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Primary mucinous adenocarcinoma of thyroid gland
Abstract
A mucin-producing carcinoma in the thyroid gland found in a 44-year-old man was first thought to be a metastatic carcinoma, possibly from salivary gland. However, follow-up examinations for 8 years have not demonstrated another neoplasm, and it seems reasonable to conclude that this lesion was a rare primary mucinous adenocarcinoma of the thyroid gland.
... 6,7 Several cases of stromal mucin-producing thyroid neoplasms have also been reported. [8][9][10][11][12][13][14][15][16] We report here on two additional cases of follicular adenoma with thick capsule and extensive stromal mucin deposits. Importantly, this is the first time that BRAF gene mutation analysis is performed in these types of lesions. ...
... The main differential diagnosis included the mucin-producing papillary carcinoma, medullary carcinoma, and mucinous carcinoma. 8,12,17,18 Mucin-producing medullary carcinoma can be distinguished from the present two cases based on the absence of amyloid, the positivity for thyroglobulin, and negativity for CEA and calcitonin. However, the cytological overlapping features such as abundant mucin, frequent nuclear inclusions, and intravacuolar globules with violaceous-colored magenta bodies of the target-like cytoplasmic vacuoles under Diff-Quik stain, prompted a differential diagnosis with mucinproducing papillary carcinoma. ...
... 24 Mucin can be seen in a wide spectrum of primary thyroid tumors including follicular neoplasms, medullary carcinoma, poorly differentiated carcinoma, papillary and undifferentiated carcinomas, and mucoepidermoid and primary mucinous carcinomas. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16]19,25,26 A mucincontaining thyroglossal duct cyst in the thyroid has also been described in the literature. 27 Most of the mucin of these tumors are of intracellular location and a large amount of mucin can cause a signet-ring appearance in signet-ring cell adenoma of the thyroid gland. ...
We report two cases of follicular adenoma of the thyroid with extensive extracellular mucin deposition. Fine needle aspiration in Case 1 showed singly discohesive polygonal cells in a granular mucinous background. They contained abundant eosinophilic cytoplasm, nuclear irregularities, and frequent nuclear inclusions with occasional bizarre mitoses. A right lobectomy was done. In Case 2, a 47-year-old Caucasian woman with multinodular goiter had total thyroidectomy and a yellow-tan nodule was found within the right lobe. Both tumors were well-encapsulated masses with thick capsules. Each was characterized by microfollicles without papillae in a mucinous stroma. Tumor cells were positive for thyroglobulin and negative for calcitonin, CEA, galectin-3, HBME-1, and CK19. The extracellular mucin stained with Alcian-blue and colloidal iron but not with mucicarmine and D-PAS. No BRAF gene mutation was detected. Because there were neither capsular nor vascular invasions, both cases were diagnosed as follicular adenomas of the thyroid with extensive extracellular mucin deposition, which as proposed by the WHO classification can be categorized as a mucinous variant of follicular adenoma. Retrospectively, frequent nuclear inclusions and the absence of nuclear grooves in the mucin-containing background of cytologic smears and histologic sections were shared by those of mucin-producing papillary carcinoma. It is unclear whether it belongs to an existing category of thyroid neoplasm with mucin production or whether it is truly a new tumor variant. Furthermore, pathologists should pay attention to avoid misdiagnosis of this variant of follicular neoplasm that shows an overlapping cytology with that of papillary carcinoma.
... However, several primary thyroid tumors have been reported to be thyroglobulin-negative or weak- ly positive. 3,5,6,8,24 It is possible that thyroglobulin production decreases as the follicular cells gain mucinous differentiation. Furthermore, it has been suggested that oncocytic tumors of the thyroid produce only small amounts of thyroglobulin. ...
... The histogenesis of mucin-producing neoplasms of the thyroid gland has been a debated and is a controversial issue. Diaz-Perez et al 5 have suggested that the mucinous thyroid tumor they described could have originated from thyroglossal vestiges, because thyroglossal cysts or sinus are lined by ciliated pseudostratified respiratory type cells, squamous cells, and mucus-secreting columnar cells. Rhatigan et al 8 speculated that salivary gland remnants trapped in the developing thyroid were responsible for their case of mucoepidermoid cancer. ...
We report a carcinoma that is, to the best of our knowledge, the first case of a mixed mucus-secreting and oncocytic carcinoma of the thyroid. We also describe the histochemical, immunohistochemical, and ultrastructural features of this tumor. A 59-year-old man complaining of severe bone pain and weight loss underwent clinical and radiologic investigations. The studies revealed a nodule in the left thyroid lobe that was “cold” by 131I scintiscan and multiple lytic lesions of the skeleton that showed increased uptake by 99mTc-Sestamibi scintiscan. Left hemithyroidectomy was performed and the surgical specimen contained a well-circumscribed nodule of 3 cm in the greatest diameter. Light microscopy showed an oncocytic carcinoma with an area of glandular and papillary proliferation of mucin-producing cells. A double histochemical approach (Alcian blue–periodic acid–Schiff and Alcian blue–high-iron diamine) combined with ultrastructural investigation confirmed the presence of true mucus, ruling out the presence of breakdown products of thyroglobulin. Ultrastructural and immunohistochemical studies, together with clinical findings, excluded a possible metastatic origin of the mucin-producing component.
