapeutic option.VIPomas are insidious functional neuroendocrine tumors.Hepatic localization of hepatic VIPomas is exceedingly rare.The immediate focus in patients with VIPomas should be on controlling the diarrhea.Transarterial chemoembolization is a safe and efficient therapeutic option in hepatic VIPomas.Long-term follow-up for patients with hepatic VIPoma is mandatory.


Duodenal varices usually occur due to portal hypertension and are rare causes of gastrointestinal tract bleeding. We report a unique case of a previously fit patient who presented with melena and was found to have isolated duodenal varices (DV) in the third part on esophagogastroduodenoscopy. No esophageal or gastric varices were noticed. The duodenal varices were successfully managed by endoscopic banding. A computerized tomography scan of the abdomen to further investigate the cause confirmed duodenal varices and revealed superior mesenteric vein thrombosis. The liver was normal with patent hepatic and portal veins. No evidence of thrombophilia was found. Apixaban was prescribed for superior mesenteric vein thrombosis and on follow-up. no further bleeding was reported. Duodenal varices (DV) are an uncommon cause of gastrointestinal tract bleeding (GITB) usually associated with portal hypertension. Isolated DV due to non-portal hypertension is rare and is limited to case reports. Here, we report a case with no past history of cirrhosis, portal hypertension, or coagulopathy disorder that presented with duodenal varices resulting in melena due to superior mesenteric vein thrombosis.A 35-year-old male, previously fit, presented to the emergency department with a history of melena and a significant drop in hemoglobin levels to 6.2 gm/dl. He was hemodynamically stable, and the general physical examination, including the abdomen, was unremarkable. A digital rectal examination confirmed the presence of melena. Other laboratory investigations, including white cell count, platelet count, lipase, and liver and renal profile were normal. The patient underwent esophagogastroduodenoscopy (EGD) that showed normal esophagus, stomach, and the first two parts of the duodenum without any evidence of varices in the esophagus or stomach or portal hypertensive gastropathy. To carefully examine the upper GI tract and find the cause of GITB, the endoscopist intubated deep into the third part of the duodenum, which surprisingly showed four enlarged varices in the third part Figure .Two of the largest duodenal varices were banded successfully Figure .Further investigation by computerized tomography (CT) angiography revealed chronic superior mesenteric vein (SMV) thrombosis Figure , duodenaDuodenal varices are part of a heterogeneous group of ectopic varices that arise due to shunting within the portal vascular bed or the systemic/mesenteric vessels . An uncoPrompt diagnosis and therapy of DV are essential to avoid massive bleeding and consequential high mortality ,7. EGD iTherapeutic management of DV is not standardized due to its uncommon occurrence and treatment is mainly based on the outcomes of case series and case reports. Endoscopic therapy for DV is reported to be effective and options include endoscopic banding, cyanoacrylate injection, thrombin injection, and hemoclip placement, with no clear benefit of one over the other ,9. HenceOur case highlights the importance of being aware of duodenal varices that may exist in isolation. In addition, it demonstrates the significance of inspecting the duodenum beyond D2, especially in upper GI bleeding cases with no apparent identifiable source. Although endoscopic therapy for DV may seem daunting, it nevertheless is feasible and effective.


