Fibroadenoma of the vulva with pseudoangiomatous stromal hyperplasia: A common neoplasm in uncommon site.

Mammary-type tissue in the vulva was first described in 1872 but has been rarely reported in the literature. This tissue was previously considered as ectopic breast tissue that occurs as a result of incomplete regression of the milk line. Similar to native breast tissue, ectopic mammary tissue is hormone-sensitive and can develop benign changes, such as fibroadenoma, as well as malignant changes. A more recent theory suggests that these benign and malignant mammary-type entities arise from mammary-like anogenital glands, which constitute normal vulvar components. We report a case of a 41-year-old woman who presented with a chronic asymptomatic cyst on the left vulva that eventually became uncomfortable, especially on standing. The cyst was located on the labium minus, measuring 1.0 × 0.5 cm, with no identified erythema or other skin abnormalities. Excision of the lesion and subsequent microscopic examination showed a circumscribed mass with a nodular overgrowth of epithelial and stromal components, resembling a mammary fibroadenoma with pseudoangiomatous stromal hyperplasia. We bring to attention this rare diagnosis and the importance of considering it in the presence of a vulvar lesion. The malignant and recurrence potential of mammary-type tissue necessitates excision with clear margins and close monitoring of these patients.

Vertebral Angiomatosis in a Dog.

A 3 yr old spayed female Cavalier King Charles spaniel was referred for insidious ataxia and paraparesis. A thoracolumbar lesion was suspected. Computed tomography showed focal osteolysis of the vertebral body and pedicles of T5. In addition, a hyperdense, extradural material within the vertebral canal, causing spinal cord compression on the right side, was present. The lesion was confirmed with magnetic resonance imaging. A T4-T5 hemi-dorsal laminectomy was performed to decompress the spinal cord. Histopathological examination was consistent with vertebral angiomatosis. After the surgery, the dog rapidly improved; however, 5 mo later the clinical signs relapsed. Vertebral angiomatosis is a vasoproliferative disorder, rarely reported as a cause of myelopathy in cats. This condition has not previously been reported in dogs. This case report describes the clinical features, the diagnostic findings, and the follow-up of a young dog with vertebral angiomatosis.

Pseudoangiomatous stromal hyperplasia of the breast: Clinical evaluation.

BACKGROUND: Pseudoangiomatous stromal hyperplasia is a rare benign breast stromal proliferative lesion of the breast. Clinical presentation ranges from rapidly growing mass to incidental identification in routine screening. This difference in manifestation and its rarity makes it difficult to be a standard treatment protocol. Therefore, we aimed to share our clinical experience in Pseudoangiomatous stromal hyperplasia. METHODS: The files of patients who underwent core biopsy or surgical excision due to a breast mass and resulted in pseudoangiomatous stromal hyperplasia between January 2013 and December 2021 were included in the study. RESULTS: 17 patients with a median age of 37 (22-68) were found Pseudoangiomatous stromal hyperplasia confirmed by surgical excision or core biopsy. Chosen treatment option was observation in 8 patients (47.1%), while surgical excision was used in 9 (52.9%) patients. The mean follow-up period was 55.24 ± 26.72 (13-102) months. None of the patients observed the Malignant transformation during the follow-up period. CONCLUSION: For Pseudoangiomatous Stromal Hyperplasia of the breast, surgical excision with clean margins or close follow-up after diagnosis confirmation by tissue biopsy is sufficient. Pseudoangiomatous Stromal Hyperplasia is not a risk factor for developing breast cancer.

Clinicopathological Analysis of Sturge-Weber Syndrome with Focal Cortical Dysplasia FCD IIIc.

Objective: To investigate the clinicopathological features of children with Sturge-Weber syndrome and to analyze the correlation between the distribution area of leptomeningeal angiomatosis, the degree of cerebral cortical calcification, and the degree of cerebral atrophy associated with epileptic seizures. Methods: 10 children were diagnosed with SWS with FCD IIIc by histopathology and immunohistochemistry. Spearman correlation analysis was used to calculate the association of SWS with FCD IIIc and seizures in children. Results: The leptomeningeal angiomatosis area was markedly positively correlated with the degree of brain atrophy in 10 children with SWS (r = 0.783, p = 0.007). The distribution of leptomeningeal hemangiomatosis, the degree of cortical calcification, and brain atrophy were not significantly correlated with epilepsy. Conclusion: SWS may be accompanied by FCD IIIc. The more extensive the cerebral lobes of leptomeningeal angiomatosis in SWS, the more pronounced the brain atrophy.

