Rare Histological Finding Research Articles

Myeloma crystal tubulopathy and Fanconi syndrome: a rare histological finding

Myeloma crystal tubulopathy and Fanconi syndrome: a rare histological finding

Hepatocellular carcinoma occurring in hepatobiliary fibropolycystic disease

Congenital hepatic fibrosis (CHF) and bile duct hamartomas (von Meyenburg complexes) are hepatobiliary fibropolycystic diseases. There have been several reports of liver neoplasias arising in hepatobiliary fibropolycystic diseases. However, most of them were cholangiocarcinomas and cases involving hepatocellular carcinoma (HCC) are rare. A 51-year-old woman was found to have multiple hepatic tumors by ultrasonography and enhanced computed tomography (CT) during a regular work-up for the recurrence of lung cancer and thyroid cancer, which had been surgically removed 4 and 3 years ago, respectively. Nodules were observed at S3, S5, and S6 (2 cm in diameter). All of the nodules were hyperattenuated at the early arterial phase, and the main tumor at S5 showed hypoattenuation at the delayed phase on dynamic CT and magnetic resonance imaging (MRI). HCC was suspected from these findings. She also suffered from multiple small cystic lesions in the liver. The surgically removed liver showed HCC arising in CHF, which is a rare histological finding.

Granulomatous Pigmented Purpura: An Unusual Histological Variant

Pigmented purpuric dermatoses (PPDs) tend to vary clinically, yet share a similar histology. Granulomas are only rarely seen, with 4 prior reported cases, designated granulomatous pigmented purpura. Historically, granulomatous pigmented purpura has been seen in Asians. In this article, we report the case of a 42-year-old, white female with granulomatous pigmented purpura, the fifth such reported case in the literature. Histopathological examination revealed a granulomatous dermatitis with eosinophils, extravasated erythrocytes, melanophages, and vascular proliferation. These findings were consistently reported on subsequent biopsy 6 months later. This case illustrates a rare histological finding in PPDs. Medications associated with PPD are reviewed and the patient's use of mesalamine and balsalazide for ulcerative colitis are deemed potential triggers, given their relative similarity to aspirin, a known trigger of PPD. Although other granulomatous processes must be excluded in such patients, one must consider the possibility of granulomatous pigmented purpura in the appropriate setting.

The Role of Pure Diffuse Mesangial Hypercellularity in Patients with Proteinuria

Background. Pure diffuse mesangial hypercellulality (DMH), in its primary form, is a relatively rare histological finding, and scant data exist in the literature regarding its clinical course and prognosis in nephrotic adults with this diagnosis. Methods. We retrospectively analyzed the clinical and histological data of 8 out of 41 patients with the above diagnosis in regard to response to the treatment, outcome and prognostic indicators. Results. Six patients received oral prednisolone as initial therapy, five of whom receiving it as monotherapy at first. The two other patients did not receive anything at all. Three out of the above six patients received prednisolone either with cyclophosphamide or with cyclosporine (CyA). Three patients responded with complete remission, two showed partial remission, and one did not respond at all. During follow-up, none of the patients with complete response appeared to have relapse. The two patients with initial partial response to steroids received CyA in combination with low dose of oral prednisolone. The other patient who did not respond at all from the beginning did not receive anything more due to his bad general condition. Plasma creatinine remained stable in those with complete or partial response to treatment. None of the clinical characteristics was found to be predictive of the degree of renal function impairment at the time of renal biopsy. The three patients with partial or no response were characterized by the severity of mesangial hypercellularity. Patients with complete or partial response to therapy did not differ with regard to age, plasma creatinine, and severity of proteinouria at biopsy. Presence of mesangial IgM was not associated with poor or satisfactory response. In general, no clinical feature at the time of biopsy was predictive of a response to therapy. Conclusions. At present, it seems that adult patients with DMH and proteinuria represent a heterogeneous group with different clinical courses despite a similar morphological appearance in initial biopsies. We conclude that serial biopsies taken at regular intervals coupled with a longer follow-up may provide answers concerning the real intentity of DMH.