Intravenous leiomyomatosis (IVL) is a rare and specific type of smooth muscle tumor that is histologically benign but has a malignant biological behavior. It is commonly associated with a history of uterine leiomyomas.A 36-year-old woman, G1P1, presented to the hospital with left lower abdominal pain for 2 months and she has accepted hysteroscopic myomectomy about 1 year ago. Ultrasound venography, echocardiography and computed tomography venography (CTV) of inferior vena cava were performed, which revealed IVL located in left intramural myometrium walls growing along the left ovarian vein reaching the level of the lumbar 5-sacral 1 disc. Laparoscopic bilateral salpingo-oophorectomy and hysterectomyis were scheduled. The IVL in the left ovarian vein and parauterine venous plexus were detected and excised completely during surgery. IVL was diagnosed by postoperative pathology and immunohistochemistry. The patient recovered well after surgery. No surgical-related or anesthesia-related complications occurred.The 3-month follow-up CTV of inferior vena cava and echocardiography examination revealed normal.The cause of IVL is unknown, this observation demonstrates that hysteroscopic myomectomy might lead to the occurrence of IVL. Intravenous leiomyomatosis (IVL) is a rare and specific type of smooth muscle tumor that is characterized by the presence of vascular extension and invasion of benign smooth muscle lesions in a worm-like manner into the pelvic and systemic vasculature system , 2. The A 36-year-old woman, G1P1, presented to the hospital with left lower abdominal pain for 2 months. She had accepted hysteroscopic myomectomy for menorrhagia and anemia caused by submucosal fibroid  about 1 year ago. During the operation, the fibroid was soft, round and hernia-like with rich blood supply. The myoma was completely resected by hysteroscopy, and all symptoms disappeared.The ultrasound examination found no abnormality 1-month post-surgery. During follow-up, ultrasound examination revealed a low echo mass with rich blood supply located in left intramural myometrium walls where the previous operation site was, and gradually increased form approximately 2.5 to 4 cm in diameter at 5 and 8 months.Until this admission, we gave her GNRH-a injection 3 times(3.75 mg/28days), but the mass grew out of the uterus,growing along the left ovarian vein reaching the level of the lumbar 5-sacral 1 disc, and ultrasound venography indicated it’s intravenous leiomyomatosis(IVL). Echocardiography and computed tomography venography (CTV) of inferior vena cava were performed and showed heart and other blood vessels with no abnormality. Laparoscopic bilateral salpingo-oophorectomy and hysterectomyis were scheduled.During this surgical operation, the uterus looked normal size, with a uneven surface, and the cord-like tissues in the left ovarian vessel was detected. During the hysterectomy, many rope and mass-like muscle tissues surrounding with abnormal proliferation blood vessels in the left inferior uterine body to the cervix were observed, and was closely related to the left ureter and the left lateral wall of the bladder.The IVL in the left ovarian vein and parauterine venous plexus were excised completely during surgeryFig.  Fig. 2 .Intravascular leiomyomatosis (IVL) is a histologically-benign, rare mesenchymal tumor which can develop from the veins of the uterus, extend into the inferior vena cava and ultimately into the right side of the heart, resulting in death.The aetiology of IVL is unclear and discussion centres around two hypothesis, namely,the proliferation of vascular smooth muscle cells and the direct invasion of uterine leiomyoma into the muscular veins and diffusion along the vascular lumen , 7.AccorAt present, the management guidelines for IVL is unavailable, and the current treatment is mainly based on limited case reports. According to previous reports, the postoperative recurrence rate is high after incomplete tumor resection, approximately 16.6–30.0% . The preWe report a case of IVL that progressed after submucosal myomectomy. The cause of IVL is unknown, this observation demonstrates that hysteroscopic myomectomy might lead to the occurrence of IVL. Due to the rarity of IVL, most current research is limited to case reports. Nevertheless, case report is limited by the quality of information available. Systematic literature review or multi-center studies about IVL is beneficial to increased awareness on the etiology and treatment of IVL. In addition, up to now, the present case has been followed up for only several months and reported no recurrence. It is possibly because the follow-up time was short, which needs to continue for life. Early detection and accurate diagnosis are imperative for appropriate treatment.


