Prognostic significance of venous invasion in node-negative head and neck squamous cell carcinoma.

BACKGROUND: Venous invasion (VI) is not frequently evaluated on routine histologic examination of head and neck squamous cell carcinoma (HNSCC), and the prognostic significance is largely unknown. Studies have shown that extramural venous invasion is an adverse prognostic factor in colorectal carcinoma. To our knowledge, this is the first study evaluating the prognostic significance of venous invasion in node-negative (without clinical or pathologic evidence of lymph node involvement) HNSCC, utilizing the elastic stain. METHODS: A total of 105 consecutive lymph node-negative (N0) HNSCC were evaluated for the presence of venous channel invasion by tumor utilizing the elastin stain. Clinical, demographic, and follow-up data were recorded. RESULTS: Of 37 patients with venous invasion, 19% had loco-regional recurrence, as opposed to 12% of those without. Univariate analysis revealed statistically significant decreased recurrence-free survival in the presence of venous invasion (log-rank [Mantel-Cox] test P-value .025). CONCLUSION: Identification of VI is greatly aided by elastic stain. In patients with node-negative HNSCC, presence of VI resulted in decreased recurrence-free survival on univariate analysis. The impact of VI as a prognostic marker should be further evaluated.

Post-radiation Mullerian adenosarcoma with sarcomatous overgrowth: rare presentation of an uncommon malignancy.

Mullerian adenosarcoma is an uncommon biphasic malignant uterine tumor. It is composed of benign epithelial and malignant stromal elements. We present a case of a 45-year-old woman who presented with post-menopausal bleeding for three months. She had a significant past medical history of pelvic irradiation for squamous carcinoma of cervix 20 years ago. Pathology revealed adenosarcoma with sarcomatous overgrowth. The patient had a recurrence of pure sarcoma three months later and unfortunately succumbed to her disease. The role of radiation in the pathogenesis of adenosarcoma has been uncommonly described compared to its well established role in the development of carcinosarcoma. Our case fulfils the criteria for a radiation induced sarcoma. We review the salient clinical and pathological features of this uncommon lesion highlighting the importance of sarcomatous overgrowth in these lesions and the possible role of radiation in the development of these tumors.

Hepatic Benign Cystic Mesothelioma in Adults: A Case Report of a Rare Hepatic Cyst.

Benign cystic mesothelioma (BCM), also known as peritoneal inclusion cyst, is a benign mesothelial lined cystic lesion, nearly always described in the pelvis of adult females. The hepatic location of BCM is rarely reported in the literature. We report a case of hepatic benign cysts in a 65-year-old woman that was incidentally discovered by imaging studies 12 years ago as a small cyst. Recently, the patient started having abdominal discomfort, distension, and anxiety. A CT scan revealed two low-density fluid-filled cystic lesions, the largest in the caudate lobe measuring up to 10.7 cm and causing a mass effect on hepatic veins and inferior vena cava. Laparoscopic marsupialization of the large liver cyst was done without complications. On gross examination, the collapsed cyst wall was a thin partly translucent pale tan to pink membranous structure with fine vascularity. No discrete nodularity or solid lesion was identified. Microscopic examination showed a thin fibro-connective wall lined by a single layer of flat cuboidal cells with no cellular atypia. The cyst lining showed characteristic calretinin-positive immunohistochemical reactivity for mesothelium, supporting the diagnosis of BCM. Hepatic BCM is among a broad differential spectrum of cystic liver lesions ranging from developmental, reactive, inflammatory, and infectious lesions, benign to premalignant or frankly malignant neoplasms with different treatment strategies. Although BCM is the rarest among the long list of differential diagnoses of hepatic cysts, its identification in this rarely reported location is essential to avoid aggressive surgical treatment.

Malignant Phyllodes Tumor With Chondroblastic Osteosarcomatous Differentiation: A Case Report.

