Is histological examination of tissue removed by general practitioners always necessary? Before and after comparison of detection rates of serious skin lesions.

OBJECTIVES: To examine whether histological examination of all tissue removed by general practitioners in minor surgery increases the rate of detection of clinically important skin lesions, and to assess the impact of such a policy on pathologists'' workload. DESIGN: Before and after comparison. SETTING: Stratified random sample of 257 general practitioner partnerships from the catchment areas of 19 English pathology laboratories. SUBJECTS: Tissue removed in minor surgery by general practitioners during the control period (September 1992 to February 1993) and intervention period (September 1993 to February 1994). INTERVENTION: General practitioners referred to their local pathology laboratory all solid tissue removed in all minor surgery, irrespective of their previous policy. MAIN OUTCOME MEASURES: Numbers of specimens referred for histology by general practitioners during intervention and control periods; numbers of primary malignant melanomas, non-melanoma malignancies, premalignant lesions, and benign lesions. RESULTS: 257/330 partnerships participated (response rate 78%). During the intervention period 5723 specimens were sent, compared with 4430 during the control period. The referral rate increased by an estimated 1.34 specimens per 1000 patient years (95% confidence interval 0.93 to 1.76, P < 0.0001). General practitioners sent 204 specimens that were malignant (including 16 malignant melanomas) in the control period and 188 that were malignant (including 15 malignant melanomas) during the intervention period (change in total number of malignancies, -1.0 per 100,000 patient years (-5.9 to 3.8, non-significant). CONCLUSIONS: The intervention was associated with a substantial increase in laboratory workload, all of which was accounted for by increases in non-serious lesions. This observation should be taken into account when considering the merits of a policy requiring histological examination in every case.     

Uveal melanoma in congenital ocular melanocytosis mimicking malignant transformation of the optic disc melanocytoma (a controlled case)

A case of a 57-year old woman with uveal melanoma of the posterior pole arising in the congenital ocular melanocytosis mimicking clinically malignant transformation of the optic disc melanocytoma is presented.     

Choroid plexus carcinoma. Cytologic identification of malignant cells in ascitic fluid

Abdominal deposits of a choroid plexus carcinoma in a patient with a ventriculoperitoneal shunt were cytologically diagnosed by examination of ascitic fluid after regression of the primary tumor. The morphology of the malignant cells in ascitic fluid was more similar to that of mesothelial cells than to the appearance of cells from this lesion in cerebrospinal fluid.     

Orbital lymphangioma

In summary, we have presented a patient with an uncommon disorder, orbital lymphangioma, with an equally uncommon outcome, restoration of normal visual acuity and ocular motility following total surgical excision. High-resolution axial thin-slice CT scanning with paraxial re-formations was used for preoperative assessment and surgical planning in the case of this 5 1/2-year-old boy with probable lymphangioma of the orbit. The tumor became symptomatic following minor local trauma. Complete excision was possible without impairment of visual or extraocular motor function.     

Cytology of primary cutaneous Langerhans cell histiocytosis with a malignant phenotype. A case report

BACKGROUND: Langerhans cell histiocytosis (LCH) is a proliferative disorder of Langerhans cells, but the nature of LCH, whether reactive, benign, or malignant and neoplastic, is controversial. We encountered a case of LCH showing a malignant phenotype initially localized in the skin of an elderly woman. Since there is no other report on the cytologic appearance of primary cutaneous LCH or on LCH with a malignant phenotype, we compared the cytologic features of this case with those of benign cases at other sites reported in the literature. CASE: A 74-year-old woman presented with a gradually enlarging and partially ulcerated skin lesion expanding both sides of her right hand. On histologic and ultrastructural analyses of surgically resected tissue, we diagnosed the lesion as Langerhans cell histiocytosis originating in the skin. Although the patient had no recurrence or metastases for six months after surgical resection of the primary skin lesion and radiation therapy, the tumor extended multisystemically, and the patient died of multiple organ failure 14 months after the initial diagnosis. CONCLUSION: Imprint and scrape cytology of multiple skin lesions six months after surgery was useful in immediately diagnosing the recurrent LCH. The tumor cells had indented, twisted or grooved nuclei, and some had intranuclear inclusions. Immunocytochemically the cells were positive for CD1a and S-100 protein. Numerous eosinophils were seen in the background.     

