Ovarian Vein Thrombosis in a Non-Puerperal Patient.

Ovarian vein thrombosis (OVT) is a relatively rare, but serious disease more commonly seen in puerperal patients. In the postpartum period, there is an unequal incidence of OVT in women with vaginal delivery (0.18%) and caesarean section (2.0%). OVT is usually associated with other conditions like pelvic inflammatory disease, malignant tumours, sepsis, enteritis, and recent pelvic or abdominal surgery. The incidence of idiopathic OVT is extremely rare and only a few cases have been reported to date in healthy patients with unknown aetiology. Patients with OVT present with abdominal pain, nausea, vomiting, fever or abdominal mass. Complications of OVT include pulmonary embolism, ovarian infarction, sepsis and death. CT scanning of the abdomen provides reliable evidence for the diagnosis of idiopathic OVT. Thrombus in most patients with idiopathic OVT is located in the right ovarian vein. This may be due to the longer length of the right ovarian vein, right shift of the uterus and relative insufficiency of the right ovarian vein valve. Treatment of OVT includes systemic anticoagulation which will achieve resolution of symptoms and prevent life-threatening complications. Our case report documents a non-puerperal patient who underwent hysterectomy a year before presenting to the ED with complaints of diarrhoea and found to have OVT on a CT scan. Our case brings attention to the fact that OVT can occur outside the puerperium and might present as a rare complication of minimally invasive surgeries for endometrial cancer. Furthermore, we posit that OVT in the non-puerperal patient may be a separate clinical entity. LEARNING POINTS: Ovarian vein thrombosis (OVT) can occur outside the puerperium and might present as a rare complication of minimally invasive surgeries.OVT should be included in the differential diagnosis of a non-puerperal postoperative woman presenting with vague abdominal symptoms.Duration of anti-coagulation varies from case to case. More research is needed to determine its exact duration.

Adrenal Myelolipoma Masquerading as an Adrenal Malignancy.

An adrenal myelolipoma presenting with suspicious features may pose a diagnostic challenge to surgeons and endocrinologists. In this case report of an adult patient with undiagnosed congenital adrenal hyperplasia presenting with bilateral adrenal masses, we review his radiographic and clinical findings which were highly suspicious for adrenal malignancy. Features of adrenal myelolipoma that may resemble malignant lesions are reviewed. This case report highlights important features of adrenal myelolipoma that the surgeon and endocrinologist should be aware of. The importance of avoiding overtreating adrenal myelolipomas presenting as tumors of uncertain malignant potential is crucial.

Diffuse Large B-Cell Lymphoma of the Left Upper Extremity Mimicking a Sarcoma.

Diffuse large B-cell lymphoma (DLBCL) can arise in both lymph nodes and extranodal sites. DLBCLs rarely present in the soft tissue of the upper extremity. We report a case of a 64-year-old woman who presented with a large left upper arm mass and underwent surgical resection under the presumptive diagnosis of sarcoma but the final pathology showed DLBCL. Sarcomas are common malignant tumors of the soft tissue of the extremities, but lymphomas also occasionally present as a soft tissue mass. It is important to keep lymphomas in mind in order to avoid unnecessary surgical excisions.

Erdheim-Chester Disease With Concurrent Lung Adenocarcinoma.

Erdheim-Chester disease (ECD) is a rare type of blood cancer characterized by infiltration of the body tissues by pathologic histiocytes, leading to widespread inflammation and damage. Clinical presentations range widely, from asymptomatic infiltration of bone to multiple organ system damage and resultant dysfunction. This report describes a case of a patient with several unusual imaging findings that led to a differential diagnosis of ECD; however, a biopsy of a mediastinal mass suspected to be due to histiocyte infiltration instead revealed primary lung cancer. Ultimately, ECD could not be ruled out, and the patient was referred to dermatology for a superficial facial xanthelasma biopsy, results of which were consistent with ECD. Concurrent ECD and adenocarcinoma is highly unusual; this case demonstrates the importance of a thorough investigation and the consideration that not all findings may be attributable to a single disease process, even when the alternative is very unlikely.

