IN RESPONSE

We thank Dr. Geller for his invaluable notes on the curious case we reported in the last issue of Autopsy and Case Reports. In fact, as stated in the microscopic description of that lung tumor, the morphological features suggested a “poorly differentiated adenocarcinoma with hepatoid features,” and we agree that this might be a better title for the report. For instance, this was the final diagnosis in the autopsy report since stringent criteria for hepatocellular differentiation as stated by Dr. Geller were not met

The first three years of Autopsy and Case Reports: an interesting journey

Autopsy and Case Reports (A&CR) completes three years of uninterrupted publication this month: March 2014. During these initial years, significant advancements have been made in the process of consolidating a reference journal in the fields of autopsy pathology, anatomoclinical correlation, and medical education

Duodenal pseudomelanosis (pseudomelanosis duodeni): a rare endoscopic finding

Duodenal pseudomelanosis (or pseudomelanosis duodeni) is a rare benigncondition characterized by black-brown speckled pigmentation of the duodenalmucosa. Collections of pigment−laden macrophages are found in the tips ofduodenal villi. The pigment is thought to be mostly composed of ferrous sulfide.Histochemichal stains for iron (Perl’s prussian blue) or melanin (Masson-Fontana) may be positive, but are usually negative or unpredictable. Duodenalpseudomelanosis occurs predominantly in middle-aged to old adults andmore commonly in females. It is associated with chronic renal failure, arterialhypertension, diabetes mellitus and gastrointestinal bleeding. Medications suchas ferrous sulfate, hydralazine, propranolol, hydrochlorothiazide and furosemideare thought to play a role as well. We report a case of a 86-year-old femalewho presented with a history of watery diarrhea and melena. The patient had ahistory of high blood pressure and ischemic stroke episodes. She was on multiplemedication including hidralazine, captopril, hydrochlorthiazide and aspirin. She wasdehydrated, her blood pressure was 96 × 60 mmHg and neurologic examinationshowed complete left hemiplegia with central VII nerve palsy. Laboratory testsshowed normal serum electrolytes and renal function. Hemoglobin level was10.7 g%. An upper endoscopy showed multiple diminutive black spots throughoutthe distal duodenal bulb and second portion. Histology showed multiple foci ofa brown-black granular pigment inside macrophages within the tips of the villi(pseudomelanosis). Stains for iron and melanin were negative. She was treatedwith omeprazol, parenteral fluid replacement with saline and partial fasting. Aftercomplete recovery she was discharged for ambulatory follow up

Testicular Regression Syndrome: a case report

Testicular Regression Syndrome (TRS) is defined as the absence or an incomplete development of the testis of varying degrees in 46XY patients with normal external genitalia. The prevalence ranges from 3-20% of cases previously diagnosed as cryptorchidism. We report the case of a 7-year-old boy who underwent surgical exploration with an initial diagnosis of cryptorchidism. Testicular structure was not identified and presumed testicular remnants were sent for histological analysis. The histological sections showed a fibrovascular nodule, structures of the spermatic cord and calcification, supporting the diagnosis of TR

Strongyloides stercoralis hyperinfection: a dreaded but still missed diagnosis

Strongyloides stercoralis (S. stercoralis), an intestinal nematode, is endemic in tropical and subtropical regions, being less prevalent in temperate climates. The number of infected persons worldwide ranges between 10 million and 100 million people. In Brazil the reported prevalence is 13%. Chronic infection may be asymptomatic or accompanied by gastrointestinal and respiratory symptoms. Under immunosuppressive conditions, the infection assumes serious proportions frequently accompanied by septic shock, disseminated intravascular coagulopathy and respiratory distress syndrome. The authors report a case of a 50-year-old female patient who was a chronic user of glucocorticoids and had been seeking medical attention for two months because of continuous gastrointestinal symptoms. She was admitted to the emergency room with clinical signs of septic shock and died after four days despite an adequate antibiotic regimen, vasopressor drugs, and ventilatory support. The autopsy revealed the unsuspected finding of  S. stercoralishyperinfection and septicemia

Fatal hemoperitoneum due to segmental arterial mediolysis

Spontaneous hemoperitoneum due to vascular injury is a life-threatening condition mostly associated with aortic or splanchnic arterial disease, which stems from atherosclerotic, inflammatory, or infectious origin. However, in 1976, Slavin and Gonzales described a nonatherosclerotic arterial disease that may render aneurysmal formation predominantly in the splanchnic arterial bed. The clinical presentation is diverse, but abdominal pain and shock prevail. We report the case of a middle-aged man who presented a hemoperitoneum due to a middle colic artery aneurysm rupture and died after undergoing a surgical treatment attempt. The preoperative imaging study revealed the presence of a huge hematoma in the epiplon retrocavity, and abdominal free liquid as well as extensive arterial disease with multiple aneurysms. The autopsy findings included hemoperitoneum, hematoma in the upper left abdominal quadrant, the surgical ligature of the middle colic artery, and histologic features consistent with segmental arterial mediolysis. The authors call attention to this rare entity and highlight the autopsy as a fundamental examination to accurately reach this diagnosi