High-attenuation pulmonary abnormalities are commonly seen on CT. These findings are increasingly encountered with the growing number of CT examinations and the wide availability of thin-slice images. The abnormalities include benign lesions, such as infectious granulomatous diseases and metabolic diseases, and malignant tumors, such as lung cancers and metastatic tumors. Due to the wide spectrum of diseases, the proper diagnosis of high-attenuation abnormalities can be challenging. The assessment of these abnormal findings requires scrutiny, and the treatment is imperative. Our proposed stepwise diagnostic algorithm consists of five steps. Step 1: Establish the presence or absence of metallic artifacts. Step 2: Identify associated nodular or mass-like soft tissue components. Step 3: Establish the presence of solitary or multiple lesions if identified in Step 2. Step 4: Ascertain the predominant distribution in the upper or lower lungs if not identified in Step 2. Step 5: Identify the morphological pattern, such as linear, consolidation, nodular, or micronodular if not identified in Step 4. These five steps to diagnosing high-attenuation abnormalities subdivide the lesions into nine categories. This stepwise radiologic diagnostic approach could help to narrow the differential diagnosis for various pulmonary high-attenuation abnormalities and to achieve a precise diagnosis.Critical relevance statement Our proposed stepwise diagnostic algorithm for high-attenuation pulmonary abnormalities may help to recognize a variety of those high-attenuation findings, to determine whether the associated diseases require further investigation, and to guide appropriate patient management.Key points• To provide a stepwise diagnostic approach to high-attenuation pulmonary abnormalities.• To familiarize radiologists with the varying cause of high-attenuation pulmonary abnormalities.• To recognize which high-attenuation abnormalities require scrutiny and prompt treatment.The online version contains supplementary material available at 10.1186/s13244-023-01501-x. High-attenuation pulmonary abnormalities on CT are often caused by calcification that develops during the healing process of infectious granulomatous diseases. Thus, such abnormalities are usually considered to be non-significant findings. However, they may also be encountered in tumors, metabolic disorders, and occupational diseases. Furthermore, substances other than calcification with high atomic numbers, such as manganese, iron, platinum, barium, iodine, and mercury, also cause high-attenuation pulmonary abnormalities. With the widespread use of thin-slice CT, the frequency of detecting tiny high-attenuation lesions, such as those represented by diffuse pulmonary ossification, and the sensitivity of detecting calcification in lung cancers or metastatic tumors is increasing. Therefore, a wide variety of high-attenuation pulmonary abnormalities on CT may be encountered in daily clinical practice, and identifying the type of lesion can reduce the patients’ burden and healthcare costs related to unnecessary scrutiny and surgery. Further, proper identification allows the clinician to promptly determine whether further examination or the initiation of treatment is needed.This article aims to outline a stepwise diagnostic approach to high-attenuation pulmonary lesions that can be applied in clinical practice and provide insights into their pathological background. We have defined high-attenuation lesions in the lung in this article as areas that exhibit higher attenuation than the surrounding muscle tissue. Thus, they range from lesions that show faint high attenuation compared to skeletal muscle, such as hematomas, to those that show dense high attenuation, such as barium sulfate. This definition encompasses lesions located in both the lung fields and in the bronchial and pleural cavities, which can sometimes be challenging to differentiate from high-attenuation lesions in the lungs.Our proposed stepwise approach consists of five steps in which high-attenuation lesions are classified into nine categories based on the presence or absence of metallic artifacts, morphology, distribution, and concomitant findings. Diseases and findings showing high-attenuation pulmonary abnormalities occupy a broad spectrum of pathologies for which differential diagnosis can be complicated. Take, for example, the differentiation of small granulomatous lesions with calcification from pulmonary ossification. Without knowledge of pulmonary ossification, small high-attenuation lesions in the lower lobes might also be considered granulomatous lesions with calcification. Both are high-attenuation lesions showing linear or micronodular patterns, though pulmonary ossification is primarily predominant in the lower lobes. Therefore, the distinction between both can be made in STEP 4, which highlights the distributional predominance. Following our proposed algorithm, a differential diagnosis can be made even in similar high-attenuation lesions, which can lead to the correct diagnosis.A summary of the stepwise approach to high-attenuation pulmonary abnormalities is given in Fig. Thin sections are needed for the imaging evaluation of high-attenuation pulmonary abnormalities on CT. For example, pulmonary ossification is a tiny