Massive Enlargement Research Articles

A Dramatic Response of a Thyroid Lymphoma to R-CHOP Chemotherapy Reversing Mechanical Airway Obstruction and Respiratory Failure

Primary thyroid lymphoma is an extremely rare thyroid malignancy that usually occurs in patients with preexisting Hashimoto thyroiditis and commonly presents in older women. The most common type is non-Hodgkin's lymphoma of B-cell origin, particularly diffuse large B-cell lymphoma (DLBCL), which is primarily treated with chemotherapy and radiotherapy. We reported an 83-year-old woman with a past medical history of hypothyroidism who suffered dysphagia and dyspnea secondary to a large thyroid mass. Her CT neck scan showed an enlarged thyroid mass with pathological cervical lymphadenopathy and marked tracheal narrowing. The ultrasound-guided biopsy confirmed the diagnosis of DLBCL of the thyroid. A few days later, she experienced respiratory distress and failure that required endotracheal intubation and mechanical ventilation. She was not considered for tracheostomy or surgical interventions, and after discussion with her family, the decision was made to start R-CHOP therapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) which resulted in a marked reduction of the thyroid size and reversal of the mechanical airway obstruction, enabling her extubation. This case report demonstrated the dramatic response of a large thyroid lymphoma to R-CHOP therapy, reducing the thyroid size and its fatal obstructive complications, including mechanical airway obstruction, within a few days of the initiation of R-CHOP therapy.

Falciform ligament abscess with disseminated intrahepatic foci: a case report

BackgroundFalciform ligament abscess (FLA) is a rare disease, and its diagnosis can be challenging without typical image findings of an abscess. We report a patient with FLA that presented as a mass, with an indistinct border between it and the liver, in addition to disseminated foci within the liver. This made it difficult to determine whether it was FLA or a malignancy.Case presentationA 69-year-old man presented with epigastric pain. Contrast-enhanced computed tomography revealed a 25-mm mass below the middle of the diaphragm. Based on an initial diagnosis of infection of the falciform ligament, we administered conservative antibiotic treatment and there was initial improvement in the patient’s clinical condition and laboratory data. However, he continued to experience mild epigastric pain. A month later, imaging studies revealed enlargement of the falciform ligament mass and the emergence of a new nodule in the liver, whereas laboratory findings showed re-elevated C-reactive protein levels. Since conservative treatment had failed, we decided to perform surgery. Considering the imaging study findings, malignant disease could not be ruled out. Based on the operative findings, we performed combined resection of the falciform ligament, left liver, and gallbladder. Histopathological examination of the resected specimens revealed extensive neutrophil infiltration and the presence of giant cells and foam cells within the lesions. These findings were indicative of abscess. Pseudomonas aeruginosa was cultured from the pus in the falciform ligament mass and bile in the gallbladder. Although multiple abscesses postoperatively developed in the residual portion of the liver, they could be treated through antibiotic therapy.ConclusionsFLA can spread to both adjacent and distant organs via its rich vascular and lymphatic networks. When FLA displays atypical image findings and/or an atypical clinical course, it can be difficult to distinguish it from malignant disease. In such cases, surgical treatment, with intraoperative pathological diagnosis, should be attempted.

Flattening of circadian glucocorticoid oscillations drives acute hyperinsulinemia and adipocyte hypertrophy.

Disruption of circadian glucocorticoid oscillations in Cushing's disease and chronic stress results in obesity and adipocyte hypertrophy, which is believed to be a main source of the harmful effects of obesity. Here, we recapitulate stress due to jet lag or work-life imbalances by flattening glucocorticoid oscillations in mice. Within 3days, mice achieve a metabolic state with persistently high insulin, but surprisingly low glucose and fatty acids in the bloodstream, that precedes a more than 2-fold increase in brown and white adipose tissue mass within 3weeks. Transcriptomic and Cd36-knockout mouse analyses show that hyperinsulinemia-mediated de novo fatty acid synthesis and Cd36-mediated fatty acid uptake drive fat mass increases. Intriguingly, this mechanism by which glucocorticoid flattening causes acute hyperinsulinemia and adipocyte hypertrophy is unexpectedly beneficial in preventing high levels of circulating fatty acids and glucose for weeks, thus serving as a protective response to preserve metabolic health during chronic stress.