Malignant phyllodes tumors (MPTs) represent the most pernicious type of intralobular stromal proliferation known as a "fibroepithelial lesion" (FEL). They comprise a small fraction of breast malignancies and can present as either a pure MPT or sometimes include a heterologous component (liposarcoma, chondrosarcoma, osteosarcoma, or rhabdomyosarcoma). Of the fraction of MPTs that include heterologous components, very little about those with chondroblastic osteosarcomatous differentiation has been described in the literature. As such, a characteristic staining profile has yet to be established, even though morphological analysis is the cornerstone of diagnosis. The few reported cases have described a poor prognosis. Therefore, we present a case of MPT with chondroblastic osteosarcomatous differentiation to contribute to the dearth of literature examining this entity.

Pathologic examination of placenta: a study on 500 live births to assess conformity to College of American Pathologists (CAP) guidelines and clinicopathologic correlation.

PURPOSE: Pathological examination of placenta is vital to understand the pathophysiology of adverse perinatal outcomes, prevention of recurring conditions in subsequent pregnancies and medico-legal risk assessment. The College of American Pathologists (CAP) has published a set of guidelines to help guide the submission of placentas to pathology. However, awareness and conformity to these guidelines are not well established and vary from one institution to the other. We aimed to examine the appropriateness of placental pathologic examination at our institution and their conformity to guidelines in this audit type study to help improve our practices. MATERIALS AND METHODS: Detailed retrospective review of obstetrical records was performed including history and delivery reports for a total of 500 consecutive live births noting whether the placenta was sent for pathologic evaluation according to CAP guidelines. Sensitivity and specificity of placental examination were calculated based on the 2 × 2 contingency table. RESULTS: The sensitivity and specificity of pathologic examination of placenta in conformity to CAP guidelines were 63.4% and 91.6%, respectively. The most common indications for submission were maternal followed by fetal and placental indications. Concordant clinico-pathologic correlation was found in 87/135 placentas submitted. CONCLUSIONS: Placenta has long been ignored with limited understanding of the value of its examination. More awareness of CAP guidelines is needed to appropriately submit placentas for pathologic examination. Each institution needs to develop their own set of guidelines taking guidance from CAP guidelines and tailored to its population. Meaningful communication between obstetricians, neonatologists, and pathologists is key to improving the utility of pathologic examination of placenta and the application of results for better patient care.

A Case Report and Brief Literature Review on Dedifferentiated Chondrosarcoma in Proximal Phalanx: A Rare Location.

Dedifferentiated chondrosarcoma (DDCS) is a rare entity, constituting only 1-2% of all primary bone tumors, and has a dismal prognosis. Nearly two-thirds of the primary tumors of DDCSs are found in the appendicular skeleton, mostly involving the femur, humerus, and pelvis. DDCS of the small bones of the hand and foot are exceedingly rare with only four cases documented in the literature so far. In this report, we present a case of a 91-year-old woman with a rapidly growing bone tumor initially thought to be a trigger finger, which, on histologic examination of the amputation, turned out to be DDCS. On a follow-up CT scan, multiple pulmonary metastases were identified. Next-generation sequencing identified isocitrate dehydrogenase 2 (IDH2) (p.R172S, c.516G>T), TERT (c.-146C>T), and TP53 (c.559+1G>A) mutations. Microsatellite instability was equivocal and tumor mutation burden was low. Due to the advanced age of the patient, she was given palliative treatment and was alive at the six-month follow-up.

Current Advances in the Management of Adult Craniopharyngiomas.

