A 55-year-old man presented with periumbilical pain associated with the development
of brownish discoloration near the umbilicus. The condition started 6 months before
seeking medical attention. The pain increased particularly at the site of the brownish
lesion and became more intense when the patient touched the site of the lesion. The
gradual increase in pain severity as well as the noticeable changes of the skin lesion
(the brownish discoloration became harder, larger and darker in hue) led the patient
to seek medical advice. The patient has not been exposed to prominent risk factors
being a non-smoker, since he works in a risk-free environment as a mechatronic engineer.
Unfortunately, he has a positive family history of malignancies, which was unfavorable
with his delayed diagnosis. His father died at the age of 72 due to lung cancer and
his mother is a survivor of basal cell carcinoma of the nasomaxillary region.
Upon skin examination, the non-ulcerated skin lesion has been revealed. Melanoma malignum
cutis has been identified after performing a preoperative dermatoscopy (Figure 1).
The tumor was subsequently removed in one piece and the tissue identified histologically
as a pT2a malignancy. The lesion has the following properties: dimensions of 2.3 ×
2 × 1 cm; Breslow thickness of 1.9 mm; mitotic index of 4 mitoses/5 HPF; margin of
at least 0.1 cm healthy tissue; positive staining patterns of HMB-45 (+), Melan A
(+) and S100 (+) markers; and negative CK AE1/AE3 (–) marker (Figure 2).
Figure 1
Surface microscopy using FotoFinder dermoscope dynamic 800HD. All axes show asymmetric
contours, irregular pigmentation, irregular dots and streaks. Additional blue whitish
Weil and atypical pigment network were noted
Figure 2
Histological staining of skin specimens show pT2a malignancy and Breslow thickness
of 1.9 mm. A – HE staining with magnification 400×, B – HE staining with magnification
600×, C – HE staining with magnification 200×, D – HMB45 staining with magnification
200×, E – HMB45 staining with magnification 400×, F – Melan-A staining with magnification
400×, G – S100 staining with magnification 200×, H – S100 staining with magnification
400×
Following tumor resection, the patient was subjected to further investigation including
abdominal and retroperitoneal ultrasound as well as pelvic ultrasound. The abdominal
ultrasound showed hypo-echoic mass with dimensions of 44 × 26 × 33 mm localized on
the left umbilical region slightly lateral to the midline suggesting a pathological
lymph node that needed further investigation. On the other hand, the retroperitoneal
and pelvic scans were negative. More radical therapy was performed on the patient
including an expanded resection but the tissue histopathology failed to show any evidence
of the presence of cancer cells.
Further findings were obtained after performing an abdominal CT scan. We found a pathological
mass of 37 × 41 mm with inhomogeneous and hypo-echogenic texture (Figure 3). Closely
adhering to the small bowel loop, we found gastrointestinal stromal tumor (GIST).
In order to ensure patient safety and due to the high morbidity and mortality of this
metastatic tumor, the patient subsequently underwent a FUSION PET (PET/CT) scan using
18F-FDG imaging on the Biograph mCT 20. Basic parameters were measured including the
blood glucose level, which showed 116 mg/dl. The patient was injected with 500 MBq
of FDG and focal mass and the data showed that 12 mm of SUV 5.0 in the descending
colon with a tumor size of 33 mm. The SUV 6.1 is localized in the mesentery alongside
of the intestinal loop on the left-hand side of the middle region of the abdomen (Figure
3).
Figure 3
The CT and PET/CT scanning images. (A) Computed tomography, the arrow indicates a
pathological mass (37 × 41 mm) with inhomogeneous and hypoechogenic echotexture closely
adhering to the small bowel loop. The primarily lesion was characterized as metastasis
or gastrointestinal stromal tumor (GIST) and (B, C, D) by positron emission tomography.
The arrow indicates tumor (33 mm; SUV 6.1) localized in the mesentery beside the intestine
loop in the left side of the middle region of the abdomen
After the PET/CT scan, the patient was referred for a colonoscopy that has been performed
using the Olympus CF-Q180AL. The colonoscopy allowed the whole large intestine to
be examined more clearly. The examination of ostium appendicis vermiformis and ileocecal
valve became clearly visible. The data showed that the patient had an enlarged hemorrhoid
while the intestinal wall and mucosa were normal. The Boston Bowel Preparation scale
(BBPS) measured as 6/9 points (2 + 2 + 2) and no other pathological changes were observed.
There were few doubts of the clinical diagnosis and the differentiation of the potential
metastatic formation of malignant melanoma due to the lack of previous reports of
similar cases. Next, the patient underwent exploratory laparoscopy and possible curative
resection (Figure 4).
Figure 4
Intraoperative imaging showed the GIST tumor and postoperative abdominal scars presentation.