First report of a patient with a mixoploidy 47,XXX/94,XXXXXX

We present a 16 years old female with a chromosomal mixoploidy and multiple phenotypic anomalies. Peripheral blood G-band karyotype was 47,XXX and her skin fibroblast karyotype revealed a mosaic with a 47,XXX cell line in 88% of metaphases and a 94,XXXXXX cell line in 12% of metaphases, consistent with a hypertetraploidy. The most prominent clinical signs were: short stature, left upper limb asymmetry, senile-like appearance, generalized hypertrichosis, and small hands and feet. Radiological examination showed bone dysplasia. The result of molecular studies demonstrated that the patient inherited the two X chromosomes from the mother and one from the father, indicating that her 47,XXX trisomy resulted from an oogenesis error in the first meiotic division. The 94,XXXXXX cell line was likely the result of a cytokinesis error. To our knowledge, this is the first documented patient with a trisomy and a hypertetraploidy.     

A case study of virilizing adrenal tumor in an adolescent female elite tennis player--insight into the use of anabolic steroids in young athletes

A 14-year-old Caucasian girl was referred to the endocrine clinic for evaluation of voice deepening, facial hirsutism, and acne starting 2 years previously. She had been a competitive tennis player since age 7 years, practicing for 4-6 hours daily. On physical examination she was noticed to have a masculine appearance with mild facial acne and moderate hirsutism. Tanner stage was 1 for breast tissue and 5 for pubic hair. Her androgen levels (testosterone, androstenedione, dehydroepiandrosterone sulfate) were extremely elevated. Adrenal ultrasonography revealed a round left 4.6 × 5.3-cm adrenal mass. Laparoscopic left adrenalectomy was performed. The histologic findings were compatible with a benign adrenocortical tumor. Postoperatively, androgen levels dropped to within the normal range. Breast development proceeded normally, menarche occurred 2 months after tumor resection, and menses has been regular since then. Muscle strength of the dominant and nondominant upper and lower extremities was measured 1 month before surgery and 1 year later, using an isokinetic dynamometer (Biodex Systems II, Biodex, Shirley, NY, USA). There was no significant decrease in overall muscle strength after removal of the virilizing tumor and the marked drop in circulating androgens. In addition, the patient maintained her age category, number 1, national tennis ranking. The results suggest that even extremely high levels of tumor-related circulating androgens had no evident effect on muscle strength and competitive performance in a female adolescent tennis player. The lack of beneficial effect on performance in adolescents, combined with the potentially hazardous side effects of anabolic steroids, suggests that teenage athletes should avoid their use.     

原发性腹膜后肿瘤46例诊疗分析

目的：总结并探讨原发性腹膜后肿瘤（PRPT）的诊断及治疗方法。方法：回顾并分析2004年1月至2009年12月收治的46例腹膜后肿瘤患者的临床资料及随访结果。结果：良性肿瘤17例,完整切除15例,部分切除2例,复发4例,均再次手术;恶性肿瘤29例,完整切除10例,部分切除15例,活检2例,复发6例,再次手术4例。随访时间0.5至5年,良性肿瘤死于其他疾病3例,死于原发性腹膜后肿瘤1例;恶性肿瘤死亡22例,其中1年内死亡8例,3年内死亡12例,5年内死亡2例。结论：对原发性腹膜后肿瘤,B超、CT及MRI检查是目前诊断PRPT方便、有效的诊断手段,手术治疗是治疗PRPT的首选治疗方式,完整切除肿瘤是影响PRPT治疗效果及其预后的重要因素。对于复发病例应选择再次手术治疗。     