Craniopharyngiomas (CPs) are slow growing, histologically benign intracranial tumors located in the sellar-suprasellar region. Although known to have low mortality, their location and relationship to the adjacent neural structures results in patients having significant neurologic, endocrine, and visual comorbidities. The invasive nature of this tumor makes complete resection a challenge and contributes to its recurrence. Additionally, these tumors are bimodally distributed, being treated with surgery, and are followed by other adjuncts, such as focused radiation therapy, e.g., Gamma knife. Advances in surgical techniques, imaging tools, and instrumentations have resulted in the evolution of surgery using endoscopic techniques, with residual components being treated by radiotherapy to target the residual tumor. Advances in molecular biology have elucidated the main pathways involved in tumor development and recurrence, but presently, no other treatments are offered to patients, besides surgery, radiation, and endocrine management, as the disease and tumor evolve. We review the contemporary management of these tumors, from the evolution of surgical treatments, utilizing standard open microscopic approaches to the more recent endoscopic surgery, and discuss the current recommendations for care of these patients. We discuss the developments in radiation therapy, such as radiosurgery, being used as treatment strategies for craniopharyngioma, highlighting their beneficial effects on tumor resections while decreasing the rates of adverse outcomes. We also outline the recent chemotherapy modalities, which help control tumor growth, and the immune landscape on craniopharyngiomas that allow the development of novel immunotherapies.

Post-Transplant Lymphoproliferative Disorder: A Rare Case of CNS Involvement following Renal Transplant.

BACKGROUND: Post-transplant lymphoproliferative disorders (PTLD) are rare immunosuppression complications affecting 5% of transplant patients. Isolated central nervous system (CNS)-PTLD without nodal or extra-nodal organ involvement is rarely reported and is difficult to diagnose due to the non-specific clinical manifestations and imaging features overlapping with other common CNS lesions. CASE PRESENTATION: We present a case of a 72-year-old female subjected to a renal transplant 11 years ago with progressively worsening headaches and confusion. Imaging revealed vasogenic edema in the left frontal and bilateral temporal lobes. She was subjected to a craniotomy and excisional biopsy to obtain tissue for diagnostic and therapeutic interventions. Pathology examination showed atypical EBV-positive lymphoplasmacytic infiltrate, consistent with Polymorphic type PTLD. CONCLUSIONS: Patients diagnosed with PTLD need to have close monitoring of immunosuppressive medications while in the hospital. Early diagnosis is essential for patient survival with PTLD, as their health can deteriorate fast.

Pitfalls in Morphologic Diagnosis of Pathogens: Lessons Learned From the Pseudo-Cystoisospora Epidemic.

Multiple groups have recently reported involvement of the gallbladder mucosa of immunocompetent patients by cystoisospora organisms. However, this has recently been disproved with the support of molecular and ultrastructural studies. Here we present a summary of these events, recounting how this pseudo-Cystoisospora epidemic began and ended. This review also highlights the important role played by ancillary techniques in supplementing the morphologic diagnosis of pathogens.

Falx Cerebelli Meningioma: Case Report of a Rarely Occurring Tumor, Management Nuances, and Literature Review.

The falx cerebelli is a small crescent fold of dura mater that is attached to the internal occipital crest and projects forward into the posterior cerebellar notch between the cerebellar hemispheres. We report a rare case of a 61-year-old female who presented with a 1-month history of headache and gait instability. Imaging findings were suggestive of a meningioma arising from the falx cerebelli. Complete surgical resection was achieved with a standard posterior fossa midline approach. Duraplasty was performed using animal allograft dura (Duraguard) and additional layers of oxidized cellulose preparation (Surgicel), fibrin sealant, and nonsuturable collagen matrix (Duragen) were utilized to reduce the risk of a cerebrospinal fluid leak. Pathology confirmed a World Health Organization (WHO) grade-I meningioma. Postoperatively, patient with asymptomatic thrombosis of the left transverse/sigmoid sinuses and later with a pseudomeningocele managed with a lumbar drain. To our knowledge, this is the second documented case in the literature. We discuss intraoperative nuances and unique aspects in the postoperative care and management of these patients.

Liberation from Dialysis Dependence in a Patient with HIV-Associated Nephropathy (HIVAN) after Combined Antiretroviral Therapy (cART).

Prior to the advent of combined antiretroviral therapy (cART), human immunodeficiency virus-associated nephropathy (HIVAN) was inevitably associated with rapidly progressive renal failure and dialysis dependence. HIV-1 seropositive patients often met with untimely deaths due to complications of end-stage renal disease (ESRD), opportunistic infections, or other HIV-related end-organ failure. Although the association between cART and improved outcomes in HIVAN has been recognized for over 20 years, no randomized trials have specifically examined this effect to date. In terms of reversal of dialysis-dependent renal failure after cART initiation, only a handful of case reports exist. The authors report a case of a 44-year-old Latino male requiring thrice-weekly haemodialysis in the setting of biopsy-proven HIVAN who was able to stop dialysis in 7 months after being initiated on cART.

