A rare case of a peripheral Ewing sarcoma primitive neuroectodermal tumor of pelvic origin.

Primitive neuroectodermal tumors (PNET) represent malignant neuroectodermal tumors composed of small round cells. They can be differentiated between originating from the peripheral nervous system or the central nervous system. Peripheral PNET (pPNET) can be further subclassified as one of the Ewing family tumors (EFT). Although rare, EFT can originate in the female genital tract and pelvic region. Here, we present a case of a middle-aged female with PNET masses in her uterus, abdomen, and hepatic lobes. We discuss the diagnostic modalities, including immunohistochemistry, histopathology, and imaging findings associated with this rare malignancy.

Carcinoma Erysipeloides: An Underdiagnosed Phenomenon of Cutaneous Metastases of Breast Cancer.

BACKGROUND Cutaneous metastatic breast cancer is a rare manifestation. Causes include vascular or lymphatic spread or iatrogenic mechanisms following surgery. A sub-type of this disease process, "Carcinoma Erysipeloides," represents subcutaneous and dermal tissue layer invasion via lymphatic spread. Diagnosis can be challenging, and therefore, obtaining a thorough history and physical, with careful inspection of prior surgical scars is essential for an accurate diagnosis. Lesions present in variable ways, including papules, plaques, ulcerations, nodules, crusting, or fungating masses, with common locations in the chest, scalp, abdomen, and less commonly the arms. When carcinoma erysipeloides is identified, it is imperative to evaluate for distant metastases. Recent literature has identified benefits with trastuzumab deruxtecan therapy instead of trastuzumab emtansine, with decreased progression rates and decreased mortality rates. Metastasis to the skin can indicate advanced disease; however, this metastatic site may be preferable to visceral organs or bones in terms of prognosis. CASE REPORT We present a rare manifestation of metastatic breast cancer in 45-year-old Hispanic woman, status post neoadjuvant chemotherapy and radical cystectomy on maintenance trastuzumab and pertuzumab. We discuss the clinical presentation variability, keys to diagnosis, treatment considerations, and outcomes for this unique patient population. CONCLUSIONS Carcinoma erysipeloides varies in clinical presentation, especially when patients develop exclusive skin lesions. We identify common etiologies for this progression of disease and discuss combination therapy which has demonstrated a reduction in mortality in this patient population.

Gynecomastia and Malignancy: A Case of Male Invasive Ductal Breast Carcinoma Treated with Neoadjuvant Chemotherapy.

BACKGROUND Male breast cancer represents a rare malignancy with identifiable risk factors, including genetics, radiation exposure, liver dysfunction, and concomitant diagnosis of Klinefelter syndrome. Gynecomastia can commonly present in these patients, and despite increased estrogen levels in adipose breast tissue, gynecomastia has not been proven to be a significant risk factor for carcinoma development. Male patients with new-onset breast masses are recommended to undergo diagnostic mammograms and breast ultrasound for further evaluation. Those diagnosed with breast cancer most commonly have invasive ductal carcinoma of the breast, and over half of these patients are found to have estrogen and progesterone receptor (ER/PR) positivity. CASE REPORT In this case report, we present a Black man with gynecomastia and an areolar lesion for a 6-month duration following a traumatic event. He was initially referred to the surgical team for further evaluation, and subsequent imaging and biopsy data revealed ER/PR-positive invasive ductal carcinoma. Multidisciplinary discussions were held, and the patient was arranged to begin neoadjuvant treatment with doxorubicin hydrochloride and cyclophosphamide, followed by treatment with paclitaxel (AC-T) chemotherapy, followed by bilateral mastectomy and adjuvant hormonal therapy. CONCLUSIONS The treatment of male breast cancer has remained relatively like that of female breast cancer, which may be due to the limited data in the treatment of male breast cancer. Thus far, studies involving neoadjuvant chemotherapy of female patients have demonstrated promising responses to expand surgical options for patients and possibly decrease the rates of recurrence. Additional studies are warranted to discern optimal therapy for the male patient population.

