International Journal of Surgery Case Reports 114 (2024) 109101

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International Journal of Surgery Case Reports

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Case report

Unusual presentation of adamantinoma with synchronous involvement of

entire-lengths of the tibia and fibula in an elderly man: A case report
Prabodh Kantiwal a, *, Divya Agrawal b, G. Laxmi Prasad c, Nitesh Gahlot a, Abhay Elhence a
a
Department of Orthopaedics, All India Institute of Medical Sciences, Jodhpur, India
b
Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Jodhpur, India
c
Kims Sunshine Hospital, Begumpet, Hyderabad, Telangana 500016, India

A R T I C L E I N F O A B S T R A C T

Keywords: Introduction and importance: Adamantinoma is a rare primary low-grade malignant bone tumor with a median age
Synchronous of 20 to 30 years with a specific predilection to the lower 2/3rd shaft of the tibia.
Epithelial cell We present an unusual presentation of a giant adamantinoma with synchronous involvement of almost entire
Basaloid
lengths of the tibia and fibula and extensive to the skin in a geriatric man.
Osteofibrous
Case presentation: An elderly male patient in their late 50s presented to us with a grossly deformed left leg with a
D240
Ulcero-proliferative fungating mass over the left leg for 5 years. X-rays showed a lytic sclerotic lesion with a honeycomb appearance
involving the entire length of the tibia and fibula. Magnetic Resonance Imaging showed a heterogeneous altered
signal intensity (T1 isointense and T2 heterogeneous hyper-intense lesion) large lobulated lesion involving the
entire length of the leg with lytic destruction of the entire tibia and fibula and associated remodeling. The
histopathological examination revealed an Invasive tumor composed of both epithelial and mesenchymal ele­
ments. On immunohistochemistry, tumor cells were positive for D240 and negative for CD31. After confirming
the diagnosis of adamantinoma of tibia and fibula radical resection of the tumor was planned and performed in
the form of above-knee amputation. The patient was disease-free at 18 months of the latest follow-up and
walking with the above knee prosthesis comfortably without any assistance.
Clinical discussion: Two morphological patterns of adamantinoma on MRI have been described, a solitary lobu­
lated focus and a pattern of multiple small nodules in one or more foci. Our case has demonstrated the second
type of morphology.
Histologically, this case presented with “classical basaloid type epithelial cells embedded in osteofibrous
dysplasia-like stroma.”
Conclusion: The diagnosis of adamantinoma was based on the clinical-radiological findings and histo-
morphology, and should be confirmed by immunohistochemistry for demonstrating epithelial cells. Ultra-
structural analysis and Cytogenetic studies may be required in the cases of unusual presentation of these tu­
mors. Wide local resection is the preferred treatment.

1. Introduction lesion with subsequent involvement of the ipsilateral tibia. Ada­

Adamantinoma, originally described as ameloblastoma of the
mandible, is a low-grade primary bone malignancy of uncertain histo­
genesis with epithelial differentiation [1] and a striking predilection to
the mid-diaphysis tibia which accounts for about 85 % of all cases.
Synchronous involvement of tibia and fibula occurs in about 10 % of
all cases. Other bones can also be involved, like the femur, ulna, hu­
merus, radius, rib, ischium, tarsal, metatarsal, capitate, and extra-
skeletal pretibial soft tissue [1–3]. The neoplasm may start as a fibular
mantinoma comprises 0.1 to 0.5 % of all primary bone tumors [4]. Male
and female patients are affected almost equally with a wide age spec­
trum but young adults are most frequently affected, and <3 % of patients
are younger than 10 years. The mean age at presentation is about 30
years. The diagnosis may be challenging since this tumor has very close
differentials like osteofibrous dysplasia, myoepithelial tumor, carci­
noma, carcinosarcoma, or myxoid chondrosarcoma. The most frequent
sites of metastases are the lungs. Lymph node metastases occur less
frequently, with rare metastases to the bone reported as well [1].

* Corresponding author.
E-mail address: prabodhkantiwal@gmail.com (P. Kantiwal).

