Rare epithelioid hemangioendothelioma in the brachiocephalic vein for long-term survival after surgery: A case report.

Epithelioid hemangioendothelioma (EHE) is a rare vascular tumor. In this report, we describe the case of a 62-year-old man who presented with pain in the left clavicle and swelling of the left upper limb. Contrast-enhanced computed tomography revealed an intravascular tumor, which was completely resected surgically. Histopathological examination and immunohistochemical staining revealed that it was epithelioid hemangioendothelioma with occurrence in the left brachiocephalic vein. It has been 6 years since the surgery was performed, and no recurrence has been observed. Epithelioid hemangioendothelioma may recur or metastasize and therefore requires careful follow-up.

A unique case of splenic tumor exhibiting a serous carcinoma phenotype.

The spleen has no epithelial element; thus, primary carcinoma of the spleen is quite rare. We present the case of a patient with serous carcinoma of the spleen. A 76-year-old woman with no significant medical history presented with a huge lesion in the spleen. Except this lesion, clinical examination, including imaging examination, revealed no remarkable findings. She underwent excision of the spleen for treatment and diagnosis. Postoperative pathological examination revealed neoplastic cells with pleomorphic and hyperchromatic nuclei, prominent nucleoli, and frequent mitotic activity. The neoplastic cells exhibited a papillary pattern with psammoma bodies. Immunohistochemistry showed positivity for cytokeratin 7, PAX-8, WT-1, p16, p53, and Ber-EP4 and negativity for cytokeratin 20, thyroid transcription factor-1, carcinoembryonic antigen, CD10, estrogen receptor, calretinin, D2-40, intelectin-1, and sialylated HEG1. We inferred that this tumor was a primary splenic serous carcinoma. Serous tubal intraepithelial carcinoma is the plausible origin of most pelvic serous carcinomas. However, the origin of serous carcinoma of the spleen remains unknown. We speculated that endosalpingiosis might be the origin of the tumor.

Natural history of inferior mesenteric arteriovenous malformation that led to ischemic colitis: A case report.

BACKGROUND: Ischemic colitis with inferior mesenteric arteriovenous malformation (AVM) is a rare disease. Although a few reports have been published, no report has described the natural history of idiopathic mesenteric AVM. CASE SUMMARY: A 50-year-old male was admitted to our hospital due to abdominal pain that had persisted for 3 mo and bloody diarrhea. He had no history of trauma or abdominal surgery. He had undergone two colonoscopies 6 mo and 2 years ago, and they showed only a polyp. He was diagnosed with ischemic colitis with inferior mesenteric AVM following contrast-enhanced abdominal computed tomography (CT) and underwent rectal low anterior resection. He has not had a recurrence of symptoms for 3 years. His history showed that he had undergone non-enhanced abdominal CT 2, 5, and 8 years ago when he had attacks of urinary stones. Retrospectively, dilation of blood vessels around the rectosigmoid colon could have been detected 5 years ago, and these findings gradually became more evident. CONCLUSION: This is the first report of the natural history of inferior mesenteric AVM.

Madelung's disease: long-term follow-up.

Madelung's disease (MD) is a rare lipid metabolic disorder of adipose tissue overgrowth, which has been reported to be related to alcohol abuse. Although it does not affect survival itself, alcoholism and metabolic disorders associated with MD can be life-threatening. Although surgical procedures and classifications have been reported, long-term follow-up has rarely been reported. Here, we report a 61-year-old Japanese man with MD who has been followed-up for 12 years. Lipectomy was performed three times over the first 3 years and a total of 4 kg of adipose tissue was excised. Relapse has not been observed. He was diagnosed with multiple hepatocellular carcinomas (MHC) and placed on dialysis due to hepatorenal syndrome caused by alcoholism. Moreover, one of the MHC ruptured resulting in hemorrhagic shock. The cause of death in MD is not fat proliferation but comorbidities. Long-term observation and multidisciplinary systemic management are necessary for MD patients.