Resection of giant malignant solitary fibrous pleural tumor after interventional embolization: a case report and literature review

BackgroundSolitary fibrous tumor of the pleura (SFTP) is a rare mesenchymal tumor that arises at various sites and typically originates from the pleura. Most patients with SFTPs are asymptomatic, unless the tumor is large. Approximately 20% of SFTP cases are malignant. There are few reports on imaging diagnoses and interventional treatments of SFTP. Here, we report a case of a giant SFTP that exhibited malignant behavior and underwent successful resection after embolization of the main supply artery of the tumor.Case presentationWe report a clinical case of a giant SFTP in a 66-year-old Chinese female patient complaining of chest tightness and cough for more than 2 months. Ten years ago, the patient had undergone a chest CT scan at a local hospital for cough. Computed tomography (CT) had revealed a mass in the right thoracic region, which was misdiagnosed as a pulmonary abscess by CT-guided biopsy. Therefore, the patient did not receive appropriate/complete treatment at that time. She was hospitalized again, because CT showed significant enlargement of the right thoracic mass, which caused her obvious symptoms of discomfort. The pathological results of CT-guided biopsy at our hospital confirmed SFTP. Considering the large size of the tumor and the rich blood supply, some of the main blood vessels were treated with embolization before surgical resection. A large tumor, about 23 cm × 16 cm × 15 cm in size, was then successfully removed by thoracic surgery. The diagnosis of malignant SFTP was confirmed by surgical pathology and immunohistochemistry.ConclusionImaging findings of SFTPs are not characteristic, especially when a tumor is large, the diagnosis is difficult, and the final diagnosis still depends on histological and immunohistochemical examinations. The two-stage surgical treatment described here, which involves first embolization of the main supplying artery of the large tumor and then complete surgical resection, is effective and safe for SFTPs. Whether needle biopsy or vascular embolization is performed, intervention plays a crucial role in the diagnosis and treatment of patients with SFTPs.

Endomyocardial fibrosis in a 7-year-old Nigerian child: a case report and review of the literature

<p>Endomyocardial fibrosis (EMF) is a poorly-understood idiopathic disorder characterized by the development of restrictive cardiomyopathy. It is a disorder of the tropics and subtropics and was once the second commonest cause of acquired heart disease in Nigeria. Its diagnosis is often made when the disease is advanced and the prognosis is invariably poor at this stage. Reports of EMF have become increasingly rare in recent years and it is frequently misdiagnosed. This report seeks to highlight EMF as a still present and potentially important cause of heart failure in Nigerian children. A 7-year-old male referred to the pediatric cardiology clinic with complaints of abdominal and facial swelling, with no difficulty in breathing and exercise intolerance. Examination revealed a classic “egg-on-stick” appearance. Apex was not displaced and heart sounds were distant. Liver was enlarged but non tender and there was demonstrable ascites. A 2D echocardiography showed massive right atrial enlargement with severe tricuspid regurgitation and fibrosis within the right ventricular cavity which was small. There was a moderate-sized pericardial effusion. Electrocardiogram showed right atrial enlargement with low-voltage complexes. Full blood count showed eosinophilia (20% total white blood cell count). Total serum protein and albumin were reduced. EMF, though increasingly rare, remains an important cause of childhood cardiac morbidity and mortality. A high index of suspicion is critical as the prognosis remains poor.</p><p> </p>

Long delay in diagnosis of a case with MEN1 due to concomitant presence of AIMAH with insulinoma: a case report and literature review