Tumoral pseudoangiomatous stromal hyperplasia (PASH) of the male breast: A systematic analysis.

<b><br>Aim:</b> The study was conducted to systematically analyze the recent peer-reviewed literature related to tumoral pseudoangiomatous stromal hyperplasia (PASH) in males.</br> <b><br>Materials and methods:</b> Articles published in peer-reviewed journals of repute from 2012 to 2023 were evaluated for eight variables: 1) the age of the patient, 2) clinical presentation, 3) laterality (unilateral or bilateral), 4) associated breast disorder, 5) the presence of systemic co-morbidity, 6) imaging modalities aiding diagnosis, 7) treatment, and 8) recurrence.</br> <b><br>Results:</b> There were 10 patients ranging in age from 1 year to 67 years (mean: 34.5 years). In 8 cases (80%), there were breast lumps; in 2 cases (20%), there were axillary lumps at presentation. Pain was not a prominent symptom. Six patients (60%) had another breast disorder at presentation and 3 (30%) had significant systemic co-morbidities, including diabetes and renal insufficiency. The greatest dimension of the lumps ranged from 2 cm to 12 cm, with 80% of the lumps being below 5 cm in size. Imaging modalities utilized in evaluation included breast ultrasound (USG), mammograms, computerized tomography (CT), and magnetic resonance imaging (MRI). All the cases were treated surgically, and the final diagnosis was made only after the analysis of the surgical specimen. Recurrence was recorded in 2 (20%) cases.</br> <b><br>Conclusions:</b> Tumoral PASH is a benign myofibroblastic proliferation disorder rarely reported in males. This condition should be considered a differential diagnosis of breast or axillary lumps, and an evaluation should be initiated accordingly. The recommended course of treatment is adequate surgical excision with regular clinical and radiological follow-up.</br>.

Diffuse dermal angiomatosis: a rare cause of painful ulceration in renal insufficiency.

Diffuse dermal angiomatosis is a rare, benign, reactive cutaneous vascular proliferation that has been reported in the context of end-stage renal failure and can rarely be associated with arteriovenous fistulas. We report a striking clinical resolution following prompt diagnosis and subsequent arteriovenous fistula reversal. This case further demonstrates that accurate diagnosis is particularly rewarding since correct therapeutic approach can be curative.

Unusual Gigantiform Angiomatosis of Jawbone.

ABSTRACT: The purpose of this paper is to highlight rare highly infiltrative massive Angiomatosis and depict surgical outcome of a rare case series of gigantic lesions in the maxillofacial region. Data were recorded from the medical records of patients. Predictor variables were drawn from demographics, age, gender, site, evaluation of surgical treatment. The outcome variables were the challenges encountered and related complications. Out of 6 patients, the youngest was 10 and the oldest was 26 years old. Soft tissue and jawbone involvement were seen in 3 patients each with a single massive lesion involving both maxilla and mandible. All 6 patients had wide excision of the lesion with one patient having 2 stage procedures. No complications or recurrence was seen at 1-year follow-up. Angiomatosis is a rare and benign lesion in the head and neck region. its diffuse infiltrating nature may give a false malignant picture. it clinically mimics Hemangioma or Arteriovenous (AV) malformation thus requires thorough evaluation and its surgical intervention is challenging as described in this case series because of its magnitude and infiltrative nature.

Systemic cystic angiomatosis mimicking metastatic cancer.

BACKGROUND: Cystic angiomatosis is a rare benign disease presents with multiple lytic and sclerotic bone lesions mimicking a metastatic malignant neoplasia with less than 50 cases have been reported in literature so far. CASE PRESENTATION: We reported a case of a 48-year-old woman who presented to an oncology clinic with multiple lytic and sclerotic bone lesions. Oncologic investigation for metastatic malignant neoplasia started. After that the negative results were obtained by evaluating the primary tumor site, a final diagnosis of cystic angiomatosis was made according to bone biopsy results. CONCLUSIONS: Cystic angiomatosis is a rare disease with unpredictable prognosis. It can mimic metastatic malignancy especially when it presents at old age.