A – Explorative laparoscopy showed tumor formation classified latterly as gastrointestinal
stromal tumor (GIST). B – The arrows indicate postoperative scars after primary MM
excision, laparoscopic GIST resection and lymph node dissection
In the exploratory laparoscopy, 6 cm of the small intestine was found and resected.
The surgical cut localizes 4.7 cm of pathological change and further 1.2 cm of non-pathological
tissue. A final gross examination confirmed the presence of GIST. The characterization
of the tumor is the following: tumor type pT2 with a low mitotic rate at stage IA
of 1 mitose/50 HPF (Figure 5). In addition, the proliferation maker of Ki67 detected
by immunohistochemical staining showed positive cells but did not exceed 5%. Other
markers showed some changes including CD34 (+), CD99 (+), CD117 (+), CK AE1/AE3 (–),
Desmin (–), DOG-1 (+), EMA (–), MA (–), Myogenin (–), S100 (–), SMA (+) and Vimentin
(+).
Figure 5
Histological examination of surgical skin specimens confirmed the presence of a gastrointestinal
stromal tumor (GIST). The results confirmed the presence of GIST, type pT2 with a
low mitotic rate at stage IA. A – HE staining – GIST with magnification 40×, B – HE
staining – GIST with magnification 100×, C – HE staining – GIST with magnification
400×, D – VIM staining – GIST, with magnification 400×, E – DOG1 staining – GIST with
magnification 400×, F – CD34 staining – GIST with magnification 200×, G – CD117 staining
– GIST with magnification 400×, H – CKAE1-AE3 staining – GIST with magnification 200×
The lymphoscintigraphy and sentinel lymph node biopsy (SNB) were also performed. The
SNB examination showed 2 nodes, the first node was 1.2 cm with lymph node metastasis
and the second node was inflamed with a size of 0.5 cm. Therefore, the patient underwent
a wider excision of these lesions. Six months later, the patient was in good condition
and he was also undergoing periodic observation as part of his postoperative follow-up.
Many studies showed the synchronous coincidence of double or multiple cancers [1,
2] and such occurrences are expected to be increased [3, 4]. Nowadays, there are only
few detailed studies of synchronous cases of melanoma and other malignancies such
as carcinomas of the large intestine, breast and prostate [5–7]. Up to now, there
has been only one published case study that refers to synchronously diagnosed malignant
melanoma (involving the right hard palate) and GIST [8]. In addition, there is one-center
analysis indicating that multiple cancer occurrence may be considered as a coincidence
but without details on the anatomical location or whether this case can be classified
as metachronous or synchronous [9].
A well-known conclusion has been made that diagnostic imaging and other novel diagnosis
modalities are important in providing early diagnosis which lead to early management
and less complications to ensure catching many tumors at the early stage [10, 11].
This is particularly true for globally synchronous GIST tumors, which are often detected
incidentally during diagnostic processes or treatment [12]. Moreover, those patients
are frequently diagnosed with an asynchronous occurrence of another malignancy during
follow-up to their treatment [13]. Those double or multiple coincidences of GIST are
mainly observed with other primary gastrointestinal malignancies [14].
However, individual cases related to other types of cancer are increasing since such
data are available for comparative geographical, demographical and epidemiological
analysis [15]. Such data analysis will be available for any research investigator
in Poland that has only detected GIST as a secondary neoplasm while studies in other
countries have observed it within the primary site of cancer [16].
The malignant melanoma is one of common types of skin cancer and can occur in many
other organs. There is only a small number of case studies that referred to the synchronous
coincidence between malignant melanoma and other neoplasms [5–17]. Such cases are
characterized as very rare and incidental cases. Examples of these cases include cases
of anorectal malignant melanoma coexisting with adenocarcinoma of the sigmoid colon
and cases of double cancer incidences that involve esophageal MM and adenocarcinoma
of the lung [18, 19].
Up to date, there has been only one published case study of synchronous double cancer
involving melanoma malignum and GIST [8]. This case concerns a 78-year-old woman that
differs from this case whereas there was no detection of pathological sentinel lymph
nodes or any details of symptoms reported by the patient linked to GIST. However,
there was a comparable absence of distant metastases and diagnostic track involving
diagnostic imaging examination. Both cases have involved quick and early diagnosis
as well as the relatively fast application of effective treatment.
So far no cases of occurrence of synchronous double cancer with both malignant periumbilical
melanoma and GIST have been published. Therefore, reporting such cases in the future
will increase our knowledge to avoid misdiagnosis and delayed therapy for the double
cancer cases. Our report highlights some questions that might need further exploration
of whether use of additional expand diagnostic protocols could help to avoid clinical
omissions in rare cases and unexpected multiple cancer coincidences. In such rare
cases, the right planning treatment will be a great benefit for those patients. The
report provides a better guide for all cases of synchronous double or multiple cancers
that are still growing with unexpected coincidences that are more likely to emerge.
Conflict of interest
The authors declare no conflict of interest.