209: Granulomatous Interstitial Nephritis Secondary to Carbamazepine Causing Acute Renal Failure

GRANULOMATOUS INTERSTITIAL NEPHRITIS SECONDARY TO CARBAMAZEPINE CAUSING ACUTE RENAL FAILURE Prashanth Podaralla, Nagaraju Sarabu, Marcos Rothstein, 1 Barnes Jewish Hospital, Washington University, St. Louis, MO. 2 St. Luke’s Hospital, Chesterfield, MO. Granulomatous interstitial nephritis (GIN) is a specific subtype of interstitial nephritis that is present in 0.5% to 0.9% of native kidney biopsies. Thus, GIN is a rare histologic finding and has been associated with medications, infections, sarcoidosis and Wegener’s granulomatosis. Common medications causing GIN include antibiotics, non-steroidal antiinflammatory drugs, allopurinol, diuretics and anticonvulsants. We present the case of a 77-year-old male with past medical history of hypertension, hypothyroidism and seizure disorder, who came with one week history of nausea, vomiting and decreased urine output. About two months prior to presentation, he was started on carbamazepine for his seizures. Physical examination was remarkable only for bilateral basilar crackles. Laboratory data showed normal white cell count with slightly increased eosinophil count, normal hemoglobin, blood urea nitrogen 132 mg/dl, and creatinine 9.4 mg/dl (baseline 1.1, 3 weeks ago). Urine analysis showed 3+ protein and spot urine protein to creatinine ratio was 2.43. He required dialysis soon after presentation. Percutaneous biopsy of the kidney showed extensive interstitial non-caseating granulomatous inflammation with eosinophils. Patient also developed a maculopapular rash all over his trunk during hospital stay. CT scan of the chest failed to show any hilar lymphadenopathy or pulmonary infiltrates. Based on the clinical presentation, temporal association with carbamazepine, and biopsy findings, the diagnosis of carbamazepine induced GIN was made. Carbamazepine was stopped and he was switched to Zonisamide for seizure control. Patient was started on prednisone, which dramatically reversed his acute renal failure, and he came off of dialysis within 4 weeks. After one year of presentation he retains his normal kidney function. In conclusion, we present a case of GIN due to carbamazepine requiring renal replacement therapy, which is rare, but a recognized severe complication of carbamazepine.

Xanthogranulomatous Orchitis in a Patient with a History of Instrumentation and Bacillus Calmette-Guérin Therapy

Xanthogranulomatous orchitis (XGO) is a rare histological finding that is generally diagnosed at orchiectomy. To date, fewer than 10 reports of XGO exist in the literature. The exact pathogenesis of XGO is unknown and patients may present with a rapid onset of symptoms or a longer, more indolent course. We report a patient who presented with symptoms of epididymoorchitis, leukocytosis, and fever after an instillation of bacillus Calmette-Guérin therapy for bladder cancer.

Intrathyroidal hematopoiesis: a rare histological finding in an otherwise healthy patient and review of the literature

Multinodular goiter represents the most common thyroid disease in Europe and the United States. Regressive changes like hemorrhage, infarction or fibrosis are frequently observed. Calcifications have also been detected by microscopic investigation of thyroid tissue. Extramedullary hematopoiesis has been described in almost every organ, especially in patients with hematologic diseases. However, true bone formation is uncommon and to date only two cases of metaplastic bone formation with extramedullary hematopoiesis in the thyroid have been reported in the literature. We present a case of total thyroidectomy due to multinodular goiter harboring an area of mature bone and hematopoiesis in an otherwise healthy female patient. Furthermore, we reviewed and summarized for the first time all available cases from the literature. We report the third case of metaplastic bone formation with foci of hematopoietic tissue in the thyroid gland. All three patients were young females without any obvious stimulus for extramedullary hematopoiesis. Isolated extramedullary hematopoiesis seems to be more frequent in the thyroid gland than metaplastic bone formation with hematopoiesis, particularly in older people with underlying hematologic disorders. It also represents an important differential diagnosis of anaplastic carcinoma of the thyroid, particularly in intra-operative frozen sections and fine-needle aspiration biopsy.

A diagnostic dilemma in breast pathology – benign fibroadenoma with multinucleated stromal giant cells

Fibroadenomas are common benign breast tumours that display a characteristic pathological morphology, although several epithelial and stromal variations exist. A very rare histological finding is the presence of multinucleated giant cells throughout the stroma of a benign fibroadenoma. Cells of this type, which are more commonly found incidentally within the interlobular stroma of breast tissue, are benign and should not be mistaken for malignant cells on microscopic examination. Unfortunately a lack of awareness of this pathological entity can lead to diagnostic confusion amongst pathologists resulting in the multinucleate giant cells being mistaken for highly mitotic cells and consequently the fibroadenoma being mistaken for a malignant lesion. This may have serious implications for the subsequent management of the patient. The presence of this unusual cell type in the stroma does not alter the prognosis of otherwise benign lesion. We encountered two such cases at our institution in a six month period recently. We present their histories along with relevant radiological, microscopic and immunohistochemical features, followed by a discussion of this unusual pathological entity.

