Metastasis of breast cancer to the thyroid gland.

Metastases to the thyroid gland from nonthyroidal malignant tumors are rare but significant. They are often asymptomatic, indicating advanced-stage primary tumors and poor prognosis. Although infrequently, breast cancer (BC) can metastasize to the thyroid gland. We present the case of a 56-year-old woman with a history of BC who presented with a nodular goiter. Physical examination and imaging revealed a thyroid nodule and cervical lymph nodes with suspicious features. Fine-needle aspiration biopsy (FNAB) confirmed the presence of atypical epithelial cells in the thyroid nodule and lymph nodes. Further evaluation, including positron emission tomography, histological biopsy, and immunohistochemistry, supported the diagnosis of metastatic BC to the thyroid gland. Due to the local extent of the disease, total thyroidectomy was not feasible. The treatment with ribociclib and letrozole was initiated, but unfortunately, the patient had an unfavorable progression with the development of metastasis in the nervous system. Metastatic carcinoma to the thyroid gland is rare but has increased due to improved diagnostic techniques. BC can metastasize to the thyroid. Diagnosis involves imaging, FNAB, and immunohistochemistry. Treatment options include surgery, radiotherapy, and chemotherapy, but the prognosis is generally poor.

Las metástasis en la glándula tiroides a partir de tumores malignos no tiroideos son raras pero significativas. A menudo son asintomáticas, lo que indica tumores primarios en etapas avanzadas y un mal pronóstico. Aunque infrecuentemente, el cáncer de mama puede metastatizar en la glándula tiroides. Presentamos el caso de una mujer de 56 años con antecedente de cáncer de mama que consultó por bocio nodular. El examen físico y las imágenes revelaron un nódulo tiroideo y ganglios linfáticos cervicales con características sospechosas. La punción aspiración con aguja fina confirmó la presencia de células epiteliales atípicas en el nódulo tiroideo y los ganglios linfáticos. Una evaluación adicional, que incluyó tomografía por emisión de positrones, biopsia histológica e inmunohistoquímica, respaldó el diagnóstico de cáncer de mama metastásico en la glándula tiroides. Debido a la extensión local de la enfermedad, no fue factible realizar una tiroidectomía total. Se inició el tratamiento con ribociclib y letrozol, pero desafortunadamente la paciente tuvo una progresión desfavorable con el desarrollo de metástasis en el sistema nervioso. El carcinoma metastásico en la glándula tiroides es raro, pero ha aumentado debido a las técnicas de diagnóstico mejoradas. El cáncer de mama puede metastatizar en la tiroides. El diagnóstico implica imágenes, punción aspiración con aguja fina e inmunohistoquímica. Las opciones de tratamiento incluyen cirugía, radioterapia y quimioterapia, pero el pronóstico generalmente es desfavorable.

Fatal Case of Naegleria fowleri Primary Amebic Meningoencephalitis from Indoor Surfing Center, Taiwan, 2023.

We investigated a fatal case of primary amoebic meningoencephalitis from an indoor surfing center in Taiwan. The case was detected through encephalitis syndromic surveillance. Of 56 environmental specimens, 1 was positive for Naegleria fowleri ameba. This report emphasizes the risk for N. fowleri infection from inadequately disinfected recreational waters, even indoors.

Canine primary ureteral leiomyosarcoma treated with unilateral left ureteronephrectomy.

Background: Primary ureteral neoplasms are extremely rare in dogs, and ureteral involvement usually occurs owing to the invasion of renal and bladder tumors. Case Description: This case report describes a 12-year-old intact male mixed-breed dog referred to a private clinic with a six-month history of abdominal distention. A physical examination revealed mild abdominal pain. Hematological tests detected normocytic-normochromic anemia (hematocrit 33.6% [reference interval-RI: 37%-55%], red blood cells 4.93 M/µl [RI: 5.5-8.5 M/µl], and hemoglobin 12.4 g/dl [RI: 12-18.0 g/dl]). The results from the leukogram, thrombogram, renal, and hepatic panels were within the reference intervals for dogs. Abdominal ultrasonography revealed a cavitary mass measuring approximately 12 cm in diameter as the largest tumor in the left abdominal region over the left hepatic lobe or mesenteric site. Chest radiography did not reveal any metastasis. Therefore, the patient underwent exploratory laparotomy, during which the left ureter was found to be affected by a 12-cm mass that adhered to the left kidney. A unilateral left ureteronephrectomy was performed, and histology and immunohistochemistry (IHC) confirmed well-differentiated primary ureteral leiomyosarcoma. The patient survived for 130 days but died of lung metastasis. Conclusion: Ureteral leiomyosarcoma should be investigated and included in the list of differential diagnoses for primary ureteral neoplasms. Regardless of the therapeutic modality, the prognosis of ureteral leiomyosarcoma may be unfavorable, as shown in this report.

