A case of Lymphoepithelioma-like carcinoma (LELC) developed in the rectum

Lymphoepithelioma-like carcinoma (LELC) is histologically similar in form to nasopharyngeal carcinoma (NPC) and is an epithelial tumor that is suggested to be involved in infection with Epstein-Barr virus (EBV), but it is rare to occur in the colon. A 35-year-old woman was found to have a rectal wall thickening by follow-up computed tomography (CT) image after sigmoidectomy and left salpingo-oophorectomy. Biopsy under colonoscopy revealed recurrence of ovarian cancer, and she underwent a low anterior resection. Pathological diagnosis was LELC. Although LELC of the stomach has been reported to have a high EBV infection rate as NPC, EBV infection was not detected in our case. Pelvic lymph node dissection was also performed, and metastasis was recognized around the iliac artery. There have been few reports of LELC occurring in the rectum, and there are no reports of distant metastasis only to the pelvic lymph node. We consider it a very valuable case, and report it with literature references.     

Primary pulmonary leiomyosarcoma. Report of a case diagnosed by fine needle aspiration cytology

BACKGROUND: Primary pulmonary leiomyosarcoma is a rare but important entity. We report a case diagnosed by fine needle aspiration cytology. CASE: A 73-year-old male presented with an asymptomatic, right, pulmonary, subpleural nodule detected by computed tomography during follow-up for chronic obstructive pulmonary disease. Fine needle aspiration cytology showed cellular smears with numerous single or loosely cohesive groups of spindle-shaped to round cells. The tumor cell nuclei were blunt ended (cigar shaped), with fine to fine-granular chromatin, prominent nucleoli and an irregular nuclear rim. The tumor cells were positive for desmin and negative for cytokeratin and S-100 protein by immunocytochemistry. Right upper lobectomy with lymph node dissection was performed. Pathologic diagnosis after microscopic, immunohistochemical and electron microscopic studies was leiomyosarcoma. CONCLUSION: To our knowledge, this is the first reported case of primary pulmonary leiomyosarcoma arising in the subpleural region diagnosed by fine needle aspiration cytology. Immunocytochemistry was useful in establishing the diagnosis in this case.     

Clinically inapparent adrenal mass in a patient with familial adenomatous polyposis

Familial adenomatous polyposis (FAP) is an autosomal dominant condition characterized by multiple colorectal adenomas that can progress to carcinoma. FAP can be associated with diverse extracolonic manifestation, including desmoid tumors and adrenal masses. We report our experience with a patient diagnosed of FAP, who developed a desmoid tumor and an adrenal mass in the follow-up. To our knowledge, this is the first case in the literature in which a hypersecretion of aldosterone and cortisol in the adrenal mass of a patient diagnosed of FAP has been demonstrated.     

Ectopic ACTH syndrome due to Grawitz tumor

An extremely unusual case of adrenocorticotropin (ACTH)-producing Grawitz tumor is reported in a 56-year-old female. The clinical feature of the patient was compatible with Cushing's syndrome. The plasma levels of ACTH and cortisol were markedly elevated. At autopsy, a left renal tumor was demonstrated and its histopathological diagnosis was renal cell carcinoma (Grawitz tumor). The adrenal gland was bilaterally enlarged with diffuse hyperplasia of the fasciculate zone. The adenohypophyseal cells were atrophic and showed Crooke's degeneration. The tumor contained extremely high levels of ACTH, beta-lipotropin and beta-endorphin. The presence of large molecular weight forms of ACTH has also been demonstrated by a Sephadex G-50 gel filtration of the tumor extract. We authors believe that this is the first documented case of ACTH-producing Grawitz tumor in the literature.     

Lymphatic mapping for Merkel cell carcinoma

Merkel cell trabecular carcinoma of the skin has a prognosis poorer than expected for a small skin lesion. An early diagnosis and prompt treatment can contribute to improve survival in cases of this aggressive skin tumor. A wide local excision is indicated for localized disease. Elective lymph node dissection is controversial. The authors report a rare case of Merkel cell tumor treated with wide local excision and sentinel lymph node biopsy.     