A Case of Thoracic Endometriosis Syndrome Presenting with Recurrent Catamenial Pneumothorax.

BACKGROUND Catamenial pneumothorax (CP) is a spontaneous pneumothorax commonly associated with menstrual periods. Endometrial tissues most commonly involve the pelvic region. However, after the pelvis, the lungs are most frequently involved. Thoracic endometriosis should always be suspected in young women presenting with CP. CASE REPORT A 30-year-old woman with history of endometriosis presented with chief complaint of umbilical pain. A computerized tomography (CT) scan of the abdomen and pelvis was performed, which showed an incidental finding of a large right-sided pneumothorax. Chest X-ray imaging showed 50% pneumothorax. A right-sided chest tube was placed, and after the procedure, a chest X-ray image showed expansion of the right lung. The patient was readmitted for elective resection of an umbilical mass and was again incidentally found to have a recurrent pneumothorax on the right side. She underwent videothoracoscopic pleurodesis with pathology, establishing the diagnosis of catamenial pneumothorax. CONCLUSIONS Thoracic endometriosis resulting in catamenial pneumothorax should be suspected in young women of child-bearing age. Treatment options still under debate include endoscopic resection and videothoracoscopic pleurodesis followed by gonadotrophin-releasing hormone (GnRH) therapy to reduce the rate of postoperative recurrence.

Diffuse Peritoneal and Bowel Wall Infiltration by Light Chain-AL Amyloidosis with Omental Calcification Mimicking Abdominal Carcinomatosis - An Elderly Female with Incidental Finding of Light Chain Monoclonal Gammopathy of Undetermined Significance (LC-MGUS).

BACKGROUND Amyloidosis is the extracellular tissue deposition of plasma proteins, which after conformational changes, forms antiparallel beta pleated sheets of fibrils. Amyloid light-chain (AL) is a type of amyloidosis that is due to deposition of proteins derived from immunoglobulin (Ig) light chains. Gastrointestinal tract (GIT) involvement most often found in amyloid A (AA) amyloidosis type. There have been no reports of obstructive GIT AL amyloid patients having monoclonal gammopathy of undetermined significance (MGUS). Our case is the first case to show two coinciding conditions; one is the association of GIT AL amyloidosis with the incidental finding of a rare type of MGUS (LC-MGUS) and the other is the radiologic presentation of GIT amyloidosis with omental calcification mimicking the GIT malignancy. CASE REPORT A 68-year-old female presented with symptoms of partial bowel obstruction, including intermittent diffuse abdominal pain and constipation. After computed tomography (CT) abdomen and pelvis, an exploratory laparotomy was needed because of suspicion of abdominal carcinomatosis due to diffuse omental calcification. The tissue sent for biopsy surprisingly showed AL amyloidosis. The patient did not report any systemic symptoms. Further workup was advised to inquire about the plasma cell dyscrasia which eventually turned into a very rare version of MGUS knows as light chain MGUS (LC-MGUS). Following adequate resection of the involved structures, the patient was then placed on chemotherapy and successfully went into remission. CONCLUSIONS This case report illustrates that in an era of evidence based medicine, it is important to show through case reports the association of GIT AL amyloidosis with LC-MGUS, as the literature on this topic is lacking. It also points to the importance of timely intervention that can greatly enhance, not only the only the chances of remission but also prevention of further complications such as malignant transformation.

A Case Report of Cystic Pheochromocytoma.