Rapidly progressive cystic lung disease in a patient with a scalp lesion.

We describe an elderly patient presenting with pneumothorax, cystic lung disease and a scalp lesion. The pneumothorax resolved after placing a chest tube and suction but recurred within a week. Progression of cystic features was also seen, and biopsies of the lung and scalp lesions were performed. Immunohistochemistry was positive for markers of endothelial cells (CD31 and ERG) and negative for markers expected to be positive in alveolar cells (keratin AE1/AE3 and TTF-1), supporting the diagnosis of metastatic angiosarcoma. Palliative chemotherapy did not prevent progression and the patient expired soon after. In describing the clinico-radiological correlation of metastatic angiosarcoma, we also briefly describe the approach to cystic lung disease. Understanding the pathophysiology of cyst formation in metastatic angiosarcoma may help clinicians to better appreciate and manage the full spectrum of cystic lung disease, especially with atypical features.

Case report of fatal immune-mediated myocarditis following treatment with davoceticept (ALPN-202), a PD-L1-dependent CD28 costimulator and dual PD-L1/CTLA-4 checkpoint inhibitor, in combination with pembrolizumab.

Engagement of programmed death-1 (PD-1) and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) can interfere with the CD28 signaling requisite for T-cell activation. While immune checkpoint inhibitors (ICIs) can relieve this suppression, they are unable to drive CD28 costimulation that may mechanistically contribute to ICI resistance. Thus, CD28 costimulation in the context of checkpoint inhibition may activate immunosuppressed T-cells in the tumor microenvironment. Davoceticept (ALPN-202) is an Fc fusion of a CD80 variant immunoglobulin domain (vIgD) designed to mediate PD-L1-dependent CD28 costimulation while inhibiting the PD-L1 and CTLA-4 checkpoints. PD-L1-restriction of davoceticept's CD28 costimulatory activity may minimize systemic T-cell activation and avoid untoward systemic toxicities. At the same time, preclinical studies have suggested that treatment with davoceticept during PD-1 inhibition may enhance antitumor activity by upregulating PD-L1, potentially synergizing with davoceticept's PD-L1-dependent costimulatory mechanism. This report details two cases of fatal cardiac events following treatment with davoceticept in combination with pembrolizumab (anti-PD-1) in the phase 1 study, NEON-2. Both events occurred in females in their 60s; one with choroidal melanoma and prior immunotherapy, the other with ICI-naïve microsatellite stable colorectal cancer. The clinical courses were fulminant with symptom onset at 2 weeks, followed by rapid decline. Cardiac autopsy from one patient confirmed immune-related myocarditis, and immunosequencing revealed expansion of a single T-cell clone that was not present in the pretreatment tumor. These cases highlight the importance of understanding risk factors that may contribute to immune-related myocarditis and other severe immune-related adverse events when CD28 agonism is targeted in the context of checkpoint inhibition.NEON-2 (NCT04920383).

Fluid Overload-Associated Large B-Cell Lymphoma with Light Chain Restriction Type Plasma Cell Infiltration: A Case Report.

BACKGROUND Fluid overload-associated large B-cell lymphoma (FO-LBCL) is a recently described malignant lymphoma that presents with serous effusions in the pleura, peritoneum, and/or pericardium but without an identifiable lymphoma mass. This report describes the case of an 80-year-old man who presented with a pleural effusion and describes the approach to diagnosis and management of FO-LBCL. CASE REPORT We present a case of an 80-year-old man who presented with right pleural effusion and shortness of breath at work. Initial radiological assessment suggested a pleural effusion on the right side, without an identifiable mass, given the patient's symptoms and imaging characteristics. Subsequently, he underwent a pleural fluid puncture and biopsy. Based on the initial pathological assessment, malignant lymphoma, a non-epithelial tumor, was considered likely, but differentiation from reactive proliferative cells was difficult, given the patient's symptoms and cytologic characteristics. Postoperatively, histopathological examination and immunohistochemistry confirmed a diagnosis of FO-LBCL. After 1 year of follow-up, the condition had progressed and the patient died due to recurrence. CONCLUSIONS This report has presented a case of FO-LBCL in an elderly man with pleural effusion and described how this rare and recently described lymphoma was diagnosed and managed.