... El carcinoma mucinoso primario de la glándula tiroides fue descrito por primera vez por Díaz Pérez y col. (6) en 1976, siendo extremadamente raro y la revisión de la literatura reveló solo ocho casos de los que se tiene conocimiento. (7) Generalmente en estos reportes, el tumor aparece caracterizado por un charco de mucina alrededor de las trabéculas o grupos de células tumorales que generalmente muestran núcleos grandes y nucléolo prominente, a menudo extracelular y mezclado con áreas de carcinoma típicode tiroides. ...
... (5,8) En estudios realizados se ha podido apreciar que la mayoría de los pacientes con diagnósticos anatomopatológicos de carcinoma mucinoso primario tienen un peor pronóstico que el carcinoma tiroideo común. (6,9) Algunos investigadores han señalado que la terapia de supresión de TSH puede ser una ISSN 2221-2434 ...
Mucinous thyroid carcinomas are very rare and in some cases may appear in association with other tumors, but it does not represent a true primary thyroid carcinoma. A case of a 66-year-old white-skinned patient of rural origin is presented with a history of suffering from ischemic heart disease and arterial hypertension, for which she was undergoing medical treatment. He went to the office with an increase neck volume in the anterior region for several months, accompanied by malaise, gallop rhythm and sometimes dysphagia to solid foods. On the physical examination a tumor was found which occupied almost the entire right lobe, corresponding to a nodule of approximately 4 cm. in diameter, mobile with swallowing. The objective of this work is to present the surgical treatment performed to a patient affected by a thyroid gland mucinous carcinoma. Due to the low frequency of this entity, its publication is considered of interest to scientific staff.
... Primary mucinous carcinoma of the thyroid gland is extremely rare, and only eight cases have been reported in English-language literature, to our knowledge. [1][2][3][4][5][6][7][8][9] It was first described by Diaz-Perez et al 2 in 1976. The histologic hallmark of this kind of tumor is small nests, trabeculae, or sheets of epithelial cells, which appear suspended in pools of abundant extracellular mucus. 1 Histologically, it resembles the mucinous carcinoma of other sites, such as the breast, colon, stomach, and pancreas. ...
... gov) revealed only eight cases reported to our knowledge. [1][2][3][4][5][6][7][8][9] Because of the rarity of this thyroid carcinoma, we discuss this case in the context of a literature review of previously reported cases (summarized in Table 1). The patients were aged 32-82 years, and there was no sex predominance. ...
Purpose
This study reports a case of primary mucinous carcinoma of the thyroid gland with signet-ring-cell differentiation, and reviews the literature to evaluate its real incidence and the prognosis of these patients.
Patients and methods
A 74-year-old Chinese woman, presenting with a mass in the right lobe of thyroid gland, came to the hospital. Computed tomography revealed a mass in the right lobe of the thyroid gland, accompanied with right neck lymphadenectasis and airway deviation caused by tumor compression. Thyroid imaging suggested a thyroid malignant tumor and suspicious lymph node metastasis. Histologically, the tumor was characterized by the tumor cells arranged in small nests or trabeculae with an abundant extracellular mucoid matrix. The tumor cells formed diffuse invasion among thyroid follicles. In the peripheral regions, prominent signet-ring-cells formed a sheet-like structure and extended into the extrathyroidal fat tissue. The tumor cells were diffusely positive for thyroid transcription factor-1 (TTF-1) and PAX8, while they were focally positive for pan-cytokeratin (AE1/AE3) and weakly expressed thyroglobulin.
Results
Based on the histological features and immunohistochemical profile, a diagnosis of primary mucinous carcinoma of the thyroid gland with signet-ring-cell differentiation was rendered.
Conclusion
Using a panel of immunohistochemical markers may be helpful for differential diagnosis and for determining whether the tumor is primary or not.
... Primary mucinous carcinomas of the thyroid gland are extremely rare. Since the first report published by Diaz et al. in 1976, only 6 such case reports were identified during our search of the PubMed database [3][4][5][6][7][8]. Furthermore, primary mucinous carcinoma of the thyroid gland with rhabdoid cells has never been previously reported. ...
... Lymph node metastasis was detected at the initial diagnosis in a high proportion of cases (6 of 7 cases). Furthermore, distant metastases developed in 3 out of 7 cases, and most patients died of the disease (5 of 7 cases); those who underwent incomplete resection died within a few months, demonstrating a poor prognosis [3][4][5][6][7][8]. Therapeutic methods for recurrence included surgery, iodine therapy, radiation therapy, and anticancer drug therapy, but none of these were reported to be effective [4][5][6]. ...