BackgroundACTH-independent macronodular hyperplasia (AIMAH) is an uncommon disorder characterized by massive enlargement of both adrenal glands and hypersecretion of cortisol. Concomitant AIMAH and multiple endocrine neoplasia type1 (MEN1) is rare to our knowledge.Case presentationHerein, we describe a 32 year old woman with long history of prolactinoma and secondary ammonhrea presented with not-severe manifestation of hypoglycemia due to concomitant presence of insulinoma with AIMAH leading to 12 years delay of MEN1 diagnosis. Laboratory tests showed severe hypoglycemia associated with hyper insulinemia (non-fasting blood sugar = 43 mg/dl, insulin = 80.6 μIU /ml, C-peptide = 9.3 ng/ml) hyperparathyroidism (calcium = 10.3 mg/dl, phosphor = 3.1 mg/dl, PTH = 280 pg/ml) and chemical evidence of an ACTH-independent hypercortisolism (serum cortisol value of 3.5, after 1 mg dexamethasone suppression test serum ACTH value of 17 pg/ml, and high urinary cortisol level). Abdominal CT scan demonstrated two enhancing well-defined masses 27*20 mm and 37*30 mm in the tail and body of the pancreas, respectively, and a 36*15 mm mass in left adrenal gland (seven Hounsfield units). Dynamic pituitary MRI revealed a partial empty sella. The physical examination of the patient was unremarkable. Distal pancreatectomy and a left adrenalectomy were performed. After the surgery, we observed clinical and biochemical remission of hyper insulinemia and gradual decrease in urinary cortisol. The histological features of the removed left adrenal gland were consistent with AIMAH. Histological examination of the pancreatic lesions revealed well differentiated neuroendocrine tumors. Genetic abnormalities in the MEN1, heterozygote for pathogenic variant chr11; 645,773,330-64577333AGAC, c.249-252delGTCT, p. (11e85Serfs Ter33) in exon 2 were found. It was recommended the patient undergoes parathyroidectomy as soon as possible.ConclusionGiven the history and presentation of our case, we recommend that the clinicians consider the possibility of autonomous cortisol production in MEN1 patients who do not show severe symptoms of hypoglycemia in the presence of insulinoma.

Endoscopic Endonasal Approach for Suprasellar Mature Teratoma in Growing Teratoma Syndrome: 2-Dimensional Operative Video.

Intracranial growing teratoma syndrome is a rare phenomenon characterized by enlargement of a germ cell tumor during or after adjuvant therapy despite normalization of tumor markers.1,2 It has been suggested that chemotherapy acts on the nonteratomatous components or induces differentiation of the immature germ cells to mature teratomatous phenotype.3 An 8-year-old boy presented with headache, emesis, and blurry vision. Neuroimaging revealed hydrocephalus with multiple central nervous system masses: pineal gland, suprasellar region, and spine (T8). Elevated serum and cerebrospinal fluid levels of alpha-fetoprotein and beta-human chorionic gonadotropin were found. A ventriculoperitoneal shunt was placed. Despite chemotherapy and decreasing tumor markers, the pineal mass rapidly enlarged, and the patient became somnolent. He underwent microsurgical resection with the diagnosis of mixed germ cell tumor. During his second chemotherapy cycle, the patient endorsed worsening vision and panhypopituitarism. Imaging demonstrated enlargement and honeycomb appearance of the suprasellar mass. The patient and family consented to the procedure, and an endoscopic endonasal approach was performed to access the retroinfundibular region. Removal of the nonfunctional pituitary gland and dorsum sellae was performed. Careful dissection of the tumor from the optic apparatus, hypothalamus, and perforating arteries allowed total resection. Reconstruction with fascia lata and nasoseptal flap was performed. Mature teratoma was found histologically. Postoperative course was complicated by flap hemorrhage resolved by surgical revision. Postoperative imaging showed complete resection. The patient was discharged without other complications and was making excellent recovery. To the best of our knowledge, this is the first reported case of hypothalamic intracranial growing teratoma syndrome successfully treated using an endoscopic endonasal approach.

Management anesthesia in carotid artery aneurysm caused by a Behcet’s disease: A case report

Objective: Arterial manifestations of Behcet’s disease are challenging and can lead to fatal complications. One of the causes of carotid aneurysm is Behcet’s disease. Extracranial internal carotid artery aneurysms are rare. This aneurysm is rare but important because it is associated with a high risk of neur thromboembolic events, cranial nerve damage, and rupture. Method: We describe a 19-year-old man with a history of Behcet’s disease who presented with a gradual enlargement of the right neck mass with shortness of breath when he is supine. Result: This report examines the challenges of treating carotid aneurysms by spreading to the mediastinum in a patient with Behcet’s disease. Conclusion: It is important to pay attention to the need for awake intubation by expanding to the mediastinum.