Aggressive multifocal form of epithelioid hemangioendothelioma--case report

Epithelioid hemangioendothelioma (EHE) is a rare tumor of the vascular origin. It was first described in its pulmonary form by Dail and Leibow in 1975 and named "intravascular bronchioalveolar tumor" (IVBAT). Since then, reports of occurrences of the tumor have been made for number of locations, but most often tumor can be found in soft tissues, liver, lungs, bone and skin. It is considered to be a low or borderline malignant tumor with, usually, slow progression, but aggressive forms have been described. We here report a case of a 46-year old female patient with multifocal malignant tumor spreading to lungs, liver, spleen and with synchronous involvement of lumbal vertebrae, illiac bones and central nervous system dissemination. To the best of the authors knowledge, no case of malignant EHE with multiorgan involvement of this proportions and synchronous central nervous system and bone involvement in one patient has been reported to this date in English-speaking literature.     

Electromyographic assessment of rectus abdominis muscle function after deep inferior epigastric perforator flap surgery

SUMMARY: The authors evaluated rectus abdominis muscle function after deep inferior epigastric perforator (DIEP) flap elevation. Fifteen consecutive patients who were operated on for breast reconstruction with a free DIEP flap were included in the study. A turn-amplitude electromyographic analysis was used. For each patient, the muscle activity was recorded in the portion of the muscle that was split for the epigastric perforator vessel dissection, and also in the similar portion of the contralateral nondissected muscle. A first electromyographic examination was carried out soon after surgery (mean follow-up, 9 weeks), and a second electromyographic examination was carried out at a later date (mean follow-up, 15 months). The mean activity of the dissected muscles was 50 percent of the activity of the nondissected muscles at the first electromyographic examination and 70 percent at the second electromyographic examination. The authors suggest that the DIEP flap procedure induces a partial denervation of the rectus abdominis muscle in the area of dissection and that reinnervation occurs over time because the entire width of the muscle and sufficient segmental motor innervation are preserved.     

A late recurring and easily forgotten tumor: ovarian granulosa cell tumor

ABSTRACT: Ovarian granulosa cell tumor (GCT) is a malignant tumor with slow progression. The recurrence of granulosa cell tumor often happens after 5 years, leading to a 'forgotten tumor' by the patient. We present the case of a 64-year-old woman with a presentation of left flank pain. An initial computed tomography scan revealed a single tumor with multiple adjacent organ invasions. Surgical intervention was prescribed and the pathological results revealed a metastatic granulosa cell tumor. We also review the literature for the follow-up and further management of this tumor.     

Interpretation of the post-surgical Somatostatin Receptor Scintigram of a Primary Neuroendocrine Tumor of the Thymus: a case report and literature review

A case of a thymic neuroendocrine tumor and the interpretation problems in a post-surgical Somatostatin Receptor Scintigraphy are presented. In a 53-year-old man with superior vena cava obstruction syndrome an atypical carcinoid of the thymus (neuroendocrine carcinoma of intermediate grade 2), was found at surgery.During his first year of follow-up a Somatostatin Receptor Scintigraphy was recommended. An area of abnormal concentration of the radiopharmaceutical was revealed in the mediastinum at this time.A thorough understanding of the mechanisms of the radiopharmaceutical uptake and of the various clinical settings in which uptake can occur are essential for a proper evaluation of the scintigraphic findings and result in the optimal use of this valuable modality.The literature review provides an overview of this rare type of tumor and insight into the clinical significance of Somatostatin Receptor Scintigraphy.     

The clinical importance of methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism in the 5-fluoropyrimidine-based therapy of metastatic colorectal tumours

The authors investigated the methylenetetrahydrofolate reductase (MTHFR) C677T polymorphism in 101 metastatic colorectal cancer patients treated with 5-fluoropyrimidine-based therapy and in 196 healthy individuals by PCR-RFLP method. There was no significant difference in genotype distribution of patients and healthy controls, and between subgroups investigated according to clinical parameters (age, gender, tumor location, grade and treatment type). However, after a 3-30 (median 18.5) months follow-up the survival of patients with T allele proved to be better than that of patients with wild type (CC) genotype (p=0.036). In case of CT and TT genotypes the survival of patients receiving only first line therapy was significantly shorter than that of patients receiving more lines of treatment (p=0.015). Determination of MTHFR C677T polymorphism has prognostic value in case of patients with metastatic colorectal cancer receiving 5-fluoropyrimidine-based therapy, and may help in designing the individual (group) tailored therapy.     

