Pathology of spiradenocarcinoma dr sampurna roy md. Benign eccrine spiradenoma genetic and rare diseases. Gves is very rare and differs from the eccrine spiradenoma in its larger size clinically and marked vascularity histopathologically. However, it rarely occurs as multifocal localized tumors or has zosteriform distribution. Malignant eccrine spiradenoma mes is an extremely rare sweat gland tumor that arises in a preexisting benign eccrine spiradenoma. Eccrine spiradenoma is a benign adnexal tumor of the sweat gland. A rare case of multiple segmental eccrine spiradenomas.
Overall, the lesion was a firm, tender, welldefined, blue nodule with a smooth surface and obscure boundaries. It is frequently found on the lower extremities, and it equally affects both sexes in the sixth and seventh decade. Spiradenoma lesions are benign sudoriferous tumors, and have also been described as cystic epitheliomas of. The presence of cylindroma and eccrine spiradenoma in one clinical nodule contributes circumstantial evidence for the similar derivation of both tumors. Eccrine spiradenoma and dermal cylindroma, journal of. Contribution to eccrine spiradenoma jama dermatology. Giant vascular eccrine spiradenoma is a rare variant of eccrine spiradenoma, characterized by its large size and florid vascularity. Eccrine neoplasms spiradenoma april 5th, 2011 spiradenomas are rare benign sweat gland tumors that usually occur on the chest, neck, face and scalp and i occasionally encounter them in both my the woodlands dermatology and conroe dermatology clinics. While eccrine spiradenoma is of eccrine derivation, the origin of cylindroma is controversial because of variable histochemical and enzyme histochemical findings. The latency period before transformation into malignant disease can range from 6 months to 70 years. Malignant eccrine spiradenoma genetic and rare diseases.
Siegel hj, saidalnaief n, long j, lopezben rr, klein m. Giant vascular eccrine spiradenoma gves is a variant of an eccrine spiradenoma. Traditionally these lesions have been categorized under either the apocrine or eccrine groups, but significant overlaps do exist. We present a malignant eccrine spiradenoma arising from a preexisting eccrine spiradenoma, which has an infrequent localization between lateral edge of nose and medial canthus. Billings, md key facts clinical issues typically present as plaque on scalp locally aggressive microscopic pathology infiltrative growth pattern, often with perineural invasion tumor composed of basaloid epithelium with uniform hyperchromatic nuclei tumor forms epithelial strands with lumen formation may have hand mirror shape similar to syringoma. Eccrine spiradenoma is an uncommon benign adnexal tumor originating from the eccrine sweat gland. A young male patient presented with multiple swellings on his chest and the nape of his neck. Eccrine spiradenoma es is an uncommon welldifferentiated benign tumor. Malignant eccrine spiradenoma mes is a rare malignancy of the eccrine sweat. The exact cause of malignant eccrine spiradenoma is unknown, though it is thought that sun exposure or problems with the immune system immunosuppression may contribute to the. Pdf on jan 1, 2016, sunilyogiraj swami and others published eccrine spiradenoma find, read and cite.
Tumours with overlap features with eccrine spiradenoma can be seen. Eccrine spiradenoma es, first described in 1956, is an uncommon, benign, dermal. There is frequently a more prominent population of s100 positive dendritic cells while in eccrine spiradenoma there is a lymphocytic infiltrate. Eccrine glands play an important role in the regulation of body temperature. The exact cause of malignant eccrine spiradenoma is unknown, though it is thought that sun exposure or problems with the immune system immunosuppression may contribute to the development. Jun 17, 2015 a young male patient presented with multiple swellings on his chest and the nape of his neck.
The prognosis for apocrine spiradenoma is excellent with suitable treatment. On histological examination a diagnosis of benign eccrine spiradenoma was made. Skin biopsy samples were diagnosed as benign adnexal neoplasm consistent with eccrine spiradenoma. There are nine cases in the literature with systemic metastases. Current reports suggest that the tumor predominates in. Malignant eccrine spiradenoma with carcinomatous and. Usually there is a history of increase in size of the mass, change in colour or ulceration of the overlying skin. An unusual case of congenital linear eccrine spiradenoma. Eccrine sweat glands are innervated mainly by terminal nerve fibers, which are expressed at low levels. Many consider it to be an eccrine neoplasm, and others favour apocrine differentiation. Medscape reference provides information on this topic. Eccrine naevi produce either a mucinous discharge or a localized area of excessive sweating hyperhidrosis, which can occur spontaneously or following provoking factors such as heat.