Subcutaneous bronchogenic cyst in a child: case report

Bronchogenic cysts are congenital anomalies originating from the primitive tracheobronchial tree in the fetal period. Although the middle mediastinum is the site most commonly affected (second only to the lungs), there have been reports of bronchogenic cysts at unusual sites, such as the retroperitoneum. We report the case of a 4-year-old boy with a cystic mass in the left scapular area since birth. The cyst grew progressively and was accompanied by recurrent episodes of local infection requiring drainage. It was then surgically removed.The histological findings were decisive for the diagnosis: a subcutaneous cyst lined by ciliated columnar epithelium and surrounded by a fibrous wall with smooth muscle tissue, cartilage, and bronchial glands. We also found chronic inflammatory infiltrate, which is consistent with previous episodes of infection. Bronchogenic cysts should be included in the differential diagnosis of cystic tumors of the chest wall and neck, particularly in children. Surgery is the treatment of choice because of the risk of infection, as in this case, and ofmalignant degeneration.nul

Nodal Epstein-Barr virus-positive T-cell/NK-cell lymphoma associated with immunodeficiency: a rare condition looking for recognition

The authors describe a peculiar form of Epstein-Barr virus (EBV)-associated T-cell lymphoma in an HIV-positive patient presenting an aggressive clinical course. Unlike most other EBV-positive T-cell/natural-killer (NK)-cell lymphomas, the disease was characterized by predominant nodal involvement at presentation. T-cell lineage was confirmed by T-cell receptor-rearrangement, and neoplastic cells exhibited strong and diffuse CD56 expression. A marked intravascular component was detected in the skin, the liver, and the lung parenchyma. This entity was not predicted in the WHO 2008 classification, but has been recently identified in immunocompromised patients. This case report refers to a middle-aged man with AIDS, who presented a 4-month history of weight loss, fever, hepatosplenomegaly, peripheral and deep-chain lymphadenopathy. A blood smear showed lymphocytosis with a marked presence of atypia. The outcome was unfavorable and the patient could not be treated. The autopsy revealed multivisceral involvement, including lymph nodes, spleen, bone marrow, liver, lungs, skin, and kidneys

Bacterial sinusitis and its frightening complications: subdural empyema and Lemierre syndrome

The symptoms of a previously healthy 14-year-old female with an initial history of tooth pain and swelling of the left maxillary evolved to a progressive headache and altered neurological findings characterized by auditory hallucinations, sleep disturbances, and aggressiveness. She was brought to the emergency department after 21 days of the initial symptoms. An initial computed tomography (CT) scan showed frontal subdural empyema with bone erosion. The symptoms continued to evolve to brain herniation 24 hours after admission. A second CT scan showed a left internal jugular vein thrombosis. The outcome was unfavorable and the patient died on the second day after admission. The autopsy findings depicted rarefaction of the cranial bone at the left side of the frontal sinus, and overt meningitis. The severe infection was further complicated by thrombophlebitis of the left internal jugular vein up to the superior vena cava with septic embolization to the lungs, pneumonia, and sepsis. This case report highlights the degree of severity that a trivial infection can reach. The unusual presentation of the sinusitis may have wrongly guided the approach of this unfortunate cas

Placental polyp: a rare cause of iron deficiency anemia

Placental polyps are defined as pedunculated or polypoid fragments of placentaor ovular membranes retained for an indefinite period of time into the uterus afterabortion or child birth. An important cause of retention is placental accretism, anabnormal adherence of the placenta into the uterine wall. Chronic cases are rarelyreported in the literature. In these cases, the placental retention in the immediatepostpartum is not followed by heavy bleeding what makes the diagnosischallenging. We report a rare case of iron-deficiency anemia in a multiparous29-year-old female patient two years after the last delivery. She sought medicalcare with clinical symptoms of anemia and recent menses alterations. Therewas no history of abortion. On gynecological examination, there was a twofoldenlarged uterus, and the pelvic ultrasound revealed an image compatible with anendometrial polyp. She underwent open hysterectomy because of uncontrollablebleeding followed by hypotension after curettage. The histolopathologicexamination revealed a partially hyalinized and necrotic placental polyp