Metastatic Struma Ovarii Treated With Total Thyroidectomy and Radioiodine Ablation

ObjectiveTo present a rare case of metastatic struma ovarii, review the related literature, and discuss the management.MethodsA case report of a patient with metastatic struma ovarii is presented. The treatment plan, postoperative care, and follow-up are discussed. We conducted a MEDLINE search of the English-language literature seeking additional cases of metastatic struma ovarii. In addition to the current case, the previous 40 cases of metastatic struma ovarii were reviewed and analyzed.ResultsThe mean age of the patients at presentation was 43 years. All patients underwent resection of the primary tumor. The most common sites of involvement for struma ovarii metastatic disease have been the peritoneum, mesentery, and omentum. After primary tumor resection, a wide range of additional treatments have been used, including chemotherapy, resection of metastatic disease, external beam radiation therapy, and radioiodine ablation.ConclusionIn cases of metastatic struma ovarii, we recommend total thyroidectomy in conjunction with radioiodine scanning and radioiodine ablation. Thyroglobulin levels should be followed as a tumor marker, and diagnostic radioiodine scans should be performed to screen for residual or recurrent disease. Although this treatment strategy is well established for thyroid cancer, long-term outcomes of this treatment for struma ovarii are still unknown. (Endocr Pract. 2009;15:167-173)     

Large Adrenal Oncocytoma with Uncertain Malignant Potential: Case Report and Review of Literature

ObjectiveTo report a case of oncocytoma, a relatively rare adrenal tumor, which most commonly is detected as an adrenal incidentaloma.MethodsWe present a case report, including laboratory, imaging, and pathologic findings, of a 47-year-old obese woman who had hypertension and an incidentally found large, left adrenal mass.ResultsOn the basis of the hormonal evaluation, this mass was a nonsecreting adrenal tumor, which histologically proved to be an oncocytoma with borderline malignant characteristics. A collective analysis of the few cases of adrenal oncocytoma published in the medical literature showed that our case corresponded to the previously published cases in preponderant location (left side) as well as the general size (11.4 cm in the largest dimension) and weight (372 g).ConclusionAdrenal oncocytoma should be included in the differential diagnosis of adrenal incidentalomas, especially if large tumors are detected. In addition, a longterm follow-up is suggested because there are no certain clues about the true potential of this tumor. (Endocr Pract. 2010;16:641-645)     

Unusual Case of Metastatic Neuroendocrine Tumor

ObjectiveTo report a rare case of metastatic growth hormone (GH)-secreting pituitary carcinoma causing acromegaly.MethodsWe present a case report and review the available literature on this topic.ResultsA 68-year-old woman presented with persistent acromegaly after treatment for a GH-secreting pituitary adenoma. Evaluation of long-standing cervical adenopathy revealed findings consistent with a metastatic neuroendocrine tumor. Further work-up revealed additional thyroid, parathyroid, and cervical masses. After operative treatment including total thyroidectomy, subtotal parathyroidectomy, partial thymectomy, and right modified radical neck dissection, the patient’s symptoms diminished, and her GH levels approached the normal range. Surgical pathology findings were consistent with a GH-secreting pituitary carcinoma metastatic to the cervical lymph nodes, multinodular thyroid hyperplasia with a focus of papillary microcarcinoma, and parathyroid hyperplasia.ConclusionOverall, pituitary carcinomas are extremely rare. To date, about 100 cases have been reported in the world’s literature, and of these, only 19 cases originated from GH-secreting cells. Our examination of the symptoms, signs, diagnosis, and treatment of our patient, in comparison with the previously reported cases, should enhance awareness of this unusual disease process. (Endocr Pract. 2007;13:72-76)     

Computed tomography-guided fine needle aspiration biopsy of adrenal myelolipoma. Case report and review of the literature

A case of adrenal myelolipoma diagnosed by fine needle aspiration (FNA) biopsy is reported. The FNA smears contained the diagnostic picture of mature adipose tissue intermixed with hematopoietic elements, including megakarycytes. The FNA diagnosis was histologically confirmed by study of the resected tumor. This case appears to be the first "giant" adrenal myelolipoma diagnosed by computed tomography-guided FNA biopsy. The literature on adrenal myelolipomas sampled by FNA using imaging guidance is briefly reviewed.     