Background
Primary mucinous carcinoma of the thyroid gland is a rare disease; only 6 cases of primary mucinous carcinoma of the thyroid have been previously reported. Primary mucinous carcinoma of the thyroid gland with incomplete tumor resection tends to be associated with a poor prognosis, resulting in death within a few months. An early and appropriate diagnosis may contribute to improvement in patient prognosis; however, it is extremely difficult to diagnose primary mucinous carcinoma of the thyroid. We present the seventh reported case of primary mucinous carcinoma in the thyroid gland; moreover, rhabdoid cells were detected, which, to our knowledge, is a novel finding.
Case presentation
An 81-year-old Japanese woman was initially diagnosed with a poorly differentiated thyroid carcinoma, and she underwent a hemithyroidectomy. Pathological examination revealed the presence of abundant mucus and agglomeration of large atypical cells. Rhabdoid cells were also seen scattered among the tumor cells. Immunostaining was performed for various markers, and on the basis of these results, we diagnosed the lesion as primary mucinous carcinoma with rhabdoid cells in the thyroid gland. Ten months after surgery, recurrence was noted in the paratracheal lymph nodes; therefore, total resection of the residual thyroid gland and paratracheal lymphadenectomy with thyroid-stimulating hormone suppression were performed. The patient is currently alive and disease-free.
Conclusions
The current case is of interest not only because of the rare histological findings, but also because the patient achieved long-term survival following diagnosis of a mucinous carcinoma. We believe this report will be helpful for diagnosing future cases of mucinous carcinoma of the thyroid.
... The follicular thyroid neoplasms with signet ring cell morphology are, in fact, a variant of a follicular adenoma or carcinoma with an intracytoplasmic deposit of substances [9,10,12]. In 1871, Mueller [16] first reported the appearance of mucinous substances in a thyroid tumor; later, Diaz-Perez in 1976 [17] reported a case of mucinous thyroid carcinoma composed by few cells with intracytoplasmic mucin. In 1984, Mendelsohn first reported signet ring cells in a follicular neoplasm, a microfollicular thyroid adenoma, and differentiated it from mucinous neoplasms [18]. ...
... Initially, it was thought that thyroid tumors with mucinous differentiation might be originating from intrathyroidal thyroglossal ducts or thyroid cysts remnants, probably lined by intestinal epithelium capable of synthesizing mucin [17,18], or even from rests of ectopic salivary glands [18]. This was based in the hypothesis of signet ring cells as ectopic elements of large mucin production, in an attempt to make an analogy with gastrointestinal neoplasms that present similar morphology. ...
Thyroid follicular neoplasms with signet ring cell morphology represent a challenging cytological and histopathologic diagnosis. The low frequency of these neoplasms and their broad differential diagnosis contribute to this difficult scenario. Here, we present an exceptionally rare case of thyroid follicular carcinoma with signet ring cell morphology in a 62-year-old female. We analyze the characteristics in fine-needle aspiration cytology, histopathology, and immunohistochemistry and compare our results in a discussion with previous literature reports.
... Primary mucinous adenocarcinoma of the thyroid gland was reported for the first time in 1976 (3). There are other examples in the literature (1,2), some of which showed morphologic features of mucoepidermoid carcinoma (6, 7). ...
... Our case is the second mucin-producing adenoma reported. The presence of mucin in thyroid tumors has been variously interpreted (3,7). In our opinion, the presence of mucin in these tumors is a particular differentiative moment at which follicular epithelium per se is able to secrete carminophilic material. ...
The authors studied a case of mucin-producing adenoma of the thyroid gland. The tumor consisted almost entirely of signet-ring cells containing mucin, which was strongly positive with PAS, with and without diastase pre-treatment, and Alcian blue stain at pH 2.5. Immunoperoxidase staining for thyreoglobulin was clearly positive within the cytoplasm of signet-ring cells and also in the follicle material, which indicates that the tumor derived from follicular epithelium.
... Given the rarity of this type of thyroid cancer, it is interesting to note the geographic distribution and timeline of documented cases. In 1976, Diaz-Perez et al documented the first known case in Michigan, US. 6 In 1980, 2 additional cases were identified in New York, US, by De- ligdisch et al. 7 Over 30 years later also in New York, Yang et al reported a case of mucinous carcinoma. 8 We present the first documented US case of primary mucinous carcinoma in over a decade. ...
... Mucinous thyroid carcinoma is an extremely rare malignant tumor; Only 10 cases have been described in detail 1,2 with a diagnostic time that ranged from 2 months to 2 years in patients aged 32-82 years (mean 63.1) without a predominance between the sexes 3 . It was first described by Díaz-Pérez in 1976 4 , as a tumor that presents nests, trabeculae, and sheets of epithelial cells at the histological level with abundant deposits of intra and extracellular mucin in areas where neoplastic cells are identified 5 . Its evolution is not well defined, so it can have a rapid or slow growth, sometimes presenting painful, "cold" thyroid nodules, with or without palpable regional lymph nodes 1,3 . ...
