Nevus Lipomatosus Cutaneous Superficialis with Spindle Cell Lipoma-like Areas: A Case Report
Correspondence Address :
Pooja Sharma Kala,
53/1, Teg Bahadur Road, Lane III Dalanwala, Dehradun, Uttarakhand, India.
Nevus Lipomatosus Cutaneous Superficialis (NLCS) is a rare occurring cutaneous hamartoma with presence of mature adipocytes in dermis.Though first reported in 1921 by Haffman and Zurhelle, fewer cases have been documented in literature since then. Authors hereby report a case of nevus lipomatosus cutaneous superficialis in an unusual age of 55-year-old male with multiple painless ill defined sessile nodules of 1.5 to 3 cm on lower back. Surgical excision was performed and histopathology revealed characteristic features as that of NLCS along with intervening areas of spindle cell lipoma. Coexistence of other lesion along with NLCS, both clinically or on histopathology is further an infrequent occurrence. No such case has been reported in literature so far.
Benign adipocytic tumours, Hamartoma, Lower back region, Nodule
A 55-year-old healthy male presented to surgery Outpatient Department with painless, slow growing multiple nodules on right side of lower back region, present for the past three years. The nodules were non tender, non pruritic and were not associated with any bleeding, ulceration, discharge. No similar lesion was present at any other site. Furthermore, there was no family history of any such lesion.
On clinical examination, there were multiple skin coloured, soft, ill-defined, sessile subcutaneous nodules over the lower back region, ranging in size from 1.5 cm to 3 cm in diameter. The nodules were not fixed with underlying structures. The lesion was resected with ellipse of skin around it and sent for histopathological examination.
On gross examination, the whole specimen measures 11×5×3 cm. Cut surface of the nodules was solid, smooth with homogenous yellowish white areas (Table/Fig 1)a,b. No haemorrhage, cyst or necrosis were identified. Multiple sections were taken from the nodules including the skin.
On microscopy, Haematoxylin and Eosin (H&E) stained section showed unremarkable epidermis with an ill defined lesion in reticular dermis, composed of variable sized lobules of mature adipocytes separated by dense fibrocollagenous bands (Table/Fig 2). The lobules show admixture of mature adipocytes alternating with myxoid areas enriched with spindle cells of bland morphology (Table/Fig 3), (Table/Fig 4). Many linear blood vessels and adnexal appendages were also seen in between the lobules. No lipoblast , atypical cell/ necrosis, mitosis were found. After considering various benign adipocytic lesions and variants of lipoma, a diagnosis of Nevus lipomatosus cutaneous superficialis with spindle cell lipoma- like areas was rendered. No recurrence was seen during the three month follow-up period.
Benign adipocytic tumors are fairly common, although rarity is not an exception among few of the variants. Nevus Lipomatosus Cutaneous Superficialis (NLCS) is one such rarely occurring cutaneous hamartoma, characterised by the presence of mature adipocytes in papillary and reticular dermis (1),(2). Coexistence of other adipocytic/non adipocytic lesion along with NLCS is even a rarer occurrence. So far, cases of giant NLCS with multiple folliculosebaceous cystic hamartomas and dermoid cysts and NLCS with localized scleroderma, like appearance has been reported in medical literature (3),(4). The authors report an unusual case of NLCS which, in addition, had areas of spindle cell lipoma.
Hoffmann E and Zurhelle E, were the first to describe NLCS in 1921 (5). This uncommon condition occur as classical (multiple) and solitary types, clinically. Both the types have similar clinical presentation and differ only in terms of age, number and site of lesion. Classical forms usually present as multiple, yellowish to skin-coloured, sessile /pedunculated, papules/nodules/plaques in a linear, zosteriform, or segmental pattern at birth or in the first two decades of life over pelvic girdle, gluteal region, lower trunk and upper thigh (6). On the contrary, solitary form often appears in third and sixth decades of life as an asymptomatic, yellowish to skin-coloured, solitary, papule /nodule with no predilection for any particular site (7). These lesions usually remain static but can gradually progress in size over years. Uncommonly, these lesions may ulcerate (8). Clinically, NLCS can be mistaken as neurofibromatosis, lymphangioma, hemangioma, fibroepithelial polyp (9). Both the forms show similar histomorphological features and surgical excision is the main modality of treatment (6),(10).
Characteristic histopathological feature of NLCS is abnormal presence of mature adipocytes or their aggregates in the dermis, especially around blood vessels or eccrine glands (6). This ectopic fat may variably occupy 10-70% of the dermis (11),(12). These adipocytes may or may not be separated from subcutaneous fat. A higher proportion of adipose tissue can be noted in larger lesions as compared to smaller lesions (12).
The epidermis remains normal but can be attenuated to acanthotic (12). Increased vascularity and perivascular mononuclear cell infiltration in dermis has been reported in NLCS (13). Also, uncommonly, adnexal structures can be reduced in number and may show perifollicular fibrosis (14). In present case, the adipocytes comprised of 60% of the dermis, while epidermis and dermal appendages were unremarkable.
On the other hand, spindle cell lipoma is also an infrequent histological variant of lipoma, usually presents as subcutaneous lesions of mature adipocytes. It usually arises on posterior neck, upper back and shoulder of elderly males between the 5th and 7th decade of life and are composed of mature adipocytes, bland spindle cells and collagen bundles in varying proportions (15),(16). Uncommonly, NLCS can also show spindle cells representing immature adipocytes focally (12). In present case, spindle cells comprised almost 35% of the lesion.
Case reports of NLCS with coexisting conditions like café-au-lait macules, leukoderma, hypertrichosis, folliculosebaceous cystic hamartomas , dermoid cysts has been reported in literature (3),(4),(17). But its existence with any other adipocytic lesion or any of its variants has not been seen.
Various theories have been postulated for the origin of dermal adipocytes in NLCS. These have been suggested to arise from pericytes, or from perivascular mesenchymal tissue (18),(19). Hoffmann E and Zurhelle E, suggested that the deposition of adipose tissue in dermis is a result of degenerative change in the connective tissue (20). Another study reported the deletion of 2p24 in NLCS, indicating a role of genetic factors in development of NLCS (21). Still, the etiopathogenesis of NLCs remains unclear.
This case report highlights one of the rare and interesting presentation of nevus lipomatosus cutaneous superficialis in terms of age, site and its co-existing histopathological features of spindle cell lipoma. Both come under the category of rare occurring adipocytic lesion. Surgical excision is the mainstay of treatment. Rare recurrences and complications have been reported though early diagnosis enables more conservative resection.
Date of Submission: Dec 09, 2021
Date of Peer Review: Jan 24, 2022
Date of Acceptance: Mar 25, 2022
Date of Publishing: Jun 01, 2022
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes
PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Jan 03, 2022
• Manual Googling: Jan 21, 2022
• iThenticate Software: Feb 22, 2022 (16%)
ETYMOLOGY: Author Origin
- Emerging Sources Citation Index (Web of Science, thomsonreuters)
- Index Copernicus ICV 2017: 134.54
- Academic Search Complete Database
- Directory of Open Access Journals (DOAJ)
- Google Scholar
- HINARI Access to Research in Health Programme
- Indian Science Abstracts (ISA)
- Journal seek Database
- Popline (reproductive health literature)