high-attenuation abnormality; thus, precise assessment requires a gapless scan of 1-mm slice thickness. Furthermore, the optimal assessment of calcification also requires thin sections to minimize volume averaging. Mediastinal window images ; level, 60 HU) are suitable for observation to assess mild calcification, whereas bone window  images are suitable for assessing dense calcification, ossification, and metallic substances. Coronal reconstruction is also useful in assessing diseases with a predilection of distribution.Evaluation of high-attenuation pulmonary abnormalities begins with ascertaining whether the lesions on CT scans are associated with metallic artifacts.This category comprises abnormalities of non-biological origin, including seed migration, barium sulfate aspiration, and metallic foreign body aspiration.Low-dose brachytherapy with a permanent implant of I-125 seeds is considered standard therapy for patients with localized prostate adenocarcinoma. Migration of I-125 seeds is reported in approximately 25% of patients and occurs in about 0.5% of cases per I-125 seed . ApproxiDiscrete high-attenuation lesions are often caused by aspiration of barium sulfate during an upper gastrointestinal study. The involved lung regions depend on the patient’s position during and after aspiration. Thus, the basal segments of the lower lobes are most often involved , formerly known as pulmonary sclerosing hemangioma, is a relatively rare, slow-growing, benign tumor that develops in middle-aged women. Although PSP generally presents as a solitary well-defined nodule, multiple nodules may also occur . PSP appCalcification has been reported in 41% of PSP on microscopic examination . HoweverPulmonary hamartoma is the most common mesenchymal tumor of the respiratory system, accounting for more than 75% of all benign lung tumors. Furthermore, hamartoma comprises varying amounts of at least two mesenchymal elements, including cartilage, fat, connective tissue, and smooth muscle, combined with respiratory epithelium.On high-resolution CT (HRCT), fat is identified in 34–50% of pulmonary hamartoma and calcification in 15–30%. The frequency of calcification increases with tumor size . CalcifiGranulomatous diseases are the most prevalent. However, tumors, such as multiple lung metastases and epithelioid hemangioendothelioma (EHE), may also develop. Amyloidosis is also an important differential diagnosis.Calcification or ossification commonly occurs with metastatic osteosarcoma and chondrosarcoma. In addition, metastatic carcinomas of the colon Fig. , ovary, EHE is a rare tumor of vascular origin, which presents an intermediate clinical course between hemangioma and angiosarcoma. Multiple small nodular pattern (< 15 mm in diameter) and multiple large nodular pattern (≥ 15 mm in diameter) were observed in 71.4% and 22.9% of patients, respectively. However, 5.7% of EHE showed a solitary lesion and may mimic lung cancer . EHE wasPulmonary hyalinizing granuloma (PHG) is a rare benign lung disease that presents as single or multiple lung nodules. The exact etiology is still unknown, although it is hypothesized to be an exaggerated immune response to antigenic stimuli caused by infection or an autoimmune process . PHG is The nodular lesions of PHG are classically described as well-defined solitary or multiple pulmonary nodules or masses. They may have irregular margins without preference for any particular parenchymal location. In their review, Lhote et al. reported that calcification was found in 6 of 138 cases (4%). Most of the PHG nodules or masses were between 1.5 and 5 cm in size  is an immunological lung disorder caused by hypersensitivity to Aspergillus other than A. fumigatus and other filamentous fungi can cause similar pathologies called allergic bronchopulmonary mycosis .Fig.Fig67].FThoracolithiasis is an entity in which one or more calcified or non-calcified loose bodies move freely in the thoracic cavity without any preceding trauma, intervention, or pleurisy. The prevalence of thoracolithiasis is approximately 0.086%. Thoracolithiasis usually manifests as a single calcified nodule and tends to occur in the left hemithorax. Most thoracoliths are located in the lower part of the pleural cavity, notably below the level of the top of each hemidiaphragm dome, which may be due to the effect of gravity. The longest diameter of thoracoliths ranges from 2.1 to 10.6 mm, and they all show calcification with an oval or round-shape. Because of the continuous movement associated with breathing, thoracoliths can become rounded and polished. Based on the presence of a necrotic fatty core in most thoracoliths and their left-sided predominance, dropping of twisted or torn epipericardial fat undergoing aseptic necrosis seems to be the most explainable etiology. As the mobility of the nodule is the critical finding in the diagnosis, comparison with older imaging studies is crucial  within the tracheobronchial tree and can be referred to as endobronchial, peribronchial, or transbronchial. Any focal process