Multiple myeloma with extensive AL amyloidosis presenting as chronic diarrhoea.

We present a 52-year-old man admitted to the hospital with diarrhoea and lower extremity weakness ongoing for the past 3 months. The patient was found to have malabsorptive diarrhoea, hypoproliferative anaemia and renal insufficiency with proteinuria. Extensive workup was performed including a bone marrow biopsy with 20% plasma cells, renal and duodenal biopsies with Congo-red staining revealed amyloid deposition. The patient was diagnosed with multiple myeloma and amyloidosis with gastrointestinal, kidney and nerve involvement explaining his presentation with diarrhoea, renal insufficiency and weakness. Throughout his admission, there were incidental findings of asymptomatic hypoglycaemia (serum blood glucose <40 mg/dL), which was later found to be caused by anti-insulin monoclonal antibodies produced by the neoplastic plasma cells. This is an extremely rare manifestation of multiple myeloma with only a few cases reported in the literature.

Symptomatic Pituitary Metastasis as Initial Manifestation of Renal Cell Carcinoma: Case Report and Review of Literature.

Metastasis to the pituitary gland is extremely rare (∼2% of sellar masses). Clinical, biochemical, and radiologic characteristics of pituitary metastasis are poorly defined and can be difficult to diagnose before surgery. We present an unusual case with pituitary metastasis as the first manifestation of renal cell carcinoma (RCC). A 70-year-old male presented with acute onset of weakness, dizziness, diplopia, and progressively worsening headache. The initial CT head revealed a heterogeneous sellar mass measuring 2.8 × 1.9 × 1.7 cm. A follow-up MRI showed the sellar mass invading the right cavernous sinus. The presumptive diagnosis was a pituitary macroadenoma. Physical examination revealed bilateral 6th cranial nerve palsy and episodes of intermittent binocular horizontal diplopia. Hormonal testing noted possible secondary adrenal insufficiency (AM serum cortisol: 3.3 mcg/dL, ACTH: 8 pg/mL), secondary hypothyroidism (TSH: <0.01 mIU/L, FT4: 0.7 ng/dL), secondary hypogonadism (testosterone: 47 ng/dL, LH: 1.3 mIU/mL, and FSH: 2.3 mIU/mL), and elevated serum prolactin (prolactin: 56.8 ng/ml, normal: 4.0-15.2 ng/ml). IGF-1 level was normal at 110 ng/mL (47-192 ng/mL). The patient was discharged on levothyroxine and hydrocortisone therapy with plans for close surveillance. However, his condition worsened over the next three months, and he was subsequently readmitted with nausea, vomiting, and hypernatremia secondary to diabetes insipidus. Repeat MRI pituitary showed an interval increase in the size of the sellar mass with suprasellar extension and a new mass effect on the optic chiasm. The sellar mass was urgently resected via a trans-sphenoidal approach. The tumor was negative for neuroendocrine markers and pituitary hormone panel, ruling out the diagnosis of pituitary adenoma and triggered workup for metastatic renal cell carcinoma, clear cell type. The diagnosis of renal cell carcinoma was confirmed by the diffuse and strong staining for renal cell carcinoma markers (Pax-8, RCC-1, and CD10). A follow-up CT scan noted large right renal mass measuring 11 × 10 × 11 cm. The patient underwent a cytoreductive robotic right radical nephrectomy for WHO/ISUP histologic grade II clear cell RCC, stage pT2b pNX pM1. He subsequently received fractionated stereotactic radiotherapy to the pituitary gland. He is presently stable with no radiological evidence of progression or new intracranial disease on subsequent imaging. Pituitary metastasis most commonly occurs from breast, lung, or gastrointestinal tumors but also rarely from renal cell carcinoma. Biochemical findings such as panhypopituitarism, acute clinical signs such as headache, visual symptoms, and diabetes insipidus and interval increase in sellar mass in a short time interval should raise suspicion for sellar metastasis.