BACKGROUND Pheochromocytoma is a rare catecholamine-producing tumor with an estimated incidence of less than 0.1% in the global population. We present a case of cystic pheochromocytoma that was diagnosed as an incidental finding. The patient presented with abdominal pain and had a history of hypertension. CASE REPORT A 64-year-old man with hypertension presented with a clinical history of intermittent abdominal pain for one year. He denied sweating, palpitations, headache or back pain. He was found to have an elevated blood pressure of 170/90 and no palpable abdominal mass. Contrast-enhanced computed tomography (CT) imaging of the abdomen and pelvis were performed that showed cystic mass measuring 9 cm in diameter arising from the left adrenal gland with contrast-enhancing mural nodules. Magnetic resonance imaging (MRI) confirmed the cystic nature of the mass. Laboratory analysis showed an elevated plasma normetanephrine (NMN) of 1,087 pg/ml and metanephrine (MN) of 372 pg/ml; 24-hour urine showed elevated levels of NMN and MN, 3,002 mg/24 h and 1,596 mg/24 h, respectively. Given the laboratory and radiologic findings, a diagnosis of cystic pheochromocytoma was made. After controlling blood pressure with the alpha-blocker, doxazosin, the patient was hydrated and scheduled for an elective adrenalectomy. The histopathology of the excised adrenal gland was consistent with a cystic pheochromocytoma. CONCLUSIONS Cystic pheochromocytoma is a very rare tumor that may present without symptoms. The clinical course of cystic pheochromocytoma is similar to that of solid pheochromocytoma. Early surgical intervention is recommended, following blood pressure control with an alpha-blocker, and adequate hydration.

Adrenal Metastasis from Uterine Papillary Serous Carcinoma.

BACKGROUND: Uterine papillary serous carcinoma (UPSC) is a highly malignant form of endometrial cancer with a high propensity for metastases and recurrences even when there is minimal or no myometrial invasion. It usually metastasizes to the pelvis, retroperitoneal lymph nodes, upper abdomen, and peritoneum. However, adrenal metastases from UPSC is extremely rare. Here, we present a case of UPSC with adrenal metastasis that occurred 6 years after the initial diagnosis. CASE REPORT: A 60-year-old woman previously diagnosed with uterine papillary serous carcinoma at an outside facility presented in September of 2006 with postmenopausal bleeding. She underwent comprehensive surgical staging with FIGO (International Federation of Gynecology and Obstetrics) stage 2. Post-operatively, the patient was treated with radiation and chemotherapy. The treatment was completed in April of 2007. The patient had no evidence of disease until July 2009 when she was found to have a mass highly suspicious for malignancy. Subsequently, she underwent right upper lobectomy. The morphology of the carcinoma was consistent with UPSC. She refused chemotherapy due to a previous history of chemotherapy-induced neuropathy. The patient was followed up with regular computed tomography (CT) scans. In October 2012 a new right adrenal nodule was seen on CT, which showed intense metabolic uptake on positron emission tomography (PET)/CT scan. The patient underwent right adrenalectomy. Pathology of the surgical specimen was consistent with UPSC. CONCLUSIONS: UPSC is an aggressive variant of endometrial cancer associated with high recurrence rate and poor prognoses. Long-term follow-up is needed because there is a possibility of late metastases, as in this case.

Breast Metastasis of Extraskeletal Myxoid Chondrosarcoma: A Case Report.

BACKGROUND: Extraskeletal myxoid chondrosarcoma is a unique and distinct clinicopathological entity in terms of its origin, morphology, and biologic behavior. Despite being a slow-growing tumor, it has a high rate of local recurrences and history of metastases to uncommon sites like the mandible, liver, retroperitoneum, right ventricle, pancreas, and central nervous system. Here, we report a very unique case of extraskeletal myxoid chondrosarcoma that metastasized to the breast, which itself is a very rare site for metastases. CASE REPORT: A 58-year-old woman presented with a large, firm, and tender soft-tissue mass (6.0×7.0 cm) underneath the sole of the left foot. A computerized tomography (CT) scan showed a heterogeneous lobulated mass in the plantar aspect of the forefoot, measuring 8.6×8.0×7.1 cm. Punch biopsies revealed histology consistent with extraskeletal myxoid chondrosarcoma. Metastatic work-up was negative. The mass was fully resected with left below-knee amputation. The histology of the resected mass was consistent with extraskeletal myxoid chondrosarcoma. A follow-up CT showed a new right breast nodule along with metastases to lung and bones. The results of the core needle biopsies of the right breast masses seen on mammogram were morphologically identical to extraskeletal myxoid chondrosarcoma. CONCLUSIONS: Although rare, metastases to the breast should be considered in the differential diagnosis of a breast mass. A close long-term follow-up is needed due to the unpredictable behavior of extraskeletal myxoid chondrosarcoma and the high frequency of local recurrences, metastases, and death due to disease.