Pulmonary blastomycosis masquerading as metastatic disease in the lung: a case report

We report a case of pulmonary blastomycosis appearing as metastatic laryngeal squamous cell carcinoma. Pulmonary blastomycosis was discovered as right lower lobe subpleural activity consistent with metastatic disease on a positron emission tomographic (PET) scan following total laryngectomy and bilateral neck dissection for locally invasive laryngeal squamous cell carcinoma. A computed tomographic (CT) scan of the chest showed a right lower lobe, subpleural pulmonary nodule. CT-guided fine-needle aspiration of the nodule revealed broad-based budding yeast consistent with blastomycosis. To our knowledge, this is the first case of a PET-positive pulmonary blastomycosis lesion mimicking pulmonary malignancy reported in the literature.     

Ultrasound absorption and entropy production in biological tissue: a novel approach to anticancer therapy

Leiomyosarcoma is a malignant mesenchymal tumor originating from smooth muscle cells, which most frequently develops in the myometrium and in the gastro-intestinal tract. Reviewing the international literature, radiation-induced sarcoma arise in 0.035 to 0.2 % of all irradiated patients. Especially in the head and neck region, radiation-induced leiomyosarcoma is an extremely rare lesion. The authors report a case of a radiation-induced leiomyosarcoma of the tonsillar region of the oropharynx in a 51-year-old male patient, who had undergone radiation therapy of this region 38 years before. The lesion was treated by radical surgery. Diagnostic steps, histological presentation and therapy are described in detail and the literature concerning radiation induced malignancies in general as well as radiation induced leiomyosarcoma in particular is reviewed. The highlights of this case are an extremely uncommon location and a rare pathological entity of radiation induced malignancies.     

Congenital asplenia and group B streptococcus sepsis in the adult: case report and review of the literature

Asplenia is associated with an increased incidence of fatal and life-threatening sepsis caused by encapsulated pathogens. Isolated congenital asplenia is a very rare condition, with only 33 cases reported in the literature. The authors report another case of this condition complicated by overwhelming Group B streptococcus sepsis secondary to paronychia that was managed successfully.     

肾嗜酸性细胞瘤的诊治体会（附11例报告）

目的：探讨肾嗜酸细胞瘤的诊断和治疗。方法：回顾分析我院2003．2009年间收治11例肾嗜酸细胞瘤的临床资料,结合文献对肾嗜酸细胞瘤的诊断及治疗进行复习讨论。结果：本组11例患者中,年龄为26—75岁,男性7例,女性4例。合并透明细胞癌者1例,术前误诊为肾上腺肿瘤者1例。7例行根治性肾切除术者,4例行肾部分切除术。术后随访11个月-7年未见肿瘤转移或复发。结论：肾嗜酸细胞瘤倾向于良性肾实质性肿瘤,临床表现无特异性,确诊需临床表现、影像学检查与病理学检查相结合。治疗首选保肾手术,但应注意并发恶性肿瘤的可能,加强随访。     

Heart failure induced by pheochromocytoma: laparoscopic treatment and intraoperative changes of several new cardiovascular hormones

Since 1992, adrenalectomy for pheochromocytoma has been recognized as a safe and efficient technique when performed by a laparoscopic approach. Most of the cases of pheochromocytomas treated as such and published in the literature were not associated with malignant hypertension and acute heart failure. We report the case of a 23-year-old woman who presented with this clinical picture and show that laparoscopic adrenalectomy may be as safe and efficient as conventional adrenalectomy when performed in this situation. The intraoperative changes in the secretion of catecholamines, endothelin-1, angiotensin II, N- and C-terminus of atrial natriuretic factor prohormone were also analyzed. Noradrenaline release during tumor dissection was associated with a stimulation of atrial natriuretic factor.     

Imaging of left main trauma during diagnostic cardiac catheterization with intravascular ultrasound

In this case report the occurrence of a catheter-induced coronary artery dissection is described. In our patient, angiography showed a mushroom-shaped exudate above the left main coronary artery. Intravascular ultrasound revealed a circular dissection with a huge false lumen connected to the true lumen by a small intimal tear. A brief review of the literature on catheter-induced coronary dissection is included. We believe that this case report provides a good illustration of the need for careful reviewing of indications for angiography. Although procedural risks are low, angiography remains an invasive diagnostic test with the potential to cause severe complications.