Mucinous carcinoma of thyroid is very rare. We report the 11th case of a mucinous thyroid carcinoma in the reviewed literature. A 68-year-old woman with a history of two types of breast cancer histologically different from each other and unrelated to mucin lakes develops this infrequent entity. By histology, it was compatible with mucinous carcinoma of the thyroid with signet ring cells, with no associated tumors to be considered a possibility of metastases, for which we report it as primary. Only 10 cases have been reported in the literature since its first finding in 1976.
... The mucinous material stains positively with several histochemical stains including PAS (with and without diastase), the iron diamine method, mucicarmine, and Alcian blue (pH 2.5) [87][88][89] (Fig. 7b, e, and g). The presence of mucin antigens (MUC1, MUC2, MUC3, and others) alone does not specify this diagnosis. ...
Thyroid pathology encompasses a heterogenous group of clinicopathological entities including rare and diagnostically challenging neoplasms. The review is focused on morphological, immunohistochemical, and molecular features of rare thyroid neoplasms that can pose diagnostic problems. The tumors are organized based on growth patterns including thyroid neoplasms with predominantly papillary, follicular, solid, and spindle cell growth pattern, as well as neoplasms with distinct cytological characteristics. A special section is also dedicated to rare thyroid tumors with peculiar patterns including thyroid carcinoma with Ewing family tumor elements and intrathyroidal thymic-related neoplasms.
... MEC is a rare primary thyroid tumor with indolent MEC is usually a malignancy of the salivary glands, although it has been reported in other locations. [5][6][7][8][9][10] In 1991, Chan et al. The most common presenting symptom is a painless neck mass or cold nodule on thyroid scan. ...
Rarely, salivary gland tumors such as mucoepidermoid carcinoma, mammary analogue secretory carcinoma and mucinous carcinoma arise as primary tumors from ectopic or metaplastic salivary gland tissue adjacent to or within the thyroid gland. We report for the first time a case of primary salivary acinic cell carcinoma (AcCC) adjacent to the thyroid gland in a 71-year-old female patient with Crohns disease and a previous history of malignant melanoma. Following the development of a nodule adjacent to the left thyroid lobe, a fine-needle aspiration biopsy was reported as consistent with a follicular lesion of undetermined significance (Bethesda III). A left-sided hemithyroidectomy was performed. A circumscribed lesion measuring 33 mm was noted adjacent to the thyroid and trapping parathyroid, it was composed of solid nests and glands with microcystic and follicular patterns. The tumor was negative for thyroid, parathyroid and paraganglioma markers, but positive for pan-cytokeratins, CK7, CD10, CD117, androgen receptor and HNF-beta. A metastasis of a thyroid-like renal cell carcinoma was suspected but ruled out, and the patient had no evident lesions on extensive radiology of the urogenital, pulmonary and GI tracts. Based on the morphology, a diagnosis of AcCC was suggested, and confirmed with DOG1 and PAS-diastase staining. Molecular analyses pinpointed a constitutional ASXL1 variant of uncertain significance, but no fusion events. The patient had no radiological or clinical evidence of parotid, submandibular or sublingual tumors postoperatively, and the excised lesion was therefore assumed to be a primary tumor. We here detail the morphological and immunophenotypic profile of this previously undescribed perithyroidal tumor.
The thyroid and parathyroid chapter of the Head and Neck book gives a broad overview from the most frequent to the rarest diseases of these endocrine glands. Considerations on epidemiology, etiopathogenesis, morphology, and resumed clinical management are provided for each disease, as well as some of the most up-to-date information on molecular markers. Exhaustive photographic documentation is provided for each disease, including macroscopic and microscopic images, as well as recent bibliographic references to guide further readings.
The rare reports of mucinous variant of follicular carcinoma of the thyroid gland have not provided enough evidence to support the recognition of these tumors as a distinct clinicopathologic entity or to understand their etiopathogenesis. We report the fourth case of mucinous variant of follicular carcinoma displaying a minimally invasive tumor with diffuse expression of thyroglobulin, TTF-1, CD56, PAX-8, cytokeratins 7 and 19, in the absence of monoclonal carcinoembryonic antigen (CEA), cytokeratin 20, chromogranin, HBME-1, P63 expression, and BRAF gene mutation, in a 51-year-old woman who is alive without signs of disease 13 months after total thyroidectomy, bilateral neck dissection, and radioactive iodine. Herein, fine-needle aspiration cytology disclosed "worrisome" cytologic features consisting of large epithelial cells arranged in clusters or singularly, with high nucleocytoplasmic ratio, nuclear grooves and evident nucleoli which were shared by those of mucin-producing papillary thyroid carcinoma. Therefore, knowledge of the cytological and histopathological spectrum of this lesion is important to avoid misdiagnosis. The morphologic clues leading to the correct diagnosis of mucinous variant of follicular neoplasm have been correlated with the data of the literature, and the differential diagnosis is briefly discussed.