[Allogeneic haematopoietic stem cell transplantation induced remission of periarteritis nodosa associated with azacytidine-refractory myelodysplastic syndrome]. / Mise en rémission par allogreffe de cellules souches hématopoïétiques d'une péri artérite noueuse associée à un syndrome myélodysplasique réfractaire à l'azacytidine.

INTRODUCTION: Periarteritis nodosa (PAN) is a vasculitis affecting medium-vessel and may be associated with myelodysplastic syndrome. This association needs a simultaneous treatment of the vascular and the hematological disease. However limited data are available on the benefit of hematological treatment, and in particular allogeneic stem cell transplantation, in this situation. CASE REPORT: A 32-year-old patient with refractory periarteritis nodosa and simultaneous myelodysplastic syndrome, was treated with chemotherapy followed by hematopoietic stem cell allograft. The symptoms relating to PAN improved, allowing to decrease the dose of prednisone down to 5mg/d. However, a hematological relapse occurred two months later leading to the patient's death. CONCLUSION: Hematopoietic stem cell allograft may represent a therapeutic option in the management of severe or refractory autoimmune diseases when the hematological indication is retained.

Congenital Mesenteric Defect with Transmesenteric Hernia in Children: A Case Series.

ABSTRACT: Transmesentric hernias are a type of internal hernia, in which there is herniation of bowel loops through a defect in the mesentery. They present with a wide variety of symptoms without any specific radiological features, leading to a delay in diagnosis and high mortality rate. Here, we present a case series of four patients with this rare but fatal cause of small bowel obstruction. Three children presented to the emergency department with small bowel obstruction. The other baby was a preterm neonate with an antenatal scan showing small bowel obstruction. All children underwent emergency laparotomy and were found to have a mesenteric defect with herniation and gangrene of the small bowel. Resection anastomosis of the gangrenous segment and closure of the mesenteric defect were done. The differential for small bowel obstruction in children should include transmesentric hernia. Laboratory or imaging investigations are often inconclusive. Timely exploration can save lives in this rare but life-threatening condition.

Recalcitrant Erythrodermic Psoriasis Complicated by Septic Shock and Mycotic Aneurysm: A Call for Treatment Guidelines.

Erythroderma is characterized by diffuse erythema and scale covering over 90% body surface area that can affect individuals with inflammatory dermatoses such as psoriasis. Complications of erythrodermic psoriasis include infection and cardiovascular compromise. Here we present a case of a 68 year-old man who was hospitalized for erythrodermic psoriasis refractory to multiple immunosuppressive and immunomodulatory therapies, ultimately developing sepsis due to bacteremia and fungemia complicated by infective endocarditis and a mycotic aneurysm. Although the widespread loss of epidermal function in erythroderma increases the risk of infection by opportunistic pathogens, water loss, and electrolyte imbalances, there are very few reported cases of psoriatic erythroderma complicated by fungemia and mycotic aneurysm. Given the high mortality associated with widespread epidermal dysfunction, there is a great need for evidence-based treatment guidelines for psoriatic erythroderma. J Drugs Dermatol. 2024;23(8): doi:10.36849/JDD.7751.

Navigating Diagnostic and Therapeutic Challenges in Primary Cutaneous Gamma/Delta T-Cell Lymphoma: A Case Study of Fatal Outcomes Within Two Months.