that results in dystrophic calcification can potentially lead to broncholithiasis. The most well-known theory of broncholithiasis is that necrotizing granulomas within the lymph nodes calcify and erode the bronchial wall, protruding into the tracheobronchial tree. In addition, necrotizing granulomas of TB can leak into the airway to facilitate transmission of the bacilli as a survival mechanism . FurtherThe incidence of broncholiths was highest in the bronchus intermedius, followed by the left upper lobe and right upper lobe  is characteristic. Subsequently, lacerations can take weeks to months to resolve as the air inside the laceration is gradually absorbed, eventually turning into a hematoma presenting as a solid, homogeneous, high-attenuation mass Fig. . HematomEhlers-Danlos syndrome subtype IV may cause spontaneous pulmonary laceration because of a defect of type III collagen. Following laceration, bleeding from disrupted alveolar walls and pulmonary vasculature results in the formation of hematoma .Micronodular pattern is characterized by calcified nodules of less than 3 mm in diameter that result from granulomatous infection and cement migration.Percutaneous vertebroplasty with acrylic cement  aims to prevent vertebral body collapse and pain in patients with pathologic vertebral bodies. As chest imaging is not routinely performed after this procedure, the true incidence of pulmonary cement embolism after percutaneous vertebroplasty is unknown. However, some studies reported an incidence of pulmonary cement embolism detected by chest radiograph and CT ranging from 4.6 to 6.8% , 87. VenThe proposed diagnostic algorithm has limitations. First, there may be more than one category for the same disease. Second, diseases that are likely to be encountered in daily clinical practice were eligible for inclusion in this algorithm. Therefore, rare diseases, such as extramedullary plasmacytoma , synoviaHigh-attenuation pulmonary abnormalities are caused by calcification, ossification, hemorrhage, iodine, and other etiologies. The majority of these abnormalities, such as granulomatous infections, are benign. However, they are also recognized in malignant diseases including lung cancers and metastatic tumors and can lead to a wide range of differential diagnoses. Some of these attenuations, such as hematomas and lipiodol embolisms, may change their morphology and characteristics over a relatively short time. Our proposed stepwise diagnostic algorithm for high-attenuation pulmonary abnormalities may help to recognize these imaging findings, to determine whether the associated diseases require further investigation, and to guide appropriate patient management.Additional file 1: Supplementary Fig. S1. Seed migration from prostate brachytherapy in an 88-year-old man. a CT image shows metallic artifact of a single seed that migrated to the right lower lobe (arrow). b Bone window image shows high punctate attenuation (arrow). Supplementary Fig. S2. Barium sulfate aspiration due to a history of nasopharyngeal carcinoma in a 55-year-old man. Bone window CT image shows barium sulfate with an obvious metallic artifact in the left lower lobe. Concurrent aspiration pneumonia is seen (arrows). Supplementary Fig. S3. Aspirated metallic dental implant in an 85-year-old man. a, b CT images show the metallic artifact of a dental implant that migrated to the right lower lobe bronchus (arrow). c Aspirated metal objects are easy to identify on the chest radiograph (arrow). Supplementary Fig. S4. Hamartoma in a 70-year-old man. a CT image shows a slightly lobulated nodule in the right lower lobe. b Mediastinal window image shows that the nodule contains focal calcification (arrows). Fat is absent. Supplementary Fig. S5. Metastatic papillary thyroid cancer in a 74-year-old man. CT image shows a soft tissue nodule with multiple nodular calcifications in the right upper lobe (arrow). Supplementary Fig. S6. Amyloidosis in a 74-year-old woman. Bone window CT image shows a mass along with the bronchovascular bundle with numerous irregular calcifications in the lingular segment (arrows), which is compatible with the nodular parenchymal type of amyloidosis. Supplementary Fig. S7. Silicosis (simple form) in a 76-year-old man. a CT image shows multiple small nodules with a perilymphatic  distribution in both lungs. b Bone window image shows calcification in the nodules (arrows). Supplementary Fig. S8. Metastatic calcification in a 47-year-old man. CT image shows lobular ground-glass opacity with a centrilobular distribution. Focal dense calcification is also seen (arrows). Supplementary Fig. S9. Metastatic calcification in a 67-year-old man. Mediastinal window CT image shows dense calcification in the bilateral upper lobes. Note vascular calcification in the chest wall (arrows). Supplementary Fig. S10. Pleuroparenchymal fibroelastosis (PPFE)-like lesion in a 64-year-old male. Bone window CT image shows subpleural opacities with high punctate attenuation (arrows). Histopathology reveals calcification and