Lymphomatoid granulomatosis (LYG) is a rare B-cell lymphoproliferative disorder predominantly involving the lungs, but poorly-recognized among clinicians and pathologists. It is an Epstein-Barr virus (EBV)-driven disease mimicking several other diseases on clinical and radiological grounds, generally showing multiple, bilateral nodular, ill-defined infiltrates of the lungs tending to coalescence and/or cavitation. LYG often affects middle-aged males with an underlying immunodeficiency and commonly involves skin and central nervous system during disease progression. Diagnosis requires a generous biopsy and careful histologic examination with immunohistochemical staining and molecular demonstration of EBV genome in large atypical B-cells. LYG is graded as I to III based on the number of large EBV-positive B-cells; grades II/III are now considered as a peculiar variant of T-cell rich diffuse large B-cell lymphoma. In this brief review, clinical, radiologic and pathologic features of LYG will be analyzed with focus on differential diagnosis, the most appropriate treatment and prognosis.
Cancers of the thyroid represent the most common endocrine malignancies. Papillary and follicular carcinomas represent the majority of thyroid cancers but many others exist. These range from low-grade malignancies to highly aggressive cancers. Also, the thyroid is occasionally the site of metastasis from other malignant primaries. Herein, we discuss the uncommon cancers of the thyroid, their diagnosis, and treatment.
An extremely rare mucoepidermoid carcinoma of the thyroid gland was found in a 44-year-old woman. By light microscopic examination, the tumor consisted of mucous cells and squamoid cells, and was similar to that occurring in any other organs. By electron microscopic examination, the mucous cells displayed prominent mucous droplets, and showed no evidence of colloid production in their cytoplasms. Abundant tonofilaments in aggregates were observed in the squamoid cells. Biochemically, thyroxine (T4) and triiodothyronine (T3) were not detectable in the tissue of this tumor. These findings indicate that this mucoepidermoid carcinoma does not retain the functional properties of thyroid follicular cells; namely, no evidence was found to substantiate a follicular epithelial origin of this tumor.
A follicular carcinoma of the thyroid with extensive mucin production was found in a 54-year-old woman. The primary nature of this rare neoplasm of the thyroid gland is supported by the presence of both colloid and mucin-producing follicles. Ultrastructural features confirm the secretion of colloid and mucin in the same neoplastic thyroid cells.
A case of primary hemangiosarcoma of the spleen in a 48-year-old woman is presented. Twenty-eight months after splenectomy the patient developed a severe anemia of the microangiopathic type, thrombocytopenia, and a leukoerythroblastic peripheral blood picture. In contrast to x-ray and conventional 99mTc-methylene-diphosphonate (MDP) bone scintigraphy, which showed only a few minor focal changes in the spine and ribs, angioscintigraphy with in vitro labeled 99mTc-erythrocytes revealed extensive pathologic accumulations throughout the spine, femurs, and the liver, indicating the presence of extremely vascular metastases. Autopsy 15 months later confirmed the scintigraphic findings. Angiography with 99mTc-labeled erythrocytes seems to be useful for monitoring metastases from hemangiosarcomas.
Solid cell nests (SCN) are found within the thyroid parenchyma on routine clinical pathological examinations, but their histogenesis and clinical significance are still obscure. From November 1987 to May 1991, a total of 3,260 patients underwent thyroid surgery at Ito Hospital and SCN were noted in 42 (1.3%) patients. Serial sections of the specimens from these patients were studied morphologically and immunohistochemically. The location and growth pattern of SCN within the thyroid parenchyma were distinct from those of hyperplastic C-cells. SCN were located in the isthmus lobe in 1 patient and even in the pyramidal lobe in 3 patients. In another patient neoplastic proliferation of SCN was observed. Immunohistochemically, SCN were negative for neuron-specific enolase, chromogranin A, and S-100 protein. These findings strongly suggest that SCN are of endodermal origin and that they may be closely related to mucoepidermoid carcinoma of the thyroid gland.
Mucoepidermoid carcinoma of the thyroid (MECT) has been recently recognized as a pathological entity. The origin of MECT is unknown but the morphology of this tumour closely resembles features seen in the ultimobranchial body (UB) vestiges. Recent studies in man have shown strong evidence that the so-called solid cell nests (SCN) of the thyroid may correspond to the human UB vestiges. To investigate whether these vestiges are the site of origin of this tumour a comparative study on SCN and MECT was undertaken. One hundred autopsied thyroids cut at 2-3 mm intervals were studied for the presence of SCN. Histochemical (H & E, Alcian blue-PAS, Mayer mucicarmine) and immunohistochemical studies (calcitonin, epidermal keratin) were performed in SCN and four cases of MECT. Sixty percent of thyroids were found to have SCN. They were mainly composed of epidermoid-like cells arranged in solid structures or lining cystic cavities, tubular and follicular structures. Solid clusters usually showed lumina containing PAS-positive and mucin-positive cell debris. Mucin stains also revealed mucinous cells placed around lumina filled by mucosubstances. Characteristic PAS-positive rounded bodies were found filling lumina as well as within some apical epidermoid-like cells, mucinous cells and cell debris. An obvious transition between these cells, cell debris and mucosubstances filling the lumina was noticed; suggesting degenerative changes undergone by the epidermoid-like cell. MECT basically presented all histological and histochemical features shown by SCN, furthermore, calcitonin containing cells were observed in 54% of SCN, while a metastatic MECT also showed scattered C cells within solid islands. The presence of epidermal keratin in all SCN and MECT, together with the previous findings, are strong evidence that MECT could originate in the SCN or human UB vestiges.