Intertendinous epidermoid cyst of the forearm.

As epidermoid cysts generally originate from hair follicle infundibulum, they appear as intradermal or subcutaneous tumors and are very rare in locations away from the skin. Here, we report a rare case of intertendinous epidermoid cyst of the forearm in a 68-year-old man that was treated surgically.

Successful Treatment with Cyclosporin A in Tocilizumab-resistant TAFRO Syndrome.

Thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly (TAFRO) syndrome is a unique clinicopathologic variant of multicentric Castleman's disease that has recently been identified in Japan. Previous reports have shown that affected patients typically respond to immunosuppressive therapy, such as prednisolone and tocilizumab. However, the optimal treatment for refractory TAFRO syndrome, which can be fatal, remains unclear. We herein report a case of tocilizumab-resistant TAFRO syndrome successfully treated with cyclosporin A, indicating that cyclosporine A may be an alternative therapy for refractory TAFRO syndrome.

Fatal fat embolism syndrome in a case of isolated L1 vertebral fracture-dislocation.

Although fat embolism syndrome is a well-known complication of fractures of the long bones or pelvis, fat embolism syndrome occurring subsequent to fracture of the lumbar spine is rare. We report a fatal case of fat embolism syndrome characterized by fat and bone marrow embolism that occurred 36 h after an isolated fracture-dislocation of the L1 vertebra. A postmortem examination was performed and pathological finding demonstrated fat and bone marrow tissue which were disseminated in the bilateral pulmonary arteries. We need to be aware of the possibility of fat embolism syndrome as a complication of spinal fractures, including isolated vertebral body fractures.

Giant cell phlebitis: a potentially lethal clinical entity.

An 83-year-old woman presented to us with a 4-week history of general malaise, subjective fever and lower abdominal pain. Despite the intravenous infusion of antibiotics, her blood results and physical condition worsened, resulting in her sudden death. Autopsy study revealed that the medium-sized veins of the mesentery were infiltrated by eosinophil granulocytes, lymphocytes, macrophages and multinucleated giant cells; however, the arteries were not involved. Microscopically, venous giant cell infiltration was observed in the gastrointestinal tract, bladder, retroperitoneal tissues and myocardium. The final diagnosis was giant cell phlebitis, a rare disease of unknown aetiology. This case demonstrates for the first time that giant cell phlebitis involving extra-abdominal organs, including hearts, can cause serious morbidity.

Pelvic tumors with normal-appearing shapes of ovaries and uterus presenting as an emergency (Review).

Abdominal pain with an associated pelvic mass is a common problem in everyday practice. Concerns about ectopic pregnancy, torsion of an enlarged ovary or malignancy usually dominate the diagnostic evaluation. On physical and imaging examination, when a palpable painful mass is present in the pelvis and the two ovaries and uterus are detected in their normal anatomical locations, the content and origin of the lesions may be significant in narrowing the pre-operative differential diagnosis. Thus, the emergent pelvic indications discussed in this review should be considered. The causes of acute abdominal pain are few in number and therefore an accurate diagnosis may be most frequently made at the time of exploratory laparotomy.

Transitional cell carcinoma of the ovary (Review).

Transitional cell carcinoma (TCC) of the ovary is a rare recently recognized subtype of ovarian epithelial cancer. Ovarian TCC has a modest response to chemotherapy, and metastatic TCC from the renal pelvis results in mortality. The clinical presentation is indistinguishable from other types of ovarian carcinoma. Histopathological examination remains the first tool used in the diagnosis of these heterogeneous tumors and in the separation of closely related tumors. Since it is generally accepted that surgical resection is the primary therapeutic approach, and patient outcomes following chemotherapy are better than for other types of ovarian cancers, it is a reasonable concept to detect tumors when they are still confined within the ovaries. Thus, the aim of this review was to describe typical cases of primary TCC, and to review the medical literature for information on TCC management in order to determine appropriate diagnostic methods and therapy.