A Huge Liposarcoma: Case Study

A 53-year-old had complaints of vague abdominal pain for the past few months. The patient had a history of diabetes mellitus, hypertension, and iron deficiency. The pain was aching, mild, intermittent, non-radiating, and generalized. An ultrasound of the abdomen, CT scan and MRI were recommended. After initial examination and reports, the patient was diagnosed with a retroperitoneal tumor. The liposarcoma found was dedifferentiated, high grade, and retroperitoneal. The tumor extended to the inked resection margin, and was large, with 24 cm and 14 cm in greatest dimension measurements. The liposarcoma was lipomatous and solid, with hot spots of mitosis. In terms of treatment, surgery was recommended to remove the enlarged mass. Two heterologous fragments, which were different in morphology, size, and color, were obtained from the retroperitoneal area during surgery. The mass was a huge grade 3 liposarcoma with a mitotic rate of > 20 mitoses per 10 high-power fields (HPF). The examination and diagnosis included some special studies such as immunohistochemistry assay, which was positive for p16, vimentin, and MDM2. A post-operative scan showed that there was no evidence of recurrence or residual retroperitoneal tumour. Chemotherapy was initiated by the Oncology Department.

English

Gigantomastia is a rare breast disease with massive bilateral enlargement of breast causing physical and psychosocial disability for the patient. Diffuse pseudoangiomatous stromal hyperplasia causing gigantomastia is a very rare presentation. We present a case of 45-year-old female with bilateral gigantomastia and cyclical mastalgia, who underwent bilateral mastectomy, postoperatively diagnosed with pseudoangiomatous hyperplasia. Gigantomastia is a rare condition characterised by excessive breast tissue growth resulting in massive enlargement of both breast. An arbitrary definition for gigantomastia is breast size more than 1.5 kg or when the breast tissue comprises 3 % or more of patients total body weight. Gigantomastia is accompanied by skin ulceration, postural problems, back pain, traction nerve injury and social stigmata. It is physically and psychosocially disabling for the patient. The aetiopathogenesis of gigantomastia is poorly understood, but generally classified as idiopathic, drug induced (penicillamine, cyclosporine) and due to hormonal imbalance (gestational and pubertal).1 Pseudoangiomatous stromal hyperplasia (PASH) is a benign mesenchymal proliferative disease of the breast which is usually an incidental finding accompanying other breast pathology like fibroadenoma, fibrocystic disease, breast cancer, gynaecomastia etc. It was first described by Vuitch, Erlandson and rosen (1986). Ibrahim et al. reported the occurrence of incidental microscopic PASH in breast specimens as around 23 %.2 Around 200 cases of PASH presenting as a tumoral palpable mass is there in literature. Another very rare and under recognised presentation is as diffuse PASH with bilateral or unilateral massive breast enlargement. Here we report a case of diffuse PASH with gigantomastia and a review of literature.

Small Cell Neuroendocrine Carcinoma in Head and Neck

Introduction: Poorly differentiated neuroendocrine carcinomas (NECs) originating from the eye are rare and very highly malignant diseases with a poor prognosis. Small cell NEC of the head and neck is a rare disease and highly aggressive. Early recognition and treatment are crucial for reducing morbidity and mortality. Case Presentation: A 19-year-old male visited our oncology surgery outpatient department due to the progressive neck mass enlargement originating from the eye. The patient was previously diagnosed with invasive choroid malignant melanoma of the left eye which had metastasized to the lymph nodes of the left neck. He underwent a surgical removal/exenteration of the left eye. The result showed that the patient’s survival with poorly differentiated tumors was about 14% while patients with well-differentiated NEC had a survival rate of 34%. It also indicates that the prognosis of these tumors is very poor with a total of over 90% of patients having distant metastatic disease. Histopathological examination showed the tumor tissue and its immunohistochemistry with positive streaks of CD56, NSE, Synaptophysin, and Ki67 suggested small cell NEC.Conclusions: it is crucial to establish an early diagnosis of these tumors to reduce morbidity and mortality. No optimal treatment for such disease has yet been established.

Is There a Functional Role of Mitochondrial Dysfunction in the Pathogenesis of ARPKD?