Syncytial giant cell hepatitis in human immunodeficiency virus–infected patients with chronic hepatitis C: 2 cases and review of the literature

Syncytial giant cell hepatitis (SGCH) among adult human immunodeficiency virus (HIV)-infected patients has been rarely described. Most cases have been reported in subjects coinfected with the hepatitis C virus (HCV), but its prevalence and outcome remain unknown. We performed a retrospective analysis of all cases of SGCH among 332 liver biopsies from HIV-infected patients seen at a tertiary center in Madrid, Spain, between 1984 and March 2004. Two hundred fifty specimens were obtained from HCV-coinfected patients. There were 2 cases of SGCH, leading to an observed overall prevalence of 0.6% (0.8% when considering only HCV-coinfected patients). In addition to histological changes secondary to chronic hepatitis C, the liver cords were replaced by syncytial giant cells with up to 30 nuclei. There was no histological evidence of measles (among paramyxoviruses) or herpes viruses group infections. In patient 1, there was a progressive clinical worsening after a 3-month course of prednisone, leading to liver failure and death. His postmortem liver biopsy showed more abundant giant hepatocytes accompanied with the development of a histologic pattern of severe fibrosing cholestatic hepatitis. The second patient received a prolonged course of pegylated interferon-alpha-2b and ribavirin with clearance of syncytial giant hepatocytes despite HCV-RNA persistence. SGCH is a rare histological finding among HIV-infected patients with chronic hepatitis C. Specific treatment with pegylated interferon and ribavirin can lead to histological resolution and biochemical improvement, even in the absence of HCV-RNA clearance.

Hemangioma in minor salivary glands: real or illusion

Hemangioma is a common soft tissue lesion in the head and neck region. Hemangioma in the context of minor salivary glands is rarely encountered in surgical pathology practice, and for this reason most pathologist are often unfamiliar with its histomorphological features. We report a rare histological finding of salivary gland structures within a cavernous hemangioma, which may or may not have originated in the minor salivary gland.

An unusual admixture of neoplastic and metaplastic lesions of the female genital tract in the Peutz-Jeghers Syndrome

Background. Peutz-Jeghers Syndrome (PJS) is a rare autosomal dominant condition with variable penetrance characterized by gastrointestinal hamartomatous polyps and mucocutaneous pigmentation. Patients with PJS have an increased risk for breast, gastrointestinal and female genital tract cancers. Case. Multiple genital tract neoplasms in a 41-year-old Italian woman with PJS are described. The patient presented with abdominal pain due to intussusception. A CT scan of the abdomen also showed a left adnexal mass, diagnosed as ovarian mixed serous and mucinous borderline tumor. An ovarian microscopic sex cord tumor with annular tubules (SCTAT) was incidentally diagnosed together with a minimal deviation mucinous adenocarcinoma of the uterine cervix. Also areas of typical hyperplasia of the tubal mucosa with mucinous metaplasia were found. Conclusion. This appears to be one of the rare cases reported in literature in which PJS is complicated by multiple and contemporaneous genital tract tumors and rare histological findings. The clinical significance of recurrence of these unusual genital tract tumors and histological alterations in PJS patients is reviewed.

Adult-onset idiopathic nephrotic syndrome associated with pure diffuse mesangial hypercellularity.