Smears showed clusters of round to plasmacytoid cells with moderate to abundant cytoplasm embedded in a chondromyxoid ground substance. View abstract siegel hj, saidalnaief n, long j, lopezben rr, klein m. Jun 17, 2015 eccrine spiradenoma is an adnexal neoplasm that continues to be designated as a tumor with eccrine differentiation, however, it is currently considered an apocrine process. Spiradenoma, also spiroma or eccrine spiradenoma, is a cutaneous condition that is typically characterized, clinically, as a solitary, deepseated dermal nodule of approximately one centimeter, occurring on the ventral surface of the body. Eccrine spiradenomas are uncommon, benign lesions, which are thought to originate from the eccrine sweat glands. Pubmed is a searchable database of medical literature and lists journal articles that discuss benign eccrine spiradenoma. In these cases, lesions combine features of both cylindromas and spiradenomas. The disease usually appears as solitary gray, purple, pink, red, or blue nodules on the upper half of the body. A literature search revealed 22 cases of multiple segmental eccrine spiradenoma. In our case, we present a 46 yearold female with a recurring exophytic tumor on the right lower extremity, without local extension.
Age and sex distribution apocrine spiradenoma is an uncommon condition that is seen in the age range of 2060 years. First case was a 20 year old female who presented with firm,nontender swelling in the left little finger measuring 1 cm in diameter. Malignant eccrine spiradenoma of the nasolabial fold. Gves is usually accompanied by bleeding andor ulceration, and of. Case report a 64yearold female patient was admitted to our department, with a 1.
Eccrine spiradenoma es is an uncommon tumor of eccrine sweat gland origin, occurring mostly in young adults. Most presentations of es are solitary, comprising more than 97 percent of cases. A rare case of eccrine spiradenomatreatment and management. Malignant eccrine spiradenoma mes is a very rare tumor. Eccrine definition of eccrine by medical dictionary. Carcinosarcoma ex eccrine spiradenoma of the vulva. Sep 05, 2018 how can eccrine spiradenoma be prevented. The monarch initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Malignant eccrine spiradenoma on the lateral margin of nose. Contribution to eccrine spiradenoma jama dermatology jama. In co ntrast, the malignant eccrine spiradenoma mes. Webpathology is a free educational resource with 10308 high quality pathology images of benign and malignant neoplasms and related entities. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.
Eccrine spiradenoma is now known as a be nign sweat gland tumor that commonly affects young adults. Eccrine and apocrine naevi are rare malformations of the glandular component of an eccrine or apocrine gland. Recurrent atypical eccrine spiradenoma of the forehead in vivo. Currently, there are no known methods to prevent eccrine spiradenoma occurrence. Malignant eccrine spiradenoma on the lateral margin of.
Eccrine spiradenoma usually occurs as solitary tender nodules. We report the case of a 32yearold woman with a 20year history of itchy lesions on the left side of the back and forearm with a zosteriform distribution. You may need to register to view the medical textbook, but registration is free. Benign eccrine spiradenoma is an adnexal tumor that occurs as either a solitary lesion or as multiple nodules. Eccrine spiradenoma is a rare benign adnexal epithelial neoplasm characterized by a bluishpink, slowgrowing solitary nodule, coursing with paroxysmal pain. Eccrine spiradenoma with chondroid syringoma in blaschkoid distribution. Denoting the flow of sweat from skin glands unconnected to hair follicles.
The gross appearance is firm and spherical nodule that is grayish white to brown in color, and usually 2 cm or less in diameter. Eccrine spiradenoma is a benign, painful tumor of the skin, which probably originates in the coil of the eccrine sweat glands and presents a characteristic clinical and pathological picture. Eccrine spiradenoma is an adnexal neoplasm that continues to be designated as a tumor with eccrine differentiation, however, it is currently considered an apocrine process. Nov 14, 2011 malignant eccrine spiradenoma is a type of tumor that develops from a sweat gland in the skin. D e s c a l z i t b e university of rochester medical center department of pathology, rochester general hospital d e p a r t m e n t of pathology, new york, u.
Eccrine spiradenoma is a tumor that may arise from sweat glands throughout the body. Printer friendly versions html, pdf benign nonmelanotic epidermal tumors or tumorlike. Eccrine spiradenoma of the scalp europe pmc article europe pmc. Malignant transformation in eccrine spiradenoma, dermal cylindroma, and cylindromaspiradenoma hybrid tumors is a rare event with only 150. Eccrine carcinoma is an extremely rare malignancy of the skin with few well documented cases reported in the literature. Hidradenoma refers to a benign adnexal tumor of the apical sweat gland. Multiple segmental eccrine spiradenoma with a zosteriform. It starts as a rapidlygrowing bump on the head or abdomen, and may cause tenderness, redness, or an open wound. Malignant eccrine spiradenoma is a type of tumor that develops from a sweat gland in the skin. Although most cases are benign, occasional malignant transformation has been reported. Malignant eccrine spiradenoma mes is a rare malignancy of the eccrine sweat glands. Eccrine spiradenomas are benign adnexal tumors with slow growth and long clinical history. Cylindroma and eccrine spiradenoma coexistent in the same. Eccrine spiradenoma, also spiradenoma, is usually a benign, painful skin thingy.