Polycystic kidney diseases (PKD) form a group of severe genetic kidney diseases that are characterized by fibrocystic changes of the kidneys and the liver. The main forms of PKD are autosomal dominant and autosomal recessive polycystic kidney disease (ADPKD, ARPKD). In general, cystic kidney diseases are the most common genetic cause of chronic kidney failure in both children and adults (1). ARPKD is a disorder that typically is diagnosed very early in life or even prenatally. Typically, the kidneys show bilateral massive enlargement although they keep their reniform shape. Kidney disease results in a need for kidney replacement therapy (KRT) in around 50% of patients in the first two decades of life (2, 3). Liver involvement is obligatory in ARPKD, typically presenting with congenital hepatic fibrosis and portal hypertension or with dilated bile ducts. Both are considered to be a consequence of a defect during development of the bile ducts (“ductal plate malformation”) (4). Adult patients with ARPKD have been described with variable disease manifestations. Hepatic disease may be the leading clinical issue in adults with ARPKD (5). In ADPKD renal cystogenesis starts early in life or even before birth but does not present with obvious clinical symptoms until adulthood in most patients (1, 6, 7). Liver involvement is typically characterized by liver cysts. Additional extrarenal symptoms in ADPKD include for example intracranial aneurysms, cardiac valve anomalies like mitral prolapse or abdominal wall diverticula in a subset of patients (1, 7). Extrarenal manifestations may be very helpful for clinical differential diagnosis and to distinguish ADPKD from ARPKD. ADPKD is mainly caused by variants in the genes PKD1 and PKD2 with the large majority of patients carrying PKD1 variants (1). Variants in other genes e.g., with atypical forms of ADPKD have been identified (7). Variants in PKHD1 are the main cause of ARPKD with phenotypical overlay e.g., of kidney disease in patients with other underlying genetic changes (1, 8), including biallelic hypomorphic PKD1 variants. Genotype-phenotype correlations in ADPKD show more rapid progression of kidney disease in patients with PKD1 variants and especially in those patients carrying variants resulting in protein truncation (7, 9). For ARPKD it was shown by various groups that biallelic truncating variants are associated with severe phenotypes. A recent study extended the findings and revealed that for missense variants the affected region in PKHD1 also seems to be important (10). In a study on 304 children with the clinical diagnosis of ARPKD and detected PKHD1 variants it was found that patients with either two missense variants affecting the amino acids 709–1837 or a null variant and a missense variant in this region less frequently showed progression to chronic kidney failure when being compared during their follow-up when compared to patients with variants affecting other regions of PKHD1. On the other hand patients with variants affecting the amino acids 2625–4074 showed less favorable hepatic outcome (10). The underlying molecular mechanisms of these associations remain to be explored.

Plasmodiophora brassicae-Triggered Cell Enlargement and Loss of Cellular Integrity in Root Systems Are Mediated by Pectin Demethylation.

Gall formation on the belowground parts of plants infected with Plasmodiophora brassicae is the result of extensive host cellular reprogramming. The development of these structures is a consequence of increased cell proliferation followed by massive enlargement of cells colonized with the pathogen. Drastic changes in cellular growth patterns create local deformities in the roots and hypocotyl giving rise to mechanical tensions within the tissue of these organs. Host cell wall extensibility and recomposition accompany the growth of the gall and influence pathogen spread and also pathogen life cycle progression. Demethylation of pectin within the extracellular matrix may play an important role in P. brassicae-driven hypertrophy of host underground organs. Through proteomic analysis of the cell wall, we identified proteins accumulating in the galls developing on the underground parts of Arabidopsis thaliana plants infected with P. brassicae. One of the key proteins identified was the pectin methylesterase (PME18); we further characterized its expression and conducted functional and anatomic studies in the knockout mutant and used Raman spectroscopy to study the status of pectin in P. brassicae-infected galls. We found that late stages of gall formation are accompanied with increased levels of PME18. We have also shown that the massive enlargement of cells colonized with P. brassicae coincides with decreases in pectin methylation. In pme18-2 knockout mutants, P. brassicae could still induce demethylation; however, the galls in this line were smaller and cellular expansion was less pronounced. Alteration in pectin demethylation in the host resulted in changes in pathogen distribution and slowed down disease progression. To conclude, P. brassicae-driven host organ hypertrophy observed during clubroot disease is accompanied by pectin demethylation in the extracellular matrix. The pathogen hijacks endogenous host mechanisms involved in cell wall loosening to create an optimal cellular environment for completion of its life cycle and eventual release of resting spores facilitated by degradation of demethylated pectin polymers.