ossification in the subpleural fibroelastosis. Supplementary Fig. S11. Secondary diffuse pulmonary ossification due to acute respiratory distress syndrome (ARDS) in a 75-year-old man. a CT image shows mixed coarse and fine reticulation with subpleural predominance in the left upper lobe. b Bone window image shows high-attenuation nodules in the area of coarse reticulation (arrows). High-attenuation nodules gradually increased during a 2-year period after suffering from ARDS. Supplementary Fig. S12. Idiopathic dendriform pulmonary ossification in a 65-year-old man. a CT image shows the lattice-like distribution of multiple linear and nodular opacities in the peripheral area of the lung bases. b Bone window CT image shows numerous high-attenuation small linear and branching structures (arrows). c Volume rendering image of the lungs. Bony lesions are shown as yellow color structures. d Magnified volume rendering image of the right lower lobe. Dendriform and coral-like shapes of bony structures are shown. Supplementary Fig. S13. Thoracolithiasis in a 50-year-old man. a, b Initial CT images show a smooth-marginated calcified nodule (arrow). c, d Follow-up CT images obtained eight years later show migration of the calcified nodule into the posteroinferior region (arrow). Supplementary Fig. S14. Tracheobronchial amyloidosis in a 22-year-old woman. CT image shows bronchial wall thickening with calcification (arrows). Supplementary Fig. S15. Postoperative status of pneumothorax in a 65-year-old man. Bone window CT image shows high-attenuation linear structures that abut the pleura, which correspond to surgical staples, are seen (arrows). Supplementary Fig. S16. Inactive tuberculosis in a 70-year-old man. CT image shows linear calcification in the right upper lobe, adjacent to the bulla (arrows). Supplementary Fig. S17. Inactive tuberculosis in a 90-year-old man. CT image shows numerous calcified nodules associated with soft tissues (arrows). Supplementary Fig. S18. Ghon focus in a 60-year-old man. CT image shows nodular calcification in the periphery of the right lower lobe (arrow). Supplementary Fig. S19. Broncholithiasis in a 58-year-old woman. Photomicrograph  depicts a concentric decalcified stone that is considered to have originated from mucus. Supplementary Fig. S20. Broncholithiasis in a 71-year-old man. CT image shows a calcified nodule within a dilated bronchus near the left hilum (arrow). Calcified lymph nodes at the left hilum and mediastinum are also shown (arrowheads). Supplementary Fig. S21. Cement migration due to percutaneous vertebroplasty in a 77-year-old woman. a CT scout view shows post-multilevel vertebroplasty (arrows). b Postoperative bone window CT image shows cement migration to the left lower lobe (arrow).


Urinary bladder masses in children are extremely rare. Certain benign conditions  can mimic malignant bladder masses. In this report, we present a unique case of a urachal cyst masquerading as a bladder malignancy. Unlike the typical location of urachal cysts along the course of the urachal tract, the cyst in this case was unexpectedly situated within the urinary bladder, leading to diagnostic difficulties.A 2-year-old Bahraini boy presented with hematuria and dysuria for 2 weeks. There was no history of fever, abdominal pain, or vomiting. Physical examination yielded normal findings. Urinalysis showed numerous red blood cells and revealed positive results for nitrites and leukocyte esterase. Abdominal ultrasound showed a well-defined soft tissue lesion with internal vascularity located at the apex of the urinary bladder. Subsequently, magnetic resonance imaging demonstrated a thick-walled cystic structure arising from the anterosuperior wall of the bladder and protruding into its lumen. The patient underwent complete excision of the bladder lesion for the presumed diagnosis of rhabdomyosarcoma. Histopathological examination showed a fluid-filled space lined by stratified squamous epithelium with areas of intestinal metaplasia, revealing an unexpected diagnosis of a urachal cyst. The patient was discharged with complete resolution of symptoms.Intravesical urachal cysts are a rare type of congenital urachal anomaly that may simulate a bladder malignancy, particularly if associated with infection. This case emphasizes the importance of considering urachal cysts in the differential diagnosis of bladder masses, especially in children, and specifically when the lesion is midline in the anterosuperior wall of the bladder. Unlike in adults, urinary bladder masses in children are extremely rare and have distinct histologic types. Except for rhabdomyosarcoma, which is the most common pediatric bladder malignancy, most bladder masses are benign. These include fibroepithelial polyps, inflammatory myofibroblastic tumors, urothelial papilloma, neurofibroma, and leiomyoma . ChildreCertain potential pitfalls may lead to erroneous reporting of bladder masses on ultrasound . For exaA 2-year-old Bahraini boy was brought to the emergency department by his mother due to