Follicular Variant of Papillary Thyroid Cancer with Bilateral Renal Metastases Discovered Incidentally During Work-Up of Primary Endometrial Cancer: A Rare Occurrence.

BACKGROUND: Follicular variant of papillary thyroid cancer (FV-PTC) is the second most common subtype of papillary thyroid cancer (PTC) after classic PTC. FV-PTC is characterized by nuclear features consistent with classic PTC but has a follicular architecture that lacks classic papillary morphology. Thyroid cancer rarely metastasizes to the kidney. Only 6 cases of FV-PTC metastasizing to the kidney have been reported in the English literature. We are reporting a case of FV-PTC with bilateral renal metastases discovered incidentally during work-up of primary endometrial cancer. CASE REPORT: A 70-year-old woman presented with post-menopausal bleeding secondary to endometrial cancer. Staging work-up showed multiple bilateral lung nodules, bilateral soft tissue kidney masses, and multinodular goiter. The pathological and immnohistochemical profile of the lung biopsy was consistent with primary well-differentiated lung adenocarcinoma. Follow-up computerized tomography scan showed stable lung nodules and enlarging renal masses, which was suggestive of bilateral renal cancer. While the histologic features of the renal biopsy were not typical, the immunohistochemical staining of renal biopsy was positive for Paired box 8, thyroid transcription factor-1, thyroglobulin, and cytokeratin 7, suggesting the thyroid as the primary cancer site. The final histopathology on surgical specimen of total thyroidectomy revealed follicular variant of papillary thyroid cancer. CONCLUSIONS: The presence of pulmonary nodules and kidney masses does not always suggest the lung or the kidney as primary tumor sites. The clinician should be aware of the possibility of metastasis and look for the primary source, which in the present case was FV-PTC. Immunohistochemistry plays an important role in determining the primary site of origin. In case of multiple-organ metastases, each metastatic lesion should be biopsied as soon as possible for definitive diagnosis and appropriate treatment.

First Reported Case of Primary Intrahepatic Cholangiocarcinoma with Pure Squamous Cell Histology: A Case Report.

BACKGROUND: In the United States, approximately 2500 cases of cholangiocarcinoma occur each year. The average incidence is 1 case/100 000 persons each year. Surgical resection is the mainstay for the treatment of cholangiocarcinoma. The result of surgery depends on location of the tumor, extent of tumor penetration of the bile duct, tumor-free resection margins, and lymph node and distant metastases. There has been an increase in the incidence of intrahepatic cholangiocarcinoma (IHCC) globally over a period of 30 years from 0.32/100 000 to 0.85/100 000 persons each year. Epidemiologically, the incidence of IHCC has been increasing in the U.S. from year 1973 to 2010. CASE REPORT: We are reporting a first case of primary intrahepatic cholangiocarcinoma of pure squamous cell histology. A 64-year-old man presented with right upper-quadrant pain, jaundice, and weight loss. Imaging studies revealed a large hepatobiliary mass, intrahepatic bile duct dilation, normal common duct, and absence of choledocholithiasis. Delayed-contrast magnetic resonance imaging of the abdomen showed peripheral enhancement of the central lesion, which is typical of cholangiocarcinoma in contrast to hepatocellular carcinoma or metastasis. Cancer antigen 19-9 was markedly elevated. Liver function tests were deranged. Endoscopic retrograde cholangiopancreatography showed high degree of left hepatic duct stricture. Brush cytopathology was positive for atypia. The patient underwent exploratory laparotomy for en-bloc resection of the hepatobiliary mass with colon resection, liver resection, and cholecystectomy. Histology revealed keratinizing squamous cell carcinoma. Based on these findings, a definitive diagnosis of well-differentiated squamous cell carcinoma of the intrahepatic bile duct was made. CONCLUSIONS: Squamous cell carcinoma of the biliary tree is very rare and the majority of tumors are adenocarcinomas. Cholangiocarcinomas containing a squamous cell component have a poor prognosis due to its aggressive behavior. However, prognosis of cholangiocarcinoma with pure SCC histology is unknown because this is the first case in the literature.