A case report of secondary neurolymphomatosis showing selective nerve infiltration and massive lumbar plexus enlargement

BackgroundNeurolymphomatosis (NL) is a rare manifestation of malignant lymphoma that shows selective infiltration to the peripheral nervous system primarily or secondarily. We report a patient with secondary NL caused by germinal center B-cell (GCB)-type diffuse large B-cell lymphoma (DLBCL) who showed selective infiltration of the lumbar plexus to the spinal cord and massive nerve enlargement resulting in severe pain.Case presentationA 72-year-old female exhibited asymmetric motor and sensory impairments and pain in the lower limbs that progressed for five months. Magnetic resonance imaging (MRI) showed an enlarged lumbar plexus, which continued to the cauda equina via the L3 and L4 spinal nerves. Her symptoms gradually worsened. Ten months after the onset of symptoms, the enlarged cauda equina filled the spinal canal space, and the spinal cord was swollen. A cauda equina biopsy was performed, and she was diagnosed with GCB-type DLBCL with CD10 positivity. The primary tumor was found in a mammary cyst. The autopsy study did not show apparent infiltration, except in the nervous system.ConclusionsAlthough there are many neurologic phenotypes of malignant lymphoma, the association between the cytological characteristics of lymphoma and the neurological phenotypes is still unclear. Several reports of CD10-positive secondary NL are available, whereas peripheral or central nervous tissue origin lymphoma cases are mostly negative for CD10. CD10 staining may be useful for distinguishing primary NL from secondary NL. NL often has a strong organotropism for peripheral nervous tissue, which makes early diagnosis challenging.

A giant juvenile fibroadenoma in a 12-year-old girl: Case report and brief literature review

Juvenile fibroadenoma is the most common breast mass in adolescents accounting for 0.5–4% of all cases of fibroadenomas. Giant fibroadenomas are rare variants and represent only 0.5% of all fibroadenomas, usually occurring in girls between the ages of 10 and 18. They are characterized by massive and rapid enlargement of a rubbery, mobile, and non-tender mass and defined as being larger than 5 cm or weighing more than 500 g. The aetiology is not well understood and is believed to be an increased sensitivity to normal oestrogen level. We present a 12-year-old Asian girl treated at the age of 8 for idiopathic precocious puberty, without known family history of breast masses or cancers, who developed spontaneous giant fibro-adenoma measuring 15cm X 17 cm. The patient was further evaluated via ultrasonography showing a sole large lesion of 15 X 17 cm in the left breast. Surgical excision with conservation of the developing breast parenchyma and nipple-areolar complex under general anaesthesia was performed. Histopathological findings after the surgical excision were suggestive of juvenile fibroadenoma. The patient has a normal breast development throughout 4 years follow-up. The rapid growth of a breast mass in such young patients can be of great concern causing a considerable impact on these patients’ psychological and emotional condition. One of the most important concerns is the impact on potential future mammary gland development. Therefore, it is crucial to promptly rule out malignant processes or phyllodes tumors and educate young patients and their families on treatment options that fit their concerns. In addition, the final cosmetic result is essential after the surgical excision; although the primary cause of asymmetrical breast enlargement is a benign mass, early surgical excision is efficient concerning the best possible cosmetic outcome. Keywords: Breast mass; juvenile fibroadenoma; giant fibroadenoma.

Massive Mixed Adenoneuroendocrine Carcinoma: A Case Report

Mixed adenoneuroendocrine carcinoma (MANEC) of the gastrointestinal (GI) tract is a rare subtype of mixed tumors, and it is scarcely described in the literature. MANEC tumors are composed of adenocarcinoma and neuroendocrine carcinoma components, each of which comprises at least 30% of the lesion. Diagnosing MANEC requires specific histological and immunohistochemistry (IHC) analysis. Typically, MANEC tumors carry a poor prognosis due to their very aggressive nature.We report the case of a 70-year-old female patient with no past medical history who presented with a three-week history of abdominal pain and one episode of hematemesis one week prior to presentation. Initial CT of the abdomen showed a large, 8 x 6 x 6-cm mass arising from the stomach and extending to the lesser sac as well as the central crus of the diaphragm with bilateral retroperitoneal lymphadenopathy. Upper endoscopy revealed an excavated, ulcerated, and partially necrotic mass on the lesser curvature of the proximal gastric body. Tissue biopsy of the lesion showed infiltrating mixed poorly differentiated adenocarcinoma and neuroendocrine carcinoma. On IHC, the adenocarcinoma component stained positively for CDX2 and pancytokeratin, and the neuroendocrine component stained positively for synaptophysin and chromogranin. Further workup included CT of the chest, which demonstrated extensive bilateral pulmonary emboli and new liver lesions with moderate ascites not seen on the initial abdominal CT. The latter was repeated and showed remarkable enlargement of the gastric mass (up to 12 cm) with extensive retroperitoneal adenopathy and mesenteric implants. Given the rapid clinical deterioration and progression of tumor burden, comfort measures were offered and the patient passed away soon after. MANEC tumors are highly aggressive subtypes of "collision" tumors, which are not well described in the medical literature due to their rarity. The etiology is poorly understood with various theories proposing different pathophysiological mechanisms. Standard therapy is not well developed at present; however, a few reports have demonstrated successful outcomes with surgery or combined chemotherapy (cisplatin with irinotecan or etoposide) if diagnosed at an early stage.