ABSTRACT: Primary cutaneous gamma/delta T-cell lymphoma (PCGD-TCL) is a rare yet highly aggressive subtype of primary cutaneous lymphoma. Characterized by its challenging diagnosis and poor prognosis, PCGD-TCL presents unique clinical and histopathological features that distinguish it from other primary cutaneous lymphoma subtypes. Here, we report the case of a 75-year-old man who initially presented with multiple erythematous indurated plaques over his back and bilateral lower extremities. The initial biopsy suggested primary cutaneous T-cell lymphoma (PCTCL) with a CD30-negative phenotype. However, within a 2-month interval, the disease progressed rapidly, manifesting as extensive skin involvement across the chest and upper extremities. A repeat skin biopsy was performed, revealing dermal atypical lymphocytes without epidermotropism. Immunohistochemical analysis demonstrated positivity for CD3, CD5, and CD4, as well as T-cell receptor delta (TCR delta) expression, along with the loss of CD8 and CD30 expression. These findings were consistent with a diagnosis of PCGD-TCL. Despite therapeutic interventions, including systemic treatments, the patient's condition deteriorated rapidly, ultimately leading to his demise within a month of receiving the PCGD-TCL diagnosis. This case highlights the diagnostic complexities associated with PCGD-TCL, emphasizing the importance of careful histopathological examination and immunophenotypic characterization. Given its aggressive nature and propensity for rapid dissemination, early recognition of PCGD-TCL is paramount for initiating appropriate therapeutic interventions. However, effective treatment options for PCGD-TCL remain limited, and the disease typically carries an unfavorable prognosis. Further research is needed to elucidate the underlying molecular mechanisms driving the pathogenesis of PCGD-TCL, to identify novel therapeutic targets, and to improve patient outcomes. In addition, increased awareness among clinicians and pathologists regarding the clinical presentation and diagnostic criteria of PCGD-TCL is crucial for facilitating timely diagnosis and management of this challenging malignancy.

Primary breast angiosarcoma: A case report.

RATIONALE: Primary breast angiosarcoma is a rare tumor, accounting for only 0.05% of all malignant breast tumors. The primary breast angiosarcoma typically presents with nonspecific clinical manifestations, which can easily lead to misdiagnosis. Potential factors contributing to misdiagnosis include skin changes that may be erroneously attributed to breast trauma-induced bruising and breast swelling that may be mistaken for inflammatory diseases or other benign tumors. PATIENT CONCERNS: A 19-year-old female was admitted to the hospital due to repeated lump formation in the left breast for 9 months after left breast trauma. DIAGNOSES: The diagnosis of primary breast angiosarcoma was confirmed on hematoma biopsy. INTERVENTIONS: Due to the patient's condition, no special treatment was given postoperatively. After then, there was a recurrence in the chest wall, and the patient received 2 cycles of chemotherapy, resulting in a reduction in the size and lightening of the recurrent chest wall mass. When chemotherapy intolerance happened, the patient chose to discontinue treatment. OUTCOMES: After an 18-month follow-up, the recurrent chest wall mass increased and the patient died from bleeding. LESSONS: Primary breast angiosarcoma has a low incidence but high malignancy, with a high recurrence and metastasis rate, leading to a poor prognosis. The adjuvant chemotherapy, radiotherapy, targeted therapy, and other treatments should be considered to reduce the local recurrence rate and prolong patient survival.

Pink urine syndrome in an anuric patient during continuous renal replacement therapy: A case report.

INTRODUCTION: Pink urine syndrome is a rare, poorly understood condition, often prompted by obesity, insulin resistance, and the drug propofol. It is characterized by pink urine or urine sediment and occurs in the absence of a heme or food-based pigment. The pathophysiology of this syndrome is not yet fully understood but is linked to a uric acid metabolism disorder. Pink urine syndrome is less familiar to anesthesiologists than other propofol infusion complications. Our case report aims to highlight this rarely encountered syndrome, whose both diagnosis and therapeutic may be challenging. We have reported the first case of this syndrome evidenced by the change in color of the effluent bag during continuous veno-venous hemofiltration (CVVHF). CASE PRESENTATION: A 61-year-old woman was admitted to the intensive care unit following a recovered cardiorespiratory arrest due to ventricular arrhythmia. She was placed in hypothermia, sedated with propofol (300 mg/h), and started on CVVHF for oligo-anuric acute kidney injury associated with severe metabolic acidosis. A few hours after initiation of CVVHF, the effluent bag turned bright pink. Given the pink color of the effluent bag and the hypothesis of propofol-induced pink urine syndrome, propofol was replaced by midazolam. After stopping propofol, the color of effluent bag lightened. Unfortunately, the patient died on the third day of hospitalization due to diffuse cerebral edema. CONCLUSIONS: We report here the first case of pink urine syndrome as revealed by the change in color of the contents of the CVVHF effluent bag in an anuric patient. This syndrome is rare but significant in anesthesia/intensive care settings, where propofol is a frequently used sedative. Knowledge of this syndrome appears to be important to avoid irrelevant additional investigations and to optimize the therapeutic strategy.

[Clinical case of generalized amyloidosis (ATTR-amyloidosis) with a progressive course of chronic heart failure. Case report].