the presence of blood in his urine and diaper for two weeks. The child also complained of painful urination and foul-smelling urine. The patient had no history of fever, bruising, abdominal pain, or vomiting, and there was no reported trauma or upper respiratory tract infection. The child had a medical history of eczema and delayed speech. The family history was unremarkable.On physical examination, the child appeared active and well-nourished, with vital signs within normal limits. Abdominal examination revealed the abdomen to be soft, non-tender, and without palpable masses, with normal bowel sounds in all quadrants. The umbilicus appeared normal, with no visible abnormalities or signs of discharge. Examination of other bodily systems also revealed normal findings.Urine analysis showed > 100 red blood cells per high-power field, 50 white blood cells per high-power field, and positive results for nitrite and leukocyte esterase. Other laboratory investigations, including hematological and biochemical parameters, showed no abnormalities.To rule out any underlying congenital urinary tract abnormalities as the cause of the urinary tract infection, an abdominal ultrasound examination was performed. The scan revealed a well-defined, lobulated, soft tissue lesion arising from the apex of the urinary bladder with internal vascularity on color Doppler. There was a focal irregularity of the bladder wall at the site of the lesion, raising the possibility of an extravesical extension. Low-level mobile echoes were also observed within the lumen of the bladder Fig. . Both kiAfter a thorough evaluation, the multidisciplinary team determined that the best course of action was laparotomy with segmental cystectomy for the suspected rhabdomyosarcoma. The decision was based on several factors, including the symptomatic nature of the lesion, highly suspicious initial clinical and radiological findings, and the technical difficulties of performing a cystoscopic biopsy in pediatric patients, where specialized equipment required for the procedure was not readily available, and delaying management was not advisable. The team also considered the potential for perforation and seeding of malignant cells into the peritoneal cavity due to the location of the lesion.The patient underwent laparotomy and complete excission of the lesion. A gross examination revealed a firm mass with frond-like papillary projections. Subsequent histopathological examination revealed a fluid-filled space lined by stratified squamous epithelium with areas of intestinal metaplasia, without any evidence of atypia or malignancy. The immunohistochemistry results were negative for ALK1, MyoD1, and cytokeratin AE1/AE3. These unexpected findings led to the diagnosis of a benign urachal cyst (Fig. Postoperatively, the medical team closely monitored vital signs and hydration status. A urinary catheter was kept in place for 1 day to monitor for bleeding and ensure proper bladder drainage. A strict regimen of hygiene practices was implemented to prevent infection, and pain management was closely monitored to ensure the patient's comfort. The patient was discharged on the second postoperative day with normal urine analysis results. At the 1-month follow-up visit, the mother reported no active complaints, and there was no recurrence of the initial symptoms.We present a rare case of intravesical urachal cyst masquerading as a bladder malignancy. The urachus is a tubular embryonic structure that connects the fetal urinary bladder to the allantois. Normally, the urachus is obliterated at birth and forms a fibromuscular structure known as the median umbilical ligament . HoweverIntravesical urachal cysts are a recently recognized congenital urachal anomaly , 6. YagiCongenital urachal anomalies are relatively uncommon , 12. GivRhabdomyosarcoma was initially suspected in the present case before the histopathological diagnosis confirmed it as a urachal cyst. Rhabdomyosarcoma typically presents within the first 5 years of life and tends to affect males more frequently . Its chaOn ultrasound, rhabdomyosarcoma appears as a predominantly solid, round or lobulated mass with internal flow on color Doppler . On MRI,The management of congenital urachal anomalies is controversial. Some authors advocate using a conservative approach in asymptomatic or even mildly symptomatic patients, as it became increasingly recognized that spontaneous regression of urachal remnant may occur , 16. TraIntravesical urachal cysts are a rare, yet important, type of congenital urachal anomaly. In unusual instances, particularly if associated with infection, urachal cysts may have atypical radiologic features that can be indistinguishable from bladder malignancy. It is important to always consider the differential diagnosis of urachal cysts in patients with a focal midline mass related to the anterosuperior wall of the urinary bladder. An accurate diagnosis of this condition is crucial to provide reassurance to patients and obviate unnecessary radical surgical procedures.