Necrotizing infundibulo-hypophysitis: case-report and literature review

Purpose We describe a rare case of histopathologic-proven necrotizing infundibulo-hypophysitis (NIH). Clinical history A 40-year-old female presented with coexistence of central diabetes insipidus and hypopituitarism. Imaging disclosed a thickened infundibulum and a diffusely enlarged pituitary mass with gadolinium rim enhancement pattern. Microsurgical endonasal transsphenoidal resection was performed. The presence of extensive liquefactive necrosis, surrounded by lymphoplasmocytic inflammatory infiltrate, allowed for the diagnosis of NIH. Follow-up cranial imaging 10 months after surgery showed no evidence of reappearance of the lesion. There was no progression to panhypopituitarism. Conclusion Surgery and histopathological confirmation are the key diagnostic feature in NIH. The current case is the fifth report of NIH and the first one with an indolent course and without progression to panhypopituitarism so far.

Presentation of External Ear Rosai-Dorfman Disease With Laryngeal Involvement.

Rosai-Dorfman disease (RDD) is a rare benign systemic histiocytic proliferation characterized by massive lymph node enlargement and sometimes associated with extranodal involvement. Even though it is considered to be benign, death can occur depending on the extent and location. Our case highlights a primary extranodal site of the right pinna with extension through the Eustachian tube to the subglottis. A previously healthy 15-year-old female presented with 1-year right pinna swelling, slowly enlarging and becoming more bothersome. An incisional biopsy was performed on the ear along with S100 staining yielding a diagnosis. After multidisciplinary case discussion, clofarabine monotherapy and systemic therapy for Langerhans cell histiocytosis has started. Rosai-Dorfman disease can be a general disorder, often affecting the lymph nodes. Unlike a nodal disease, extranodal disease could involve any site on the patient's anatomy. Head and neck lesions are the most common extranodal lesions. Rosai-Dorfman disease is self-limited in more than 20% of the cases with spontaneous regression without intervention; 70% of the patients have noticeable symptoms and vital organ involvement requiring treatments such as surgery, steroids, radiation, and chemotherapy. In our case, the patient had wide involvement and presented without any serious breathing difficulties; we decided to start with monotherapy with chemotherapy and systematic glucocorticoid treatment.

Metastatic Adrenocortical Carcinoma Co-Secreting Multiple Steroid Hormones

Background: Adrenocortical carcinoma is a rare disease which may be complicated by co-secretion of multiple steroid hormones.Clinical Case: A 53- year-old female was discovered to have 1 cm left and 1.7 cm right adrenal nodules by Chest CT scan in 2004. She had a follow-up abdominal CT scan in 2011 revealing enlargement of the left adrenal mass to 5.7 cm and a stable 1.8 cm right adrenal nodule. A laparoscopic left adrenalectomy was performed in 2012 and the surgical pathology diagnosis was benign adrenal hyperplasia. In 5/2016 the patient developed left abdominal pain and a CT scan revealed a 1.7 cm mass in the left adrenalectomy surgical bed, a 1.4 cm nodular density adjacent to the left diaphragm and the stable 1.8 cm right adrenal nodule. 6/2016 lab tests: 24 hr urinary cortisol 15 ug/24 hr (<50 μg/24hr), aldosterone 8.7 ng/dL (<31 ng/dL), renin activity 0.7 ng/ml/hr (0.5–4 ng/mL/hr) and DHEA-S 94 ug/dL (32–240 μg/dL). A re-examination of the 2012 surgical pathology resulted in an addendum diagnosis of an adrenal cortical neoplasm of indeterminate malignant potential. In 1/2018 she underwent an exploratory laparotomy with surgical resection of the 1.7 cm mass in left paracolic gutter and biopsy of numerous small retro-peritoneal and multiple liver lesions. Pathology revealed metastatic adrenocortical carcinoma with low grade mitotic activity (3 mitoses per 10 HPF) and intermediate grade Ki-67 (15–25%). 5/2018 lab results: 1. aldosterone 20 ng/dL, 2. renin activity 0.2 ng/mL/hr and 3. testosterone 34 ng/dL (<75 ng/dL). Mitotane was started in 06/2018 but was discontinued in 9/2018 due to side effects. In 3/2020 she was hospitalized for generalized weakness and was discovered to be severely hypokalemic K+ 1.5 mmol/L (3.6–5.2 mmol/L) with an aldosterone of 300 ng/dL and renin activity of 0.1 ng/mL/hr. She was treated with IV KCl to correct her hypokalemia and was discharged on oral KCl 20 meq bid and spironolactone 50 mg bid. She was readmitted to the hospital on 10/12/2020 after a near-syncopal event and lab tests revealed a K+ of 1.4, aldosterone 508 ng/dL, renin activity 0.7 ng/mL/hr, AM cortisol 13.6 μg/dL (5–20 ug/dL), testosterone 161 ng/dL, and DHEA-S 377 ug/dL, indicating co-secretion of multiple steroid hormones. Her hypokalemia was treated with IV KCl and her spironolactone dose was increased to 100 mg bid.Conclusion: This case report details the rare occurrence of an adrenocortical carcinoma which was hormonally silent but eventually metastasized and became hormonally active, co-secreting multiple steroid hormones with a predominance of aldosterone. Serial adrenal hormone lab profiles are important for optimal management of patients with this disease.