Pure diffuse mesangial hypercellularity (DMH), in its primary form, is a relatively rare histological finding and few data exist in the literature regarding its clinical course and prognosis in nephrotic adults with this diagnosis. We retrospectively analysed the clinical and histological data of 28 adult nephrotic patients (13 male) with this diagnosis with regard to response to the treatment, outcome and prognostic indicators. Of 25 patients treated with prednisolone (Pred), nine (36%) showed complete remission (CR) of proteinuria, eight (32%) partial remission (PR) and eight (32%) did not respond at all (NR). The combination of cyclosporin treatment with prednisolone of those with PR or NR produced one further complete and two partial remissions. At the end of follow-up (mean 64 months), 10 patients (40%) were in CR, nine (36%) in PR and six (24%) were NR and remained nephrotic. Renal function remained unchanged in patients with CR or PR. In contrast, the six non-responders progressed to end-stage renal disease (ESRD). Compared with non-responders, patients who responded to Pred were older and had normal renal function at presentation. This group also had less mesangial sclerosis and severe tubulointerstitial fibrosis and none showed synechiae with Bowman's capsule. IgM mesangial deposits were observed in 22% of patients with CR in response to Pred, in 37% of those with PR and in 100% of non-responders, who finally progressed to ESRD. A multivariate analysis of clinical and histological features at biopsy showed persistent nephrotic syndrome (P<0.001), the severity of DMH (P<0.03) and the presence of mesangial IgM (P<0. 01) to have independent predictive value for ESRD. This analysis also demonstrated that only mesangial sclerosis (P<0.03) and the presence of mesangial IgM (P<0.002) independently predicted the response to therapy. DMH associated with idiopathic nephrotic syndrome is a heterogeneous entity. Patients who respond to therapy (completely or partially) have a benign course similar to that of minimal change nephrotic syndrome. They are usually older and have normal renal function at presentation, whereas 'sclerotic' lesions are less frequent findings in initial biopsies. Non-responders tend to be younger and progress to ESRD. Most of them have impaired renal function at first assessment and more prominent 'sclerotic' lesions on initial biopsies. Mesangial IgM is an independent marker of poorer response to treatment and progression to ESRD but it lacks specificity.

汗管に沿って増殖を認めたＡｃｒａｌ ｌｅｎｔｉｇｉｎｏｕｓ ｍｅｌａｎｏｍａ（ＡＬＭ） ｉｎ ｓｉｔｕの１例

A 50-year-old Japanese female of acral lentiginous melanoma in situ involved into deeper eccrine duct epithelium on the right middle finger was described. Histopathologically, increased number of atypical melanocytes were proliferated involving both lower epidermis and deeper portion, 643μm from epidermal basal layer, of eccrine sweat duct epithelium, which is a rare histological finding in usual ALM. It is well known and interesting that malignant melanoma in situ, especially ALM in situ often proliferate involving into superficial portion of eccrine duct epithelium, however, our case was exceptional.We supposed that ALM in situ cells could proliferate with affinity to eccrine duct epitherlium. Taken together, we need careful treatment and follow-up for ALM in situ with proliferation into the deeper portion of eccrine duct as our case.

CASE REPORT OF COLON CARCINOMA WITH A RARE HISTOLOGICAL FINDINGS

From 1970 to 1985, 286 cases were operated on for colon or rectal carcinoma, and out, of the 286 cases 5 with rare histological findings were reported. Case 1-A man aged 34 years had an undifferentiated carcinoma of the rectum. Around 3 months after Mile's operation he experienced liver and lung metastases and died with the prognosis of 2 months. Case 2-A man aged 62 years had small cell carcinoma of the rectum, who died of the recurrence 9 months after surgery. Case 3-A man aged 63 years was found to have a caecal carcinoma and caecal resection was carried out. Histologically it was undifferentiated carcinoma. His prognosis was 8 months. Case 4-A woman aged 60 years admitted because of peritonitis carcinomatosa had a colon ascendens carciona. Excisional biopsy revealed adenoacanthoma. She died 3 months later. Case 5-A woman aged 61-years was diagnosed to have a malignant melanoma of the rectum and underwent Mile's operation, with the prognosis of 6 months. In those 5 patients no chemotherapy could yield cures and their prognoses were very poor.

Report--British Federation against the Venereal Diseases

Delayed gastric emptying can be due to muscular, neural, or humoral abnormalities. In the absence of an identified cause, gastroparesis is labelled as idiopathic. We present the case of a patient with severe idiopathic gastroparesis. Pharmacological approaches failed, as well as reduction in gastric emptying resistance with pyloric injection of botulinum toxin and pyloroplasty. Therefore, subtotal gastrectomy was performed. Histological and immunohistochemical study of the resected specimen showed hypoganglionosis, neuronal dysplasia, and a marked reduction in both myenteric and intramuscular interstitial cells of Cajal. To our knowledge, this is the first time these rare histological findings have been described in a patient with idiopathic gastroparesis.