This study was carried out to investigate the fate of the ultimobranchial contribution to the thyroid in man when the thyroid lobe is not fully descended. Thyroid abnormalities were recorded in 41 of 29000 autopsies at the London Hospital—a prevalence of 1·41/1000. The 18 major abnormalities included four cases of lingual thyroid, in two of which cystic structures were noted in the neck in the region of the upper parathyroid. Three further cases of lingual thyroid with upper parathyroid cysts were collected from other sources.
The multilobulated cystic structures were up to 1·5 cm in diameter and showed a variety of histologic features. In four cases there were glandular nodules of up to 1 mm across lying in the intercystic connective tissue. These nodules showed solid areas of irregularly distributed cells that stained positively for calcitonin and calcitonin gene‐related peptide, intermingled with, and sometimes apposed to, follicular structures that showed follicular cells and colloid that stained for thyroglobulin.
This study has provided conclusive evidence that the ultimobranchial body contributes both C cells and follicular cells to the thyroid in man, has documented the prevalence of congenital defects of thyroid development, and has described the human ultimobranchial gland as showing a wide developmental potential which may have implications in the histogenesis of thyroid neoplasia.
One hundred forty-two cases of thyroid carcinomas were studied to assess the frequency with which mucinous deposits occur within the neoplastic cells. In contrast to the literature, which contains only a few scattered reports on this topic, the production of mucin by carcinomas of the thyroid was relatively frequently observed in our material. Almost one half of the cases displayed mucinous substances, which occurred in about 50% of the papillary and the medullary carcinomas, 35% of the follicular types, and about 21% of the anaplastic varieties. The amount of mucinous substances varied considerably: 28 cases were weakly positive, 30 cases were moderately positive, and 6 cases were strongly positive. These findings are discussed with respect to diagnostic and differential diagnostic problems, especially with respect to the interpretation of mucinous deposits that occur in metastatic carcinomata.
Although textbooks often state that demonstration of mucin in a metastatic carcinoma excludes the possibility of a thyroidal primary tumor, mucin recently has been reported in various types of thyroid carcinoma, particularly medullary carcinoma. The presence of mucin in papillary carcinoma has not been extensively studied, even though this tumor not uncommonly presents with lymph node metastasis. We stained 40 lymph nodes containing metastatic papillary carcinoma of the thyroid for mucin. Mucin was demonstrable by mucicarmine stain in the colloid, luminal borders, and cytoplasm in 18 (45%), 9 (22.5%), and 7 (17.5%) cases respectively; 17 cases (42.5%) were completely negative. With alcian blue staining, mucin was seen in 9 (22.5%), 9 (22.5%), and 7 (17.5%) cases; 25 cases (62.5%) were negative. Most of the cytoplasmic vacuoles were target-like, with a peripheral rim of sulfated acid mucin and a central core of neutral mucin. The psammoma bodies stained consistently with mucicarmine, alcian blue, and periodic acid-Schiff. We conclude that papillary carcinoma of the thyroid should be included in the differential diagnoses for a mucin-producing metastatic carcinoma.
Forty primary carcinomas of the thyroid of different histological types were reviewed and studied histochemically, with the aim of identifying and assessing "mucin secretion". The patterns of extracellular "pure alcianophilia" and "mixed alcianophilia" were noted in 7.5% and about 50% of these tumours, respectively. A critical review of the pitfalls in methods and interpretation of mucin histochemistry--as performed in previously reported cases of "mucin secreting" or "mucinous" primary thyroid tumours--is presented. The apparent "mucin secretion" described in these unusual neoplasms could be due to histochemical staining of carbohydrate components or breakdown products of thyroglobulin and colloid.
An unusual case of a mucin secreting benign microfollicular adenoma of the thyroid in a 30 year old euthyroid woman is reported. Histologically, the lesion was characterised by follicular cells with the appearance of signet ring cells. Histochemistry showed the mucin content of these cells to consist uniformly of sulphated acid mucins; positive thyroglobulin immunostaining was also shown. The published work on primary mucin secreting tumours of the thyroid gland is reviewed. Dual differentiation is thought to be responsible for combined mucin secretion and hormone production in this type of neoplasm.
A case of thyroid carcinoma in a 38-year-old male with a high serum level of calcitonin is reported. The tumor was composed of polygonal argyrophilic cells, signet ring Alcian blue-positive cells, and a minority of amphicrine elements. The same polymorphic cellular component was seen at the ultrastructural level. Anticalcitonin immunoperoxidase revealed that the majority of cells were positive, including the amphicrine cells. In spite of calcitonin production it is felt that cases similar to the present one should be distinguished from "ordinary medullary" carcinoma and the terms "amphicrine" or "composite calcitonin and mucin-producing carcinoma" are proposed.