Early diagnosis of malignant-transformed ovarian mature cystic teratoma: fat-suppressed MRI findings.

The most common form of malignant transformation developing from a mature cystic teratoma is squamous cell carcinoma, representing 80% of malignant transformations, while adenocarcinoma accounts for approximately 5%. Because of this rarity, few reports exist of preoperative diagnosis of this tumor by magnetic resonance imaging, in particular with fat suppression techniques. Here, we report magnetic resonance imaging findings and clinical features of a 79-year-old woman with mucinous adenocarcinoma arising from a mature cystic teratoma (measuring 5×6 cm), classified as surgical stage IA. Because of the poor prognosis of malignant transformation, when mature cystic teratomas are detected (even smaller than 5 cm tumor size) in postmenopausal women, serum tumor marker carcinoembryonic antigen levels and fat-suppressed magnetic resonance imaging may be potential indicators of malignant transformation.

[A case of bronchocentric granulomatosis associated with Aspergillus clusters that was difficult to distinguish from lung cancer].

A 69-year-old man was examined for investigation of a nodular shadow with calcification detected on computed tomography of the chest. He showed no features of allergic bronchopulmonary aspergillosis (ABPA), such as bronchial asthma or eosinophilia. We could not distinguish his disease status from lung cancer and performed upper lobectomy of his right lung. Pathological examination revealed bronchocentric granulomatosis (BCG) with Aspergillus clusters. Subsequent serological testing demonstrated high levels of IgE-RIST and IgE-RAST (Aspergillus) whereas anti-Aspergillus precipitating antibody was not detectable. We believe that Aspergillus clusters may have been present in this patient for several years, and the pathological findings might be manifested as an asymptomatic localized allergic inflammation. BCG is a pathological component of ABPA. This case, of a localized allergic reaction, is considered very valuable for understanding the underlying mechanism of the onset of ABPA.

[A case of pulmonary Mycobacterium abscessus infection with asbestosis in a patient given multidisciplinary treatment].

A 68-year-old man consulted our hospital because of a giant cavity in the upper lobe of the right lung, which was detected in a medical examination for asbestosis. Mycobacterium abscessus was cultured from his sputum in 2 separate cultures. Therefore we diagnosed pulmonary Mycobacterium abscessus infection and prescribed amikacin, imipenem/cilastatin, and clarithromycin. After 2 months, lobectomy of the upper lobe of the right lung was performed. The 3 antibiotics were continued for another 6 months, and the patient recovered completely. Mycobacterium abscessus infection is one of the most intractable non-tuberculous mycobacterial infections, and it is important to include surgery in the primary treatment plan.

Muscle hematoma: a critically important complication of alcoholic liver cirrhosis.

An iliopsoas hematoma can occur either spontaneously or secondary to trauma or bleeding tendency due to hemophilia and anticoagulant therapy. Although liver cirrhosis is commonly associated with coagulopathy, iliopsoas hematoma is very rare. We herein, present a case of bilateral iliopsoas hematoma in a patient with alcoholic cirrhosis, and review the literature on muscle hematoma associated with cirrhosis. A 56-year-old man with alcoholic cirrhosis was admitted in a state of shock with anemia. The cause of anemia could not be detected, and the patient was treated conservatively. The site of bleeding was not detected with either gastroduodenal endoscopy or upper abdominal computed tomography, the latter of which did not include the iliopsoas muscle. He died on the 10th day of admission and bilateral iliopsoas hematomas were found on autopsy. An iron stain was positive in the iliopsoas muscle. Eight cases of muscle hematoma associated with cirrhosis, including the present case, were found in a review of the literature. Four of these cases involved the rectus abdominis muscle, 3 involved the iliopsoas muscle and 1 involved combined muscles. Alcoholic cirrhosis accounted for 75% of the cases. One case (12.5%) was associated with virus-related cirrhosis, and another with combined virus- and alcohol-related cirrhosis. The mortality rate was 75% despite early diagnosis and low risk scores for cirrhosis. Muscle hematoma in patients with cirrhosis is closely related to alcoholism, and the mortality rate of the condition is extremely high. In conclusion, muscle hematoma should be recognized as an important complication of cirrhosis.