Leydig cell hyperplasia revealed by gynecomastia

Leydig cell tumors are rare and represent 1% to 3% of all tumors of the testis. Leydig cell tumors affect males at any age, but there are 2 peak periods of incidence: between 5 and 10 years and between 25 and 35 years. Their main clinical presentation is a testicular mass associated with endocrinal manifestations that are variable according to age and appearance of the tumor. Our patient, a 17-year-old adolescent, presented with an isolated and painless hypertrophy of the right mammary gland. Clinical examination found gynecomastia and no testicular mass. Hormonal levels and tumor markers were normal. Testicular sonography showed an ovular and homogeneous right intratesticular mass 6 mm in diameter. We treated the patient with an inguinal right orchidectomy. The anatomopathological study found a nodule of Leydig cell hyperplasia. The patient recovered without recurrence at 8-month follow-up. The patient opted for mammoplasty 2 months after his orchidectomy rather than wait for the spontaneous gradual regression of his gynecomastia, which requires at least 1 year. Leydig cell hyperplasia manifests in the adult by signs of hypogonadism, most frequently gynecomastia. Although many teams prefer total orchidectomy because of the diagnostic difficulty associated with malignant forms, simple subcapsular orchidectomy should become the first-line treatment, provided it be subsequently followed by close surveillance, as it preserves maximum fertility, and these tumors usually resolve favorably.     

Locomotion of T cells from patients with cutaneous T-cell lymphoma (Sezary syndrome and mycosis fungoides)

Peripheral blood T cells from eight patients with cutaneous lymphoma (four each with Sezary syndrome or mycosis fungoides) and T cells from skin tumor of one patient each with Sezary syndrome or mycosis fungoides were studied for their locomotor responses to the chemoattractant, casein. Nonmalignant peripheral blood T cells from each patient with mycosis fungoides moved normally. Malignant T cells from skin tumor of patients with mycosis fungoides or Sezary syndrome did not move in the presence of casein. Peripheral blood malignant T cells (Sezary cells) from three of four patients with Sezary syndrome either moved very poorly or did not move at all. The circulating Sezary cells from the fourth patient with Sezary syndrome responded moderately to the chemoattractant, casein. Two of three patients with Sezary syndrome with poor or no locomotor response of T cells underwent therapeutic leukopheresis without any demonstrable effect on their skin infiltration. The patient whose malignant T cells demonstrated moderate locomotor response to casein had a leukemic blast crisis and at that time her skin became free of malignant cells. A repeat study of her circulating T cells at that time demonstrated almost normal locomotor response to casein. These results demonstrate that the locomotor properties of malignant T cells in patients with Sezary syndrome may have prognostic significance.     

Cytologic features of metanephric adenoma of the kidney during pregnancy: a case report

BACKGROUND: Metanephric adenoma (MA) is a relatively rare neoplasm derived from metanephric blastema and composed of well-differentiated epithelial nephroblastic cells. In view of its invariably benign clinical outcome, a preoperative diagnosis of this tumor could be of critical importance. Since computed tomography and ultrasound imaging are not per se sufficient to unequivocally distinguish between MA and malignant neoplasms, fine needle aspiration cytology (FNAC) could be the only accurate method to establish a preoperative diagnosis of this tumor. However, cytologic appearance of MA is not well characterized. CASE: A 33-year-old pregnant woman presented with erythrocytosis. Transabdominal ultrasound examination disclosed a mass in her left kidney. FNA smears showed small, uniform cells with bland nuclei arranged in compact acinar and follicular structures; immunocytochemical staining revealed a diffuse, positive reaction for CD57, WT-1 and vimentin, and epithelial membrane antigen and alpha-methylacyl-CoA racemase yielded negative results. These cytologic and immunocytochemicalfindings led to a preoperative diagnosis of MA. After delivery, the diagnosis was confirmed on the surgical specimen. CONCLUSION: A diagnosis of MA could be established by FNAC supported by immunocytochemical analysis. The present case illustrates the clinical impact that this diagnosis could have on patient management.     

