Epithelial myoepithelial carcinoma of the parotid gland with malignant ductal and myoepithelial components arising in a pleomorphic adenoma: a case report with cytologic, histologic and immunohistochemical correlation

BACKGROUND: To describe the cytologic, histologic and immunohistochemical findings of a case of epithelial myoepithelial carcinoma (EMC) arising from a pleomorphic adenoma (PA) of the parotid with both malignant epithelial and myoepithelial components. CASE: A 29-year-old female presented with a 1.5 x 1.5-cm, palpable mass of the left parotid of 7-8 months' duration with recent enlargement and pain. Fine needle aspiration biopsy (FNAB) revealed biphasic epithelial (small cell) and myoepithelial (large/clear cell) clusters arranged in a pseudopapillary and trabecular pattern with abundant hyaline material with many naked nuclei, together with areas typical of pleomorphic adenoma (PA) was noted. The cytology was reported as salivary gland neoplasm, "suggestive of adenoid cystic carcinoma, less likely pleomorphic adenoma." The mass was excised and histologically reported as "pleomorphic adenoma, with focal invasion of one resected margin." Four months later the tumor recurred, and FNAB showed almost the same cytologic features as did the previous aspirate. Due to early recurrence, previous histologic sections were reviewed, and typical areas of a biphasic pattern of EMC with atypicality and mitosis of both components was found. The final diagnosis was EMC ex PA. CONCLUSION: Although previous reports mention the difficulties in diagnosing EMC and differentiation from the more common salivary gland neoplasms such as PA, we like to emphasize the cytologic confusion that results when the tumors coexist.     

Extramedullary plasmacytoma of the parotid gland. Report of a case with extensive amyloid deposition masking the cytologic and histopathologic picture

BACKGROUND: Extramedullary plasmacytomas are uncommon. Although approximately 90% occur in the head and neck region, parotid gland localization is extremely rare. CASE: This report describes fine needle aspiration and histopathologic findings in an extramedullary plasmacytoma arising in the left parotid gland of a 62-year-old man. Aspiration smears showed multiple amorphous clumps of material admixed with ductal epithelial cells, multinucleated giant cells and inflammatory cells rich in plasma cells, suggestive of pleomorphic adenoma. In surgical material, excessive amyloid deposition was observed. Six months later the tumor recurred, and in the second surgical specimen clusters of atypical plasma cells among amyloid deposits was noted. Clinical and laboratory examination excluded multiple myeloma. After local recurrence, radiotherapy was applied. Ten months later the patient was well and without systemic involvement. CONCLUSION: Two points are important. First, in the salivary gland region, because of the focal metachromasia of amyloid with Giemsa stain, amyloid can be confused with the chondromyxoid matrix of pleomorphic adenoma. Second, although localized forms of amyloid tumor exist, one should keep in mind that amyloid may be so abundant that it may mask an underlying plasma cell neoplasm, as in our case.     

CANCER OF THE LARYNX—The Advantages of More Conservative Treatment

Properly administered irradiation therapy for cancer of the larynx in properly selected cases can be successful. Reported herein is the case of a woman 49 years of age with a Grade 3 squamous cell carcinoma of the larynx, who at first was scheduled for radical operation but was treated by irradiation therapy with an estimated total dose of 5292 r given in 29 treatments. The tumor and all subjective and objective symptoms disappeared. The patient was still well when last observed a year later.     

Cancer of the larynx; the advantages of more conservative treatment

Properly administered irradiation therapy for cancer of the larynx in properly selected cases can be successful. Reported herein is the case of a woman 49 years of age with a Grade 3 squamous cell carcinoma of the larynx, who at first was scheduled for radical operation but was treated by irradiation therapy with an estimated total dose of 5292 r given in 29 treatments. The tumor and all subjective and objective symptoms disappeared. The patient was still well when last observed a year later.     

Clear cell carcinoma arising from pleomorphic adenoma of a minor salivary gland: Report of a case with fine needle aspiration, histologic and immunohistochemical findings

BACKGROUND: Malignant changes in pleomorphic adenoma (PA) of the salivary gland (carcinoma ex pleomorphic adenoma) are not common. Clear cell carcinoma is a rare form of salivary gland tumor and involves mostly minor salivary glands, especially those of the palate. Only 3 cases of clear cell carcinoma arising in PA have been reported, 2 in submandibular glands and 1 in a minor salivary gland of the palate. CASE : A 53-year-old man presented with an enlarged mass on the left side of the palate. He had a history of palate mass about 30 years earlier; it was excised and reported as PA. Since then the tumor had recurred twice in the same place; it had been excised and was diagnosed as PA again. A few years later the mass showed rapid growth over a few months. Fine needle aspiration of the mass showed epithelial clusters with bland nuclear features and myxohyaline material typical of PA. Also noted were large and small papillary, trabecular and well-circumscribed clusters of neoplastic cells with a moderate amount offoamy, vacuolated cytoplasm with distinct borders. Glandlike and acinar structures with hyaline globule material resembling cannonballs were also noted. The cytology was suspicious for malignancy. Incisional biopsy was reported as PA. Due to the suspicion of malignancy, the whole mass was excised up to the floor of the orbit. The final diagnosis was clear cell carcinoma expleomorphic adenoma. CONCLUSION: Due to nonspecific cytologic findings in clear cell carcinoma and a mixture of elements of PA in this case, we did not consider clear cell carcinoma as the malignant component.     