Despite the presence of various signs of cardiac amyloidosis ("red flags"), the introduction into routine practice of new non-invasive diagnostic methods (Speckle Tracking technology using echocardiography, myocardial scintigraphy with technetium pyrophosphate, genetic testing, screening for free light chains of immunoglobulins to exclude AL-amyloidosis), which have high specificity and sensitivity, transthyretinic (ATTR) cardiomyopathy is still a difficult to diagnose disease, especially in the early stages when treatment is most effective. The article presents a clinical case of ATTR-amyloidosis with predominant heart damage, manifested by severe diastolic heart failure resistant to treatment. The timing, from the moment of the first episode of decompensation of heart failure to death, is 4 months, which confirms the rapid progression of severe biventricular dysfunction of the heart. Despite the presence of cardiac and extracardial "red flags" of ATTR-amyloidosis in the patient, the diagnosis was established at autopsy. The paper analyzes possible errors of early diagnosis at the outpatient and inpatient stages of patient management.

Cardiac Mass in a 78-Year-Old Patient With a History of Cancer: Diagnostic and Treatment Challenges.

Primary cardiac angiosarcoma is a rare, aggressive malignancy that commonly metastasizes to various organs. The presenting symptoms are typically nonspecific, so a comprehensive examination is required to confirm the diagnosis promptly. This case report describes the presentation of an older patient with a history of neoplasms. Echocardiography and biopsy were performed, but despite surgical intervention to resect a large right atrial mass, the patient died. A final diagnosis of primary angiosarcoma was made based on the resected specimen.

Arsenic Poisoning-Induced Sensorineural Hearing Loss: A Case Report.

Arsenic is frequently used in alternative medicine, and it is critical to promptly identify and treat suspected arsenic toxicity in patients. In a case study, a female patient presented with several symptoms, including nausea, vomiting, bilateral tinnitus, hearing loss, vertigo, and other associated complaints. After admission, the patient showed lethargy, and topical application of Chinese herbal medicine was found on her left breast, along with visible pigmentation on her torso. Examination revealed severe bilateral sensorineural deafness, liver and kidney injury, and pancytopenia. Due to the presence of broken skin, toxicological analysis detected elevated levels of arsenic in both blood (113 ng/mL) and urine (865.4 ng/mL). The patient was diagnosed with arsenic poisoning and received symptomatic treatment, including detoxification. Unfortunately, the patient died due to long-term exposure to arsenic. Therefore, early identification of the etiology is crucial for managing cases of arsenic poisoning.

Sildenafil-induced priapism in a dog : an unusual case report.

BACKGROUND: Priapism is defined as erection that lasts for more than 4 h without sexual stimulation. There are various causes of priapism, but there are no reports of sildenafil-induced priapism in dogs. In human medicine, there were no pre-marketing reports of priapism caused by sildenafil, but post-marketing surveillance has shown that it is rare. In cases of pulmonary hypertension in dogs, sildenafil is the first-line drug of choice for symptomatic relief. CASE PRESENTATION: An 11-year-old neutered male Maltese dog that presented with tachypnea and cough was diagnosed with myxomatous mitral valve disease, American College of Veterinary Internal Medicine (ACVIM) stage C, and was treated medically. Eighteen months after the diagnosis, severe pulmonary hypertension occurred due to left heart disease. At 20 months postdiagnosis, pleural effusion occurred, and sildenafil (2 mg/kg twice daily) was added to the existing treatment. Two weeks later, the dyspnea recurred, confirming pleural fluid recurrence, and sildenafil was increased to 2 mg/kg thrice daily. One day later, the patient developed persistent erections and penile pain. Penile amputation and urethrostomy were recommended but were refused; therefore, analgesia and palliative care were provided. The patient died of acute dyspnea 22 months after the first presentation, with no specific priapism recurrence at the time of death. CONCLUSION: To the best of our knowledge, this is the first report of sildenafil-induced priapism in a dog with pulmonary hypertension.

Concomitance of Pericardial Tamponade and Pulmonary Embolism in an Invasive Mucinous Lung Adenocarcinoma with Atypical Presentation: Diagnostic and Therapeutic Pitfalls-Case Report and Literature Review.