ACTH- and prolactin-producing pituitary gland microadenoma with biphasic features of atypia and intermediate filament expression

Herein, we report the case of a 28-year old woman clinically presenting with unclear weight gain over the last years. The patient displayed facial and neck edema in combination with unobtrusive striae distensae. Endocrinological examinations led to the diagnosis of Cushing's disease. Neuroradiological examination revealed an intrasellar tumor mass of 7 mm in diameter. Subsequently, transsphenoidal tumor resection was performed. Histological and immunohistochemical investigations revealed a pituitary gland adenoma showing a biphasic tumor growth pattern with two morphologically different tumor areas producing ACTH and prolactin respectively. Co-expression of ACTH and prolactin is exceedingly rare in pituitary adenoma. To our surprise, both tumor areas exhibited features of atypia consisting in elevated MIB-1 proliferation index in the ACTH-producing portion as well as p53 expression selectively in the prolactin-producing tumor parts. To our knowledge, this is the first case of an ACTH- and prolactin-producing pituitary gland adenoma exhibiting biphasic features of atypia.     

Herpes simplex keratitis in renal transplant recipients.

Herpes simplex epithelial keratitis developed in four renal transplant recipients while they were receiving high-dose systemic corticosteroid and immunosuppressive therapy. In all cases the presentation was atypical, and in two cases the course was protracted, leading to some visual loss from corneal scarring and opacification. With the immunocompromised patient it is important to seek early ophthalmologic consultation when even minor ocular symptoms develop.     

Malignant fibrous histiocytoma of the chest wall masquerading as medullary breast carcinoma: a case report

BACKGROUND: Cytologic diagnosis of malignant fibrous histiocytoma can be problematic, as these neoplasms are known to mimic multiple other conditions. CASE: A fine needle aspirate from a 60-year-old woman was diagnosed at 2 institutions as medullary carcinoma of the breast. The patient received neo-adjuvant chemoradiotherapy before the tumor war excised. Gross pathologic examination and histomorphology on routine staining were compatible with the cytologic diagnosis. The accurate diagnosis of pleomorphic-storiform-type malignant fibrous histiocytoma was a surprise and was established with immunocytochemical stains. In retrospect, it was thought that clinical and radiologic overlap, creating a high index of suspicion for a breast neoplasm and compounding the cytologic appearance of a medullary carcinoma with spindle cell metaplasia and syncytial cells, was responsible for the error. CONCLUSION: This case highlights a potential cytodiagnostic pitfall and the importance of establishing a definitive tissue diagnosis in the face of equivocal cytologic findings.     

Ascitic fluid cytology in a case of metastatic malignant mixed mesodermal tumor of the ovary

The cytologic features of the abdominal fluid from a patient with a malignant mixed mesodermal tumor (MMMT) of the ovary are presented. Both malignant epithelial and stromal elements were cytologically appreciated and confirmed by histologic examination. Other ovarian neoplasms that can present with malignant sarcomatous elements or mixed epithelial and sarcomatous elements are discussed; this case documents the importance of recognizing these features when staging patients with unusual ovarian neoplasms. To our knowledge, this is the first complete report of the ascitic fluid cytology of an MMMT of either ovarian or uterine origin.     

Second primary malignant tumors in patients with ocular melanoma

Multiple primary malignant tumors have been documented with increased frequency over the last two decades. Continuously increasing success of modern oncotherapy has led to long-term remissions in many cases, but this success rate poses a growing risk for the development of second primary malignancies. The incidence of those involving an intraocular tumor is relatively rare. In the present study we report five ocular melanoma patients with second primary malignant tumors diagnosed during a fourteen-year period in our department. We wish to emphasize that an intraocular mass lesion in a patient with a history of a previous malignancy should not be dismissed as a metastatic lesion. The diagnosis of an intraocular lesion as a separate primary tumor drastically changes the prognosis and the therapeutic approach.     