Leukocytosis and PTHrP-Mediated Hypercalcemia: Ominous Signs in an Indolent Thyroid Nodule

Introduction/Background: Anaplastic thyroid cancer is a rare thyroid malignancy with a bad prognosis. There are few systemic markers, as thyroglobulin is not usually synthesized in these poorly differentiated malignancies. Clinical Case - Diagnostic Evaluation, Treatment, and Follow Up: A 70-year old man presented in 2016 with a right thyroid nodule, slowly growing since 2012. The nodule was 10cm on neck CT and ultrasound showed a hyperechoic mass with central hypoechoic area, and coarse calcifications. Fine needle biopsy showed a follicular neoplasm. By 2019, neck CT showed enlargement of the nodule to 13 cm. Repeat thyroid biopsy was benign. Lobectomy was recommended due to size but not performed. In 2020, he developed neck discomfort and painful thigh swelling. WBC was significantly elevated at 27,000 (4000-10000) /mm3. Ultrasound showed no evidence of necrosis or abscess in the nodule. WBC rose progressively to 96,000/mm3 (ANC 94,200). CT chest showed further enlargement of the thyroid mass, a soft tissue shoulder mass and lung nodules suspicious for metastases. Laryngoscopy revealed a distorted larynx from external compression, and possible vocal-fold involvement (motion impairment) without airway compromise. Biopsy of the shoulder mass, lung nodule, and thigh masses all showed metastatic anaplastic thyroid cancer. Full infectious disease evaluation and molecular testing for BCR-ABL and JAK2 mutations were negative. Thus, leukocytosis was determined to be reactive in the setting of malignancy. Serum calcium of 14.7 mg/dL (8.5 - 10.6mg/dL) prompted hospital admission. Bone metastases were not visible on imaging. Hypercalcemia was treated effectively (to 8.3mg/dL) with IV fluids, calcitonin and IV bisphosphonates. Workup revealed suppression of PTH to 4 pg/mL (15-65 pg/mL) and low 25 Vitamin D of 21 ng/mL (30-80ng/L). Subsequently PTHrP was elevated at 53.4 pmol/L (<2.3pmol/L) and 1,25 Vitamin D inappropriately normal at 48.9 pg/mL (20- 79.3pg/mL), suggested humoral hypercalcemia of malignancy. The patient declined further treatment beyond hospice care. Clinical Lesson/Conclusion: Significant, sudden leukocytosis and hypercalcemia prompted further evaluation of his long-standing apparently benign thyroid nodule leading to a diagnosis of anaplastic thyroid cancer. Presumably this mass had been a follicular thyroid cancer, misdiagnosed on cytology, that subsequently de-differentiated. There are uncommon reports of leukocytosis complicating anaplastic thyroid cancer, where elevated GCSF and IL-6 were noted. We suspect this same etiology here. Hypercalcemia is extremely rare other than one report of PTHrP-secreting anaplastic thyroid cancer. Thus, high, rising calcium and PTHrP levels may reflect level of disease activity in anaplastic thyroid cancer.