A Case Report of Chemo-sensitive Intimal Pulmonary Artery Sarcoma

The incidence rate of pulmonary artery sarcoma is very low, but its prognosis is extremely poor. In this case report, after various initial diagnoses at the early stage, pulmonary artery sarcoma was confirmed by surgery. 1 year later, the tumor recurred. After chemotherapy, the patient showed improvement of the subjective complaint of tightness in the chest, and radiological lesion decreased in size. The survival time was extended by 2.5 years. This is the first case report of pulmonary artery sarcoma with such chemo-sensitivity.     

Metastatic minor salivary gland colloid carcinoma in the parotid region after parotidectomy for pleomorphic adenoma: a case report

BACKGROUND: Primary colloid or mucinous carcinoma of the salivary glands is extremely rare. Only a few cases have been reported that originated in the minor salivary glands. an even more exceptional presentation is as a metastatic tumor in the parotid region subsequent to superficial parotidectomy for pleomorphic adenoma. The case presented here posed a diagnostic dilemma that could be resolved only after a thorough reevaluation of the previous cytologic and histologic material and detection of the occult primary tumor in the hypopharynx following an extensive clinical and radiologic workup. CASE: A 75-year-old female underwent fine needle aspiration of the left parotid and was diagnosed as having pleomorphic adenoma. A superficial parotidectomy removed the tumor completely, and the diagnosis was confirmed. Six months following the surgery, the patient developed an enlarged nodular mass in the ipsilateral parotid region, with fine needle aspiration showing colloid (mucinous) adenocarcinoma, which proved to be a metastatic colloid (mucinous) carcinoma on excisional biopsy. The clinical and radiologic workup in search of a primary lesion led to an occult tumor in the left hypopharyngeal mucosa. CONCLUSION: Unusual presentations of rare tumors can cause considerable diagnostic difficulties to both the clinician and cytopathologist. Awareness of these rarities is important to ensure the best patient care and to avoid unnecessary investigative and therapeutic procedures.     

Cytologic findings of a pleomorphic adenoma of the breast: a case report

BACKGROUND: Pleomorphic adenoma of the breast is a rare benign tumor. Only a few cases have been reported. The histologic features have been described well. However, the cytologic findings have been described in only a few papers. CASE: A 47-year-old female presented with a left breast mass of several months' duration. The clinical and mammographic findings were highly suspicious for malignancy. Following an aspiration biopsy diagnosis of "positive for malignancy," the mass was excised. The histologic diagnosis was pleomorphic adenoma (mixed tumor of salivary gland type) rather than carcinoma. CONCLUSION: The cytologic presentation of pleomorphic adenoma of the breast can masquerade as that of a malignant tumor, in this case colloid carcinoma. This case delineates the cytomorphologicfeatures of pleomorphic adenoma, which may mimic carcinoma.     

Significant gene expression of insulin-like growth factor II and proliferating cell nuclear antigen in a rapidly growing recurrent pituitary acth-secreting adenoma.

BACKGROUND: We quantified the expression of various growth-related factors in an adrenocorticotropic hormone (ACTH)-secreting adenoma that had recurred very rapidly as invasive macroadenoma. METHODS/RESULTS: A 43-year-old woman underwent successful transsphenoidal surgery for Cushing's disease. Seven years later, she was admitted to our ward for further endocrine examinations. In spite of a very high plasma ACTH level, the serum cortisol level was normal. Discrepancies between ACTH and cortisol levels were detected on the basis of diurnal rhythms, dexamethasone suppression tests, and corticotropin-releasing hormone test. The patient showed no clinical features of Cushing's disease. Magnetic resonance imaging of the pituitary showed an almost empty sella, and no microadenoma was found. These results, along with those of Sephadex column gel filtration and high-performance liquid chromatography of plasma-immunoreactive ACTH, suggested that the patient's residual corticotrophs secreted biologically inactive ACTH. Two years later, the patient suddenly developed diplopia and right abducens nerve palsy. She was slightly moonfaced and centrally obese. Her plasma ACTH and serum and urinary free cortisol levels were elevated, although discrepancies between ACTH and cortisol still existed. Magnetic resonance imaging revealed a large pituitary mass with suprasellar and cavernous sinus extensions. The tumor was excised, and the proopiomelanocortin gene and the expression of growth-related factors were analyzed. No mutations were found in the ACTH-coding region of the proopiomelanocortin gene. A significant expression of insulin-like growth factor II and proliferating cell nuclear antigen mRNAs was demonstrated. A high MIB-1 antibody labeling index was also detected in the adenoma tissue, suggesting high Ki-67 expression. CONCLUSION: These growth- and proliferation-related factors might be involved in the rapid growth and aggressiveness of this patient's pituitary adenoma.     