The invasive mucinous adenocarcinoma of the lungs (LIMA) is an uncommon histological subtype of the mucinous adenocarcinoma. In this article, we present the case of a patient with a very high cardiovascular risk profile, diagnosed with LIMA, pericardial tamponade due to secondary dissemination, and pulmonary embolism, whose management rouses many challenges. Despite receiving the correct anticoagulant and antiaggregant therapy, our patient developed repeated acute major cardiovascular events leading to a fatal outcome. To gather additional information on LIMA and the above cluster of pathologies, we performed the first research of the international medical literature for scientific articles published in the last eight years on PubMed, ResearchGate, Clarivate, and Google Scholar. As the first literature research failed to identify any case similar to our patient, we performed a second study of the same databases for subjects with lung adenocarcinoma instead of LIMA and the same comorbidities, and we found 10 cases. LIMA is a less frequent type of adenocarcinoma, with polymorphic radiologic appearances on the chest computed tomography, frequently mimicking pneumonia, and thus delaying the diagnosis and therapy. It has a worse prognosis and higher mortality than the common adenocarcinoma, but information on its secondary dissemination and complications is still required.

Primary Lung Squamous Cell Carcinoma With Intestinal Metastasis: A Case Report and Literature Review.

Lung squamous cell carcinoma (LUSC) is characterized by a high rate of metastasis and recurrence, leading to a poor prognosis for affected patients. Intestinal metastasis of LUSC is a rare clinical occurrence. Treatment options for LUSC patients with intestinal metastasis are limited, and no standard therapy guidelines exist for managing these cases. In this review, we discuss the clinical features, diagnosis, and treatment of LUSC patients with intestinal metastasis and present a rare case of LUSC with intestinal metastasis. We describe a patient who presented with a severe cough and chest pain and diagnosed with LUSC and bone tumor. Initially, the primary LUSC and bone tumor were controlled with standard treatments. However, the primary LUSC reoccurred shortly after treatment, this time with intestinal metastasis, for which effective treatments are lacking. Our observation from the case suggests that LUSC metastasizing to intestinal tract is associated with a poorer prognosis.

Primary exophytic urothelial carcinoma of the bladder: A case report.

RATIONALE: Bladder urothelial carcinoma (UC) is a common urinary system tumor that is generally diagnosed by cystoscopy combined with pathological biopsy. However, complete exophytic UC of the bladder is very rare and difficult to diagnose. Early diagnosis and accurate identification of such tumors, followed by aggressive surgical treatment, is essential for the management of these patients. PATIENT CONCERNS: An 84-year-old man was admitted to the hospital with dysuria, a poor diet, and significant weight loss. DIAGNOSIS: Pelvic computed tomography and magnetic resonance imaging revealed an exteriophytic round mass on the right lateral wall of the bladder. Cystoscopy revealed a necrotic mass on the right lateral wall of the bladder cavity, and no tumor cells were found following the biopsy. The tumor was removed via partial cystectomy, and the pathological result indicated high-grade muscle-invasive UC. INTERVENTIONS: The patient refused radical cystectomy and underwent laparoscopic partial cystectomy plus pelvic lymph node dissection followed by cisplatin plus gemcitabine chemotherapy. OUTCOMES: The patient's mental state and appetite were significantly improved after the urinary tube was removed 1 week after surgery. His general state was significantly improved after 1 month of follow-up but died of acute cerebral infarction 3 months after surgery. LESSONS: UC of the bladder may grow completely out of the bladder without symptoms such as gross hematuria; thus, early diagnosis is difficult. For high-risk individuals, regular imaging tests may help to detect tumors early. Partial cystectomy is a reliable surgical modality for bladder preservation in such patients.

Primary Pulmonary Angiosarcoma Found Incidentally in a Complicated Patient: A Rare Case Report.

INTRODUCTION: Primary pulmonary angiosarcoma (PPA) is a highly aggressive and rare malignancy originating from the endothelial cells of blood vessels in the lungs. PPA is an extremely rare subtype, with less than 30 cases reported to date. PPA is not only challenging to diagnose but also has a poor prognosis, often resulting in a high mortality rate within a year of diagnosis, regardless of the treatment approach. METHOD: We present the case of a 33-year-old woman with no significant past medical history who presented with abdominal pain and was incidentally found to have a right hilar mass with pleural effusion and empyema. After undergoing surgery for a perforated gastric ulcer, her pulmonary lesions were further worked up. Despite an extensive diagnostic evaluation, including imaging, bronchoscopy, and thoracotomy, establishing a diagnosis was challenging. Ultimately, PPA was diagnosed on surgical lung biopsy, and the patient was started on pazopanib and paclitaxel chemotherapy but expired after 1 month due to multiple complications. CONCLUSION: This case highlights the difficulty in diagnosing this rare tumor and its poor prognosis regardless of therapy. Greater awareness of PPA and more research are needed to improve early detection and treatment options for this deadly disease.