Coincidence of mammary and sentinel lymph node papilloma.

This report describes an unusual case of mammary intraductal papillomas coexistent with sentinel lymph node papilloma. A 47-year-old Japanese female underwent 5 needle manipulations and 2 surgical biopsies for recurring papillomas in the right breast over 5 years before having a simple mastectomy. During the mastectomy, the ipsilateral sentinel node was found to be extensively occupied by completely benign papilloma that measured 6 mm in its greatest dimension. The clinical history led us to put forward the working hypothesis that the nodal papillary lesion may develop from the epithelial cells that are displaced from the mammary papillomas during needle procedures and mechanically transported to the sentinel lymph node. To test the hypothesis, we retrieved surgical biopsies (dochectomy and excisional biopsy), mastectomy, and sentinel lymph node specimens for histopathologic, immunohistochemical, and molecular studies. The presence of myoepithelial layer in each papillary tumor was confirmed by immunostains with specific myoepithelial markers, p63 and CD10. The excisional biopsy specimen exhibited displaced fragments of benign epithelial cells within granulation tissue at the needle manipulation site, indicating that iatrogenic epithelial cell displacement did occur in this case. However, loss of heterozygosity at 16p13 and 16q21 was only observed in the papillomas of the dochectomy and the excisional biopsy; no loss of heterozygosity was detected in the papillomas of the mastectomy and the sentinel lymph node. It remains undetermined whether the nodal papilloma was derived from the papilloma of the mastectomy or if it arose de novo from the breast tissue inclusion of the sentinel node.

Segmental arterial mediolysis: unrecognized cases culled from cases of ruptured aneurysm of abdominal visceral arteries reported in the Japanese literature.

Segmental arterial mediolysis (SAM) is a rare nonatherosclerotic and noninflammatory arteriopathy that was proposed by Slavin et al. [Segmental mediolytic arteritis. A clinical pathologic study, Lab. Invest. 35 (1976) 23-29]. It mainly involves abdominal visceral arteries and is characterized by lytic degeneration of the media, resulting in intraabdominal bleeding. We collected 27 unrecognized cases of SAM by reviewing microscopic slides of cases of ruptured aneurysms of visceral arteries, except splenic and hepatic aneurysms, reported in the Japanese literature. This paper describes the pathological and clinical features of these cases. The symptom at onset was abdominal pain associated with intraabdominal bleeding in all cases. The most involved artery was the middle colic artery, accounting for 14 (50%), followed by gastric and gastroepiploic arteries, (6 and 5, respectively). Seventy-eight percent of aneurysms were of dissecting type and the rest of pseudoaneurysm type, except for one. Multiple aneurysms were found in 9 cases (33.3%). Pathological lesions were acute in all. The outcome of those who had surgery was good, even in those who had surgery for 1 ruptured aneurysm, leaving the others unmanaged. The relationship of SAM to fibromuscular dysplasia is discussed. Secondary changes in the wall of the accompanying vein to the affected artery are briefly described. It is emphasized that the majority of aneurysms of abdominal visceral arteries are gathered together as SAM as a definite clinical and pathological entity.

Longitudinal study of patients with idiopathic isolated TSH deficiency: possible progression of pituitary dysfunction in lymphocytic adenohypophysitis.