A Rare Case of Paraneoplastic Syndrome of Inappropriate Secretion of Antidiuretic Hormone in Cervical Squamous Cell Carcinoma; A Case Report and Literature Review.

SIADH is more commonly associated with small cell lung cancer but has also been associated with other malignancies such as oropharyngeal, gastrointestinal, genitourinary, endocrine, lymphomas, and sarcomas. There have been few reports of small cell carcinoma of the cervix complicated by SIADH; however, not many cases are associated with squamous cell carcinoma of the cervix. We present a case of a patient with squamous cell carcinoma of the cervix with a paraneoplastic syndrome of inappropriate secretion of antidiuretic hormone. The pathophysiology, clinical picture, and treatment are also discussed.

Chemotherapy Port Induced SVC Thrombosis in a Patient with Non-metastatic Breast Cancer.

Cancer patients are at a higher risk of complications such as venous thromboembolism (VTE). This risk increases in patients who receive chemotherapy. Despite the increased risk, common locations for VTE are similar to those in patients without cancer. Chemotherapy-port-related thrombosis represents a rare complication due to the location and frequent use of access (with Heparin flushes) as part of the standard care. Attention should be made to this rare complication, which may progress to superior vena cava (SVC) syndrome. SVC syndrome typically presents in females around the age of 57 years old. Management of this syndrome can be difficult and generally requires the initiation of systemic anticoagulation therapy. Here, we present a rare case of a 45-year-old female who presented to the Emergency Department with right arm swelling, found to be secondary to her chemo port thrombosis, causing SVC syndrome.

How we dealt with the double whammy! Acute pulmonary embolism with abdominal aortic clot and renal infarcts.

Pulmonary embolism (PE) is the third most common cause of cardiovascular mortality in the United States, and the submassive PE accounts for 20%-25% of all acute PE. In the last decade, endovascular therapy with catheter-directed thrombolysis (CDT) intervention has shown great success in the treatment of submassive PE. There is limited data regarding using these devices to treat patients with concomitant abdominal aortic and renal vessel clots. Herein, we present a case of a 23-year-old male who presented with submassive PE associated with abdominal aortic thrombosis and renal infarcts. The patient was successfully treated with CDT with complete resolution of pulmonary and bilateral renal artery clots.

Pulmonary Carcinosarcoma: A Rare Disease With Challenging Diagnosis and Treatment.

Pulmonary carcinosarcoma (PCS) is a rare type of non-small cell cancer. Overall, middle-aged and older smokers are the most affected age and sex groups. The diagnosis of PCS is difficult due to the absence of characteristic imaging findings. Additionally, preoperative biopsies do not usually reflect the heterologous nature of this tumor. Given the rarity of such tumors and the challenging diagnosis, the prognostic factors have not been established, and the overall prognosis remains poor. The valid therapeutic options are still limited. Here, we report a rare case of metastatic PCS that was accidentally discovered by imaging and properly diagnosed after surgical resection. The clinicopathological features, diagnostic tools, genetic theories, prognosis, and therapeutic options of this rare cancer are also discussed.

An Unexpected Transformation: Malignant Spindle Cell Carcinoma Developed From Primary Basal Cell Carcinoma.

Spindle cell carcinoma (SpCC)/sarcomatoid carcinoma is a biphasic tumor with molecular and histopathological properties of both epithelial and mesenchymal tumors. SpCC usually occurs either in sun-exposed areas like the head, neck, upper extremities, and chest or in the areas of skin with prior radiation exposure or in immuno-suppressed individuals. Cutaneous SpCC is a very rare disease, with only a handful of reported cases so far. SpCC differs from conventional squamous cell carcinoma (SCC) with dermal infiltration of atypical keratinocytes as single cells with hyperchromatic eosinophilic cytoplasm and elongated, pleomorphic nuclei with multiple nucleoli, in contrast to cohesive nests or islands in SCC. The objective of this study is to complete a review of the current literature and present a rare manifestation of malignant SpCC which developed from a localized basal cell carcinoma following excision and radiation therapy (RT) in a 79-year-old female. We plan to elucidate the importance of a timely and accurate diagnosis of this disease in order to maximize treatment options and improve survival outcomes.