Clinical and morphological features of three cases of primary mucoepidermoid carcinoma of the thyroid are described. The tumours were composed of two cell types. One of these resembled squamous epithelium and ultrastructurally showed tonofilaments and numerous desmosomes. The other cell type contained Alcian blue and mucicarmine positive mucin and, on electron microscopy, showed mucigen granules. Marked stromal fibrosis and psammoma bodies were seen in all tumours. Immunohistochemical studies showed that the tumour cells were negative for thyroglobulin. A few calcitonin-containing cells were seen in one metastatic tumour. One tumour showed, in addition to the histological features of mucoepidermoid carcinoma, anaplastic areas with obvious transition between the two histological patterns. The same thyroid also had a small thyroglobulin-positive papillary carcinoma in the opposite lobe. All tumours presented lymph node metastases. In two cases the primary tumour was confined within the thyroid capsule but that with anaplastic areas invaded surrounding structures. This patient died 13 months after diagnosis; the other patients are alive and symptomless one and 10 years since diagnosis. Mucoepidermoid carcinoma of the thyroid appears to be a clinicopathological entity that resembles papillary carcinoma in its natural history. The origin of the tumour is unclear. There is, however, some histological and immunohistological data suggesting that the tumour might be related to the ultimobranchial system although some histological features also appear to favour a common origin with papillary carcinoma.
A case of medullary carcinoma of the thyroid with the presence of mucus-secreting (goblet) cells was reported. Ultrastructurally, the tumor was composed of two types of cells, one with neurosecretory granules and the other with mucus granules. The secretion of calcitonin and mucus was demonstrated by histochemical methods. The presence of two cell populations in this tumor casts doubts on the "APUD" concept, which proposes a neuroectodermal origin for parafollicular (C) cells of the thyroid gland. The significance of this tumor with regard to the histogenesis of the cells involved, is discussed.
Forty-four thyroid carcinomas with an insular component (IC) were reviewed from 2457 tumors diagnosed as papillary (PC) or follicular carcinoma (FC). These tumors were classified as FC with an IC (FCIC; 30 cases) and PC with an IC (PCIC; 14 cases). Both tumors were composed of solid cell nests in some areas and had a tendency toward a characteristic nuclear size: FCIC had a small nucleus and PCIC contained a nucleus of an intermediate type or a large nucleus similar to that of PC, although there were numerous tumors with an exceptional nuclear size. The mean age and tumor diameter were the highest and largest in FCIC, respectively, followed by PCIC. Among the 44 cases, 17 patients died of the disease, two were alive with the disease and 18 were alive without the disease. From 13 clinicopathological factors, the presence of an IC, age, non-encapsulation, tumor size, vascular invasion and necrosis were found to be independent variables for actual prognosis of FC and PC based on univariate analysis followed by multivariate analysis. The results of the present study indicate that the presence of an IC is an independent aggressive prognostic factor for patients with PC and FC.
The aim of the study was to report a recent case of mucoepidermoid carcinoma (MEC) of the thyroid, review the pertinent literature, and highlight the importance of multimodal treatment in this potentially aggressive malignancy.
The study comprise a case report and a review of literature.
A 42-year-old woman had initially presented to another hospital with a painful left neck mass. She subsequently underwent left hemithyroidectomy. Surgical pathology was significant for lymphocytic thyroiditis and MEC. One month later, she presented to us with a right neck mass.
The patient underwent completion thyroidectomy and selective neck dissection, including level 6, for high-grade MEC. Operative findings included 3 lymph nodes positive for metastasis and tumor invasion of the transverse cervical vein. Postoperative chemoradiation therapy was administered in 2 cycles separated by 1 month. The patient remains disease-free 22 months after the last treatment.
Mucoepidermoid carcinoma is a rare malignancy of the thyroid with less than 35 cases reported to date. The origins of this tumor are not completely understood, with debate regarding whether it arises from solid cell nests of the ultimobranchial apparatus or from follicular epithelium. Although MEC of the thyroid has frequently been reported as low grade, the aggressive presentation in our patient suggests the appropriateness of multimodal therapy.
We report on a case of mucinous carcinoma of the thyroid in an 82-year-old Japanese woman. The 3 x 2 x 2-cm thyroid tumor located in the patient's right lateral lobe was soft and yellowish gray on the cut section. Microscopically, the tumor was composed entirely of strands or solid clusters accompanied by extensive extracellular mucin. Extracellular mucin was positive with Alcian blue stain and negative with periodic acid-Schiff stain. Immunohistochemically, the tumor cells were positive for thyroglobulin, thyroid-specific transcription factor 1, and low-molecular-weight cytokeratin but negative for carcinoembryonic antigen, calcitonin, and high-molecular-weight cytokeratin. The MIB-1 labeling index was rather high (11%), and immunoexpression of p53 was detectable in the nuclei of carcinoma cells. From these findings, we classified the present tumor as a mucinous carcinoma of the thyroid (poorly differentiated thyroid carcinoma producing massive extracellular mucin). The patient died of multiple metastases 4 years after the initial operation.