https://doi.org/10.1016/j.ijscr.2023.109101
Received 9 November 2023; Received in revised form 29 November 2023; Accepted 3 December 2023
Available online 15 December 2023
2210-2612/© 2023 Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
P. Kantiwal et al.
We aim to discuss the preoperative planning, operative execution,
and follow-up outcomes.
2. Case presentation
A 57-year-old male patient presented to us with a grossly deformed
left leg with a fungating mass and painful discharging sinuses in the
anterior aspect over the left leg for 5 years. An expansile and grey-white
coloured ulcero-proliferative growth was seen measuring around 15 ×
10 × 10 cm in the lower half of the leg on the anterior aspect disfiguring
the entire leg. The growth was externally ulcerating the skin showing
haemorrhagic areas and necrosis. Distal pulses were very feeble as
compared to the opposite side, indicating that swelling was grossly
compressing or involving underlying vascular bundles. The fibula was
not appreciable and palpable separately.
Plain radiographs showed a large area of expansile lytic sclerotic
lesion with a honeycomb appearance involving the entire tibia and
fibula with anterior bowing of the shaft of the tibia and fibula. Innu­
merable radiolucencies surrounded by ring-shaped densities are very
clearly visible in the whole length of the tibia and distal half of the fibula
(Fig. 1).
Magnetic Resonance Imaging showed a heterogeneous altered signal
intensity large lobulated lesion involving the entire length of the leg
with lytic destruction of the entire tibia and fibula and associated
remodeling. This was a T1 isointense and T2 heterogeneous hyper-
intense lesion involving all compartments of the leg predominantly
the anterior and deep superficial compartments, involving the skin with
sinus tracts anteromedially in the lower one-third of the leg. There was
encasement of posterior tibial vessels and a broad area of contact with
anterior tibial and peroneal vessels. On diffusion-weighted images, there
was diffusion restriction. There was heterogeneous contrast enhance­
ment on post-contrast images with internal non-enhancing areas sug­
gestive of necrosis. The lesion extends up to sub-articular margins of the
tibia proximally and distally without intra-articular extension (Fig. 2).
A metastatic workup was done with Contrast-Enhanced Computed
Tomography (CECT) and Tc99m-MDP Bone Scan and found to be
negative for the distant spread of the disease. In the Bone Scan, a large
area of expansile lytic sclerotic lesion with heterogeneous radiotracer
uptake was noted involving the entire left tibia and fibula with spared
knee and ankle joints.
A large photogenic area in the lower 1/3 leg was noted with soft
tissue component and associated sinus tract with few air pockets (Fig. 3).
Fig. 1. Antero-posterior (figure a) and lateral (figure b) views showing a
grossly deformed leg with expansile lytic sclerotic lesion with a honeycomb
appearance involving the entire tibia and fibula with anteromedial bowing of
the distal leg. Innumerable radiolucencies surrounded by ring-shaped densities
are very clearly visible.
2
International Journal of Surgery Case Reports 114 (2024) 109101
Based on the radiological and nuclear medicine findings, primary car­
cinoma with sarcomatous transformation, adamantinoma, the active
phase of Paget's disease with possible superimposed infection, and less
likely metastasis were considered.
A core-needle biopsy was done and a histopathological examination
revealed an Invasive tumor composed of both epithelial and mesen­
chymal elements. The epithelial component comprises basaloid cells
disposed in islands, anastomosing trabeculae, cribriform pattern with
peripheral palisading basaloid cells. The tumor cells have a high N:C
ratio, and hyperchromatic nuclei with scant cytoplasm. The intervening
mesenchymal element was composed of loosely arranged spindle cells
along with occasional lymphocytic inflammatory infiltrate.
Few areas of myxoid degeneration were noted. Necrosis was identi­
fied, both macroscopically and microscopically. Mitosis index was 10/
10 HPF (in 0.5 mm 2 field diameter). Tumor infiltration into peripheral
skeletal muscle bundles was seen (Fig. 4). In immunohistochemistry,
tumor cells were positive for D240 and negative for CD31 (with positive
internal control in endothelial cells).
Clinico-pathology TNM classification as pT2N0M0 was made (p
TNM, AJCC 8th Edition).
After confirming the diagnosis of adamantinoma of the tibia and
fibula and staging of the disease, radical resection of the tumor was
planned and performed in the form of above-knee amputation, because
the attempt of limb salvage was not feasible due to gross involvement of
the entire leg with the tumor, with the ulcero-proliferative fungating
mass lesion, invasion into vascular structures and superadded local
infection. The post-operative period was uneventful and he recovered
well. He was disease-free at 18 months of the latest follow-up and
walking with the above knee prosthesis comfortably without any
assistance.
This study has been reported in line with the SCARE guidelines
criteria [16].
3. Discussion
The first report of a case of the primary bone tumor with epithelial
characteristics, observed in the ulna, is attributed to Maier in 1900 [5].
In 1913, Fischer provided the first detailed description and named the
lesion “primary adamantinoma of the tibia” because of its striking his­
tologic resemblance to the jaw adamantinoma (ameloblastoma),
asserting that adamantine epithelium was located in both the tibia and
intraoral enamel during the embryonic period [6].
In 1951, Schulenberg suggested a unifying histogenetic concept for
the gnathic ameloblastoma, pituitary adamantinoma, and ada­
mantinomas of the appendicular skeleton [4]. In 1957, Changus et al.
suggested the vascular origin of this lesion and proposed close similarity
with angioblastomas [7]. This theory has been supported by a few pieces
of evidence from ultra-structural studies, which showed that ada­
mantinoma cells have basement membranes, microvilli, and tonofibrils
forming desmosomes [8]. A few studies have shown the synovial tissue
origin of adamantinoma [9,10]. A history of significant trauma has been
noted in approximately 60 % of the 200 cases reviewed by Moon and
Mori [1]. Thus, the pathogenesis of this tumor is still debatable, and has
been described differently by various theories, like congenital epithelial
cell implantation theory [6], and traumatic implantation theory [1,11],
and articular origin theory [9,10].
In 1958, Jaffe reported the origin of adamantinoma from epithelial
cells [12]. This has been supported by numerous ultra-structural studies,
electron microscopy, and immunohistochemistry (IHC). The IHC dem­
onstrates adamantinoma tumor cells as keratin-positive and reinforces
their epithelial originSo, and this fact explains the predilection to the
anterior border of the tibia as the skin is very close here, and this was
hypothesized, that there was a trapping of skin epithelium during the
fetal period [3].
As per histopathology, adamantinoma is distinguished into classic
adamantinoma and osteofibrous-like adamantinoma. The epithelial cell
P. Kantiwal et al. International Journal of Surgery Case Reports 114 (2024) 109101