A huge chest wall angiomatosis with pleural and rib invasion: A case report.

RATIONALE: Angiomatosis is a rare non-neoplastic proliferative vascular lesion that typically develops during childhood or adolescence with a female predominance. Management of angiomatosis is challenging because of the manifestation of a wide variety of lesions as well as their invasive and highly recurrent nature. PATIENT CONCERNS: We report the case of a 74-year-old man who presented with a right lower back mass that persisted for a decade. The mass progressively enlarged and had been painful in the previous month. DIAGNOSIS: Computed tomography (CT) revealed suspected lipomatous sarcoma with invasion of the ribs, pleurae, and lung parenchyma. The final pathological examination revealed angiomatosis. INTERVENTIONS: The patient underwent wide composite excision of the tumor along with excision of the pleura and lung nodules in the right lower and middle lobes via video-assisted thoracoscopic surgery (VAST). Fasciocutaneous rotational flap reconstruction was performed immediately after the wide composite excision and video-assisted thoracoscopic surgery (VAST). OUTCOMES: The patient recovered uneventfully, was discharged without complications, and tolerated the daily activities well. LESSONS: Angiomatosis is a rare benign vascular tumor that frequently mimics malignancy. Even if the patient profile does not match the reported epidemiology of this disease, differential diagnosis should be considered. Complete resection is the mainstay of treatment for the prevention of recurrence.

Rare mimic of angiosarcoma: Erythema ab igne with reactive angiomatosis.

Erythema ab igne is an uncommon physical dermatosis that presents with localized patches of reticulated erythema and hyperpigmentation corresponding with the underlying dermal venous plexus. The rash occurs in response to chronic heat exposure that does not meet the threshold for thermal burn of the skin. The histopathologic findings are characterized by atrophy and thinning of the epidermis, focal hyperkeratosis, and keratinocyte atypia. The dermis displays dilated capillaries, evidence of pigment incontinence, and prominent elastotic material. We report a case of a 65-year-old male who presented to his primary care physician with a 1-year history of reticular erythema and hyperpigmentation with focal ulceration on his right lateral leg. Histopathology on biopsy revealed mild hyperkeratosis and focal epidermal atrophy; however, the most striking finding was a proliferation of dermal vascular spaces lined by pleomorphic endothelial cells and numerous mitotic figures, which was morphologically compatible with angiosarcoma. However, clinicopathologic correlation and immunostaining revealed an actual diagnosis of erythema ab igne with reactive angiomatosis. Reactive angiomatosis-morphologically mimicking angiosarcoma-is a rarely reported feature of severe erythema ab igne, and dermatopathologists should be aware of this possibility to avoid misdiagnosis of erythema ab igne as angiosarcoma.

Expanding the phenotypic spectrum of FINCA (fibrosis, neurodegeneration, and cerebral angiomatosis) syndrome beyond infancy.

Fibrosis, neurodegeneration, and cerebral angiomatosis (FINCA, MIM#618278) is a rare clinical condition caused by bi-allelic variants in NHL repeat containing protein 2 (NHLRC2, MIM*618277). Pulmonary disease may be the presenting sign and the few patients reported so far, all deceased in early infancy. Exome sequencing was performed on patients with childhood interstitial lung disease (chILD) and additional neurological features. The chILD-EU register database and an in-house database were searched for patients with NHLRC2 variants and clinical features overlapping FINCA syndrome. Six patients from three families were identified with bi-allelic variants in NHLRC2. Two of these children died before the age of two while four others survived until childhood. Interstitial lung disease was pronounced in almost all patients during infancy and stabilized over the course of the disease with neurodevelopmental delay (NDD) evolving as the key clinical finding. We expand the phenotype of FINCA syndrome to a multisystem disorder with variable severity. FINCA syndrome should also be considered in patients beyond infancy with NDD and a history of distinct interstitial lung disease. Managing patients in registers for rare diseases helps identifying new diagnostic entities and advancing care for these patients.