Acute and chronic lower back pain can be commonly caused by intervertebral disc prolapse. This prolapse usually occurs in the dorsal direction and towards the anterior epidural space. In extremely rare cases, this migration/herniation can be seen approaching the posterior epidural space. One such rare instance has been recorded and described in our patient, a 53-year-old with a history of hypertension who presented with persistent lower back pain, radicular in nature, and recent acute aggravation, leading to mobility impairment. The patient experienced numbness in the lower limbs, urinary incontinence, and irregular bowel movements. Sensory deficits were noted along the L3 dermatome.The patient underwent an L3 laminectomy, revealing extruded disk fragments causing the compression. After surgery, the patient's power in the lower limbs began to improve, with significant recovery by discharge and complete resolution of bowel and bladder incontinence. This case highlights the diagnostic and therapeutic challenges of posterior epidural mass-like lesions in the lumbar spine, emphasizing the importance of prompt surgical intervention in restoring neurological function. The successful outcome underscores the significance of early diagnosis and intervention in such cases, ultimately improving the patient's quality of life. Sequestration of a disc is described as the herniation of the disc, which perforates the posterior longitudinal ligament, ultimately leading to the migration of the disc fragments into the epidural space . The disThe rarity of this condition has resulted in a limited understanding of the root cause behind this condition. Furthermore, despite the potential for severe neurological impairment associated with PEMLIF, diagnosing and treating this condition remains quite challenging. By documenting a unique case of right-sided PEMLIF mimicking an epidural mass, this study aims to shed light on the atypical manifestations of this condition. The study seeks to contribute to the existing medical literature and assist healthcare professionals in effectively recognizing and treating PEMLIF. To the author’s best knowledge, this is the first case report documenting an unusual case of right-sided PEMLIF mimicking an epidural mass.Patient informationA 53-year-old man with a history of hypertension came to the Neurosciences Outpatient Department with a recent complaint of persistent lower back pain that has been bothering him for two years. The pain was radicular in nature and had an acute aggravation for the last five days, which impeded the patient's ability to ambulate. Furthermore, the patient described experiencing numbness in his lower limbs, urinary incontinence, and irregular bowel movements. Notably, the patient did not mention any previous instances of injury or swelling in the affected area.Clinical findingsThe clinical examination of the lower limbs indicated normal muscle bulk and tone. However, there was reduced power, graded at 4/5, in both hips. The power in the left knee was also noted as 5/5, whereas it was 3/5 in the right knee. Furthermore, the power was graded at 1/5 on dorsiflexion and 2/5 on plantarflexion, bilaterally in the ankles. The examination also revealed a positive straight leg test and reduced sensation along the L3 dermatome.Diagnostic approachFollowing a magnetic resonance imaging (MRI) of the spine, the results revealed the presence of a posterior epidural mass measuring 3.5 cm×2 cm×1 cm at the L2-L3 level. This mass appeared isointense with the intervertebral disc on both T1- and T2-weighted images. Additionally, there was evidence of a disrupted outer annulus in the adjacent disc and tract-like enhancement extending into the right posterior epidural space (Figure Therapeutic interventionThe patient underwent an L3 laminectomy using a posterior approach. Following the visualization and removal of the ligamentum flavum, extruded disk fragments that resembled an epidural tumor were observed. These fragments were embedded in fibrous epidural tissue, causing thecal sac compression Figure .On histological examination, the diagnosis of PEMLIF was confirmed.These fragments were removed with great precision, and the L2-L3 space was examined. A rupture in the posterior longitudinal ligament was discovered, and L2-L3 discectomy was carried out.Outcome and follow-upOn postoperative day one, the patient began to regain power in the lower limbs, recorded 3/5 bilaterally. On discharge, the patient regained the power of 4/5 bilaterally with no bowel and bladder incontinence. At three months of follow-up, the patient could walk without support and had no bowel and bladder involvement.The above-discussed case is extremely rare and was initially described in 1973 by Vincenzo Lombardi . While tClinical signs of free fragment migration are usually associated with radicular pain, but the posterior migration of fragments can also lead to CES . The cliThe preferred method for diagnosing this condition is a lumbar spine MRI scan enhanced with gadolinium, considered the gold standard. A study conducted in the recent past reported a 39.2% incidence of posterior migrations of the disc at the L3-L4 level due to the migration-conducive anatomy at that level [The scan of our lesion showed a hypointense T1-weighted image and around 80% of cases demonstrated a hyperintense T2 scan . This hyRegarding the other differentials considered, facet cysts have peculiar signal intensity on scans with distinct anatomical associations. An epidural abscess may present findings similar to PEMLIF, but the absence of hallmark signs and symptoms of infectious etiology rules out this diagnosis. Hematoma usually presents with isointense or hyperintense T1-weighted images with a history of trauma. The migrated fragments often show no continuity with the disc space of origin. On the other hand, hematomas maintain this continuity . Many caPosterior epidural migration of lumbar disc fragments is an exceedingly rare condition. It may manifest with symptoms such as radicular compression, lower back pain, or even the complex presentation of CES. Rim augmentation, which can resemble the symptoms of an intraspinal extradural tumor, makes it difficult to make a preoperative diagnosis. It is believed that an inflammatory reaction, manifested by the growth of vascular granulation tissue around the disc mass, is responsible for the peripheral enhancement of the disc fragment seen in contrast-enhanced imaging investigations.To arrive at an accurate diagnosis of PEMLIF, considering factors like the patient's medical history, a detailed analysis of MRI scans to identify similarities in signal intensities between the disc fragment and the adjacent intervertebral disc and peripheral rim enhancement is crucial for the precise diagnosis of this rare spinal condition.