Urinary bladder paraganglioma presenting as micturition-induced palpitations, dyspnea, and angina.

BACKGROUND: Sympathetic urinary bladder paragangliomas are rare catecholamine-secreting neuroendocrine tumors arising from neural crest cells. They are uncommon urinary bladder neoplasms. Symptoms classically include micturition-related or unrelated palpitations and syncope with hypertension, headaches, diaphoresis, and hematuria. Other than being attributable to vasovagal reactions, micturition-induced cardiovascular symptoms should prompt a search for catecholamine-secreting tumors such as a urinary bladder paraganglioma, as in this case. CASE REPORT: A 45-year-old asthmatic African-American female presented with episodic hematuria that began 4 years ago and episodes of micturition-induced palpitations, dyspnea, substernal tightness, sweating, and throbbing headaches. Computed tomography with contrast revealed an enhancing mass along the anterior urinary bladder wall, measuring 2.4×3.5 cm. On Positron emission Tomography with [18F] fluorodeoxyglucose integrated with computed tomography (18F-FDG PET/CT), the urinary bladder mass was 18F-FDG avid. Serum normetanephrine and supine plasma norepinephrine were significantly elevated and there was mild elevation of supine plasma epinephrine. Transurethral resection of the bladder mass revealed a neoplasm with microscopic features and immunohistochemical profile positive for synaptophysin and chromogranin, with negative screening cytokeratin AE1/AE3, suggesting a paraganglioma. Following resection of the paraganglioma, there was complete resolution of micturition-induced cardiovascular symptoms on long-term follow-up. CONCLUSIONS: Micturition-related cardiovascular symptoms are commonly attributed to vasovagal reactions. However, urinary bladder pathologies must be ruled out as a cause, as in this rare case of a urinary bladder paraganglioma exhibiting catecholaminergic symptoms.

Postchemotherapy histiocyte-rich pseudotumor involving the spleen.

We report 2 cases of splenic postchemotherapy histiocyte-rich pseudotumor. Each patient had a history of diffuse large B-cell lymphoma, treated with multiagent chemotherapy. Computed tomography scans performed on both patients showed splenic masses. A positron emission tomography scan performed on 1 patient showed increased metabolic activity. The preoperative diagnosis in both patients was recurrent lymphoma, prompting splenectomy. The splenectomy specimens showed multiple, tan-white, firm nodules, up to 3.5 cm in diameter, that were histologically composed of central necrotic B cells (CD20+/CD3-), consistent with necrotic lymphoma, surrounded by numerous lipid-laden (xanthomatous) histiocytes. Clinical staging studies at the time of splenectomy showed no other sites of disease. We conclude that these histologic and immunophenotypic findings represent chemotherapy-induced tumor necrosis with a florid histiocytic reaction mimicking residual viable lymphoma. Others have used descriptive terminology or the term xanthomatous pseudotumor for these lesions that have been only rarely reported in the spleen previously.