A rare case of metastatic pancreatic adenocarcinoma presenting as a pulmonary embolism from nonbacterial thrombotic endocarditis.

Nonbacterial thrombotic endocarditis (NBTE) also called, "Marantic endocarditis" occurs due to an underlying hypercoagulable state causing tissue damage and upregulation of the coagulation cascade, with noninfective vegetation formation on heart valves. Mitral and aortic valves are most commonly involved. NBTE is rare, with an incidence of 1.6%, with 65 cases identified during a 10-year autopsy analysis. The most common malignancies associated with NBTE include gynecological cancers, lung cancer, gastric cancer, and pancreatic cancers with adenocarcinoma histology being the greatest risk. Herein, we present a rare case of a 55-year-old male who presented with acute hypoxic respiratory failure secondary to pulmonary embolism due to nonbacterial thrombotic endocarditis. He was found to have advanced pancreatic adenocarcinoma on further investigation of the 2.2 cm hypodense cystic mass in the distal pancreatic body and tail, and complex liver masses which were incidentally found on computed tomography angiography (CTA) of the chest. This is a rare phenomenon and clinicians have to consider the hypercoagulable state associated with cancers, particularly pancreatic adenocarcinoma, and the risk of NBTE.

Recurrent Hypokalemia as an Unusual Presentation for Metastatic Adrenocortical Cancer.

Adrenocortical cancer is a rare malignancy of the endocrine system. Therefore, when this malignancy presents with metastatic disease, this leads to further difficulties in management. Due to the rare and ambiguous nature of this malignancy, diagnosis is generally made at later stages, with limited options for patients. Symptoms may include weight gain/loss, muscle weakness, abdominal discomfort/bloating, hyperglycemia, hypertension, electrolyte imbalance, hirsutism and virilization in females, gynecomastia and hypogonadism in males. Due to the variety of conditions presenting with one or more of these symptoms, diagnosis can be difficult. Many adrenocortical tumors, malignant and benign, are usually detected incidentally on imaging performed for evaluation of another condition, also known as "incidentalomas." Here, we present a rare case of metastatic adrenocortical malignancy in a 56-year-old female patient who presented with isolated recurrent episodes of hypokalemia.

Hemosuccus Pancreaticus: Challenging Diagnosis and Treatment.

Hemosuccus pancreaticus (HP) is defined as bleeding from the ampulla of Vater through the pancreatic duct. It is a rare complication associated with acute or chronic pancreatitis. The source of bleeding can be from the pancreas itself or surrounding vessels, with the splenic artery most commonly involved. Diagnosing HP is challenging and computed tomography angiography remains the gold standard for diagnosis. We present the case of a 62-year-old male with recurrent pancreatitis complicated with HP. Imaging and endoscopy were consistent with bleeding from the section portion of the duodenum, which resolved without intervention. LEARNING POINTS: Hemosuccus pancreaticus is a rare complication associated with acute or chronic pancreatitis.CT angiography is the gold standard for diagnosing hemosuccus pancreaticus.Arterial embolization is the first-line treatment of hemosuccus pancreaticus.

A Case Report of Autoimmune Encephalitis: Could Post-COVID-19 Autoimmunity Become a Lethal Health Issue?