The relationship between isolated TSH deficiency and hypophysitis was studied. Six patients (five women and one man) with idiopathic isolated TSH deficiency were longitudinally investigated with an interval of 31 to 60 months. Clinical symptoms, laboratory results and endocrine function were investigated as well as pituitary magnetic resonance imaging (MRI) at the start and the end of the study. Clinically, initial symptoms due to hypothyroidism were ameliorated by the thyroid hormone replacement in all patients. Oligomenorrhea newly appeared during the study in three patients, although no other symptoms appeared. Serum fT3 and fT4 levels were within the reference ranges, and serum TSH level and its response to TRH stimulation remained low in all patients. Peak plasma GH level during GRH stimulation was significantly (p<0.03) decreased, at the end of the study as compared with the start. Peak plasma FSH level to LHRH stimulation was significantly (p<0.03) decreased as well as basal FSH level. In contrast, peak of prolactin during TRH stimulation was significantly (p<0.03) increased at the end of the study as compared with the start as well as basal prolactin level. Endocrine features at the end of the study were compatible with those of lymphocytic adenohypophysitis (LAH). MRI of the pituitary gland showed empty sella in one patient and slight swelling in two patients. These findings remained unchanged during the study period. One patient underwent pituitary biopsy, with histological examination showing atypical form of LAH. LAH can cause idiopathic isolated TSH deficiency and can functionally progress to combine dysfunction of the pituitary gland.

Hypopituitarism and diabetes insipidus with localized hypertrophic pachymeningitis (Tolosa-Hunt syndrome) associated with Hashimoto thyroiditis.

We report a 69-year-old woman with intracranial pachymeningitis showing hypopituitarism, diabetes insipidus, and Tolosa-Hunt syndrome associated with Hashimoto thyroiditis confirmed by autopsy. A large tumorous lesion of the hypothalamo-pituitary gland was revealed on magnetic resonance imaging, after the patient complained of gait and visual field disturbance. These symptoms subsided after thyroid hormone supplementation. Hypopituitarism and diabetes insipidus were diagnosed after cessation of the treatment by the patient herself. Multiple cranial nerve palsies and orbito-frontalgia appeared. Methylprednisolone pulse therapy improved the symptoms, but they recurred when the dose of glucocorticoid was decreased. The patient died of brain thrombosis. Autopsy revealed typical findings of Hashimoto thyroiditis and intracranial pachymeningitis involving the cranial base and pituitary gland. The high titer of rheumatoid factor and Hashimoto thyroiditis in this patient suggest an immunological role in the pathogenesis of pachymeningitis.

Variant of pre-clinical Cushing's syndrome: hypertension and hypokalemia associated with normoreninemic normoaldosteronism.

The case of a 48-year-old woman with a left adrenocortical adenoma and showing hypokalemia, hypertension and normoreninemic normoaldosteronism is reported. Basal plasma adrenocorticotrophic hormone (ACTH) and cortisol levels were within the reference ranges. The patient's plasma cortisol level decreased insufficiently at night, and was insufficiently decreased by nighttime administration of dexamethasone. She showed no Cushingnoid stigmata. Iodocholesterol scintigraphy revealed tumor-sided uptake alone. The plasma dehydroepiandrosterone-sulfate level was low-to-normal for her age. Metabolic alkalosis and increased potassium clearance after sodium thiosulfate loading were revealed. The plasma aldosterone level was within the normal range, but it was statistically higher than the range for patients with pre-clinical Cushing's syndrome. However, peripheral plasma renin activity (PRA) increased normally after the patient resumed an upright posture following furosemide administration. After adenomectomy the hypokalemia and hypertension were resolved, and the plasma ACTH, cortisol, and PRA remained within the reference ranges. The plasma aldosterone level decreased slightly, but also remained within the reference range after adenomectomy. Paradoxical hyperplasia in the non-neoplastic adrenal glomerulosa zone, which indicates primary aldosteronism, and slight atrophy of the non-neoplastic adrenal cortex, which indicates pre-clinical Cushing's syndrome, were demonstrated. These findings satisfied the criteria of pre-clinical Cushing's syndrome, but did not completely satisfy those of primary aldosteronism. However, the level of CYP11 B2 mRNA in the tumor was in the lower-limit of the range for adenomas associated with primary aldosteronism and was higher than the ranges for adenomas associated with pre-clinical Cushing's syndrome and overt Cushing's syndrome. Based on these results, this case was suspected to constitute a variant of pre-clinical Cushing's syndrome with slight hypersecretion of aldosterone.