Keratoacanthoma Arising in an Organoid Nevus During Childhood: Case Report and Literature Review

We report an 11-year-old girl with an organoid nevus of the cheek. She represents the first report of a keratoacanthoma arising within an organoid nevus in childhood.     

皮脂腺痣继发肿瘤16例临床与组织病理分析

目的 探讨皮脂腺痣继发肿瘤的组织病理学特征。方法 回顾性分析所收集到的16例皮脂腺痣继发肿瘤患者的临床、病理资料和组织病理学特征。结果 ≥18岁12例,11 ~ 17岁4例。7例伴有乳头状汗管囊腺瘤,4例伴有管状顶泌汗腺腺瘤,3例伴有毛鞘瘤,2例伴有基底细胞癌,汗孔角化样汗孔和真皮导管痣及鲜红斑痣各1例。6例有1个以上的肿瘤,尤以乳头状汗管囊腺瘤伴管状顶泌汗腺腺瘤最多,占有4例。结论 皮脂腺痣基础上有任何继发肿瘤发生,都应密切关注。     

Syringocystadenoma papilliferum: report of the first case on the lower leg

Syringocystadenoma Papilliferum (SCAP) is a benign adnexal tumor which most frequently arises from an organoid nevus on the head and neck. Although they are rarely found on the trunk and limbs, we treated a case of this disorder on the lower leg. A 26-year-old man had an asymptomatic tumor on his lower leg. Histopathological examination showed it to be a typical SCAP on organoid nevus. This is the first report of SCAP on the lower leg.     

Proliferating trichilemmal tumor with apocrine sweat glands

A 43-year-old man with a proliferating trichilemmal tumor is described. Since the tumor had appeared on a pre-existing alopecia and accompanied ectopic apocrine sweat glands, it is supposed that the pathogenesis of the tumor of our patient might be similar to that of an organoid nevus.     

Trichoblastoma,syringocystadenoma papilliferum,desmoplastic trichilemmoma and tumor of the follicular infundibulum with signet‐ring cells,all arising in nevus sebaceus

Herein, we describe a 63‐year‐old male with multiple tumors arising within a nevus sebaceus on the posterior scalp. On histopathologic examination, four distinct tumors were identified: trichoblastoma, syringocystadenoma papilliferum, desmoplastic trichilemmoma and tumor of the follicular infundibulum (TFI). Within the TFI component of the nevus sebaceus, there was intracytoplasmic accumulation of eosinophilic keratin, as shown on pancytokeratin‐stained sections, imparting a signet‐ring appearance to the cells. To our knowledge, this is the first report of signet‐ring cells arising within a TFI occurring in a nevus sebaceus. We discuss this case as well as review the literature on multiple tumors arising within nevus sebaceus and signet‐ring cell changes in primary cutaneous tumors.     

Pedunculated basal cell epithelioma which is not Pinkus tumor

A 33-year-old female with pedunculated basal cell epithelioma was reported. She had noticed a cutaneous tumor on the scalp for two years before admission. It developed gradually and clinically resembled fibroma or pigmented nevus. Total resection was performed, and its histopathology revealed the solid or cystic type of basal cell epithelioma.     

Desmoplastic sebaceoma arising from nevus sebaceus: a new variant

Nevus sebaceus is known to have the potential to develop into various secondary tumors. We observed a sebaceoma arising from a nevus sebaceus excised from the left cheek of a 51‐year‐old woman. This sebaceoma showed desmoplastic change similar to that observed in desmoplastic trichoepithelioma and desmoplastic trichilemmoma. This heretofore undescribed desmoplastic variant of sebaceoma should not be mistaken for invasive sebaceous carcinoma.     

Syringocystadenocarcinoma papilliferum in a linear nevus verrucosus

Syringocystadenocarcinoma papilliferum (SCACP), the malignant counterpart of syringocystadenoma papilliferum (SCAP), is a rare form of adenocarcinoma of the skin. Only 11 well‐documented case reports of SCACP have been published so far. An 83‐year‐old woman with a linear nevus verrucosus (LNV) on her right arm had a history of a nodule arising within this nevus that was diagnosed as SCAP by skin biopsy 7 years earlier. Since then, the nodule had enlarged gradually and formed an exophytic tumor with a moist surface, measuring 3 × 2.5 cm. The tumor was excised and studied by histologic examination. Although histologically the overall architecture of the tumor still resembled SCAP, transition to SCACP was obvious by the presence of areas of cytonuclear atypia, increased proliferative activity and infiltrative growth. The edges of the excised ellipse flanking the tumor showed typical microscopic features of LNV, but no organoid components of nevus sebaceus (NS).We report the 12th case of SCACP, the first case of SCACP on the arm and the first case of SCACP arising from pre‐existing SCAP, in what appeared to be an epidermal nevus. Hoekzema R, Leenarts MFE, Nijhuis EWP. Syringocystadenocarcinoma papilliferum in a linear nevus verrucosus.     