Osteogenic Sarcoma of the Sella After Radiation Treatment of a Pituitary Adenoma

ObjectiveTo report an uncommon case of osteogenic sarcoma of the sella turcica after radiation treatment of a pituitary adenoma.MethodsWe present the clinical history, physical findings, laboratory data, imaging studies, and pathologic findings in a patient found to have osteogenic sarcoma of the sella after radiation therapy for a nonfunctioning pituitary adenoma.ResultsSix years after transsphenoidal resection and postoperative fractionated radiation therapy for a nonfunctioning pituitary adenoma that extended to the cavernous sinus, a 45-year-old man presented with a sinus infection, diplopia, and ophthalmoplegia of the right eye. A computed tomographic scan of the head showed a mass in the sella with involvement of the optic chiasm and right cavernous sinus. Transsphenoidal resection and debulking of the tumor revealed an osteogenic sarcoma. The patient was discharged from the hospital with residual diplopia and ophthalmoplegia. He was treated with levothyroxine, testosterone, and hydrocortisone. Six weeks later, the patient was readmitted after he was found unresponsive, and computed tomographic scans disclosed a massive cerebrovascular accident. He died a few days later.ConclusionOsteogenic sarcoma is a rare, late complication of radiation treatment of pituitary adenoma. Although radiotherapy remains an effective adjunctive treatment in patients with pituitary adenomas, particularly those with residual or recurrent tumor, potential complications must be acknowledged. (Endocr Pract. 2004;10: 335-338)     

Localized early mesenteric Castleman's disease presenting as recurrent intestinal obstruction: a case report

Introduction

Pleomorphic adenoma is the most common benign neoplasm of the salivary glands. Extensive lipomatous involvement of the tumor is, however, a very rare finding.

Case report

Herein, a rare case of lipomatous pleomorphic adenoma arising in the parotid gland of a 14-year-old Japanese woman is presented.

Conclusion

This is the sixth case of lipomatous pleomorphic adenoma in the English literature. Recognition of this rare subtype of pleomorphic adenoma is important for clinical diagnosis and management. On CT scan, it may not be detected possibly due to the extensive fatty component.     

Metastatic carcinoma with myxoid stroma in the salivary gland: a case report

BACKGROUND: Most epithelial salivary gland tumors with a myxoid stroma are pleomorphic adenomas. Rare metastatic carcinomas have prominent myxoid stroma and therefore can mimic pleomorphic adenomas cytologically. CASE: A 62-year-old man presented with a left canthal tumor. A biopsy and computed tomography revealed an adenocarcinoma of the left ethmoid sinus with medial canthal extension. The patient was treated with tumor resection and chemoradiation. An enlarging, left parotid mass developed that was reported as a pleomorphic adenoma on a fine needle aspirate. However, a parotidectomy showed metastatic adenocarcinoma with a myxoid and fibroblastic stroma in an intraparotid lymph node. CONCLUSION: Before concluding cytologically that a biphasic epithelial/myxoid stromal salivary gland lesion is a pleomorphic adenoma, the patient's previous malignancies should be reviewed, and the smears should be scrutinizedfor the absence of diffuse epithelial atypia and presence of spindle cells transitional between the 2 tissue phases.     

Role of FNAC in metastasizing malignant mixed tumor of the external auditory canal. A case report

BACKGROUND: No previous report of metastasizing mixed tumor (pleomorphic adenoma) of the external auditory canal (EAC) has been described. CASE: A 12-year-old, Chinese girl with a history of mixed tumor of the EAC presented with a locally recurrent, aggressive tumor and metastases to the lung and bone five years later. The primary, locally recurrent and metastatic lung tumor showed epithelial and myoepithelial elements with duct formation, chondromyxoid stroma and mitotic activity in the cellular areas on histology. Fine needle aspiration cytology (FNAC) disclosed the presence of spindle cells blending into chondromyxoid fibrillar ground substance in the recurrent and metastatic lung tumors. CONCLUSION: In primary mixed tumor of the EAC, FNAC plays a useful role in the diagnosis of recurrent and metastatic disease. Its ability to identify ominous features, such as increased mitoses in this case, may be limited by sampling. Since cytology and histology cannot reliably prognosticate, long-term follow up of mixed tumor of the EAC after complete excision is advocated.     