While it is primarily thought of as a respiratory illness, COVID-19 is now recognized as a multi-organ disease that can present with a wide range of clinical manifestations. Particularly in patients with severe respiratory illness, neurological manifestations ranging from headaches, and loss of smell to strokes have been associated with the virus. In the setting of resolving respiratory illness, it is important to consider autoimmune encephalitis (AE) in the instance of new-onset neurological manifestations. The typical patient presentation includes altered mental status, fever, seizures, and/or focal neurological deficits. These neurological manifestations make it crucial to consider either underlying COVID-19 infection or post COVID-19 autoimmunity so as not to delay the administration of the appropriate treatment. Herein, we present the case of an 88-year-old female with new-onset right leg weakness, and dysarthria, that progressively developed to altered mental status months after having respiratory symptoms of COVID-19. According to the criteria of AE diagnosis, the patient's clinical course and work-up findings proved the diagnosis.

A Favorable Outcome of Grade 3 Follicular Lymphoma Treated With Surgery and Obinutuzumab Combined With Chemotherapy: A Case Report and Literature Review.

Follicular lymphoma is the most common type of low-grade non-Hodgkin lymphoma and the second most common type of lymphoma. Primary extranodal follicular lymphoma is rare compared with nodular follicular lymphoma involving the gastrointestinal (GI) tract. There has been uncertainty regarding follicular lymphomas due to the heterogeneous presentation and severity in which they present. However, studies showed that patients diagnosed with primary gastrointestinal follicular lymphoma do not typically differ in their presentation from those diagnosed with nodular follicular lymphoma involving the GI tract. Furthermore, recent literature identifies that patients diagnosed with grade 3 follicular lymphoma tend to have similar genetic and molecular entities to those diagnosed with diffuse large B-cell lymphoma (DLBCL). Based on these results, current studies have shown that patients with grade 3 follicular lymphoma who are treated with anthracycline-based regimens have similar outcomes to those with diffuse large B-cell lymphoma. However, additional studies are warranted to demonstrate the benefit of managing grade 3 follicular lymphoma with more aggressive anthracycline/rituximab-based regimens. Here, we present a case of a 44-year-old male diagnosed with grade 3 follicular lymphoma involving the gastrointestinal tract, who demonstrated an excellent treatment response following therapy similar to the treatment of bulky diffuse large B-cell lymphoma despite a tumor burden size below 7.5 cm.

A Rare Case of HHV-6 Encephalitis in an Immunocompetent Host: Case Report and Literature Review.

Human herpesvirus-6 (HHV-6) is a virus known for causing the highly contagious infection, roseola infantum, and has been associated with causing encephalitis in pediatric patients and less commonly in adult patients as well. Regardless of the patient's age, the primary HHV-6 infection could be complicated by neurological sequelae including encephalitis, acute encephalopathy with biphasic seizures syndrome, or demyelinating disease. HHV-6 encephalitis does occur in an adult as a primary infection or reactivation. However, immunocompromised, hematopoietic stem cell transplantation patients, and solid organ transplant recipients are the most affected population. Here we present a rare case of HHV-6 encephalitis in a 26-year-old healthy immunocompetent male. HHV-6 viral DNA was detected in the cerebrospinal fluid during the acute stage of the disease, and the diagnosis was confirmed by quantitative polymerase chain reaction (PCR). The patient was treated with ganciclovir and had a complete response to treatment without any further complication. The pathophysiology, clinical course, and treatment in otherwise immunocompetent adult patients are also discussed.

Unintentional Dual Benefit: Improvement in Secondary Polycythemia in a Patient Receiving Chemotherapy for Metastatic Clear Cell Renal Cell Carcinoma.

Complete blood counts are frequently collected from cancer patients, but laboratory findings may be misleading. Secondary polycythemia can occur in renal cell carcinoma (RCC) due to erythropoietin (EPO) stimulation. Therefore, complete blood counts should be closely monitored to prevent complications such as thrombosis. We discuss the case of a 47-year-old man with metastatic RCC who presented with secondary polycythemia that improved with chemotherapy. His secondary erythrocytosis was anticipated, but his haemoglobin levels were lower than expected after therapy. This article discusses the treatment and diagnosis of secondary polycythemia in patients with RCC. LEARNING POINTS: Haemoglobin and haematocrit levels should be closely monitored in renal cell carcinoma (RCC) patients as they may develop secondary polycythemia as a result of their malignancy.Secondary polycythemia can be managed with chemotherapy and immunotherapy in RCC, with anti-cancer agents preventing the need for phlebotomy.Chemotherapy may benefit RCC patients by decreasing tumour burden, preventing progression, and by lowering haemoglobin levels, thus improving secondary polycythemia.