Nevus marginatus: a distinct type of epidermal nevus or merely a variant of nevus sebaceus?

Epidermal nevi (EN) represent a heterogeneous group of mosaic skin lesions frequently following the lines of Blaschko. They are divided into organoid and nonorganoid types. Herein we report on a 35-year-old man with a rather unusual type of EN. A linear lesion involving the trunk and following Blaschko's lines was conspicuously bordered by dark brown papules. The flat reddish central area histopathologically showed sebaceous hyperplasia consistent with nevus sebaceus, an organoid EN type, whereas the elevated margin showed features of a common nonorganoid keratinocytic EN. For this peculiar disorder, we propose the term 'nevus marginatus'. So far it is not clear whether nevus marginatus represents a distinct entity or merely an unusual clinical variant of nevus sebaceus.     

Sebaceous carcinoma arising in nevus sebaceus of Jadassohn: a clinicopathological study of five cases

The authors report a small series of sebaceous carcinoma developing in nevus sebaceus of Jadassohn (also known as organoid nevus) and analyze similar cases reported in the literature. All of our patients were females (age range 57-71 years; median 60 years) who had the organoid nevus on the scalp, face, or nuchal area, the rest of which was recognized clinically and/or histologically. All sebaceous carcinomas manifested unquestionable architectural (asymmetry, invasive growth) and cytological features of a carcinoma (cellular and nuclear pleomorphism, plentiful atypical mitoses, necroses en masse), demonstrated unambiguous sebaceous differentiation in the form of mature sebocytes, and lacked other differentiations. The sebaceous carcinoma was always accompanied by other benign or malignant adnexal lesions such as sebaceoma, syringocystadenoma papilliferum, syringocystadenocarcinoma papilliferum, trichoblastoma, tricholemmoma, desmoplastic tricholemmoma, or syringoma. In three cases, prominent mucinous metaplasia of sweat ducts and glands was seen. In two of these cases, sweat ducts exhibited hyperplastic changes. The analysis of the previously published material and our cases indicates that sebaceous carcinoma arising in organoid nevus has a female predilection and tends to occur in elderly patients. It may involve any site where nevus sebaceus typically occurs. Clinically, the tumor presents as a solitary nodule, ulcerated tumor, or mass, often with a recent history of rapid growth. It may arise alone, but it occurs more frequently as part of multiple benign and malignant adnexal tumors. The lesion does not seem to be associated with Muir-Torre syndrome. The rest of organoid nevus is usually recognized both clinically and microscopically, although large tumors may overgrow and mask the nevus. The tumor seems to be a low-grade carcinoma in terms of clinical behavior.     

Three Cases of Organoid Nevus on the Trunk and Extremity

Organoid nevi are most commonly observed on the scalp. Although they are rarely found on the trunk and extremities, we were able to study 3 cases of organoid nevi involving distributions of this sort. These included a 39-year-old male with a 2 × 6 cm brown area on the left flank (case 1), an 8-year-old female with a 5 × 6 cm hypopigmented macule on the lower left leg (case 2), and a 45-year-old male with a 1.5 × 3 cm brown spot on the back (case 3). All these were histopathologically confirmed to be organoid nevi. The lesion in case 1 revealed basal cell epithelioma, that in case 2 exhibited hypohidrosis caused by poorly developed sweat glands, and the lesion in case 3 was typical, as was an additional one on the same patient's scalp.     

Metastasizing adenocarcinoma and multiple neoplastic proliferations arising in a nevus sebaceus

Nevus sebaceus of Jadassohn is a hamartoma of multiple skin structures. Many neoplasms have been reported to arise in association with nevus sebaceus, most commonly trichoblastoma/basal cell carcinoma and syringocystadenoma papilliferum. We report a case of a 66-year-old woman with an adenocarcinoma as well as multiple neoplastic proliferations arising in a long standing nevus sebaceus on the scalp, with subsequent occipital neck metastatic disease. On histologic evaluation, the epidermis showed changes reminiscent of tumor of the follicular infundibulum as well as basaloid proliferations resembling superficial trichoblastoma. A focus suggestive of syringofibroadenoma was also present. A small dermal collection of basaloid and more mature sebocytes was consistent with a sebaceoma/sebaceous epithelioma. Most of the lesion was composed of an adenocarcinoma with areas showing ductal differentiation with decapitation secretion, well-formed papillae and focal cribriform structures. Other portions demonstrated a high-grade neoplasm with prominent nuclear atypia and a solid pattern of growth resembling high-grade breast carcinoma. Anti-epithelial membrane antigen strongly labeled tumor cells and highlighted ductal structures. Less than 1% of cells expressed progesterone or estrogen receptors. Her2/neu reactivity was focally present, showing 1+ membranous reactivity in 10% of cells. Anti-p63 labeled basaloid cells surrounding the tumor lobules. A breast primary was ruled out by clinical and radiologic examination. This report illustrates an extraordinary case of adnexal neoplasia displaying various lines of differentiation arising in association with nevus sebaceus.     