Ultrastructural Characteristics of Gallbladder Epithelial Inclusions Mimicking Cystoisospora.

OBJECTIVES: There is recently reported increased prevalence of Isospora organisms in cholecystectomy specimens from immunocompetent patients, especially in acalculous cholecystectomies. We performed an ultrastructural and molecular evaluation of these specimens. METHODS: From 28 gallbladders with intraepithelial inclusions, two specimens with diffuse involvement of the gallbladder epithelium were analyzed by electron microscopy. Polymerase chain reaction was performed on five samples for the ITS2 region of C belli and eukaryotic 18S region. The 18S products were sequenced by next-generation sequencing. RESULTS: Electron microscopic analysis showed cytoplasmic condensations leading to vacuole formation. In contrast with true C belli, there were no identifiable organelles or organization. None of these cases showed amplified products other than human on molecular analysis. CONCLUSIONS: Electron microscopic analysis demonstrates that the inclusions are condensed cytoplasmic material and not true organisms.

CADASIL disease, an inherited slowly progressive vascular dementia: case report with radiologic and electron microscopic findings.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) disease, a genetically determined arteriopathy, is a rare cause of vascular dementia. We present a case report of a 50-year-old man with migraines associated with left-sided weakness since age 34 years, a stroke at age 43 years, followed by progressive dementia. Magnetic resonance imaging revealed diffuse leukoencephalopathy with involvement of bilateral anterior temporal lobes. Skin biopsy was performed because of clinical suggestion of CADASIL and positive family history. Electron microscopy revealed granular osmophilic material deposits in dermal arterioles, diagnostic for CADASIL disease. A brief discussion of reasons for false-negative skin biopsy findings, differential diagnosis, and treatment of patients with CADASIL disease is presented.

Waldenström's macroglobulinemia and large B-cell central nervous system lymphoma: a case report.

BACKGROUND: Waldenström's macroglobulinemia is a low-grade B-cell lymphoma characterized by monoclonal synthesis and secretion of IgM antibodies [3]. The classic symptoms of WM result from blood hyperviscosity. Neurologic symptoms, such as fatigue, dizziness, and blurred vision, are common. The metastatic spread to the CNS, which rarely has been reported, can occur either diffusely or in the form of a mass lesion. CASE DESCRIPTION: We present the case of a 73-year-old woman with intracerebral large B-cell lymphoma and concurrent WM. CONCLUSION: This case illustrates the importance of histopathologic diagnosis by brain biopsy despite an existing diagnosis of systemic neoplasm.

Desmoplastic fibroblastoma of the left upper arm.

An elderly female patient presented to the clinic with a several-week history of a mass in her left upper arm that was tender to the touch. The mass was initially thought to be a schwannoma of the left radial nerve based on imaging and was surgically removed. The pathology report revealed an uncommon diagnosis of desmoplastic fibroblastoma.

Intramedullary mature teratoma of the cervical spinal cord at C1-2 associated with occult spinal dysraphism in an adult. Case report and review of the literature.

There is a well-recognized association between dysontogenetic tumors of the spinal cord (including teratomas and enterogenous cysts) and dysraphic congenital spinal malformations. The authors present a case of an adult with an intramedullary mature teratoma (IMMT) at the level of C1-2 of the cord associated with dysraphic congenital spinal malformations. Intramedullary mature teratomas of the cervical region of the spinal cord are very rare in adults; only four such lesions have been reported, two of which involved upper cervical segments. Despite the potentially critical location of the tumor, monitored microsurgery resulted in complete removal of the tumor with an intact surrounding capsule, associated fibrous tract, and ellipse of skin with a central dimple. There was an excellent postoperative neurological outcome. The clinical features, imaging studies, treatment options, postoperative outcome, and plausible pathological correlations of IMMTs are discussed.