A thyroid tumor with extensive extracellular mucin deposition is extremely rare. We herein describe a case of a thyroid adenoma with prominent myxoid stroma. A 63-year-old man presented with a mass in his right anterior neck. Radiological examinations showed this mass to be a thyroid tumor with a cystic component. The histopathological findings showed the stroma of this tumor to consist of abundant myxoid materials which stained gray-bluish for hematoxylin-eosin. The myxoid material was positive for alcian blue, whereas periodic acid-Schiff did not stain this material. No intracytoplasmic mucin was identified. In an immunohistochemical study, the tumor cells were negative for cytokeratin 19. Furthermore, positive staining was observed for thyroglobulin while a negative finding was seen for calcitonin.
Mucinous carcinoma of the thyroid gland is an uncommon tumour that from the histological point of view, resembles mucinous carcinoma of others sites. Although a mucinous appearance has sometimes been reported in association with cases of typical thyroid carcinoma, true mucinous carcinoma is exceptionally rare. We describe two cases of thyroid tumours with mucinous differentiation studied with immunohistochemistry. Both cases disclosed a similar histological appearance, with small nests and sheets of malignant epithelial cells associated with extensive extracellular mucin that substituted and entrapped the follicular parenchyma of the thyroid. Thyroglobulin and focally thyroid transcription factor (TTF) 1 were positive in one case. From these findings, we classified this tumour as primary mucinous thyroid carcinoma. Thyroglobulin and TTF-1 were negative in tumour cells of second case; on the contrary, positivity to the carcinoembryonic antigen and CA-125 was strong and generalized. However, successfully, the patient presented ascites associated to right ovarian mass. In this case, thyroid tumour represents the first clinical sign of an ovarian mucinous adenocarcinoma, and it has not been previously described in literature. Both patients died after few months to diagnosis. In conclusion, primary and secondary mucinous carcinoma are rare and unusual tumours of the thyroid gland that can be a cause of pitfall in differential diagnosis. In these cases, for a correct diagnosis, a complete clinical history, restricted histological criteria and immunohistochemical panel are necessary.
In the International Classification of Diseases all malignant tumours of a specific site are usually classified into one group using one code number. This is generally-done also in epidemiologic studies. It has been found, however, that the malignant tumours of most organs do not form a homogeneous group but are composed of many individual types which differ in their clinical course and epidemiology. For example, in the nodular sclerosing type of Hodgkin’s disease the age and sex distribution and the course of the disease are very different from those of the other histologic types of Hodgkin’s disease (Lukes et al., 1966, Franssila et al., 1967). This concurs with the hypothesis based on epidemiologic studies that Hodgkin’s disease is actually composed of two different diseases (e.g. Macmahon, 1966). It is also well known that adenocarcinoma of the lung does not show the same sex difference or the same correlation with smoking as epidermoid carcinoma.
In spite of a complex variety of microscopic patterns, thyroid carcinoma can be classified pathologically into four basic subtypes, each with a more or less predictable rate of growth, mode of spread, and expected outcome after treatment. These four subtypes are designated as papillary, follicular, medullary, and anaplastic. The name selected for each subtype is not intended to describe the total microscopic structure, but merely to call attention to one diagnostic facet of each type. Thus, papillary cancer, the common tumor among children and young adults, usually is composed of a mixture of papillations and neoplastic follicles. However, either of these structural patterns may dominate the microscopic picture, or the tumor may contain a large, solid component. The exact makeup of microscopic structure in this subtype, while of possible interest in terms of uptake of 131I, does not influence mode of spread, surgical treatment, or prognosis. Tiny papillary tumors arc extremely common, and they come to the attention of clinician or pathologist only when associated with palpable, metastatically involved lymph nodes or when discovered incidentally in a surgical or autopsy specimen. Follicular carcinoma is that subtype of well-differentiated thyroid carcinoma characterized by encapsulation, slow growth, and a tendency toward vascular invasion. Spread is via the bloodstream to lungs or bones, but cervical lymph nodes arc seldom inolved. Microscopically, papillary structure is not seen, but rather, various mixtures of solid and follicular pattern are identifiable; Hürthle cell variants are also included in this subtype. Uptake of 131I, important in the treatment of distant metastasis, depends on the extent of follucular differentiation in a given tumor. Tumors of medullary type, derived from parafollicular cells, are of undifferentiated microscopic structure and contain varying amounts of stromal amyloid. The tumors grow slowly and spread locally to cervical nodes and distantly to lungs or liver. “Anaplastic carcinoma” is the name applied to a subtype of undifferentiated tumors with a variety of microscopic patterns. All are characterized by microscopic evidence of rapid growth and a uniformly fatal prognosis.