Nevus of Ota and choroid melanoma

A 59-year-old patient presented with metastatic uveal melanoma that developed in a nevus of Ota. The nevus of Ota or oculodermal melanocytosis contains an increased number of dermal melanocytes in the distribution of the ophthalmic and maxillary divisions of the trigeminal nerve. Malignant transformation arising in a nevus of Ota may occur in all anatomical sites influenced by the nevus. Most often the choroid is involved. Although the nevus of Ota is rare in Caucasians, associated melanoma is more common than in Asians or black people. The dermatologist should be aware of this problem and aim at an interdisciplinary management of these patients.     

Multiple Agminated Juvenile Melanoma Arising on a Hyperpigmented Macule

A case of multiple agminated juvenile melanoma (MAJM) arising on a hyperpigmented macule is reported. The patient, a 1-year-old boy, had 9 small nodules on an irregular, hyperpigmented macule on the right shoulder. Histologically, the nodules contained nests of spindle-shaped cells in their epidermis and at the dermoepidermal junction, and the hyperpigmented macule exhibited increased melanin granules in the basal layer and small nests of epithelioid cells. We concluded that MAJM on a hyperpigmented macule is the subtype of the nevus spilus.     

Malignant melanoma arising in a nevus spilus

Malignant melanoma arising within a nevus spilus is rare. Nevus spilus is characterized by darkly pigmented macules and papules with background hyperpigmention. We report a 65-year-old woman who presented with a melanoma arising in a nevus spilus that had been present since birth.     

Phacomatosis pigmentokeratotica: a 20-year follow-up with malignant degeneration of both nevus components

Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. The disorder is a consequence of the so-called twin spot genetic mechanism. We describe the first occurrence involving malignant degeneration of both nevus components, giving rise to three basal cell carcinomas over the sebaceous nevus and a malignant melanoma of the superficial spreading type over the speckled lentiginous nevus. This observation, in concert with the other instances reported in the literature, points to the need for adequate patient follow-up to ensure early detection and treatment of any possible associated malignant degeneration.     

2 basaliomas of the lower leg in a 60-year-old female--one of them simulating ulcus cruris

We report on a 60-year-old female patient with an ulcer on the left lower leg, which was treated for 2 years without success. Histologically, a biopsy specimen showed a partly fibrotic and partly solid basal cell epithelioma. When all of the patient's skin was examined, a small skin-colored nodule was found on her right lower leg that histologically also turned out to be a basal cell epithelioma. The patient had no history of predisposing factors like exposure to arsenic or actinic skin damage. Our case is compared with the literature and briefly discussed.     

Multiple tumors arising in nevus sebaceus.

A forty-three year old woman with nevus sebaceus of Jadassohn involving the scalp is described. The unusual features of this case include the presence of adenoidal basal cell epithelioma, pigmented basal cell epithelioma, syringocystadenoma papilliferum, and nevocytic nevus. This case suggests the importance of prophylactic removal and close follow-up of nevus sebaceus.     

Association of nevus sebaceus with an unusual type of "combined nevus"

A case of nevus sebaceus with development of a basal cell epithelioma is described. This case of nevus sebaceus was characterized by an association with extensive nevoid growths of melanocytes in the same lesion. The nevoid growths of melanocytes showed features of an unusual "combined nevus", representing a combination of speckled lentiginous nevus with blue nevi. We discuss the relationship between nevus sebaceus and nevoid growths of melanocytes.     

Metastatic malignant melanoma arising from a common blue nevus in a patient with subacute cutaneous lupus erythematosus

We describe a 66-year-old black male who first presented with subacute cutaneous lupus erythematosus (SCLE) and was later found to have malignant melanoma adjacent to a common blue nevus. The melanoma was located on the left buttock and in several left inguinal lymph nodes. Following complete resection and lymph node dissection the patient developed widespread leukoderma. To our knowledge this is the first report of malignant melanoma arising in a common blue nevus. The literature on SCLE and leukoderma in the setting of melanoma is reviewed.