Urine cytology of localized primary amyloidosis of the ureter: a case report

BACKGROUND: Morphologic findings of amyloid in urine cytology material have rarely been reported because amyloidosis of the urinary tract is a relatively uncommon disorder. We present a case of primary amyloidosis of the ureter, including catheterized urine cytologicfindings. CASE: A 78-year-old man had pollakiuria and dysuria for 5 years before admission after transurethral resection of the prostate. Clinical examination revealed left hydronephrosis and stricture of the lower part of the left ureter, and a malignant ureteral tumor was suspected clinically. In catheterized urine cytology, many clusters of epithelial cells, inflammatory cells and abundant, amorphous, waxy material were observed. The amorphous material stained light green by the Papanicolaou method and positive with direct fast scarlet (DFS), showing yellow-green birefringence under polarized light. Positivity with DFS staining was not affected by treatment with potassium permanganate. Immunocytochemically the material was AL-type amyloid protein. Atypia were absent from epithelial cells. The patient had no history of diseases that could cause secondary amyloidosis. The present case was considered to be primary amyloidosis localized to the left ureter because no particular morphologic change in the epithelial cells of the urinary tract was observed. CONCLUSION: Amyloid can be present in urine and should not be overlooked or confused with tumor diathesis when a malignant tumor is suspected clinically.     

Advanced primary clear cell carcinoma of the vagina not associated with diethylstilbestrol

BACKGROUND: Primary vaginal clear cell carcinoma occurs in young women exposed to diethylstilbestrol (DES) in utero. Primary vaginal clear cell carcinoma not associated with DES is very rare. We report the clinicopathologic and cytopathologic features of a patient with advanced, sporadic primary vaginal clear cell carcinoma with metastases to liver, lung and paraaortic lymph nodes. CASE: A postmenopausal, 63-year-old woman presented to our department with genital bleeding. A hemorrhagic tumor found in the vagina was diagnosed as a clear cell carcinoma by cytopathologic examination of the tumor smear and by histopathologic examination of a biopsy specimen. A chest radiograph revealed multiple lung metastases, and metastases to the liver and paraaortic lymph nodes were noted on computed tomography and magnetic resonance imaging. The tumor was diagnosed as primary clear cell carcinoma of the vagina, stage IVb (FIGO) based on a normal cytopathologic examination of the cervix, endometrium and ascites; normal appearance of the uterus, ovaries and kidneys on magnetic resonance imaging; and absence of detectable tumor in the urinary tract. The patient died of respiratory failure 31 days after hospitalization. The tumor demonstrated overexpression of p53 protein and did not show microsatellite instability. CONCLUSION: This patient was the second reported Japanese woman with advanced primary vaginal clear cell carcinoma not associated with DES.     

A case of resectable lung adenocarcinoma associated with sarcoidosis

A 71-year-old woman with uveitis was referred to our hospital for further examination of the possible underlying diseases. In roentgenological examination with plain X-ray and CT scan, hilar and mediastinal lymphadenopathy and a mass shadow in the right upper lung field was observed, whereas fibrotic changes were not obvious in both lung fields. Transbronchial lung biopsy with fiberoptic bronchoscope revealed granulomatous interstitial pneumonia. CD4-positive lymphocytes were increased in bronchoalveolar lavage. The patient was diagnosed as having sarcoidosis. Subsequently, right upper lobectomy was performed, and Stage I lung adenocarcinoma was diagnosed. The patient is under follow up without medication and the disease has been stable for two years. A relationship between epithelioid granulomatosis and malignant diseases is discussed and a review of the literature is given. Since it is still controversial as to the incidence of malignant diseases in sarcoidosis patients, it is important to accumulate data on these associations.     

Crush cytology of melanocytoma of the spinal cord. A case report

BACKGROUND: Primary melanocytic neoplasms of the central nervous system are rare. There is no previous report on the intraoperative crush cytology of these neoplasms. CASE: A melanocytoma occurred in a 65-year-old woman who presented with back pain, and weakness and numbness of the right lower extremity. Magnetic resonance imaging revealed an enhancing mass in the conus medullaris. The tumor was subtotally removed. Crush smears revealed dispersed and perivascular aggregates of monotonous oval or spindle tumor cells with round or oval, uniform nuclei; small nucleoli; and long cytoplasmic processes. Some tumor cells contained fine or coarse cytoplasmic brown pigments. The pigments were easier to discern in the crush smears than in the frozen sections. CONCLUSION: Crush cytology appears to be helpful, alone or in conjunction with frozen sections, in the intraoperative diagnosis of meningeal melanocytoma.