Fig. 2. Figure a1, a2, a3) T1 weighted images showing isointense lesion occupying whole length of tibia and fibula; figure b1, b2, b3) T2 weighted images showing
heterogeneous hyper-intense lesion; figure c1, c2, c3, c4) STIR images.

Fig. 3. Triple-phase bone scintigraphy images showing the anteroposterior whole-body image (fig. A) and left leg images (fig. C) demonstrating heterogeneously
increased radiotracer uptake in the left tibia and lower 2/3 of the fibula with associated deformity; figure b, & d) SPECT CT images of bilateral legs region.

components are the basis of various histological sub-types of classical reported [13].
adamantinoma, like (i) tubular (the most frequent), (ii) basaloid, (iii) The epithelial component exhibits co-expression of cytokeratins 5,
squamous, and (iv) spindle variant. A very rare histological sub-type of 14, and 19; epithelial membrane antigen, P63, E-, P- and N-cadherin,
Ewing's sarcoma-like adamantinoma (the least frequent) has also been osteonectin, and vimentin, but lacks the immunohistochemical

3
P. Kantiwal et al. International Journal of Surgery Case Reports 114 (2024) 109101

Fig. 4. Figure a) Photograph shows a biphasic tumor composed of epithelial and mesenchymal elements intermixed with each other (hematoxylin and eosin 20×);
figure b) the tumor was infiltrating native mature bony trabeculae (hematoxylin and eosin, 40×); figure c) the epithelial component was in the form of mildly
atypical epithelial cells as tubular structures and strands, embedded in osteofibrous dysplasia-like stroma (hematoxylin and eosin, 100×); figure d) tumor cells are
negative for CD31 (with positive internal control in endothelial cells, CD31 immunohistochemistry, 100×); & figure e) tumor cells are positive for D240 (D2–40
immunohistochemistry, 100×).

expression of keratins 8 and 18, which differentiates them from many Funding
other skeletal or soft-tissue tumors. These tumors are negative for
osteopontin and osteocalcin [3,13]. None.
On X-ray, this classically appears as an eccentric and sometimes
central lesion in the distal two-thirds part of the tibia, but in our case, Ethical approval
this involved the entire length of the tibia and the distal half of the
fibula. The involvement of the fibula may be explained by the direct This study is not required for ethical approval in our institution
dissemination from the long-standing distal third tibial lesion. Classical because this is anonymous and does not contain any personal
multifocal radiolucencies surrounded by ring-shaped densities are very information.
clearly demonstrable. The tumor remains usually inside the cortex, and
in our case too, despite a long-standing neglected case scenario, most of CRediT authorship contribution statement
the tumor content was confined inside the cortical boundaries.
Two morphological patterns on MRI have been described, a solitary The following authors were responsible for the drafting of the text,
lobulated focus and a pattern of multiple small nodules in one or more sourcing and editing of clinical images, investigation results, drawing
foci [14]. Our case has demonstrated the second type of morphology. original diagrams and algorithms, and critical revision for important
Histologically, this case presented with “classical basaloid type intellectual content:
epithelial cells embedded in osteofibrous dysplasia-like stroma.” Ada­
mantinoma contains a malignant epithelial component with a reactive 1. Prabodh Kantiwal
osteofibrous layer [15].
The following authors gave final approval of the manuscript, they
4. Conclusion were not directly involved in the patient's care, but they contributed to
the manuscript by:
Adamantinoma is a rare bone tumor, occurring primarily in the tibia.
Synchronous involvement of the tibia and fibula can also be manifested 1. Nitesh Gahlot – manuscript editing
as the first presentation in rare circumstances or neglected long-standing 2. Laxmi Prasad G – manuscript editing
cases. The lesion may primarily originate from the fibula and dissemi­ 3. Divya Agrawal – Histopathological analysis
nate to the tibia thereafter. Confirmation of histological diagnosis using 4. Abhay Elhence – manuscript editing
immunohistochemistry, ultra-structural studies (electron microscopy),
or cytogenetic studies (molecular analysis) might be required in unusual
presentations of adamantinoma. However, this is a tumor of low ma­ Declaration of competing interest
lignancy but might metastasize to the lungs and neighboring lymph
nodes, and very rarely to other bones, liver, and brain. Thus, a proper None.
metastatic workup should be included in the management plan. Finally,
long-term follow-up is important as early diagnosis is essential for References
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