They are common in young adults and are without a sex predilection. Eccrine spiradenoma a painful dermal nodule j shushan shweta, sharmila sp, rajaram t, sushma ta department of pathology, rajarajeswari medical college and hospital, bangalore, karnataka, india abstract eccrine spiradenoma is an uncommon benign adnexal neoplasm occuring mainly in young adults. However, in rare cases, some spiradenomas are known to transform into malignant lesions. Cytogenetics of a case of eccrine spiradenoma sciencedirect. Flow cytometry of the primary tumor and the two metastases showed a dna index of 1.
Physical examination revealed multiple small papulonodular swellings measuring 0. Pdf eccrine spiradenoma arising from the breast skin. We describe a case of malignant eccrine spiradenoma of a 41yearold male with a history of swelling over nape of neck f or 6yrs and recent onset of increase in size since 6 months. Mar 16, 2020 webpathology is a free educational resource with 10308 high quality pathology images of benign and malignant neoplasms and related entities. Monarchs tools are designed to make it easier to compare the signs and symptoms phenotypes of different diseases. A case of eccrine spiradenoma in a patient with neurofibromatosis. Malignant eccrine spiradenoma with squamoid and chondroid. Eeccrine spiradenoma with chondroid syringoma in ccrine. These tumors occur primarily in middleaged females and have a predilection for the head region. It arises from smooth muscle of the blood vessels and the most common symptom is pain. Introduction eccrine hidradenoma is also referred to as nodular hidradenoma, eccrine spiradenoma, or clear cell hidradenoma. A case of multiple eccrine spiradenoma occurring on the scalp and mimicking multiple cylindroma. Mri features characteristic to angiomyomas include a. Click on the link to view a sample search on this topic.
Hidradenomas are often subclassified based on subtle histologic differences, for example. Angioleiomyoma of the hand is a rare differential diagnosis of painful soft tissue nodule in the extremity. Spiradenoma, also spiroma or eccrine spiradenoma, is a cutaneous condition that is typically characterized, clinically, as a solitary, deepseated dermal nodule of approximately one centimeter, occurring on the ventral surface of the body 666 spiradenoma lesions are benign sudoriferous tumors, and have also been described as cystic epitheliomas of the sweat glands. Eccrine spiradenoma es, as first described in 1956, is a rare, benign adnexal neoplasm that is able to present on any part of the body, with 15 of cases occurring in the extremities 4. Pdf eccrine spiradenoma es is a rare, benign adnexal neoplasm that may easily be mistaken for glomus lesions or angioleiomyoma due to its. Eccrine spiradenoma definition of eccrine spiradenoma by. They presumably arise from eccrine sweat glands and do not show any apocrine differentiation. A case of multiple eccrine spiradenoma occurring on. The presentation is often that of a single nodule that may or may not be tender. Eccrine spiradenoma, giant vascular eccrine spiradenoma, sweat gland neoplasms introduction giant vascular eccrine spiradenoma gves is a variant of an eccrine spiradenoma.
The glands within a sweat gland naevus are increased in size and number from birth. Eccrine spiradenoma of the hand clinically mimicking a lipoma. A case in which a young woman presented with a longstanding lesion of the forearm sustained after trauma is reported. Eccrine spiradenoma and dermal cylindroma eccrine spiradenoma and dermal cylindroma gerber, j. Cylindroma in this tumour the low power view shows discrete polygonal tumour islands. Outcomesresolutions the prognosis for eccrine spiradenoma is excellent with appropriate treatment, since it is a benign tumor.
It usually presents as a single, small skincolored lesion, and is considered distinct from the closely related poroma. Malignant eccrine spiradenomas mes are rare but have been divided into two categories. Differential diagnosis of eccrine spiradenoma pathology. Eccrine spiradenoma cogitates a category of painful dermal tumours with the nomenclature of blend an egg. Eccrine spiradenoma es, first described in 1956, is an uncommon, benign, dermal tumor of apocrine differentiation derived from cutaneous sweat glands. It is crucial to take eccrine spiradenoma into consideration in superficial. Here we report a case of eccrine spiradenoma of the breast skin in a 39yearold woman who presented with a breast nodule for 10 years. The tumor is located at the interface of the deep dermis and superficial breast tissue, wellcircumscribed with a thick fibrotic capsule a and b.
Eccrine spiradenoma is a benign adnexal neoplasm that has been historically designated as a tumor of eccrine differentiation, although current reconsideration indicates an apocrine process. The classical description of the eccrine spiradenoma by kersting and helwig 1 in 1956 defined this condition as a distinct clinicopathological entity. Sweat gland lesions generally have variable apocrine or eccrine differentiation. Eccrine spiradenoma of the left scapular region with axillary and lymph node metastases. In the majority of cases the benign component merges with the areas of malignant. Brookespiegler syndrome no differences are seen between sporadic eccrine spiradenoma or those occurring in the context of this syndrome.
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