A Rare Case of Benign Histiocytic Neoplasm of Cutaneous Rosai-Dorfman Disease: A Case Report and Literature Review.

Rosai-Dorfman disease (RDD) is a rare disorder characterized by the proliferation of histiocytes in lymph nodes. It can occur anywhere in the body but commonly involves the cervical area of the neck. Its clinical presentation varies and patients with skin manifestations may develop papules, nodules, plaques, or pustules. Histologically, it typically presents with emperipolesis, where intact lymphocytes are found within histiocytes. The definitive treatment of RDD is not well established given the rarity of the disease and indeed skin lesions can regress spontaneously. Therapeutic treatment options include cryotherapy, radiation, or topical agents such as steroids or retinoids. Here we describe the case of a 24-year-old Hispanic female who presented with skin manifestations which proved to be histologically positive for Rosai-Dorfman disease. The patient clinically improved following the administration of intralesional steroids. LEARNING POINTS: RDD is a rare self-limiting benign lymphoproliferative disorder, which can be associated with autoimmune disease, hematological malignancies, and post-infectious conditions.Skin lesions can be self-limiting in many cases of RDD.Therapeutic treatment options include cryotherapy, local radiation, and topical steroids and retinoids.

A Rare Case of Endocervical Adenocarcinoma of Gastric Type.

Gastric adenocarcinoma of the cervix (GAC) represents a rare mucinous endocervical cancer unrelated to human papillomavirus (HPV). GAC has been found to comprise approximately 10% of cervical adenocarcinomas internationally. As more cases have been identified, GAC has been further classified into subtypes such as poorly differentiated versus well-differentiated (also referred to as mucinous or adenoma malignum). This cancer coined the term "gastric" subtype due to its similarity to the pancreaticobiliary and gastric tissue lining. With limited data and similar histological and genetic features of GAC, this malignancy poses a challenge for clinicians when differentiating between metastasis from the gastrointestinal tract and GAC. Here, we present a case of a 55-year-old female who presented with postmenopausal bleeding and was found to have stage IA1 endocervical adenocarcinoma of gastric subtype. The purpose of this article is to introduce a rare type of gastric adenocarcinoma with a unique site of origin in order to better understand this disease process and potentially help clinicians better diagnose and treat patients with this malignancy in the future.

Can a Cerebral Congenital Anomaly Present in Adulthood?

Colpocephaly, a congenital anomaly in the ventricles, is usually discovered early in infancy and rarely during adulthood. Partial or complete agenesis of the corpus callosum or Chiari malformations (developmental arrest of white matter formation in early fetal development) can lead to abnormal asymmetrical ventricular enlargement. Minimal literature about colpocephaly is available for clinicians, making diagnosis and treatment very challenging. Colpocephaly in adults is an infrequent condition, mostly found as an incidental finding with no neurological or cognitive impairment. Symptoms usually represent the affected lobe in the brain as our patient's visual hallucination may be attributed to the lesioned occipital horn. Differentiating from normal pressure hydrocephalus, representing new-onset dementia, can help avoid unnecessary procedures. Neurological and psychiatric consultation should be practiced to exclude other causes of neurological and cognitive impairment. While there is no definitive treatment for this condition, seizure prophylaxis has been helpful. Cognitive behavioral therapy, psychotherapy, and social skills training are recommended in some literature. Typical and atypical antipsychotics can control symptoms with uncertain efficacy.