Effectiveness of chronic treatment with ketoconazole in a patient with diabetic Cushing's disease resistant to surgery

Without treatment, Cushing's disease has significant morbidity and mortality. Where a surgical approach may not be feasible, or is refused by the patient, medical therapy becomes the only option. In this case report, we discuss the effects of two years of ketoconazole treatment on diabetes regulation and insulin resistance in a patient reluctant to agree to surgery. A 62 year-old female patient with uncontrolled type 2 diabetes mellitus was investigated. Cushing's disease was confirmed by the results of high urine free cortisol level and dexamethasone suppression tests. We discuss the effects of two years of 600 mg/day ketoconazole treatment on diabetes regulation and insulin resistance in a patient with Cushing's disease reluctant to agree to surgery. This case report illustrates the beneficial long-term effects of 24 months of ketoconazole treatment on the clinical and laboratory findings and also on steroid and glucose metabolism.     

Diagnosis of pleomorphic adenoma in a heterotopic salivary gland: a case report

BACKGROUND: The histologic diversity encountered in pleomorphic adenoma may cause diagnostic difficulty in fine needle aspiration cytology (FNA) due to limited and selective sampling. CASE: A 40-year-old woman presented with a mass in the anterior aspect of the neck along the sternocleidomastoid muscle. FNA revealed a cellular tumor with a chondrimyxoid background and epithelial cells intermingled with a few mesenchymal cells. The diagnosis of pleomorphic adenoma was confirmed on histopathology. CONCLUSION: Primary ectopic pleomorphic adenoma can confidently be diagnosed by FNA. This technique is a useful tool in the initial assessment of the tumor.     

Benign neurilemmoma (schwannoma) masquerading as a pleomorphic adenoma of the submandibular salivary gland

A case of solitary benign neurilemmoma (schwannoma) arising in the submandibular region is presented. The tumor was mistaken clinically for an enlarged submandibular salivary gland. Fine needle aspiration cytology made an erroneous diagnosis of a pleomorphic adenoma, predominantly stromal in composition. Histology of the resection specimen resulted in the correct diagnosis of a benign schwannoma. Review of the needle aspirate demonstrated cytologic features that should enable both the correct diagnosis of this neoplasm and its distinction from pleomorphic adenoma, which it mimicked in this location.     

A cell line from pleomorphic adenoma of the rat salivary gland

Pleomorphic adenoma of rat salivary gland is extremely rare, and culture of cells obtained from rat salivary gland tumors has not been reported. We have established a long-term cell line from a pleomorphic adenoma of a Sprague-Dawley rat submaxillary gland. The pleomorphic adenoma was composed of oval or spindle-shaped cells occasionally forming a small duct. Alcian blue-positive intercellular matrices, consisting mainly of glycosaminoglycans, were abundant. The cultured cells showed characteristics similar to those of the original tumor. This cell line should be useful for biological and biochemical studies of glycosaminoglycan-synthesis of pleomorphic adenoma cells.     

An unusual case of recurrent left atrial myxoma.

In a patient who had a calcified left atrial myxoma resected, recurrence developed 31 months later. Although complete radical resection of the recurrent tumour presented a special problem, the patient survived the second operation. The tumour recurred again and the patient had two episodes of cerebral embolism 1 1/2 and 2 years later, respectively, and died 3 1/2 years after the second operation. The erythrocyte sedimentation rate correlated with the size of the tumor, and the recurrent tumour seemed to grow more rapidly than the primary tumour. Experience with this case and a review of the nine reported cases of recurrent left atrial myxoma suggest that a radical approach is necessary at the primary operation.     

Interferon-Induced Hypothyroidism Causing Reversible Pituitary Enlargement

ObjectiveTo present the first reported case of interferon-induced hypothyroidism with radiographic confirmation of secondary pituitary hyperplasia.MethodsWe describe the case of a woman with recurrent malignant melanoma, outline the details of her endocrine work-up, and illustrate the serial findings on magnetic resonance imaging of the head.ResultsA 26-year-old woman underwent surgical excision of a melanoma of the left thigh and 10 years later had a second melanoma removed from her right knee. Metastatic work-up revealed evidence of tumor involvement in the cervical and mediastinal lymph nodes. After treatment with interferon for 1 year, persistent fatigue and menstrual irregularities led to the laboratory diagnosis of hypothyroidism, and magnetic resonance imaging revealed pituitary enlargement. Both her endocrinopathy and the pituitary hyperplasia resolved with discontinuation of the interferon treatment and with institution of thyroid replacement therapy.ConclusionClinicians should be aware of the potential adverse effects of interferon therapy to avoid inappropriate diagnosis of a pituitary adenoma or metastatic lesion in patients with cancer who are treated with interferon. In addition, screening for hypothyroidism should be performed in patients receiving interferon. (Endocr Pract. 2008;14:219-223)